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A 20-year-old male patient presented to the emergency department of a tertiary care center with complains of intermittent, vague, dull aching pain in the abdomen localized to the periumbilical region for 2 years managed conservatively with medications from a local practitioner aggravated since 1 day, now localized to the right lower abdomen and associated with one episode of nonbilious vomiting. There was no history of per rectal bleed, melena, or loss of weight and appetite. On clinical examination the patient had tachycardia. Abdominal examination revealed tenderness and guarding in the right iliac fossa. A clinical diagnosis of acute appendicitis was made. An erect abdominal X-ray did not show free gas under the right dome of diaphragm. An ultrasound scan of the abdomen was reported as acute appendicitis with suspected rupture with free fluid in the pelvis. A computed tomography scan of the abdomen and pelvis was obtained. This was suggestive of a thickened segment of fluid-filled distal ileum with no intraluminal oral contrast, with surrounding free fluid and extraluminal air specks in addition to reactive mesenteric lymphadenopathy with minimal free fluid in the pelvis tracking down from the right iliac fossa (\n). The appendix appeared normal. The patient underwent emergency exploratory laparotomy for perforative peritonitis.\nA 15-cm long isolated tubular ileal duplication (\n), 30 cm proximal to the ileocecal junction supplied by a separate mesenteric pedicle arising from the base of the ileal mesentery (\nand\n) with a 1-cm wide perforation on the mesenteric aspect of the duplication was found. There was 200 mL mucoid contamination of the peritoneal cavity. Rest of the small and large bowel was normal.\nThe ileal duplication was resected along with its mesenteric pedicle (\n). After a thorough peritoneal lavage, the abdomen was closed. We did not place any drains in the abdomen. He was ambulatory and the nasogastric tube was removed on the first postoperative day (POD) and the patient started on oral sips. Once tolerated, he was progressed to liquids and then soft diet on POD 3. He was discharged on POD 4. Patient was followed up in the outpatient department on POD 10 for suture removal. He had an uneventful postoperative recovery.\nHistopathology report confirmed an ileal duplication cyst lined by gastric mucosa with foveolar hyperplasia and chronic gastritis. |
A healthy, 40-year old woman presented to the emergency room with pain near the region of her right ear. She had furthermore experienced vertigo with nausea for several months. Physical and radiographic examination by the neurologist and Ear-Nose-Throat-surgeon showed no abnormalities at the time of presentation. The only abnormal finding at this time, however, was enlargement of the right condyle and the patient was therefore referred to the maxillofacial surgeon ().\nComputed tomography (CT) showed a relatively large (34 × 30 × 28 mm) bone tumour in the right condyle with cystic deformation without involvement of the inferior alveolar nerve or other surrounding structures (). Magnetic resonance imaging (MRI) showed an expansive lesion of the right condyle with heterogeneous signal intensity on all recordings (T1, T2, and after intravenous contrast). The tumour in the right condyle caused compression of the parotid gland but did not show invasive growth into this gland or other surroundings structures. The differential diagnoses for this bone tumour included giant cell tumour, aneurysmatic bone cyst, ameloblastoma, fibrous dysplasia, and myxoma. A biopsy of the lesion was performed under general anaesthesia.\nMicroscopic examination by a specialized pathologist showed mainly pre-existing bone with a high bone turnover and bone formation. Fibrous dysplasia was deemed most likely, but a certain diagnosis was not possible. The biopsy showed no signs of malignancy.\nApproximately four months after initial presentation at the emergency room, the patient was referred to a maxillofacial surgeon who specialized in bone tumours. Physical examination showed a narrow outer ear canal on the right side without signs of infection at the time of inspection. The masseteric and pterygoid muscles were hypertrophic and painful during palpation. The maximum mouth opening was 13 mm with a deviation to the right side and a reduced laterotrusive movement of 4 mm to the right side (compared to 10 mm to the left side). Computed tomography showed a stable situation compared to the CT-scan that was taken four months previously. Bone scintigraphy showed a monostotic active fibrous distension in the right part of the mandible ().\nBisphosphonate treatment was commenced with olpadronate, 100 mg daily. The patient was furthermore referred to a physiotherapist to treat the symptoms of craniomandibular dysfunction. Besides an increase of the maximum mouth opening to 17 mm, this however did not have the desired effect. Bisphosphonate treatment did somewhat relieve the pain, but the patient still experienced high morbidity due to the remaining pain symptoms. During follow-up, the patient experienced infectious symptoms around the right ear twice, which was diagnosed as otitis of the outer ear canal due to compression and subsequent narrowing of the canal. The patient had to be admitted for four days and received intravenous antibiotics to reduce the infection.\nBecause of the repeated functional deficits with considerable morbidity for the patient, surgical therapy was therefore indicated. After consultation with several specialists in the field, resection of the affected bone (condylectomy) and reconstruction with either rib bone or a custom total joint were believed to be the two best options. Total joint replacement with a custom total joint was preferred by the surgeon and planned approximately two years after the first presentation at the emergency room.\nThe patient was preoperatively scanned in her intended occlusion to fabricate the custom total joint. A stereolithographic model of the jaw was printed to define the extent of the pathology and the margins for resection. Because the custom joint contains nickel, the patient was tested for nickel allergy, which was not present.\nUnder general anaesthesia, the condyle was resected through a pre-auricular and submandibular approach (). A custom made resection template was used to define the margins. After resection, a custom-made Biomet temporomandibular joint prosthesis, including the condylar head and articular fossa was inserted and fixated with titanium screws.\nPathologic examination of the resected tissue showed a predominantly expansive lesion with some cystic formations, highly increased bone turnover, and reactive/pre-existing bone formation including formation of neocortex. This was diagnosed as fibrous dysplasia with cystic degeneration ().\nAfter uneventful healing, the patient was treated by the gnathologist and physiotherapist to help retrieve complete masticatory function. Approximately 5 months after surgery, the patient reported complete function without any pain or morbidity. The occlusion showed no changes. The maximum mouth opening was 25 mm after treatment with Therabite. During the latest follow-up, approximately 22 months after surgery, the patient was free of complaints and satisfied with the result (). |
A 3-year-old definitely negative behavior female child reported to the department of pediatric and preventive dentistry with a chief complaint of pain and fractured right primary maxillary central incisors. Her medical history was not significant and dental history revealed that discoloration of maxillary incisor was seen a few years ago, and chipping of the tooth has occurred progressively, leading to loss of complete coronal structure. Intraoral examinations revealed that right maxillary central incisor is caries extending up to the cervical third with an exposure of the pulp chamber, restored and pulp therapy done 54, dental caries in 51, 61, 62, and 64. Orthopantomogram confirmed the clinical findings and presence of permanent teeth. Pulpectomy followed by intra-radicular biological post and core with crown was planned for 52; restoration of 51, 61, 62, and 64; and stainless-steel crown for 54.\nExtracted root stump is placed in a sodium hypochlorite 3% solution in a day for dissolution of organic structure []. The surface layer is removed using an ultrasonic scalar and washed thoroughly with saline and hydrogen peroxide. This removes any remnants of organic structure as well as conditions the root stump. It is disinfected and sterilized at 121°C at 15 lbs pressure for 15min.[] This is stored in saline mixed with hypochlorite till donor is available.\nPulpectomy for 51 was performed and zinc oxide eugenol was used as obturating materials. A thinner mix of zinc oxide initially is used to coat the canal walls. A thick mix of zinc oxide eugenol is condensed in the canal with a help of cylindrical condenser. About 6 mm of cement is removed from the coronal end of the root on the same day. Cotton is placed in the root canal space and appointment is given after 1 day for post and core preparation. This time interval allows the cement to set and form a hard base.\nIn this case, biological post was prepared from the palatal root of human primary maxillary first molar. For the second time, the post is sterilized at 130°C for 5min before checking for fit. On the basis of the prediction of the root canal shape of the restored teeth, it is cut buccolingually (if it is conical) or mesiodistally (if it is ribbon shaped) with a metal disc. It is then washed with saline and checked for fit []. After minor adjustments and checking for mild resistance, intraoral periapical radiograph is taken to check for fit, which appears as mild radiopacity as it is demineralized.[] It is cemented with glass ionomer cement with the post at least 3–4 mm above the cervical line and clearance of at least 1–2 mm from the opposing teeth should be maintained [ and ]. The coronal part of the post is restored with a thin layer of glass ionomer cement later on with a composite by sandwich technique. Composite crown built up is performed with a pedo jacket crown []. |
A 67-year-old man developed chest tightness and recurrent cerebral infarction. No obvious cause of cerebral infarction was found in the local hospital, so the patient was subsequently referred to our hospital for further evaluation. The patient had no family history of heart disease. A physical examination demonstrated left-downward displacement of the cardiac apex. Subsequently, transthoracic echocardiography (TTE) displayed a slightly enlarged left ventricle (left ventricular end-diastolic diameter was 55 mm) with decreased segmental motion of the ventricular wall and ventricular aneurysm formation at the apex of the left ventricle. Cardiac function was reduced, with a left ventricular ejection fraction (LVEF) of 45%. The left ventricle had several abnormal muscle bands connecting the posterior wall of the left ventricle and the interventricular septum, which separated the left ventricle into two parts. There was a relatively small communication between the main chamber and the aneurysm, with a diameter of 20 mm in diastole, and color Doppler flow imaging (CDFI) showed no obstruction to blood flow in the communication. Doppler detected a velocity of 190 cm/s during systole (Fig. ).\nTo identify the cause of the ventricular aneurysm formation, we performed an electrocardiogram (ECG), coronary angiography (CAG) and cardiac magnetic resonance imaging (CMR) to identify a common etiology. There was no obvious abnormality in the ECG, and CAG showed no obstructive lesions. Even after an extensive investigation, we could not identify any common cause of ventricular aneurysm formation. We repeatedly inquired about the medical history of the patient. He complained of discomfort in the precordium since he was young and underwent TTE in 1989 and 1995. The TTE indicated the existence of a large abnormal muscle bundle that separated the left ventricle into two lumens including a proximal chamber and a distal chamber; however, there was no ventricular aneurysm at that time. The diameter of the communication measured 15 mm in diastole. Different from the present situation, we detected a mean systolic gradient of 35 mmHg and a diastolic gradient of 10 mmHg between the two chambers by continuous wave Doppler. Therefore, we believe that the formation of the apical ventricular aneurysm was related to hemodynamic changes caused by the multiple abnormal bundles in the left ventricle. Subsequent CMR confirmed the existence of the abnormal muscle bundles and the apical ventricular aneurysm with a wall thickness of 4 mm (Fig. ). During diastole, blood flowed into the distal chamber by passing a bottle neck from the proximal chamber, and during systole, blood was squeezed from the distal chamber into the proximal chamber. Due to the blockage by the abnormal muscle bundles, the distal chamber needed enhanced contraction to push blood into the main chamber during systole, which resulted in a chronic increase in contractive pressure in the distal chamber and led to the formation of the apical ventricular aneurysm. This process is akin to the formation of an apical aneurysm in hypertrophic cardiomyopathy with mid-ventricular obstruction (MVO). We believe that the patient’s multiple cerebral infarctions were also associated with hemodynamic changes in the distal chamber. The stagnation of blood may have led to the formation of small thrombi in the aneurysm. Open surgery for the correction of the aneurysm was not proposed because cardiac function was stable. Instead, warfarin was prescribed to prevent intraventricular thrombus formation, with subsequent long-term follow-up. |
A 32 year old man presented to us with a 12 month history of infection around his lower right tibia and ankle. There was no history of fracture; rather, the infection was attributed to a soft tissue injury sustained after impact with a concrete bollard whilst running. Before presentation to us he had undergone incision and drainage of a large volume of pus as well as windowing of the tibia and two bony debridement procedures, the last 6 months previously. A wound swab taken at the time of injury had grown a fully sensitive Group A haemolytic streptococcus, and a single intraoperative sample from the right distal tibia taken at the time of the last debridement had grown Staphylococcus aureus (resistant to erythromycin). Blood cultures had remained negative throughout. He had completed a short course of oral clindamycin 300mg QDS (5 days) and oral flucloxacillin 500mg QDS prescribed by his GP at the time of initial presentation, as well as a second short course of oral flucloxacillin and two short courses of oral amoxicillin immediately after each of his surgical procedures. He was a smoker, but had no other significant medical co-morbidities.\nAt the time of our assessment he was afebrile and systemically well, though complained of ongoing ankle discomfort and swelling. On examination there was a scar over the anteromedial border of the tibia, with a small amount of skin discolouration and dry crust over the lower third. He had strong pedal pulses, normal sensation and a good range of ankle movement. Pre-operative imaging was consistent with Cierny and Mader Anatomic Type 3 osteomyelitis of the right lower tibia (Figure ).\nHe was taken to theatre for surgical excision, deep tissue sampling and reconstruction. The previous bone window was extended and the cavity was curetted back to healthy bleeding bone. Five deep samples were taken using a validated sampling protocol ,. Briefly this protocol recommends that when suspecting infection, 5 or more deep samples are taken with minimal manipulation of the target area using separate, unused instruments for each sample. Preferably samples should be taken prior to administration of intra-operative antibiotics, and after withholding antibiotics for at least 2 weeks prior to sampling. No pus was seen, but a significant area of dead infected bone was removed leaving a cortico-medullary bone defect. The resection site was washed with 0.05% aqueous chlorhexadine and the bone defect was filled with gentamicin eluting, bioabsorbable composites (Herafil® beads G [Heraeus Medical] and Cerament™ G (20mls) [Bone Support]). The soft-tissue defect was reconstructed with a free gracilis muscle flap and split skin graft (Figure ). Post operatively he was treated empirically for five days with vancomycin 1g BD and meropenem 500mg TDS.\nIn the laboratory, a preparation from each deep tissue sample was inoculated into both a BD BACTEC™ Plus aerobic/F bottle and a BD BACTEC™ Lytic/10 Anaerobic/F bottle and incubated at 35-37 degrees within a BD BACTEC™ FX system. After 48 hours incubation C. cadaveris grew in four out of five samples incubated under anaerobic conditions (sensitive to penicillin, tetracycline, erythromycin, fusidic acid, rifampicin, linezolid, vancomycin and metronidazole, as assessed by disc diffusion). No other organisms (including further Staphylococci or Streptococci) were isolated. Deep tissue histology was consistent with active chronic infection, but no organisms were seen with Gram stain. He was commenced on a 3 month course of oral clindamycin 450mg TDS and discharged on post-operative day ten. |
A 38-year-old man was admitted to the emergency room with sudden-onset severe resting pain in his right arm. A year before, the patient had complained of right shoulder pain, radiculopathy of the right arm, and coldness of the right hand, and was diagnosed with arterial thoracic outlet syndrome (TOS) at that time. He refused a recommendation for surgery and chose to be treated with rehabilitation instead. On his subsequent admission to the emergency room, the patient’s physical examination showed ischemia of the right hand along with diminished radial and brachial arterial pulses. There was an acute occlusion caused by an embolism in the distal brachial artery, and the right subclavian artery was patent in anatomical position. When the patient was in abduction position with an angle of more than 90 degrees, total occlusion of the right subclavian artery was observed (). Initial lab findings were not significant.\nEmergency surgery was performed on the patient. Under local anesthesia, a transverse skin incision was made in the right antecubital fossa, after which embolectomy of the brachial artery was performed using a Fogarty balloon catheter. Due to the absence of backflow, another incision was made in the wrist in order to approach the radial artery for embolectomy. The operation had to be discontinued when Doppler ultrasound confirmed continuity of the palmar arch. After surgery, the patient’s arm was maintained in the adduction position. The next day, the patient complained of severe pain in his right arm, and pulses were completely absent in the brachial and radial arteries. Angiography revealed that the occlusion in the lower part of the brachial artery had recurred at the same site (). Unobstructed blood flow was observed following a percutaneous mechanical suction thrombectomy. However, reoperation seemed inevitable, since progressive ischemia of the right arm was observed as soon as four hours after the thrombectomy. The reoperation was initiated with a transverse incision one fingerbreadth above the right clavicle. The medial half of the right clavicle was disarticulated from the sternum, and a resection of the clavicle was performed in order to expose the proximal portion of the right subclavian artery. Resection was also hoped to result in decompression. Inflammation surrounded the impinged area of the right subclavian artery. The great saphenous vein, harvested from the left leg, was reversed and used for a bypass conduit. The proximal anastomosis was performed in an area of the right subclavian artery more proximal than the compressed area, and the distal anastomosis was performed at the radial artery by making a subcutaneous tunnel up to the incision point in the wrist and passed through the subcutaneous tunnel. Angiography taken in anatomical position after the surgery showed that the blood flow from the right subclavian artery to the native brachial artery was maintained, as well as the blood flow of the radial artery from the right subclavian artery through the reversed great saphenous vein conduit in abduction position (). Persistent vasospasm of the palmar arch was evaluated. Coldness and tingling sensations also continued; consequently, right T3 sympathicotomy was performed three days later using video-assisted thoracoscopic surgery. The patient took warfarin and aspirin for three months following the surgery, after which he was maintained on aspirin alone. Upon discharge, the patient received rehabilitation therapy for decreased motor function, decreased sensation, and tingling sensations, recovering after eight months of therapy. The patient was unable to carry out everyday tasks at work before surgery, but one month after surgery, he was able to return to his job and has been working since then with no difficulties. |
In June 2002, a 36-year-old woman presented to her primary health care doctor with a history of flushing, diarrhoea, night sweats, and a clinically detectable mass in her left medial supraclavicular fossa. Her past medical history consisted only of essential hypertension for which she did not require prescribed therapy. Her family history included a brother with a diagnosis of sarcoma and two other non-first-degree relatives with primary brain malignancies.\nFine needle aspiration confirmed the diagnosis of medullary thyroid cancer, and in July 2002, she underwent total thyroidectomy with left-sided modified radical neck dissection and central compartment clearance. At this point, concerns were raised regarding optimal cytoreduction as the appearances of the central compartment, level 4 and level 5 nodes, were that of extensive disease. In order to maximise local disease control, she received adjuvant radical radiotherapy delivering 60 Gy to the thyroid bed.\nTwo years after completion of treatment, in February 2004, follow-up repeat imaging reported a recurrent nodule at level 4 of her neck. Subsequent resection confirmed this to be recurrent medullary thyroid cancer with no evidence of distant spread at the time. She continued to be monitored at the oncology clinic and remained disease-free until four years later, in May 2008, when computer tomography (CT) imaging revealed new pulmonary parenchymal metastases. These were closely monitored for the next 2 years with repeat imaging and measurement of calcitonin levels. In May 2010, it was decided that the patient should embark on systemic anticancer treatment.\nShe was offered participation in a phase 2 clinical trial with the agent lenvatinib (E7080) and she commenced treatment with 24 mg once daily in May 2010. One week into therapy, it was noted that she was marginally hypertensive with a blood pressure of 140/100 mmHg. No proteinuria was identified at this point, but she was commenced on 5 mg of amlodipine to manage hypertension. Monitoring of blood pressure and urinalysis continued as per study protocol.\nAfter two completed cycles of lenvatinib, CT imaging reported a reduction in size of all lesions. Further tumour assessment after 4 months confirmed a partial response to treatment with a 50% reduction of the sum of the long diameters of target lesions. She was experiencing various grade 1 toxicities throughout this time but was keen to maintain treatment given the good response. Due to the multiple low-grade toxicities, the dose of lenvatinib was initially reduced to 20 mg and thereafter to 14 mg.\nIn December 2011, 19 months after starting lenvatinib, she developed mild ankle oedema. Urinalysis carried out at the time identified proteinuria. A subsequent 24-hour urine collection identified 3.1 g/litre of proteinuria, equating to a urinary protein creatinine ratio (UPCR) of 625. The patient had not started any other medications and the incidence of proteinuria was felt to be lenvatinib related. Treatment with lenvatinib was ceased; however, due to concerns regarding possible intrinsic renal disease, she underwent screening for glomerulonephritis which was negative.\nA subsequent renal biopsy showed focal segmental glomerulosclerosis (FSGS) in two of twelve viable glomeruli, with tuft-capsule adhesion, hyalinosis, segmental intracapillary hypercellularity, and segmental splitting of capillary walls, predominantly in regions of segmental sclerosis. There was mild tubular atrophy, interstitial fibrosis, mild/moderate arterial intimal fibroelastic thickening, and mild arteriolosclerosis. Immunofluorescence showed no staining in glomeruli. Electron microscopy showed mild patchy reduplication of the basement membrane and effacement of only 20% of podocyte foot processes. There were no widespread electron dense deposits and no endothelial cell tubule-reticular inclusions. The endothelial cells showed no evidence of activation or damage. The appearances were consistent with a diagnosis of focal segmental glomerulosclerosis (FSGS). The lack of widespread podocyte foot process effacement suggests a secondary form of FSGS, which in the context of anti-VEGF treatment, could be mediated by microangiopathy. Whilst there was no histological evidence of acute thrombotic microangiopathy, it is possible that some of the pathological changes seen (splitting of glomerular capillary walls and mild arteriosclerosis) could be related to chronic low-grade endothelial cell damage. Based on histology, it is not possible to be certain whether the FSGS was caused by direct podocyte injury or whether it was related to endothelial cell injury. Histological slides are illustrated below in Figures , , and .\nThroughout this time, excretory function remained stable. Treatment with an ACE-inhibitor (ACEi) was introduced but due to poor tolerance and the quick improvement of the proteinuria after cessation of lenvatinib, the ACEi was stopped and patient's blood pressure was monitored closely.\nWithdrawal of lenvatinib had a marked effect on the levels of proteinuria, as illustrated in .\nThe patient continued follow-up at the renal clinic on a regular basis until July 2013, when she was discharged with no evidence of proteinuria, normotensive and with normal excretory renal function.\nAfter her discharge from the renal clinic and between 2013 and 2017, the patient was treated with vandetanib, nintedanib, and cabozantinib with no evidence of recurrent renal disease.\nE7080, also known as lenvatinib, is a potent inhibitor of the receptor protein kinases VEGFR-2 and VEGFR-3 but also displays inhibitory binding properties against VGFR-1, FGFR-1, and PDGFRα/β, albeit at significantly higher IC50 (half maximal inhibitory concentration, IC50). Its ability to restrain angiogenesis was shown on human umbilical vein endothelial cells (HUVEC) where E7080 inhibited VEGFR-2 phosphorylation and thereby capillary tube formation []. Apart from angiogenesis, E7080 decreased lymphangiogenesis in both the primary tumour of human breast adenocarcinoma cells in xenografts as well as in metastatic nodules in the lymph nodes of nude mice bearing these tumours []. Glen et al. showed in preclinical experiments that abrogation of FGFR and PDGFR signalling by E7080 inhibited invasion and migration of human melanoma cells lines (DX3) and human osteosarcoma epithelial cells (U2OS) []. Its potency against FGFR-1 differentiates E7080 from other currently approved tyrosine kinase inhibitors with antiangiogenesis properties [, ].\nThe preclinical data above were confirmed in several early phase human trials with E7080 in 2011 and 2012 in US, Europe, and Japan. Whilst establishing pharmacokinetic and pharmacodynamic properties of the drug, safety and preliminary efficacy was also well described. Lenvatinib was well tolerated at doses from 10 mg BID to 25 mg OD [–] and was associated with a reduction in disease activity biomarkers [], partial response, and stable disease according to response evaluation criteria in solid tumours []. These findings were further established in phase 2 trials and notably responses were demonstrated in thyroid cancer [–].\nApproval in thyroid cancer was granted in light of significant improvement in progression-free survival (PFS) compared with placebo in patients with radioiodine-refractory differentiated thyroid cancer in a phase 3 study (SELECT study) []. Lenvatinib improved median PFS over placebo by almost 15 months (HR 0.21; p < 0.01) and induced an objective response rate of 64.8%. The median survival results were diluted due to crossover of the patients from the placebo arm to the treatment arm; nevertheless, a subgroup analysis on patients stratified by age showed that older patients (>71 years old) had a survival advantage when treated with lenvatinib compared to placebo (HR, 0.53; p = 0.02), and the younger subgroup achieved a PFS of 20.2 months versus 3.7 m (p < 0.001) [].\nProteinuria and hypertension are the two most commonly reported side-effects of VEGF inhibitors and frequently the cause for therapy discontinuation. Proteinuria is used as a surrogate marker for glomerular damage and hypertension often accompanies and aggravates this.\nThe pathophysiology of proteinuria and glomerular damage in anti-VEGF therapy remains complex and far from well understood. Biopsy-proven cases of glomerular disease in anti-VEGF therapy are few; however, most have demonstrated changes in keeping with glomerular thrombotic microangiopathy (TMA) histology, with predominant endotheliosis and membranoproliferative changes [, ]. Other histological changes documented include cryoglobulinaemic glomerulonephritis, acute interstitial nephritis, collapsing and crescentic glomerulonephropathies, and FSGS plus TMA [–].\nIt has been theorized that hypertension is caused by decreased vascular production of nitrous oxide induced by inhibiting VEGF. This leads to renal haemodynamic compromise and subsequent proteinuria (much akin to exercise-related proteinuria) []. However, a mouse model study showed that glomerular injury preceded hypertension [] and many cases document glomerular injury in the absence of hypertension [], indicating that it cannot be the only trigger for proteinuria in anti-VEGF treated patients.\nInhibition of VEGF in podocytes (by injection of anti-VEGF antibodies or VEGF gene deletion) results in loss of endothelial fenestrations in glomerular capillaries, proliferation of glomerular endothelial cells, loss of podocytes, and proteinuria in mice [, ]. VEGF appears to be a crucial endothelial survival factor and its inhibition often manifests as TMA, a histology strikingly similar to that of severe preeclampsia—as placenta overproduces a soluble VEGF receptor (fms-like tyrosine kinase 1) that acts as a VEGF antagonist.\nIzzedine et al.'s 8-year follow-up study results from 2014 shed great light in anti-VEGF-related renal injury. It showed that in 100 patients who developed renal disease whilst on anti-VEGF treatment, the main histology associated with TKIs was minimal change disease and/or collapsing-like focal segmental glomerulosclerosis (MCN/cFSGS), a FSGC variant which is considered a separate entity to FSGS. In the same analysis, TMA histology was most frequently associated with VEGF-ligand targeted therapy (such as bevacizumab and aflibercept) suggesting two, possibly distinct pathophysiologies [, ] between renal damage caused by targeting the VEGF ligand as opposed to targeting the VEGFR tyrosine kinase domain. This could potentially be explained by considering the associations and signal transduction pathways between podocytes, endothelial cells, and VEGF. Podocytes produce vascular endothelial growth factor (VEGF), whereas VEGF receptor tyrosine kinases (RTKs) are expressed by both podocytes and glomerular endothelial cells.\nOur case demonstrates a secondary form of FSGS pathology which cannot confidently be attributed to TMA but could potentially represent the end result of chronic low-grade endothelial cell damage. The moderate histological findings were in keeping with a less-severe clinical course of the FSGS, with fast resolution of proteinuria and hypertension. More significantly, rechallenging the patient with additional three agents blocking the VEGF axis did not result in recurrence of the renal damage. |
The patient is a 14-year-old female with no significant past medical history or known trauma who presented with fever and a dry cough. She was initially treated for respiratory tract allergic bronchitis for 3-4 months. Due to a lack of improvement and the recent development of shortness of breath at rest, a chest X-ray (CXR) was ordered demonstrating a globular shadow of pericardial effusion. The patient was then referred to a cardiologist who performed a subsequent echocardiogram to assess the etiology of the pericardial effusion. The echocardiogram demonstrated moderate-to-severe pericardial effusion and tamponade. The patient was immediately taken to the catheterization suite for percutaneous drainage of the effusion under fluoroscopy. At this time, the cardiologist noted that there was an intracardiac metallic foreign body located behind the sternum in the wall of the right ventricle, moving with each heartbeat (Figures -).\nCardiac surgery was consulted during the procedure and the decision was made to drain the effusion surgically via a subxiphoid approach under fluoroscopy and to assess whether the foreign body could be retrieved in the same manner. Preparations were made to convert to conventional median sternotomy under cardiopulmonary bypass if needed. After incising the pericardium from the subxiphoid incision and draining the hemorrhagic effusion, the surface of the heart was palpated under fluoroscopic guidance. We were able to visualize the tip of the foreign body that was emerging from the anterior wall of the right ventricle. The sternum was retracted superiorly to improve visualization and the surgeon used his index finger to press on the area around the tip of the foreign body in an effort to force it to protrude, so that it could then be grasped by forceps. Ultimately the FB was removed from the right ventricular wall using artery forceps, carefully pulled out in the same axis of its insertion to avoid causing any further damage. The FB was found to be an intact domestic sewing needle (Figures -). Subsequent fluoroscopy revealed no additional foreign bodies inside or around the heart.\nA sample of the pericardial effusion fluid was sent for gram stain, culture, and sensitivity to tailor the post-operative antibiotics accordingly. The pericardial space was washed out with normal saline, a pericardial drain was inserted, and the wound was closed around the drain. The patient was taken to the cardiothoracic intensive care unit (CTICU) and started on intravenous antibiotics. The following day, CXR and echocardiography were performed ensuring that there was no collection present and that no damage was caused to the tricuspid valve or the interventricular septum intraoperatively. The patient was determined to be stable and transferred to the surgical floor for further recovery and monitoring. After five days, she was discharged home with complete resolution of her symptoms. |
A 16-year-old male patient presented to the emergency department with mild chest pain that had arisen after he was crushed by a slow-moving vehicle while riding his bicycle. The patient was under the car for only a brief period, and no abnormalities were found on the physical examination, except mild tenderness and swelling of the anterior chest. The patient showed an alert mental status and blood pressure in the normal range, with an elevated heart rate of 120 bpm. No dyspnea was present, and arterial oxygen saturation was 99%. Because a plain-film chest X-ray showed mild pneumomediastinum, a chest computed tomography (CT) scan was performed to identify potential injuries of the thorax, but no evidence of bronchial or esophageal injury was found in the unreconstructed axial CT images. Thus, oxygen therapy via a nasal cannula was planned for the patient without further examination. However, the patient suddenly developed severe dyspnea, and his arterial oxygen saturation decreased rapidly 1 hour after his arrival at the hospital. An emergency intubation was performed with single lumen endotracheal tube, but the decreased arterial oxygen saturation did not recover. A new chest X-ray showed a massive pneumoperitoneum that had suddenly developed and subcutaneous emphysema without any evidence of pneumothorax. Another CT scan was performed on the abdomen to check for any visceral perforation, but no evidence of visceral injury was seen (). However, rupture of the right main bronchus was revealed on the initially-obtained chest CT image, which was only reconstructed after some delay (). Since the patient showed hypotension and hypoxia, a double-lumen endotracheal tube was inserted into the left bronchus to ventilate the left lung and to block the path to the perforated right main bronchus. A few minutes after intubation, the patient was stabilized and transferred to the operating room. Exploration via an emergency right thoracotomy was performed under general anesthesia with the pre-positioned endotracheal tube. After the unperforated mediastinal pleura was divided, the completely amputated right main bronchus was found, with no injury to the lung parenchyma (). End-to-end anastomosis of the trachea to the right main bronchus was performed. Bronchoscopy performed 2 days later indicated an intact anastomosis without any evidence of leakage. After a few days of recovery, the patient was discharged without any complications. |
A 20-year-old female presented to the emergency department with chest pain, dyspnea, fever, and chills for 3 weeks. She was an active intravenous heroin abuser at the time and had been attempting to wean herself off heroin. During her evaluation, her blood cultures were notable for Streptococcus mitis. The diagnosis of bacterial endocarditis with severe aortic valve vegetation was made after a transthoracic echocardiography was performed. The patient was started on intravenous antibiotics on the day of admission and a cardiothoracic surgery consultation was made. On hospital day number 2, the patient was taken to the operating room for a bovine pericardial patch repair of a fistula and aortic valve replacement with a 21 mm bioprosthetic valve. The patient remained hospitalized for 36 days and was discharged to a drug rehabilitation program once she was medically cleared.\nWhen the patient presented to cardiology for follow-up 1.5 years after her aortic valve replacement, the patient was 22 years old and 20 weeks pregnant with a recorded body mass index of 29 kg/m2. The patient had been enrolled in a methadone maintenance program and had not used intravenous heroin for 6 months. She was promptly referred to our maternal-fetal medicine and cardiology joint program, a multidisciplinary collaboration created to manage pregnant women with preexisting or acquired cardiac conditions. An echocardiogram was performed during her first prenatal care visit showing a heart rate of 61 bpm, peak velocity of 360.5 m/s, peak gradient across valve of 52 mmHg, mean gradient across valve of 28.9 mmHg, and dimensionless index 0.36.\nJoint prenatal care and cardiology clinic visits were scheduled, during which routine prenatal care and cardiology evaluations were performed. The patient was placed on 81 mg of aspirin for the duration of her pregnancy to decrease her risk of thrombosis. Echocardiogram was repeated at 32 weeks and 37 weeks of gestation. At 32-week gestation, the echocardiogram showed a heart rate of 69 bpm, peak velocity of 386.9 m/s, peak gradient across valve 59.9 mmHg, mean gradient across valve 36.1 mmHg, and dimensionless index 0.43. At 37-week gestation, heart rate was 76, peak velocity of 339.3 m/s, peak gradient across valve 46.1 mmHg, mean gradient across valve 22.6 mmHg, and dimensionless index 0.45. The patient was asymptomatic and denied any cardiac symptoms.\nA multidisciplinary meeting included subspecialists of cardiology, maternal-fetal medicine, anesthesiology, critical-care, and labor and delivery nursing to create a delivery plan. A consensus was reached that a vaginal delivery would be safe for this patient without an assisted second stage, unless obstetrically indicated. A plan was made for telemetry monitoring immediately postpartum given higher risk of arrhythmia at that time.\nThe patient presented to the labor and delivery triage unit at 40-week 0-day gestation complaining of contractions and was found to be in spontaneous labor. As discussed during our multidisciplinary meeting, the patient received endocarditis prophylaxis and underwent expectant management of her labor. The patient did not require an assisted second stage of delivery and proceeded to have a spontaneous vaginal delivery. Within 10 hours of admission, she delivered a healthy male infant with Apgar scores of 9 at 1 min and 9 at 5 min, weighing 3770 grams with estimated blood loss of 350 ml. Her postpartum care was uneventful. Her echocardiography on postpartum day 1 showed a heart rate of 63 bpm, peak velocity of 325.8 m/s, peak gradient across valve 42.5 mmHg, mean gradient across valve 25.3 mmHg, and dimensionless index 0.36. The patient was discharged on postpartum day 3 without any complications. At her 6-week postpartum visit, the patient received an intrauterine device (IUD) for contraception. |
A 37-year old homeless male, with a past medical history of peripheral vascular disease, type 1 diabetes mellitus, hypertension, and depression, presented to the emergency department with intermittent chest pain and progressive shortness of breath for a few weeks. The patient denied intravenous drug use, although admitted to the use of recreational marijuana. The patient’s vitals on admission were stable except for low saturation on a pulse oximetry of 86% on room air. On physical examination, the patient was tachypnic and had fine crackles in the bilateral lung fields on auscultation. Laboratory results on admission showed a normal complete blood count and basic metabolic panel, but a urine drug screen test was positive for opioids. Because of persistent hypoxia, a D-Dimer was checked and came back highly elevated. The patient underwent a computed tomography angiography (CTA) of the chest to rule out pulmonary embolism. The CTA was negative for pulmonary embolism, however, it displayed extensive miliary densities throughout the bilateral lung fields (). The patient was admitted to the floor with a differential diagnosis of military tuberculosis versus fungal infection. Human immunodeficiency virus (HIV), fungal, and Quantiferron testing were negative. The cardiac work up and autoimmune serology were also unremarkable. The patient was then started on intravenous steroids and inhaled albuterol, although no improvement was seen. The patient remained hypoxemic despite therapy, and, therefore, underwent a bronchoscopy with a lung biopsy to find out the etiology of the disease process. The lung biopsy showed alveolated lung tissue with a miliary pattern of perivascular foreign body histiocytes containing refractory material suggestive of microcrystalline cellulose material (). There was no evidence of malignancy and there were no fungal or acid fast bacilli organisms identified on special stains. The histological features suggested intravenous injection of foreign material and upon further questioning the patient admitted to injecting oral opiates. The patient was started on intravenous steroids, although his clinical condition continued to decline. The patient developed hypercapnic respiratory failure, which required intubation, and eventually suffered from a cardiopulmonary arrest and passed away. |
A 60-year-old female, weighing 57 Kg, ASA physical status Class II, scheduled to undergo open cholecystectomy presented for preanesthetic check-up in our tertiary care hospital. She had a history of an allergic reaction during anesthesia 4 months back in another hospital when she was posted for the same surgery. Brief notes of previous anesthesia revealed that the patient was given injections of midazolam, fentanyl, propofol, and vecuronium intravenously in the given sequence. After few minutes of intubation, she had developed hypotension, bradycardia and swelling over the face and the whole body. She was managed as a case of suspected anaphylaxis with adrenaline, antihistaminics, steroids, and crystalloids given intravenously. She responded to the treatment. She became hemodynamically stable and swelling over face and body decreased gradually. Her surgery was deferred. She was extubated and shifted to the Intensive Care Unit (ICU) on oxygen inhalation by mask for observation. She remained hemodynamically stable in ICU. She did not require any inotropes and by morning swelling over her face and body had completely subsided. She maintained SpO2 of 97–98% on room air. She was shifted to the ward by afternoon. There was no mention of serum tryptase levels in the notes. She was sent for skin prick test and intradermal test after 6 weeks to an allergy clinic both of which were positive and confirmed IgE-mediated immune reaction to vecuronium. She was found to be not sensitive to midazolam, propofol, and fentanyl on skin testing. The patient was referred to our hospital for surgery and anesthesia. The patient gave a history of having undergone a cataract surgery under peribulbar block using bupivacaine and lignocaine 10 months back and a laparoscopic surgery for ligation of fallopian tubes 20 years back under local anesthesia and sedation. She was a diabetic well controlled on oral hypoglycemic drugs. There was a history of back pain since 2 years. There was no history of asthma or any other drug or food allergy. There was no other significant finding on history, examination, and investigations.\nThe patient was planned for regional anesthesia, but patient did not give consent for spinal/epidural anesthesia due to a backache. The alternate plan was a general anesthesia using an NMBD, which was not sensitive on intradermal test as surgeons required good abdominal muscle relaxation. Informed consent for high risk of anaphylaxis was taken, and a bed in ICU was reserved as a precautionary measure. All the drugs such as adrenaline, steroids, and antihistaminic drugs were loaded in syringes and equipment required for resuscitation and management of anaphylaxis was kept ready.\nThe only alternate NMBD available to us was atracurium. On the day of surgery, Patient was shifted to the operating room. Monitors for SpO2, ECG and blood pressure, were attached, and intravenous access was established. The patient was given an intradermal injection of atracurium (10 mg/ml solution diluted to 1:1000 in saline) to raise a bleb of 4 mm in diameter on the volar surface of forearm according to the guidelines.[] The test was read and interpreted according to the guidelines at 20 min, and there was no wheal and size of the bleb did not double or become more than 8 mm in diameter.[] The patient was found to be not sensitive to atracurium.\nHer preoperative pulse rate was 70/min; blood pressure was 140/90 mm of Hg and SpO2 was 98% on room air. Air entry was bilaterally equal on auscultation of the chest. Another large bore intravenous cannula was inserted, and the patient was preloaded with 500 ml of Ringer's lactate solution. Premedication was done with injection hydrocortisone 100 mg, injection pheniramine maleate 22 mg, and ranitidine 50 mg intravenously (blockers of histamine 1 and 2 receptors). The patient was preoxygenated with 100% oxygen. Injection fentanyl 2 μg/kg and propofol 1.5 mg/kg titrated to loss of verbal response were given separately and slowly intravenously. Muscle relaxation was achieved with injection atracurium (0.5 mg/Kg) given slowly intravenously over 2 min to prevent histamine release. After 3 min of ventilation ProSeal laryngeal mask airway, number 3 was inserted, and its correct placement was confirmed by bilateral auscultation of chest and square wave capnography. Anesthesia was maintained with isoflurane 0.6–0.8% in 1:2 mixture of oxygen and nitrous oxide. One supplemental dose of atracurium 5 mg was given intravenously. Intraoperatively, hemodynamic parameters and SpO2 were well maintained. No tachycardia, bradycardia, hypotension or bronchospasm was noted. The surgery lasted 75 min and total anesthesia time was 100 min. Intra- and post-operative analgesia was maintained with hourly bolus doses of fentanyl and infusion of paracetamol 1 g given 8 hourly intravenously. The patient had received paracetamol several times without any reaction before this surgery. Wound infiltration was done with 0.25% of bupivacaine as it had been given previously two times without any reaction. The patient was reversed with injection neostigmine 0.05 mg/kg and injection glycopyrrolate 0.01 mg/kg given intravenously, which were not tested as patient had already received them uneventfully in previous anesthesia. The patient was shifted to postoperative recovery room and monitored for 4 hours. She maintained stable hemodynamic parameters and SpO2 of 98% on room air. She was shifted to the ward and subsequently discharged after 3 days. She was given a letter containing all the details of her tests, safe and unsafe drugs and anesthesia procedure. |
A 59-year-old postmenopausal woman complaining of vaginal bleeding and pelvic pain presented to the gynecological department in May 2019. The woman had a significant past medical history of deep vein thrombosis (DVT) and her past surgical history included appendectomy 45 years ago. Her family history is negative for malignant ovarian and breast cancer in first-degree relatives. Previous screening for cervical cancer had not been performed.\nClinical and gynecological examination revealed no abnormalities, while transvaginal ultrasonography (TVS) was performed and revealed a cystic lesion 10 × 7 cm in diameter on the left ovary and a cystic lesion 1 × 1 cm in diameter on the right ovary. She underwent colposcopic examination followed by punch biopsy and endocervical curettage. Histological analysis of punch biopsy and endocervical curettage revealed possible endocervical mucinous adenocarcinoma. The differential diagnosis included either endocervical gastric-type mucinous adenocarcinoma, or metastatic mucinous colorectal or ovarian adenocarcinoma. Pelvis magnetic resonance imaging (MRI) revealed a 10 × 8 cm sized cervical mass () and enlarged retroperitoneal lymph nodes.\nIn November 2019, the Council of Gynecologic Oncology decided that the tumor was inoperable, because it had a large diameter and there was a suspicion that the tumor had spread to the bladder. The patient received 6 cycles of chemotherapeutic agents with carboplatin, paclitaxel and bevacizumab from December 2019 to March 2020. Although there was a decrease in the level of all tumor markers after chemotherapy, a new pelvis MRI showed enlargement of the cervical mass, which measured 24 × 21 × 12 cm.\nThe patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy, bilateral pelvic and aortic lymph node dissection and complete omentectomy. Intraoperatively the tumor was determined to be about 30 cm () and had invaded part of the bladder, as well as the sigmoid colon, so a left hemicolectomy and partial cystectomy was also performed. Her final histopathology report indicated a gastric-type mucinous adenocarcinoma of the cervix with low differentiation. The surgical margin was all free from everywhere. The patient was discharged and referred to the oncology department for further management. Three months after surgery, a CT scan of the abdomen and pelvis was performed with the absence of the uterus and ovaries, without lymph node enlargement or other changes. Further management was continued by the oncology team for possible adjuvant chemoradiation therapy after radical surgery. |
A 33-year-old white woman was referred to our outpatient clinic after suffering four atraumatic fractures between the age of 30 and 32 years. The fractures included a spontaneous fracture of the third right metatarsal bone with a spontaneous refracture 2 months later, a longitudinal fracture of the left tibia with protracted healing, as well as a fracture of the right femoral neck treated surgically, likewise with protracted healing. The patient reported contracting a hepatitis B virus infection in her childhood from a blood transfusion after a spinal surgery for neuroblastoma. Consequently, she has been treated with tenofovir disoproxil over the course of 15 years. Furthermore, she also received a course of radio- and chemotherapy as a part of the treatment. In addition, medical history revealed an early colon cancer at the age of 25 years, which was treated by surgery and chemotherapy. A genetic testing for most common genetic causes of breast and colon cancer was declined by the patient. Laboratory records showed low iron levels, hypophosphatemia, and hypouricemia, as well as a high alkaline phosphatase. Further blood and urine tests performed at 2 and 3 months before admission in our institution also revealed glycosuria and proteinuria with decreased bicarbonate levels in presence of normal blood glucose levels (Table ). One year before the first presentation at our institute, the patient received oral vitamin D (cholecalciferol) and calcium carbonate substitution as well as one cycle of denosumab treatment, due to the sustained fractures. The antiviral therapy was stopped 3 months before the first presentation in our outpatient clinic and an oral phosphate substitution was then started.\nThe patient's mother had also sustained a femoral fracture in the past. Two female siblings aged 25 and 35 years, as well as her 3-year-old boy were healthy. The family history also included cases of breast cancer in the family, with patient's mother, aunt, and grandmother being affected. The patient negated having spontaneous fractures or tooth loss during her childhood. Nicotine, alcohol, and substance abuse were ruled out. The patient reported having regular menstrual cycle and regular gynecologic checks, which did not yield any pathological findings.\nThe blood analysis performed at admission to our outpatient clinic revealed a normal blood phosphate level due to the ongoing oral phosphate substitution and after stopping the tenofovir regimen 3 months before the presentation. The patient's renal function was normal (creatinine 0.93 mg/dL [0.51–0.95]), uric acid levels were lowered (2.2 mg/dL [2.6–6.0]), while alkaline phosphatase level was markedly elevated (235 U/l [30–120]). The parathyroid hormone was within normal range (69 pg/mL [12–88]), FGF-23 levels were decreased (10.9 pg/mL [23.2–95.4]), and calcitriol was slightly above the range of normal (99.0 pg/mL [19.9–79.3]). The patient's urine sample revealed elevated levels of total protein (70 mg/dL [0–29]) and glucose (100 mg/dL [0–15]). A subsequent quantitative analysis of patient's urine showed decreased specific gravity (1011 kg/L [1012–1030]) in presence of an increased total protein to creatinine ratio (660 mg/g [0–149]). The patient presented with normal level of cholecalciferol (25-OH-vitamin D, 120 nmol/L [75–250]) since she was receiving an adequate substitution that was continued up to the transiliac bone biopsy, which took place 1 month after the presentation at our department. Furthermore, a single course of glucose 1-phosphate substitution was initiated 2 months before the admission. The patient received tetracycline labeling before biopsy to assess the dynamic bone formation. The patient also received a course of calcitriol (1,25-vitamin D), which was discontinued due to low tolerance. However, the patient's serum calcitriol levels remained within the normal range even after the cessation of the substitution. Upon acquisition of the transiliac bone biopsy results, the patient received an oral phosphate substitution with 6.4 mmol two times daily as well as an oral cholecalciferol substitution. The phosphate substitution was augmented by additional 6.4 mmol per day. Regular blood checkups showed normal levels of 25-OH-vitamin D and 1,25-vitamin D over the course of the treatment.\nBone scintigraphy showed an increased tracer uptake at the fracture sites, without any signs of malignant process. Bone mineral density (BMD) measurement by dual energy x-ray absorptiometry (DXA) revealed only osteopenic T-scores at the lumbar spine (L1 to L4: −1.6) and hip (femoral neck: −1.3, total hip −1.5). High-resolution peripheral quantitative computed tomography (HR-pQCT) showed a severe trabecular structural defect at the distal radius and even more at the tibia in presence of normal cortical thickness (Table ). The examination of oral cavity upon admission in the outpatient clinic yielded a partial anodontia (five teeth), periodontal disease as well as reduction of the osseous substance of the jaw. The patient underwent genetic testing because hereditary osteomalacia, osteogenesis imperfecta, and osteoporosis were initially suspected as the possible cause of multiple fractures. No pathological gene sequences were found in following genes: COL1A2, IFITM5, LEPRE1, PLS3, LRP5, WNT3A, WNT1, DMP1, PHEX, CLCN5, ENPP1, FGF23, SLC34A1, and ALPL. The only pathological finding was the presence of the Sp1-Polymorphism of the COL1A1 gene in a heterozygotic state. A physical examination revealed moderate swelling of the left forefoot with presence of tenderness and incalescence. Furthermore, the patient showed a limping gait with dragging of the right foot due to suffered fracture of the femur neck. |
We present the case of a 57-year-old gentleman who was admitted from the community with a 5-day history of worsening lower back pain. Nine months previously he had been diagnosed with advanced bladder transitional cell carcinoma (pT2G3N1) and treated with cystoprostatectomy. He was receiving adjuvant chemotherapy (gemcitabine and cisplatin) and his last cycle was 10 days prior to admission. His back pain had started 1 month previously after lifting a heavy object from the floor. He had been investigated with magnetic resonance imaging of his spine, which showed an acute biconcave benign fracture of L1 vertebra with a normal spinal canal. A bone scan at the time did not show any evidence of bone metastases and he was being managed with a spinal brace by the spinal team with good effect. The back pain was improving until 5 days prior to admission. It was worse on movement, without any radiation and there were no symptoms of cord compression. He denied any chest pain or discomfort at any point but complained of diaphoresis and mild breathlessness on exertion, which was initially attributed to the severe lower back pain. Apart from the bladder cancer, he did not have any other significant past medical history and there was no family history of ischaemic heart disease. He had stopped smoking 2 years ago after smoking 4–5 cigars per day for the last 30 years and that was his only cardiovascular risk factor. He was taking paracetamol 1 g QDS (four times per day), ibuprofen 400 mg TDS (three times per day), morphine sulphate 20 mg BD (twice a day) and diazepam 5 mg BD for his back pain. Clinical examination revealed normal cardiac sounds, clear chest and normal neurology without any evidence of cord compression. His chest X-ray was clear and lumbosacral X-rays did not reveal a new fracture. However, his electrocardiogram (Fig. ) showed fixed ST elevation in V1–2 and anterolateral ST depression. His troponin T was elevated at 1327 ng/l (normal <14) and he was started on aspirin, clopidogrel, fondaparinaux and secondary prevention including beta-blocker, ACE inhibitor and statin. The rest of his blood tests revealed Hb = 14.7 g/dl (normal 13.5–17.5 g/dl), WBC = 10.5 × 109/l (normal 4–11 × 109/l), Plt = 139 × 109/l (normal 135–450 × 109/l), Ur = 9.1 mmol/l (normal 2.5–8.5 mmol/l) and Cr = 105 µmol/l (normal 65–120 µmol/l). The next day he had another episode of diaphoresis while at rest with worsening ST depression laterally and so he was started on nitrate and tirofiban infusions, and plans were made for urgent transfer to the regional cardiothoracic centre. Coronary angiography showed an occluded left anterior descending (LAD) artery (Fig. , white arrow and ), severe lesions in the circumflex artery (Fig. , black arrow and ) and the right coronary artery (Fig. , black arrows) and collaterals from mid-circumflex to distal LAD. Following discussion with cardiothoracic surgery, his LAD and circumflex arteries were successfully treated with drug-eluting stents. Opening the occluded LAD was challenging due to semi-organised thrombus throughout the mid-LAD. It required serial pre-dilatations, thrombus aspiration with a Pronto LP catheter which produced limited yield, administration of Abciximab (ReoPro) and serial doses of vasodilators before deployment of the 2.75 × 38 mm Xience drug-eluting stent from the mouth of the bifurcation with the diagonal to just above the second diagonal in the mid-vessel. The disease in the circumflex artery was treated with pre-dilatation and a further 2.5 × 33 mm Xience drug-eluting stent. The final angiographic result was excellent as shown in Fig. and . Intervention to the right coronary artery lesion is planned as a staged procedure. |
In this article, we report the case of a 9-year-old female patient with no previous medical history and no significant family history of osteosarcoma or any other bone tumor, who presented in our clinic on January 2016 with a 1 year history of pain in her low back and left lower limb. She was diagnosed with osteosarcoma of the left tibia with a solitary metastasis in her L3 vertebrae on February 2016. She was subsequently treated with a left below knee amputation and L3 corpectomy with posterior spinal fusion and instrumentation from L1 to L5 with decompression laminectomy at L2-3, and L3-4 through a posterior and left thoracoabdominal approach in March 2016 ().\nShe was found to have an abnormal bone scan with a lesion at the L3 level 3 months after completing chemotherapy in November 2016. MRI scan and ultrasound guided fine-needle aspiration cytology (FNAC) of right paraspinal psoas tissue confirmed recurrent osteoblastic osteosarcoma on December 2016 (). On physical examination, she was able to ambulate with use of a below knee prosthesis and demonstrated no neurological deficits. The patient was started on second line drugs, including two cycles of ifosfamide/etoposide. Previous implants were well in place. Imaging was performed with radiographs, CT scan, bone scan, PET scan, and MRI scan with contrast enhancement to confirm only a single metastatic site (). For therapeutic strategy determination, the patient was introduced to our local tumor board. Preoperative workup was completed, and surgery was planned for a complex en bloc resection of L2, L3, and L4 with removal of deep spinal implants with anterior and posterior spinal fusion and instrumentation (). The option of nonoperative palliative care was offered to the patient and her family, but they elected to proceed with en bloc resection to maximize her chances of survival, in spite of high surgical risk and an overall poor prognosis. They were informed preoperatively that a complete resection would require sacrificing her nerve roots at L2, L3 and L4. A palliative decompression was not offered for the revision procedure as a treatment option as the patient was not complaining of pain or neurological symptoms, and it would not have improved her life expectancy. |
The 24-year-old male patient was a driver who participated in a car accident; he was admitted to the Emergency Department of University Clinical Hospital No. 2 in Lodz with multiple injuries. The car driven by the patient rolled over, and the victim fell through the windshield; according to the report of the ambulance team leader, the victim was not wearing a seat belt. On admission, the patient was stable and in deep pharmacological analgesia.\nA full-body computed tomography (CT) scan was performed. The examination revealed a number of post-traumatic lesions including subdural hematomas in the area of the temporal lobes (thickness: approx. 11 mm). The patient had multiple fractures in the craniofacial area: a frontal bone fracture, a fracture of the left zygomatic process of the frontal bone, fractures of all the walls of the left eye socket, fractures of the anterior and posterior walls of both maxillary sinuses, a nasal bone fracture, and hematomas in the paranasal sinuses. Chest CT revealed an esophageal rupture at the height of the clavicle, which caused an extensive subcutaneous emphysema (); the examination also revealed hemorrhagic contusions within the lower lobes of both lungs. There were no abnormalities in the abdominal cavity. Examination of the pelvic region revealed a fracture of the right upper part of the right femur.\nAfter the patient's vital signs were stabilized, the trauma team, having discussed the wide extent of the injuries, decided that the priority was to repair the ruptured esophagus.\nTaking into consideration the left-sided position of the esophagus, an incision was performed on the left side of the neck from the height of the left sternoclavicular joint to the level of the mandibular angle. After the esophagus was exposed, the perforation site was located; it turned out to be several centimeters above the location indicated by the CT scan. The perforation site was stapled using single-layer interrupted sutures (). A suction drain was placed near the suture line.\nAfter the surgical procedure, the patient was transferred to the intensive care unit (ICU) for further treatment. Additional maxillofacial and orthopedic procedures were performed in the subsequent days. The perforation site healed properly, and the protective drain was removed on the third day after the initial surgery. The patient remained in the ICU for 3 weeks; after this period, he was transferred to the neurological department for further conservative treatment. |
A 61-year-old postmenopausal woman presented with right outer breast pain, redness and swelling of 3 days duration. Mammogram was essentially normal at that time. Ultrasound of her right breast revealed no masses but increased echogenicity and skin thickening in the outer half of her breast, likely due to inflammation. Clinical impression was that of right mastitis. She was treated with 1 week of oral antibiotics and responded with resolution of her symptoms. A follow-up ultrasound performed 1 month later showed improvement of the inflammatory changes in the right breast.\nThe patient then remained well until she presented 8 months later with another similar episode of right mastitis over the previously affected area. An ultrasound performed this time again showed skin thickening in the right outer breast with underlying inflammatory changes. No discrete nodule or collection was seen. The patient’s symptoms again improved with antibiotics with complete resolution of the redness and swelling of her right breast.\nHowever, the patient returned 6 months later presenting with another similar episode of right breast redness, swelling and pain. Physical examination then revealed right nipple retraction and signs of inflammation over the previous area of interest. Repeat ultrasound (Figure ) showed marked inflammatory changes in the right breast with skin thickening, suggestive of an inflammatory process. Though the patient’s symptoms again responded with antibiotics, an underlying breast malignancy, especially inflammatory breast cancer, needed to be excluded in view of recent nipple retraction.\nA skin punch biopsy and core biopsy of the inflamed breast tissue were carried out which revealed mild superficial dermatitis on skin biopsy. Core biopsy showed DCIS with focal chronic mastitis.\nThe patient underwent a right total mastectomy and sentinel lymph node biopsy. Final histology showed a 75 mm high-grade DCIS with areas of necrosis. There was, however, no invasive component. Some of the involved ducts (up to 50%) showed features of cystic hypersecretory DCIS, characterized by cystically dilated ducts containing thyroid colloid-like eosinophilic secretions (Figure ). There was no lymph node involvement and the margins were clear. The estrogen and progesterone receptors status were both negative. Her2 neu score was positive. Her postoperative recovery was uneventful and the patient did not require any further treatment. |
A 24-year-old female presented a thumb-sized subcutaneous tumor mass in the right side of the pubic region for two years. Because she felt that the tumor size and the pain were gradually increasing, she consulted us for medical care. She had never been pregnant or experienced dysmenorrhea.\nManipulation in the right groin region showed that the mass was located just above the right edge of the pubic tubercle and was a 2 × 3 cm subcutaneous tumor with a slightly rough surface, unclear borderline, and mild tenderness. While no adhesion to the skin and only slight adhesion to the subcutaneous fat tissue were observed, the tumor was firmly attached to the floor without mobility. No remarkable skin region was observed. The laboratory data showed no signs of inflammation with WBC 6400/μl and CRP 0.1 and only slight anemia with Hb 11.8 g/dl. Image analysis of a pelvic CT revealed an irregular subcutaneous mass just above the right edge of the pubic tubercle with the same X-ray absorbance density as that of the muscle. The radiographic diagnosis was that of an inflammatory tumor. Consequently, as the preoperative diagnosis, we considered an inflammatory reaction of a lymph node or a dermoid cyst.\nDuring the operation, we easily approached the mass through an incision on the medial side of the right groin region. The mass could be manually released from its adhesion to the subcutaneous fat tissue, but was firmly attached to the uterine round ligament with a poorly demarcated borderline. Therefore, we removed the tumor with a part of the uterine round ligament attached. Neither an inguinal hernia nor a sac was observed. From the macroscopic view, fat tissue was attached to the surface of the tumor. The cross section presented a whitish-yellow color with an irregular round shape; the indistinct boundary adhered to the surrounding fat tissue. Small spots containing brownish mucus were observed. The H&E staining of the removed tissue showed several small hollow glands scattered inside the tumor with a stromal structure. The hollow glands were lined with columnar epithelial layers and surrounded by proliferated stromal cells (Fig. ). Immunohistochemical analyses revealed that antibodies against CA125 (Fig. ), estrogen receptor (Fig. ), progesterone receptor (Fig. ), CD10 (Fig. ), and COX-2 (Fig. ) stained positively, but no staining of the CA19-9 antibody was observed. Although five antibodies that we chose had positive staining in the endometrial tissue, there were several differences in the degree of stainability among them. The CA125 antibody stained weakly, mainly in the inner and outer surfaces of the cytoplasm in the glandular endothelial cells, without staining the nuclei of the endothelial and stromal cells. The estrogen receptor, progesterone receptor, and COX-2 antibodies stained both in the stromal and endothelial cells. However, estrogen and progesterone receptor antibodies stained positively only in the nucleus and not the cytoplasm. The estrogen receptor antibody stained more positively in the endothelial cells than in the stromal cells, while the progesterone receptor antibody stained strongly and more positively in the stromal cells than in that of the endothelial cells. COX-2 had stained stronger in the cytoplasm and the nuclei of the endothelial cells than in that of the stromal cells. CD10 stained strongly positive in only the cytoplasm of the stromal cells. These findings indicated that the tumor that had adhered to the uterine round ligament had originated from the endometrial tissue.\nAfter the operation, we consulted an obstetrician and a gynecologist. Neither uterine adenomyosis nor pelvic endometriosis was detected using manipulation, ultrasound examination, and MRI scan. The serum CA125 level was 19.9 U/ml, which is within the normal range. No recurrence of the tumor was observed one year after the operation. |
A 33-year-old woman presented to the emergency department at our hospital with recurrent episodes of right-upper-quadrant pain associated with multiple episodes of vomiting. She had an ultrasonographic scan of the abdomen that showed multiple mobile stones in the gallbladder with normal intra- and extrahepatic bile ducts with no other abnormality. The blood workup was normal including liver function tests. She was operated for a laparoscopic cholecystectomy on an ambulatory surgery basis. The operation was uneventful except for the finding of a 1.5-cm nodule within the triangle of Calot (Fig. ). This well-circumscribed, spherical lesion was attached to the common hepatic duct and embedded within the triangle of Calot with no communication to the hepatic parenchyma or gallbladder. Upon this abnormal finding, which we assumed to be an abnormally large cystic lymph node, an on-table cholangiogram (Fig. ) was performed that showed normal biliary anatomy, no filling defects within the biliary tract and normal flow of the contrast material into the second part of the duodenum (Fig. ). The node was bluntly and sharply dissected off the common hepatic duct, and it showed not to be related to the cystic artery and was separate from the cystic lymph node. No communication between the cystic structure and the common hepatic duct, the cystic duct or any vascular structures could be demonstrated, except for thick fibrous tissue attaching the mass to the distal part of the common hepatic duct. The operation was otherwise uneventful for any complications, and the patient was discharged on the same day. Histopathological examination showed the gallbladder to be chronically inflamed and the node to be 1.5 cm in greatest diameter. This node is a unilocular cyst (Fig. ) lined with pseudostratified ciliated epithelium admixed with mucinous cells. Underlying this epithelial layer, connective tissue stroma, a thin layer of smooth muscle cells and an outer fibrous layer were identified. No communication or ductal structures could be found. Furthermore, immunohistochemical staining showed the cyst to be cytokeratin 7 (CK7) positive and cytokeratin 20 (CK20) and CDX2 negative. These histological findings are consistent with a ciliated foregut cyst. With this final diagnosis, the pre-operative ultrasonographic scans were reviewed retrospectively (Fig. ). The possibility of the presence of a cystic structure separate from the gallbladder and the common hepatic duct was entertained. Obviously, such a subtle finding would have been easily missed pre-operatively taking into consideration the patient’s presentation, described above.\nCiliated foregut cysts (CFC) are extremely rare, benign, congenital cystic lesions that arise from the embryonic primitive foregut []. These cysts are most often solitary and unilocular characterised by an internal pseudostratified, ciliated, mucin-secreting, columnar epithelial lining []. These lesions are usually located above the diaphragm [, ] with a few reports of ciliated cysts in relation to abdominal organs.\nDuring embryonic development, the differentiation of the primitive foregut cells gives rise to the oropharynx, oesophagus, larynx, tracheobronchial tree, lungs, stomach, proximal duodenum, pancreas, liver and biliary system [–]. The presence of cysts with a respiratory-type epithelial lining in relation to abdominal organs is aberrant. However, it is postulated that the same mechanisms underlie the development of CFC in relation to abdominal organs and the respiratory system [, ].\nConsequently, the association of CFC with the biliary system is quite exceptional. The first description of these lesions in the gallbladder was by Kakitsubata et al. in []. A ciliated cyst of the common bile duct was reported by Baranger et al. in []. In 2000, Nam et al. were the first to introduce the term ‘ciliated foregut cyst of the gallbladder’ in their report []. Five other reports described ciliated cysts found within the wall of the gallbladder [–, , ].\nThe differential diagnosis of hepatic CFC would include biliary cyst, parasitic cyst, mucinous cystic neoplasm and various cystic metastases, such as cystic neuroendocrine tumour or necrotic metastases. When identified in the gallbladder fossa, a pancreatic pseudocyst, choledochal cyst or gallbladder duplication should also be considered [].\nTo our knowledge, this is the first description of a ciliated foregut cyst in the triangle of Calot presenting as a unilocular cyst that is attached by fibrous tissue to the common hepatic duct and is totally extramural and without any communication to the gallbladder or the hepatic ducts [].\nThis case highlights the diagnostic challenges and management options posed by this pathological rarity. In this case, the pre-operative ultrasound scan could not specifically identify the presence of the cyst, and an intra-operative cholangiogram could not prove a communication between the cyst and the biliary tracts. Furthermore, there were reports on the transformation of hepatic ciliated foregut cysts into primary squamous cell carcinoma [, ]. The exceptional presentation of such a lesion precludes firm conclusions in this regard. As malignant potential cannot be totally excluded and in the absence of well-defined surveillance criteria, the excision of these tumours, when diagnosed, would be a rational approach mainly in young and symptomatic patients. |
A 36-year-old female was referred to our institution for evaluation of symptoms of orthostatic intolerance. Consent was obtained from the patient to publish this as a case report, with documentation of verbal consent in the electronic medical record. She reported symptoms including dizziness, palpitations, nausea, and a warm sensation while upright that improved with assuming a recumbent posture. She also reported symptoms during exertion, documented as lightheadedness and feeling of her heart racing when physically active, as well as a decreased tolerance to walking. Her symptoms occurred a few times per week up to several times per day and were worse during the beginning of her menstrual cycle. Her symptoms significantly improved with wearing compression stockings. She also reported two episodes of loss of consciousness. With the first episode, she was lying on the couch then sat up and felt nauseated, and then she stood up and had an abrupt loss of consciousness. With the second episode, she was walking in the kitchen, had a brief prodrome of not feeling well, and then had an abrupt loss of consciousness. She had evaluations at an outside institution with a Holter monitor showing episodes of sinus tachycardia, an echocardiogram that was reportedly normal, and a tilt table test reporting a vasovagal reaction following the administration of nitroglycerin. She reported that her father had multiple episodes of syncope but never sought medical treatment; she did not report any family history of POTS.\nShe underwent further evaluations at our institution. A passive 70-degree head up tilt table test showed a postural increase in heart rate from 74 to 114 bpm at the end of the tilt, with some blood pressure oscillations but overall stable blood pressures (from 115/70 mmHg supine to 118/82 mmHg at the end of tilt) while eliciting her typical symptoms of nausea, warmth, a spinning sensation, and feeling like she would lose consciousness. She requested the test to be stopped early at 36 minutes out of a 45-minute protocol due to her symptoms. Of note, she was taking fludrocortisone 0.1 mg daily at the time of the head up tilt table test. She was diagnosed initially with POTS due to her clinical features and tilt table test results. She underwent blood volume and hemodynamic testing. Blood volume measured using a I-131-HSA-tagged human serum albumin technique with a Daxor BVA-100 system (Daxor Corporation, NY, USA) showed normal plasma volume at −2.8% from ideal (gender matched, weight, and height). Hemodynamic testing to assess circulatory kinetics was done using 99 m Technetium-RBC radionuclide imaging []. At 45-degree head up position, the heart rate increased by 18 bpm, cardiopulmonary volume fraction decreased by 34% (a marked decrease), cardiac index decreased from 2.6 L/min/m2 to 1.777 L/min/m2, stroke volume decreased from 79 ml to 50 ml, total peripheral resistance increased from 40 to 61 μm2, and mean pulmonary transit time was rapid at 6.8 seconds. Plasma metanephrine and normetanephrine levels were checked and were within normal limits. An evaluation of autonomic function was done. Quantitative sudomotor axon reflex testing showed normal values at the right forearm, proximal leg, distal leg, and foot. The heart rate and blood pressure responses to Valsalva showed a normal Valsalva ratio of 1.47 and normal blood pressure responses in phase II and phase IV. An exercise treadmill test done as part of the evaluation for cardiac rehabilitation showed fair functional capacity at 8.6 METS, an increase in heart rate to 99% of maximal predicted heart rate, stable blood pressures, and no arrhythmias.\nA resting echocardiogram repeated at our institution showed a normal EF of 71%, mild septal left ventricular hypertrophy measuring 1.3 cm, with a prominent posterior papillary muscle. At rest, there was chordal systolic anterior motion (SAM) of the mitral valve with trivial mitral regurgitation, and a left ventricular outflow tract (LVOT) gradient of only 7 mmHg. After Valsalva maneuver, the LVOT gradient increased strikingly to 75 mmHg () with a further increase to 81 mmHg after administration of amyl nitrite. A subsequent cardiac MRI demonstrated an apically displaced, multiheaded posteromedial papillary muscle. There was a suggestion that the more anteriorly displaced head of the posteromedial papillary muscle had aberrant chordal attachments to the anterior mitral valve leaflet that contributed to the SAM (). There was mild hypertrophy of the anteroseptum (measuring 1.3 cm) with a normal left ventricular mass index of 57 gm/m2 (normal 48–77 gm/m2) and no delayed enhancement to suggest interstitial fibrosis. A subsequent transesophageal echocardiogram (TEE) demonstrated the mobile anteriorly displaced posterior papillary muscle with SAM ().\nAfter consultations with specialists in structural heart disease and cardiothoracic surgery, the patient ultimately underwent cardiac surgery consisting of very gentle septal myectomy focusing more midventricular at the basilar septum, reorientation of the posterior medial papillary muscle head, resection of the tethering secondary chordae to the A1 segment of the mitral valve, and chordal shortening and tacking of the chordae to the A1 and A2 segments of the mitral valve. During her several post-op visits with Cardiology and Cardiothoracic Surgery, she reported a significant improvement in her prior orthostatic symptoms and no longer reported syncope. |
We present the case of a 56-year-old female with history of syncope due to third degree atrioventricular heart block presenting initially with onset of stroke symptoms six days after pacemaker placement and two days after hospital discharge. At 5 PM she developed abrupt onset of left facial droop along with left upper and lower extremity weakness. The patient was initially treated at an outlying hospital and received alteplase at 6:35 PM for treatment of acute ischemic stroke.\nA chest radiograph performed at the outlying hospital prior to alteplase administration demonstrated an enlarged cardiac silhouette when compared to prior radiographs showing only borderline cardiomegaly. Upon administration, the patient reported mild chest pain and was given nitroglycerine and morphine. Her chest pain resolved and she was transferred to our comprehensive stroke center for admission. The patient presented to our emergency department at 10:10 PM with a heart rate of 122 beats per minute (bpm) and a blood pressure of 109/41 millimeters of mercury (mmHg).\nAt 11:20 PM the patient went for a computed tomography angiogram (CTA) after an initial assessment by the emergency physician in consultation with the stroke-team attending physician. After CTA at 10:28 PM, she was documented to have a blood pressure of 49/25 mmHg and heart rate of 109 bpm. She was returned to the resuscitation bay for re-evaluation. Cardiac tamponade was suspected due to the extreme hypotension in the setting of thrombolytic administration after recent pacemaker placement.\nOn reassessment, the patient had become confused with a Glasgow Coma Scale of 14. The emergency physician performed a POCUS, which demonstrated a pericardial effusion with features of cardiac tamponade including diastolic collapse of the right ventricle (). At that point the diagnosis of cardiac tamponade was made. The patient was alert and responsive, so an intravenous bolus of normal saline was given while a stat surgical consult was obtained. The surgical team evaluated the patient at the bedside within minutes and was able to review the POCUS findings. As the patient was conscious, they elected to take her immediately to the operating room rather than perform a bedside pericardiocentesis.\nWhile in the operating room, approximately 400 milliliters of coagulated blood were evacuated from the pericardial sac with 150 milliliters of surgical bleeding. The operative report notes resolution of tachycardia following this intervention with heart rate trending down to a range of 80–90 bpm with concomitant improvement in blood pressure. She was discharged two days post-operatively with a pericardial catheter in place. Echocardiogram performed on day of discharge noted a small, residual pericardial effusion. |
In July 2001, a 28-year-old male reported with a chief complaint of pain and swelling of the left lower jaw causing facial asymmetry. The patient reported of similar symptoms on the same side 8 years back. He had visited several dentists over this period of time with a complaint of mobility of teeth on both sides of the jaw and swelling on the left side. He was advised removal of 48, 47, and 38 followed 2 years later by removal of 37, 36, 35, and 34. Since the pain and mobility of the teeth did not subside, the patient reported to us. He did not carry any past histopathological records with him. His past medical history was unremarkable and his general physical examination revealed the presence of skin lesions on the forehead [].\nClinical examination of the head and neck region showed left facial swelling along with an expanded buccal and preauricular area leading to facial asymmetry. He denied any neurosensory problem associated with inferior alveolar nerve.\nIntraoral examination revealed a firm, hard swelling on the crest and buccal vestibule of left mandibular alveolar ridge, around 5 cm in the anterior–posterior direction and overlying the crest of 36, 37, 38. There was no discharge intraorally. All other hard and soft tissue components of oral cavity appeared normal.\nThe OPG showed a multilocular radiolucency on the left side in the body of the mandible. One radiolucent lesion was seen extending from the condylar, coronoid processes up to the body of mandible on the same side. There was another smaller radiolucent lesion evident on the distal aspect of 35 [].\nThe computed tomography (CT) scan confirmed that the lesion was multiloculated and expansion of the cortical bones of the inferior and posterior borders on the left side of mandible was evident. Another radiolucency was seen just adjacent to the main lesion. In our opinion, it could have been an extension of the same lesion, which radiographically appeared as “daughter cyst.”\nOn histopathological examination, the diagnosis of OKC was made [].\nIn July 2000, under general anesthesia, hemi-mandibulectomy was performed followed by placement of reconstruction plate. In June 2002, the reconstruction plate was removed and a nonvascularized iliac crest graft was placed. In subsequent visit, impacted 18 was removed along with the cystic lining and its contents under local anesthesia. The lesion was reported to be a dentigerous cyst [].\nIn 2004, the orthopantomograph showed evidence of resorption of graft. Also, it was noticed that the skin lesions on the face and axilla appeared to be enlarging slowly. Dermatological consultation resulted in agreement with diagnosis of Basal Cell Nevous Syndrome. On submission of histopathological specimen, the skin naevi were reported to be basal cell carcinomas []. Chest radiograph did not show any evidence of bifid rib [].\nIn the clinical and radiographic follow-up from 2007 to 2009, the OPG showed increase in the level of resorption of the graft, although the patient did not have any functional or aesthetic problem. In the recent follow-up visits, the BCC lesions have gradually shown an increase in size as well as number [].\nThe patient is scheduled for regular periodic follow-up with dermatology and maxillofacial department. |
The patient was a 37-year-old woman (G3, P2), and written informed consent for publication was obtained. She has been taking oral prednisolone for controlling membranoproliferative nephropathy from the age of 19 years. Her past history included uneventful vaginal delivery at the age of 26 and cesarean delivery for preeclampsia at the age of 31. On this occasion, she was admitted to our hospital at 27 weeks and 3 days of gestation due to deterioration of kidney function and hypertension. On the day of admission, she received the first anesthetic evaluation, and her information was uploaded after excluding personally identifiable items in our list of high-risk pregnant women on the BCT.\nAt 30 weeks and 1 day of gestation, a stuff of the obstetric anesthesia team found that she had been taking icosapentate for hypertriglyceridemia through the regular anesthetic evaluation preparing for a possible cesarean delivery and discontinued its prescription after discussing with the relevant department. At that time, it was planned that anesthetic method for cesarean delivery was general (≤ 7 days) or regional (> 7 days after stopping icosapentate) according to the Japanese guideline []. Then this plan was recorded in her medical chart and updated in the BCT.\nAt 30 weeks and 4 days of gestation, because of progressing hypertension and deteriorating kidney function, cesarean section on 30w6d, after administration of betamethasone for two days to avoid infant respiratory distress syndrome, was scheduled. Although it was Saturday when her cesarean section was fixed, a resident of the obstetric anesthesia team visited the patient and explained general anesthesia for cesarean section. He also proposed transversus abdominis plane block for postoperative pain management and uploaded her acceptance on the BCT.\nIn Sunday morning, a senior anesthesiologist found the updated information on the BCT at home and left his comment that anesthetic plan should be reconsidered after checking the indication of peripheral nerve block for the patient receiving icosapentate before the surgery. After reading this comment, another resident, who was in charge on Sunday, confirmed that transversus abdominis plane block was relatively contraindicated in such situations. Therefore, he re-visited the patient to change the anesthetic plan for postoperative pain treatment from transversus abdominis plane block to intravenous patient-controlled analgesia. As she accepted this modification, the modified plan was updated on the BCT.\nOn the day of surgery (Monday), the patient started fasting from 12:00 AM and was later placed under general anesthesia to undergo cesarean section. The surgery was completed without any complications. Postoperative pain management was well-controlled by intravenous patient-controlled analgesia. |
The patient was a 41-year-old female with past medical history of appendectomy and dysmenorrhea. She presented to the emergency department with nausea, severe vomiting, and acute pain in the lower abdomen. She reported having experienced asthenia and weight loss for one month. On clinical examination, abdominal distension and tenderness were discovered. Blood tests revealed leukocytosis with neutrophilia, and a contrast-enhanced abdominal computed tomography (CT) showed a 7 × 7 × 4 cm hyperenhanced mass in the cecum that caused complete bowel obstruction (). Also, a 5 × 3 × 3 cm right adnexal mass that compromised the ovary with intimate contact with the uterus was found (). Furthermore, the CT showed dilated loops in the small bowel (>4 cm), some of which had an enlarged wall thickness and presence of intraluminal fluid stasis ().\nWith these findings, particularly the observation of a mass through the CT scan, and due to the evident weight loss that the patient had undergone, neoplasia could not be ruled out. Surgery was decided, and at laparotomy, a volume of 200 ml of inflammatory fluid was found in the cavity. Most of the loops of the distal ileum were dilated, and a 7 × 7 × 3 cm cecum mass was discovered, which compromised the ileocecal valve and caused complete bowel obstruction. Surgical decision was straightforward, the cecum mass was completely resected, and a right hemicolectomy was executed. An ileocolic anastomosis was also performed during the procedure. Furthermore, the right adnexal mass that was previously identified through the CT scan (measuring 4 × 3 × 2 cm) was observed to be firmly attached to the ovary and the fimbriae and displayed a pale external capsule surrounded by a cystic component. Gynecology consultation was required, and due to the size of the mass and its characteristics, surgical removal of the right adnexal mass was performed. After completion, closure of the abdominal wall was performed, and the remainder of the procedure continued without any complications.\nPathology revealed a 4 × 3 × 2.5 cm blueish heterogenic mass that occluded 90% of the lumen of the cecum and the ileocecal valve. Microscopy revealed that the colon wall was invaded by glands and endometrial stroma. The colonic epithelium showed inflammatory changes and was negative for malignancy (Figures and ). In the ovarian parenchyma, an endometrial cyst was discovered, covered with siderophages. Glands and endometrial stroma were observed in the fallopian tube as well ().\nThe postoperative course of the patient was uneventful. She initiated clear liquids a day after surgery and was discharged once full diet was resumed. On follow-up controls, the patient was completely asymptomatic, without any pain or complications. |
A 42-year-old man presented with symptoms of right-sided chest pain, not related to physical activity or exertion. He also complained of shortness of breath on exertion. There was no history of cough, fever, or weight loss. Physical examination did not reveal anything significant. His haematological parameters were normal. The chest radiograph (PA view) showed a well-defined radio opaque shadow with no calcification or cavitations with its medial border merging with the right border of the heart, which was suggestive of an anterior mediastinal mass (). Lateral view confirmed the anterior mediastinal location of the mass (). An axial CT scan showed a heterogeneously enhancing right anterior mediastinal mass with areas of necrosis which was adherent to the pericardium (). Bronchoscopic examination showed extrinsic compression of the trachea and the right main bronchus. With a provisional clinical diagnosis of a lymphoma versus a teratoma, surgical excision was planned through an anterolateral approach. Per operatively, a 6 × 7 cm mass was found in the anterior mediastinum which had a smooth surface. The mass showed plenty of adhesions to the right lung and pleura. The mass was removed in piecemeal and was sent with a portion of the lung for histopathological evaluation.\nThe gross specimen consisted of about 250 gm of grey white soft tissue with extensive areas of necrosis. Also received was a lobe of lung measuring 9 × 9 × 2 cm. On microscopic examination, a biphasic neoplasm was noted with well formed glandular epithelial structures admixed with a spindle cell component (). The spindle cell component was monomorphous and showed a fascicular arrangement. The cells showed elongated vesicular nuclei with mild to moderate nuclear atypia. Mitosis ranged from 3–5 per 10 high power fields. The glandular spaces were lined by cuboidal cells with vesicular nuclei and moderate cytoplasm and showed eosinophilic secretions within their lumens which stained positively with the Alcian blue stain (). The toluidine blue stain failed to reveal any mast cells. There were foci of calcification and also large areas of necrosis and haemorrhage. The lung parenchyma and pleura showed no evidence of infiltration by tumour.\nOn immunohistochemistry, the glandular component showed cytoplasmic positivity for the epithelial markers cytokeratin and epithelial membrane antigen (EMA). The spindle cell component stained strongly with vimentin and bcl-2 (). The tumour did not stain with leucocyte common antigen LCA, desmin, S100, smooth muscle actin (SMA), CD34, or CD99. The proliferation marker Ki67 showed nuclear positivity in up to 10% of the tumour cells. With the combination of histopathological features and immunohistochemical findings, a diagnosis of a primary synovial sarcoma of the mediastinum was offered. The patient received adjuvant chemotherapy with Ifosphamide and Doxorubicin. Eight months later, he presented again with distant metastasis. |
An 82-year-old white female with past medical history significant for chronic obstructive pulmonary disease atrial fibrillation, chronic hyponatremia, congestive heart failure, stroke, and hypertension who presented to the radiology department in our tertiary care center for computed tomography (CT) guided biopsy for further evaluation of recently diagnosed 1.9 cm right lower lobe pulmonary nodule and mediastinal lymphadenopathy highly suspicious for primary lung malignancy. A 19-gauge guiding needle was advanced to the vicinity of a small mass in the peripheral third of the mid right lower lobe of the lung. Three 22-gauge Chiba biopsy needles were sequentially placed through the guiding needle and cytologic material was aspirated and reviewed. Subsequently three 20-gauge core biopsy specimens were obtained from the mass. There was volume of hemorrhage in area of biopsy but no pneumothorax or other immediate complications were noted. When she was turned over after the lung biopsy, she became unresponsive and developed cardiopulmonary arrest. A code blue was called; cardiopulmonary resuscitation was performed following ACLS guidelines. She could regain spontaneous circulation with epinephrine. She was intubated for mechanical ventilation and admitted to the hospital in medical intensive care unit. CT chest was performed immediately after resuscitation which showed frothy air dense material in the left atrium and one of the right pulmonary veins suggesting a Broncho venous fistula with air embolism (). CT head was obtained as well at the same time that showed no acute intracranial findings. Bedside transthoracic echo with contrast performed a few hours later was completely normal. There was no evidence of air bubble in atria or ventricles, ejection fraction was 55–60%, and right ventricle size and function were normal as well. Patient was placed in Trendelenburg position and was subsequently sent to hyperbaric oxygen chamber for treatment of air embolism. Patient tolerated the hyperbaric oxygen therapy very well. Patient was kept on the mechanical ventilator overnight. She was successfully weaned off from the mechanical ventilation and extubated on the next day of admission. |
The case of a 54-year-old male patient is reported. The patient, an ex-smoker, with systemic arterial hypertension (SAH) and heart failure (Chagasic cardiomyopathy with implanted pacemaker) had been submitted to coronary angioplasty. On being admitted to the emergency room of the University Hospital de Base in São José do Rio Preto, the patient reported he had pain in the right lower limb associated with edema, paresthesia, and coldness that had started suddenly. He also complained of pain in the left leg but said the pain was less intense. A duplex ultrasound of the iliac arteries showed total occlusion by an intraluminal thrombus on the right side with the superficial femoral and popliteal arteries occluded along their complete length. The popliteal artery of the left leg was also distally occluded (bifurcation). Bilateral embolectomy was performed without complications.\nOn the following day, he complained of “burning” pain and said that even the blanket on his legs bothered him. He had difficulty bending and straightening his knee and the sole of his foot. On physical examination, the patient had edema of the lateral and posterior compartments, and a lot of pain on compression, especially of the anterior compartment that was highly sensitive to slight pressure.\nThe chosen conduct was intermittent manual compression (massage) of the musculature of the compartments with the objective of stimulating lymphatic and venous return during the physical examination. The posterior compartment had a 100% improvement in the pain after 20 minutes of massage, the lateral compartment improved the pain completely after around 40 minutes, and the anterior compartment improved the pain by about 70% within one hour. With this improvement, the patient spontaneously bent his knee (without pain) and the sole of his foot (with a little pain in the anterior compartment). The patient was asked to evaluate his pain using an analog scale with the worst imaginary being 10 and no pain at all being 0. This was the only therapy used to reduce the edema, because the patient does not tolerate cardiac overload. The following day, the patient was walking around the ward without difficulties. |
A 35-year-old woman of East Indian descent presented to a hospital with a long-standing complaint of vague epigastric discomfort for 18 months. She noted that the upper abdomen became “full” over this time but there were no other symptoms present. After an abdominal ultrasound suggested the presence of a pancreatic tumor, a multiphase contrast-enhanced computed topography (CT) scan was ordered ().\nThe CT scan images revealed a well-circumscribed lesion in the pancreatic tail that measured approximately 6 cm in diameter. There were peripheral enhancement and a central area of cystic degeneration present. Minimal calcifications were noted. Serum assays of carcinoembryonic antigen and CA 19-9 were within normal levels.\nBased on characteristic findings on cross-sectional imaging in this young female, a diagnosis of pancreatic SPN was entertained and the patient was taken to the operating room for a distal pancreatectomy. This was completed uneventfully using the laparoscopic approach, with tumor extraction through an upper midline incision. A 19 Fr drain was placed at the pancreatic bed. The patient recovered uneventfully. The drain was removed on the fourth postoperative day and the patient was discharged home shortly after.\nOn gross pathologic examination, an encapsulated tumor 60 mm in maximal diameter was seen in the tail of the pancreas (). There was a distance of 1 cm between the tumor and the pancreatic resection margins. The tumor was composed of small polygonal cells with small centrally placed nuclei. Histiocytes with large inclusion vacuoles within their cytoplasm were seen occasionally. Centrally, there were multiple areas of tumor necrosis with cystic degeneration. There was an area of haemorrhagic necrosis that obscured the capsule at the distal margin. There were also areas of vascular invasion noted on high power examination. The cells stained positively for antitrypsin, vimentin, and neuron specific enolase on immunohistochemistry. All other stains were negative.\nA diagnosis of a pancreatic SPN was made and this patient underwent adjuvant systemic treatment with intravenous gemcitabine. After four years of surveillance, there has been no evidence of local or systemic disease recurrence in this patient. |
The patient was a 41-year-old female with past medical history of appendectomy and dysmenorrhea. She presented to the emergency department with nausea, severe vomiting, and acute pain in the lower abdomen. She reported having experienced asthenia and weight loss for one month. On clinical examination, abdominal distension and tenderness were discovered. Blood tests revealed leukocytosis with neutrophilia, and a contrast-enhanced abdominal computed tomography (CT) showed a 7 × 7 × 4 cm hyperenhanced mass in the cecum that caused complete bowel obstruction (). Also, a 5 × 3 × 3 cm right adnexal mass that compromised the ovary with intimate contact with the uterus was found (). Furthermore, the CT showed dilated loops in the small bowel (>4 cm), some of which had an enlarged wall thickness and presence of intraluminal fluid stasis ().\nWith these findings, particularly the observation of a mass through the CT scan, and due to the evident weight loss that the patient had undergone, neoplasia could not be ruled out. Surgery was decided, and at laparotomy, a volume of 200 ml of inflammatory fluid was found in the cavity. Most of the loops of the distal ileum were dilated, and a 7 × 7 × 3 cm cecum mass was discovered, which compromised the ileocecal valve and caused complete bowel obstruction. Surgical decision was straightforward, the cecum mass was completely resected, and a right hemicolectomy was executed. An ileocolic anastomosis was also performed during the procedure. Furthermore, the right adnexal mass that was previously identified through the CT scan (measuring 4 × 3 × 2 cm) was observed to be firmly attached to the ovary and the fimbriae and displayed a pale external capsule surrounded by a cystic component. Gynecology consultation was required, and due to the size of the mass and its characteristics, surgical removal of the right adnexal mass was performed. After completion, closure of the abdominal wall was performed, and the remainder of the procedure continued without any complications.\nPathology revealed a 4 × 3 × 2.5 cm blueish heterogenic mass that occluded 90% of the lumen of the cecum and the ileocecal valve. Microscopy revealed that the colon wall was invaded by glands and endometrial stroma. The colonic epithelium showed inflammatory changes and was negative for malignancy (Figures and ). In the ovarian parenchyma, an endometrial cyst was discovered, covered with siderophages. Glands and endometrial stroma were observed in the fallopian tube as well ().\nThe postoperative course of the patient was uneventful. She initiated clear liquids a day after surgery and was discharged once full diet was resumed. On follow-up controls, the patient was completely asymptomatic, without any pain or complications. |
A 55-year-old female diabetic and hypertensive presented with a history of recurrent episodes of paroxysmal palpitations since six years despite oral verapamil. Physical examination was unremarkable. ECG during tachycardia showed narrow QRS complexes at the rate of 200 beats/min with ECG in sinus rhythm being normal. Echocardiogram was otherwise normal except dilated coronary sinus. In view of recurrent supraventricular tachycardia, she was taken up for electrophysiology study and radiofrequency catheter ablation.\nInitially a 6F decapolar catheter (2-5-2 spacing) was positioned in the coronary sinus (CS) through right internal jugular vein. The left femoral vein was cannulated and a 6F quadripolar catheter was advanced under fluoroscopy guidance. As the catheter was advanced, it was noticed to be on left side of the spine. Hence 20 ml of non-ionic contrast medium was injected through the vascular introducer sheath. This revealed the presence of inferior vena cava on the left side of the spine (), which continued as dilated hemiazygos vein, which in turn drained to dilated coronary sinus through a persistent left superior vena cava (). Subsequently a 6F quadripolar (5mm spacing) catheter was advanced through anomalous venous channel and could be manipulated to the RV apex. But a second quadripolar catheter could only be advanced to low RA which was left in place for programmed electrical stimulation as stimulation from the CS catheter failed to capture the atrium due to large CS. Since the third catheter could not be manipulated, right internal jugular vein was cannulated and a 7F deflectable quadripolar catheter was used because of its stability to record the His bundle electrogram ().\nDuring EP Study patient was found to have a dual AV nodal physiology. A narrow QRS tachycardia was easily induced with programmed electrical stimulation with tachycardia cycle length of 280ms and an activation sequence similar to AV node reentry tachycardia. We performed third right internal jugular vein puncture to introduce the ablation catheter. The tip of the catheter was positioned at the posterior atrial septum just above the coronary sinus ostium (). The ablation electrode was stabilized at a target site that was associated with an AV ratio of 1:4 to 1:8. Radiofrequency current was delivered at maximum power of 40W and a maximum temperature of 60ºC for a maximum duration of 30 seconds each. Delivery of RF energy was associated with an accelerated junctional rhythm. A total of eight RF applications were applied. There was no inducible SVT after the procedure with programmed stimulation without and with isoproterenol infusion and the procedure was uncomplicated. Ultrasonography of the abdomen was done before discharge showed normal visceral situs, direct drainage of hepatic veins into the RA and the presence of left sided IVC ascending posterior to the aorta. No recurrences of tachycardia were noted at six months follow up. |
The patient is a 28-year-old man with a history of major depressive disorder, hepatitis C, biliary colic (status postcomplicated cholecystectomy), multiple concussions, and chronic back pain who presented to the emergency department with abdominal pain clinically concerning for acute appendicitis. He localized his pain to the right lower quadrant, complained of right lower quadrant pain with palpation of the left lower quadrant (Rovsing's sign), and indicated his pain was reproducible with extension of the right hip (psoas sign). His vital signs on presentation were notable for the absence of fever or tachycardia and his initial laboratory findings revealed normal chemistries and a normal white blood cell count of 4.2. He received intravenous morphine and an urgent CT scan of his abdomen. Enroute to the CT scanner, the patient mentioned that he had been struggling with sadness and suicidality since his pregnant fiancée had recently been killed in an automobile accident. The emergency physicians felt the pretest probability for acute appendicitis was extremely high and asked the psychiatry consult-liaison team to “evaluate him quickly before he goes to surgery.” Ultimately, the imaging was negative for acute appendicitis, surgery was cancelled, and his disposition from the emergency department was left to psychiatry.\nReview of the electronic medical record showed that the patient had established care at this institution one month prior to this presentation when he presented with biliary colic resulting in a lengthy hospitalization for a complicated cholecystectomy, challenging postoperative pain management, and a new diagnosis of hepatitis C. During that hospitalization, he was seen by social work who documented that he was baffled as to how he had contracted hepatitis C but offered that he had endured many losses in his life including the death of his mother when he was 6 years of age and the death of his brother when he was 19, so felt he had developed strong coping skills. In terms of his social history, he reported that he was engaged to be married and worked as a mathematics and physics professor at a prestigious university as well as an engineering consultant in the private sector. He also reported that he had sustained a number of musculoskeletal injuries resulting in chronic pain while playing Division I football in college and that he had been drafted by the National Football League. Upon discharge from that admission, he established care with a primary care physician for ongoing management of chronic pain. At his initial appointment he reiterated the social history he provided to the social worker and signed a narcotics agreement.\nOn initial assessment in the emergency department by the psychiatry consult-liaison team, the patient was observed to be a young, overweight Asian male dressed in a plain white T-shirt and track pants. His hair was greasy, his fingernails were long and dirty, and one of the lenses of his eyeglasses had a small crack. He made poor eye contact and focused his gaze on his tablet computer for most of the interview. His affect was withdrawn and had minimal range. On interview, he began the conversation by requesting placement at an inpatient psychiatric facility for electroconvulsive therapy, reporting that he had been suffering from very low mood since his pregnant fiancée had been killed by a drunk driver eight months ago. Because the electronic medical record indicated that he had been engaged to be married just one month ago, we asked him to confirm the date of his fiancée's death, which he could not recall. He answered all questions in a matter-of-fact tone and showed very little range of affect. In terms of his mood, he did not elaborate on his experience other than to say he felt “depressed and suicidal” with the vague plan of jumping in front of a train. He evaded further discussion of his mood symptoms by spontaneously offering details of his social history. He spoke about his profession as a tenured mathematics and physics professor at a prestigious university although when asked about the nature of his research he could only vaguely describe studying “time bends in space using some of Einstein's old formulae.” He spoke in some detail about his career as a varsity football player, citing football injuries as the source of chronic back pain. When asked to provide a collateral contact, he reported that both of his parents, multiple siblings, and cousins had died during his early childhood.\nThe patient was accompanied by a male friend who was casually but neatly dressed and of approximately the same age. The patient provided us with permission to speak with his friend who appeared uncomfortable, saying “I didn't know I'd have to talk!” He reported that he and the patient were work colleagues who had known each other for a few years but refused to reveal where they worked. He provided no further information, other than saying “He's been really depressed and I just know he needs help before something happens.” He then quickly left the hospital without saying goodbye to the patient.\nAlthough, the patient initially reported that both parents were deceased, his father was listed as an emergency contact in his medical record. When confronted with this, the patient said that this was his step-father and gave consent for contact. The father reported that, five years ago, his son graduated with poor grades from a university which does not have a Division I football team. He confirmed the patient had played football in high school and had sustained multiple concussions. The year after graduation, the patient had lived with his parents briefly, but because of escalating narcotic use and lack of employment was asked to leave. Since then, he has suffered from severe opioid use disorder and has been homeless and unemployed. He has travelled to various hospitals within the city and even out of state to seek pain medication and care and has told a similarly fictional narrative to other physicians.\nWe obtained information from a local emergency community outreach agency which indicated that the patient had presented with the chief complaint of suicidality to multiple emergency departments in the city resulting in two previous inpatient psychiatric stays over the past year. We obtained records from his most recent inpatient psychiatric hospitalization about six months ago, where he presented with depression related to his girlfriend's putative recent breast cancer diagnosis. He was discharged on an antidepressant, a mood stabilizer, and oxycodone for chronic back pain.\nWhen gently confronted with these inconsistencies, the patient appeared unperturbed and easily provided further elaborate details to explain them. However when further pressed, he stated he believed he needed a “dramatic” reason for his depression and suicidality to receive help and asked, “Can I just be depressed and suicidal?” He appeared perplexed as to why we attempted to clarify his previous statements or their relevance for his care. Despite this, he continued to state that he felt very depressed and would not be able to maintain his safety in the community. |
A 55-year-old male patient fell from a height of approximately 5 m on November 26, 2016. During the fall, due to other objects, the welding electrode in his left hand was inadvertently inserted into his brain. After the injury, there was bleeding at the location of foreign-body penetration. The patient reported dizziness and headache but no nausea, vomiting, bleeding from the ears or nose, poor physical activity or convulsions. The patient was admitted to the hospital 3 hours after the injury. The patient exhibited a normal body temperature, a Glasgow coma scale score of 15 points, clear consciousness and speech, and cooperation during the physical examination. The welding electrode was penetrating the brain from the outer sidewall of the left eye socket (Fig. A). Both pupils were equal and round, with a diameter of 3.0 mm, with direct and indirect light reflex intact. The muscle strength and muscular tension of the limbs were normal. Bilateral Babinski signs were not induced. The patient was given a preoperative test immediately after admission, as well as an intramuscular injection of tetanus antitoxin and an intravenous infusion of antibiotics. CT was performed and showed an object of metallic density penetrating the skull and entering the brain parenchyma in the frontotemporal region. The sagittal reconstruction of intracranial nerves and vessels was carried out according to the CT images of the head before operation, and whether the electrodes injured the intracranial nerves and vessels was determined. The results showed that there was no neurovascular injury. Accurate preoperative assessment. (Fig. B, C). Surgical treatment was performed about 45 hours after injury due to family economic reasons. During the surgery, the metal (electrode) was observed to have passed through the skull wall from the bottom of the anterior cranial fossa, and the surrounding brain tissue was necrotic with hemorrhage (Fig. A). Fully exposing the metal foreign body after achieving hemostasis showed that it was slanted upward, reaching beneath the cerebral falx. After the welding electrode was cleared from the surrounding blood vessels, it was slowly pulled out from the outer corner of the eye, and no bleeding was observed (Fig. B). The total length of the electrode is about 17 cm and the intracranial segment is about 7.2 cm (Fig. C, D). The cranial wall was ruptured, and the cavity was repeatedly irrigated with 3% hydrogen chloride solution to completely stop the bleeding. Then, dura mater was closed tightly with tension-reduced sutures. The surgery was continued from this point by the ophthalmology department to complete the eye treatment. Postoperative head CT scans were reviewed (Fig. A, B), anti-inflammatory treatment was administered, and the patient was closely monitored for cerebrospinal fluid leakage (no colorless fluid flowed through the nostril and external auditory canal) and fever. There were no complications during surgery, and the patient was administered broad-spectrum antibiotics and prophylaxis for seizures and tetanus. Postoperatively, the surgical site showed no signs of infection (Fig. C). The patient was followed for 2 months, no significant complications, such as cerebrospinal fluid leakage or fever, were observed during the patient's review (Fig. D) or follow-up. |
A 43-year-old female was in her usual state of health until about January of 2011 when she developed nausea, vomiting and diarrhea. Diarrhea persisted for a couple of months and she sought medical help in March 2011. Initial conservative management followed by a detailed workup done by a gastroenterologist was negative. Later, in August 2011 the patient presented to ED with complaints of melena. Her CT scan revealed a 9-cm mass in the tail of her pancreas with hepatic metastasis. Liver biopsy confirmed grade 2 neuroendocrine tumor (Ki-67 of 6%). The patient was initiated on Sandostatin LAR 30 mg every 30 days. She noticed rapid improvement in her energy level. She subsequently underwent Y90 radio-embolism of hepatic metastatic disease first in the right lobe of the liver followed by the left lobe in the months of September and October 2011. The patient had a stable course until December 2011, she had another episode of GI bleed. In January 2012 she underwent partial pancreatectomy, splenectomy, partial gastrectomy and left hepatic lobectomy. She was started on sunitinib in February 2012, which she had to rapidly discontinue within a month due to severe fatigue. She was started on capecitabine and temozolomide (CAPTEM) in April 2012. The patient tolerated CAPTEM well and started gaining weight. An abdominal MRI from May 2013 showed mild worsening of some of the hepatic lesions, however the rest of the disease was stable. She underwent two more doses of Y-90 radioembolism. She was continued on long acting somatostatin analog and CAPTEM and her subsequent surveillance scan in November 2013 showed stable disease. A follow-up MRI of the abdomen in February 2014 showed mild progression of one of the hepatic lesions. She was taken off CAPTEM and started on everolimus 10 mg daily. We had to reduce the dose of everolimus to 7.5 mg daily due to stomatitis. The patient did exceptionally well on the reduced dose of everolimus and had stable disease until October 2015, at which time she was enrolled into a Phase I clinical trial of fosbretabulin for progressive disease in the liver and retroperitoneum. The patient got the first dose of fosbretabulin in November 2015. She only received three cycles of fosbretabulin before she developed disease progression in the left supraclavicular and left axillary lymph nodes. In February 2016 she was started on off-label pembrolizumab. She had stable disease on pembrolizumab for 16 months before her disease progressed in axilla and breast. Figure shows the current disease burden of patient with help of gallium DOTATATE scan. |
A 56 year-old female was transported to the emergency department by EMS after physically threatening her husband with a knife. According to the patient, she experienced altered sensorium for one week prior to presentation, primarily resulting in suicidal and homicidal command hallucinations instructing her to overdose on NSAIDs and kill her husband. She denied any headaches, vertigo, fevers, head trauma, urinary symptoms, or use of illicit substances. There was no history of psychosis, schizophrenia, mania, or depression and a review of her medication list for any potential hallucinogenic agents did not indicate a pharmacologic etiology. Her past medical history was comprised of two prior strokes, the most recent being two years ago that involved the right frontal lobe. MRI of the brain at the time also showed evidence of an old right parietal lobe infarct. An echocardiogram during that hospitalization revealed a severely decreased left ventricular function and the patient was initiated on warfarin for anticoagulation. Since the two prior cerebral infarcts, the patient and her husband denied noting any behavioral changes, cognitive impairment, or any focal neurologic deficits. On presentation, she appeared disheveled and exhibited a flat affect with minimal verbalization. Physical examination revealed only a left hemianopia without hemiplegia. All cranial nerves were evaluated as well as gait, cerebellar function, and proprioception, which were all found to be normal. The patient was alert and oriented with intact mentation.\nShe was initially admitted to the psychiatric unit for further evaluation where the patient participated in daily activities and reported no symptoms. Given the acute onset of her symptoms, laboratory studies and brain images were obtained in order to rule out an organic etiology. A complete blood count was normal and a basic metabolic panel revealed normal electrolytes and renal function. Additional labs included liver function tests, lipid panel, cardiolipin antibody, and TSH, which were all normal and RPR was nonreactive. A urine sample was negative for urinary tract infection and 8-panel urine drug screen was negative. An MRI of the brain was obtained, which revealed old ischemic infarcts within the right parietal and frontal lobes along with a new acute right occipital lobe infarct with hemorrhagic transformation (). The patient was subsequently transferred to the medical unit for further workup and management.\nThe patient was placed on telemetry, which showed normal sinus rhythm. Given the involvement of multiple brain territories and circulations, a cardioembolic source of stroke was highly suspected. A cardiologist evaluated the patient and performed a transesophageal echocardiogram that revealed a low ejection fraction of less than 20%. In addition, a bubble study was performed that was positive for a patent foramen ovale. Given her significantly low ejection fraction, the patient underwent placement of an automated implantable cardioverter-defibrillator. The patient's presenting symptom of psychosis, primarily in the form of auditory hallucinations, was self-limited and resolved on day two of hospitalization without requiring the use of antipsychotic medications. |
A 45-year-old male with a previous history of tympanoplasty and functional endoscopic sinus surgery with septoplasty 10 years earlier presented to the ear, nose, and throat (ENT) clinic with several months of left moderate-to-severe otalgia and a sensation of ear blockage in his left ear accompanied by ipsilateral hearing loss. He gave a history of multiple failed ear wax removal in his left ear that had been performed at several ENT clinics, despite the use of alkaline ear drops.\nOn examination, the patient was comfortable and afebrile, and his vital signs were stable. Otoscopic examination of the left ear revealed impacted left ear wax covering the tympanic membrane, which could not be assessed. Otoscopic examination of the right ear also demonstrated mild ear wax, and the tympanic membrane was unremarkable. Oropharynx examination was unremarkable, the lymph nodes of the neck were not palpable, and all cranial nerves were intact upon examination. Nasal endoscopy revealed no pathologies. Ear wax removal under suction was attempted and failed. Another trial of removal after using alkaline ear drops for several days was also attempted but was unsuccessful. However, the surgeon became suspicious that the patient had KO rather than impacted ear wax because the wax was thick, had the appearance of keratin plugs, and was hard to remove after several attempts, despite the use of ear alkaline drops.\nBlood test results of the patient were within normal limits. The patient was planned for microscope-guided examination of the ears under general anesthesia. The examination revealed that the left ear was full of wax that was accumulating in the skin and contained a thick keratinous plug that had dilated the external auditory canal (EAC) with pockets and bone remodeling. Furthermore, the patient ear canal was circumferentially distended with a normal annulus. The tympanic membrane became visible and was intact. The keratinous plug was removed, and a diagnosis of KO was established (). An ear pack was draped with antibiotics and placed in the left ear. The patient was extubated, shifted to the ward without any complications, and discharged the same evening with the ear pack, which was removed after 3 weeks in the outpatient clinic. The patient was started on ciprofloxacin ear drops and analgesia for 1 week.\nIn the follow-up, the ear pack was removed, his hearing returned to normal level, and the pain disappeared. Pathological analysis of the removed plug revealed acellular lamellated keratin flakes and keratinous material (Figures and ), which confirmed our diagnosis. |
One day old male baby presented to the orthopedics emergency by his parents with bluish discoloration of entire left forearm and hand 12 hours after delivery. The baby was delivered by a trained birth attendant in a village by vaginal delivery but with hand prolapse presentation. The baby was forcibly delivered by the same attendant with vigorous pulling of the hand of the extremity. The baby was pre-term with birth weight of 2.2 kg. The mother never underwent any antenatal checkup. As per statement given by the parents the affected forearm after birth was discolored and the skin temperature was less as compared to normal side 6 h after birth.\nOn clinical examination after 12 hours of delivery, the bluish discoloration of the extremity from left elbow downwards was noticed. The child was not in shock. His vitals were stable and looked apparently healthy. The affected extremity look longer. A gangrenous patch of 1” × ½” size over the lower end of forearm with ensuing gangrene of all the fingers was seen. The left hand and forearm were noted to be swollen, cool, cyanosed and tip of the fingers were necrosed []. Skin blisters were present on the elbow area. The infant had no spontaneous finger or wrist motion. There was neither any distal pulsation nor any sensation in the hand or forearm and the consistency of forearm was firm to hard. There was no other visible injury in the body.\nX-ray of the affected extremity including shoulder, elbow and wrist showed a wide gap of 6–7 cm between forearm bones and distal end of humerus []. The humerus and forearm bones appeared normal. A Doppler study of the affected limb revealed absent flow in the forearm distal to the elbow. Routine hematologic investigations including blood sugar estimation were done for the mother of the baby and she was found to be nondiabetic.\nThe baby was given parenteral antibiotics to prevent sepsis. After 3 days, a clear-cut demarcation line developed between the gangrenous and healthy area at elbow level []. Above elbow amputation was done after taking consent from the parents []. Intraoperatively, it was seen that the lower end of the humerus was separated from the forearm bones at the level of distal humeral physis. The vessels and nerves were found to be stretched out. The postoperative period was uneventful. |
A 69-year-old female with no smoking history or family history of paragangliomas or other neuroendocrine tumors initially noted discomfort in her left neck in 2007 which was thought to be related to a cervical disk pathology; however, by March 2010, her symptoms had persisted and then she had laryngitis for about 5 weeks. She was seen by her doctor who could not hear her left carotid, and she was also noted to have a grayish appearance. A left tonsillar lesion was noted, which was felt to be secondary to medial disposition of the tonsil from some extrinsic compression. A mass was noted at the left angle of the jaw in level 2. She was prescribed antibiotics; however, they did not improve her symptoms and vascular surgery was consulted. A CT angiogram of the neck was performed in 2010. Contiguous axial CT images were obtained of the neck using 80 mL of Isovue-370 IV contrast followed by 60 mL of normal saline infused at 6 mL/seconds. A 4.6 × 3.2 × 5.5 cm heterogenous attenuation, hypervascular mass appearing to arise from the left retrostyloid parapharyngeal space was most consistent with a paraganglioma, likely globus vagale. Encasement and marked narrowing of the left cervical internal carotid artery were noted ().\nMRI was performed which further demonstrated a large left cervical mass causing markedly diminished signal in the cervical left internal carotid artery likely representing diminished flow due to compression by the mass. Prior to surgery, she was put on alpha and beta blockade. She underwent embolization as well as excision of the tumor by a combination of transcervical and transoral approaches with lip and mandibular split. Her tumor was encroaching the left jugular foramen with a persistent tumor evident extending up to the jugular foramen at the termination of the procedure, despite intentional sacrifice of the cranial nerves 9, 10, and 12, given their relationship to the tumor. There was a small portion of the residual tumor that could not be resected at the superior portion of the jugular foramen. The pathology showed an extra-adrenal pheochromocytoma (paraganglioma) from the left carotid space, superior to the carotid bifurcation. Immunohistochemical staining was positive for chromogranin, synaptophysin, and S-100 and was faint for glial fibrillary acidic protein (GFAP). It measured 4.3 × 3.8 × 2.5 cm positive lymphovascular invasion, the mitotic rate is 1/10 high-power field, and necrosis was present, likely due to embolization. The margins were positive. She was on surveillance after surgery.\nSince her surgery, sequential MRI scans of the brain showed a slowly progressive recurrence. Surgery was not recommended due to potential morbidity. In 2015, she underwent stereotactic radiosurgery at a dose of 15 Gy to the left paraganglioma. In 2016, MRI of the neck with and without contrast revealed a mass lesion centered within the left jugular foramen, appearing mildly increased in size with an unchanged mild mass effect upon the adjacent left cerebellar hemisphere. The size was 20 mm in maximal dimension as opposed to 17 mm compared to previous imaging 6 months prior (). A CT scan of the chest showed scattered noncalcified pulmonary nodules. The largest was noted to be in the right middle lobe, demonstrating no discernible growth based on the volumetric analysis compared to previous imaging. Her PET/CT revealed FDG uptake in the right middle lobe pulmonary nodule. She was also found to have FDG uptake within bony lesions involving T3, T7, L3, and the T1 transverse process consistent with metastasis. FDG uptake was noted within the biopsy-proven left jugular foramen paraganglioma. She underwent CT-guided FNA of a 6 mm right middle lobe lesion which revealed neoplastic, low-grade tumor, favoring a neuroectodermal tumor, and after discussion in MDC, it was concluded that her paraganglioma disease was likely metastatic to the lung and spine. She underwent stereotactic ablative radiation therapy to the T7 and T3 paraganglioma metastasis to the spine to a dose of 1600 cGy.\nIn 2017, she had a T4 vertebral body biopsy and kyphoplasty for her back pain secondary to metastasis. In the fall of 2017, she was found to have a compression fracture and underwent T3 vertebroplasty with improvement in pain. Her MRI of the thoracic and lumbar spine showed multiple osseous lesions (). It also showed metastatic disease including the stable lung nodule which was biopsied and mild degenerative disc disease. In January 2018, CT of the chest abdomen pelvis showed stable disease (T3 and T7). CT of the neck showed stable disease in the left jugular foramen. In the spring of 2018, she was diagnosed with a T8 compression fracture and underwent T8 vertebroplasty. She was started on monthly denosumab. In the fall of 2018, CT chest showed stable lung nodules and CT abdomen pelvis showed mixed sclerotic and lytic lesions at L1 and L3 with increased compression of the left side of the superior endplate of L3 since the prior CT. MRI showed that the metastatic lesions in the L-spine were slightly larger without any new lesions. Soft tissue mass in the left jugular foramen appears to be stable without any cervical lymphadenopathy; however, there was a concern of C3 metastatic lesion and metastatic lesions in the thoracic spine that also increased in size. She was referred for genetic testing, and the results came back positive for a mutation in SDH-C, c.397C>T (p.Arg133∗), which is associated with autosomal dominant hereditary paraganglioma-pheochromocytoma syndromes, gastrointestinal stromal tumors, and renal cell carcinoma []. This sequence results in a premature translational stop signal in the penultimate exon of the SDHC mRNA at codon 133 which is expected to delete the last 37 amino acids of the SDHC protein. This variant has been reported in several individuals with paragangliomas [–].\nHer case was discussed with local and national experts, and it was recommended to get a gallium 68 dotatate PET scan (as MIBG is not helpful after the lesions have been radiated). She underwent a PET/CT GA-68 dotatate scan in the spring of 2019 which revealed multiple scattered metastatic lesions to the spine with subtle increase in size and conspicuity of a few sacral and lumbar lesions (). She was started on lutetium 177 dotatate (Lu 177), a radiolabeled somatostatin analog, in April 2019 and received a half dose in the first cycle due to concerns around catecholamine storm. She had borderline elevated 24-hour urine metanephrine and mildly elevated norepinephrine and dopamine level on plasma catecholamine fractionation and was treated with alpha and beta blockade prior to Lu-177 treatment. Other labs performed prior to treatment include a basic metabolic panel which was all within normal limits, liver function tests which were within normal limits, a complete blood count with a hemoglobin of 10.2 gm/dl, normal platelet count of 368 K/cmm, and normal white blood cell count of 4.82 K/cmm. Her folate was elevated at 24 ng/ml. She tolerated the first cycle and received the full second dose in June 2019. A follow-up PET/CT GA-68 dotatate scan in December 2019 showed a modest response to therapy with no new lesions identified while previously these lesions had been progressively getting worse (). During the most recent oncology follow-up visit in February 2020, the patient reports some continued fatigue that is beginning to improve. |
A 34-year-old gentleman was first referred to orthopaedic services with a history of persistent knee pain, located over the right femoral condyle near the origin of the lateral collateral ligament. Eleven years prior to presentation, the patient was involved in a road traffic accident where he sustained a fracture of the right femoral neck and ipsilateral shaft. Initial management consisted of open reduction, fixation of the femoral neck fracture with cannulated screws and the ipsilateral shaft fracture with plating. The patient later had a revision of the plate to a femoral nail. Union was subsequently achieved with the femoral shaft fracture; however, a significant external rotation deformity was noted, and discomfort to the knee.\nThe patient was referred on to our tertiary referral orthopaedic centre for femoral de-rotation surgery. An initial CT scan performed revealed an external rotation deformity of 45 degrees. The patient procedure included removal of femoral nail, osteotomy with de-rotation surgery, and subsequent exchange nail. The femoral nail was fixed proximally and locked into place distally, following the corrective 45 degrees of internal rotation achieved to the distal femur during osteotomy, performed under intraoperative radiographic guidance. The post-operative rehabilitation regime consisted of initial touch weight bearing only, with gradual increments in weight bearing status. At six months follow-up, the patient complained of distal lateral femur pain. A repeat CT scan was performed, revealing a delayed femoral union. This was initially thought to be the cause of the patient’s symptoms. The patient was admitted for dynamization of the femoral nail with an injection of bone graft substitute, and iliac crest graft. Despite eventual union, the patient continued to complain of pain at the distal lateral femur, with a cracking sensation on movement now noted.\nRepeat clinical examination revealed a palpable crepitus over the distal iliotibial band with a snapping sensation, as it appeared to catch. A palpable small, solid swelling was noted at the posterolateral right knee. Ultrasound and CT revealed intra-articular loose bodies lying within the lateral para-patellar gutter. The patient subsequently underwent arthroscopy with removal of loose bodies. Despite this, at six-months follow-up to arthroscopy, the patient continued to complain of posterolateral knee pain. MRI showed no evidence of abnormality to the popliteus tendon or muscle, but highlighted the fabella embedded within the lateral head of gastrocnemius at the posterolateral corner of the knee. (Image ). The fabella was also notable on radiographs (Image ) and CT (Image ). Ultrasound scan confirmed the fabella as mobile on movement and associated with tenderness. The diagnosis of Fabella Syndrome was made, and the patient planned for a Fabellectomy procedure.\nAn initial arthroscopy was performed to review the knee joint, and to potentially assist in surgical excision of the fabella. A complete diagnostic arthroscopy was performed, with the fabella unable to be identified through the posterior capsule. After arthroscopy, open fabellectomy was performed. In view of the patient’s extensive scarring from prior orthopaedic procedures, a short 2cm incision was made directly over the palpable fabella (Image ). The common peroneal nerve was identified and spared. Subsequent incision left a cuff of biceps femoris to protect the nerve. Gastrocnemius was split and incised directly over the fabella, which was removed in entirety (Image ). Histopathology confirmed the diagnosis.\nThe patient was followed-up at two and ten months postoperatively. At both follow-ups, he described the complete resolution of his posterolateral knee pain. |
The patient was a 38-year-old nulliparous woman who suffered from infertility of unknown origin. She had been treated with assisted reproductive technologies including artificial insemination and in vitro fertilization for over four years. During her treatment for infertility, cytological review followed by colposcopic biopsy revealed an invasive nonkeratinizing squamous cell carcinoma (SCC). A 1 cm mass was identified in the uterine cervix, but a pelvic MRI did not describe the cervical mass or parametrial invasion. Additionally, a submucosal leiomyoma of 15 mm in diameter was found in the uterus (). CT scans showed no signs of lymph node swelling or distant metastases. Based on these findings, she was diagnosed with stage IB1 cervical squamous cell carcinoma. We offered radical hysterectomy and pelvic lymphadenectomy as standard treatment although she strongly desired fertility preservation. The submucosal leiomyoma may have been the cause of her infertility, and she was keen to resect the myoma during the same procedure. Submucosal leiomyomas can usually be resected with hysteroscopy but was not advised in this case from the oncological viewpoint. As such, we obtained informed consent and performed an abdominal radical trachelectomy followed by abdominal myomectomy.\nDuring the surgery, we first drained the ascites in the pelvic cavity, resected bilateral pelvic lymph nodes, and sent them for intraoperative pathology. They were reported to be negative. The paravesical and pararectal spaces were then developed. The ureters on either side were resected to their insertion into the bladder. The uterine arteries were ligated and cut at the origin where they branched from the internal iliac arteries. Next, the uterosacral ligaments were divided. A colpotomy was performed circumferentially, and the cervical specimen was excised together with the parametrium at least 2 cm below the internal os. During the surgery, a frozen section procedure was performed for histology. The patient was found to have a 5 mm free cervical margin. A permanent cerclage was placed at the level of the isthmus. The uterus was then reanastomosed to the vagina. We then performed resection of the submucosal myoma via a uterine vertical incision. An intrauterine device (FD-1; Fuji Latex Co., Tokyo, Japan) was placed in the uterine cavity. The operation duration was 339 min, and blood loss was 500 ml. The surgery was completed with no complications.\nThe final histological specimen confirmed the diagnosis of squamous cell carcinoma, keratinizing type of cervix uteri, pT1B1. Exocervical, endocervical, and deep margin regions were negative. There was no metastatic lesion in the lymph nodes or lymphovascular space invasion. Leiomyoma of the corpus uteri showed no malignancy. No adjuvant treatment was administered, and no recurrence has been reported for at least 18 months postoperatively.\nSix months after the surgery, she became pregnant following the postoperative first embryo transfer. The fetus was appropriate for gestational age. At 21 weeks of pregnancy, she claimed vaginal bleeding, and her lower uterine segment lengths were shortened from 23 mm to 13 mm. She was diagnosed with threatened abortion, and tocolysis was started. At 25 weeks, preterm premature rupture of membranes occurred. She received antibiotics, and intramuscular betamethasone was administered. At 26 weeks, a male baby weighing 980 g was delivered with an Apgar score 3/5/7 by caesarean section due to chorioamnionitis. The baby received general care in a neonatal intensive care unit for four months and weighed 4520 g when discharged. He is now 6 months old and is well. There has been no recurrent disease of her cervical cancer for 18 months from the trachelectomy and myomectomy. |
A 39-week term, 41-day-old male infant with hypoxic-ischemic encephalopathy initially presented to the emergency department for evaluation of a rapidly enlarging mass located on the medial aspect of the right upper extremity. The mass was noted to be pulsatile with no effects on the radial and ulnar pulses. The patient had normal motor function, with full active movement of all extremities and mobility appropriate for his age. The patient's parents had noticed the mass 5 hours earlier and reported that it had been rapidly enlarging since then (). The patient had undergone PICC placement in the affected area during the neonatal period for treatment of Lennox-Gastaut syndrome without status epilepticus.\nThe physical examination was notable for a pulsatile mass. Capillary refill occurred within <2 seconds. The pulses for all extremities were 2+, his extremities were warm, and he had no abnormal color and no edema. Computed tomography angiography showed a pseudoaneurysm of the right distal brachial artery measuring 1.8 × 1.6 × 2.1 cm (). Communication between the pseudoaneurysm and the lumen of the brachial artery was seen, with no other vasculature affected and no other abnormalities. Further evaluation with contrast-enhanced magnetic resonance imaging showed a 2.1 × 1.7 × 1.1 cm mass along the right brachial artery with vascular flow (). Because of concerns for a possible vascular abnormality from the previous iatrogenic injury, the patient was admitted to the neonatal intensive care unit for observation. During the next 2 days, the mass continued to grow, and the plastic surgery and vascular surgery divisions were both consulted.\nRoutine preparations for surgery were performed, and the parents provided written informed consent for microscope-assisted brachial artery repair planned by the primary plastic surgery team with intraoperative assistance from vascular surgery.\nWith the patient under general anesthesia, a curvilinear incision was performed over the ulnar aspect of the elbow. Next, the brachial artery pseudoaneurysm was exposed. Once the brachial sheath had been identified with dissection, the pseudoaneurysm was isolated from its superior and inferior attachments to the brachial artery. Once the pseudoaneurysm had been adequately mobilized, the brachial artery was dissected off the pseudoaneurysm down to the level of the defect. After clamping the proximal and distal vascular structures, the cavity of the pseudoaneurysm was entered and evacuated. The pseudoaneurysm pouch was completely resected and removed from its remaining attachment to the brachial artery. After the diseased segments of the brachial artery had been excised, the brachial artery was deemed conducive for direct microvascular end-to-end anastomosis to repair the brachial artery defect proximal to the antecubital fossa at which the vessel size was 2 mm. The repair was performed under an operating microscope with 9-0 nylon interrupted suture. No leak was noted when blood flow across the artery was restored. The artery was sprayed with papaverine, and a warm surgical sponge was placed over it for several seconds. This was a tensionless repair. Next, the patient underwent systemic heparinization. After hemostasis was achieved, the incision was closed. The blood loss was minimal at ∼15 mL. The surgery lasted 3 hours, 9 minutes.\nIn the postoperative period, the patient was given low-dose intravenous heparin (10 U/kg/h) for 48 hours. During the postoperative period, the patient was noted to have warm, dry, well-perfused, symmetric capillary refill in both upper extremities with no frank drainage or bleeding at the surgical site. After an uncomplicated postoperative course, the patient was discharged on postoperative day 3 with an arm splint and instructions to receive an oral suspension of acetaminophen 160 mg/5 mL. The patient was followed up by the plastic surgery service after 2 years, and the surgical site was well healed (). The patient's hypoxic-ischemic encephalopathy led to complaints in the range of extension of the left upper extremity. However, the patient had better function with the right upper extremity, where the operation had occurred, than with the left upper extremity. The brachial, ulnar, and radial pulses were present bilaterally, and the wrist circumference was equal. The patient had a full range of motion in the right upper extremity without any neurologic deficits. |
A 66-year-old woman was admitted to hospital with severe backache. This patient had undergone ascending aortic replacement surgery to treat type A AD six months prior to the presentation . The medical history of the patient included hypertension for the past 25 years, nephrectomy due to nephrolithiasis eight years earlier, polio sequela and a motor deficit of the left leg.\nHer systolic blood pressure was 130 mmHg on the right arm and 110 mmHg on the left arm. All arterial pulses were determined by manual examination. Contrast-enhanced computed tomography revealed a type B dissection flap involving the left subclavian artery with retrograde progression. The diameter of the true lumen had narrowed significantly to < 10 mm, and the total diameter (with false lumen) was 43.7 mm at the widest section . The peak aortic diameter was measured at 67.2 mm. We therefore initiated preparation for the TEVDAR surgery.\nThe patient underwent surgery under general anaesthesia. During the operation, an initial exploration of the right common femoral artery was conducted. A 5-F pigtail catheter was inserted into the ascending aorta through the right brachial artery. This artery was chosen to facilitate proximal imaging, as delivery of the catheter through the left brachial artery could have been inhibited by the presence of the thoracic aortic stent placed six months previously.\nContrast imaging of the aortic arch revealed the brachiocephalic truncus sourced from the left common carotid artery (bovine arch). Moreover, the origin of the retrograde dissection flap was identified 1 cm distal to the left subclavian artery in the contrast view .\nFollowing the completion of the measurements, a 40 × 212-mm tube stent–graft was implanted into the descending aorta, including the proximal subclavian section. The placement of the tube stent–graft was challenging because of the narrowing of the true lumen and the high-angled aortic progression. The graft was placed using forced external manoeuvres. An extension tube stent–graft with a diameter of 42 × 112 mm was placed through the right common femoral artery. The correct placement of the extension tube stent–graft was confirmed with angiography and the application was concluded . Primary repair of the right common femoral artery was conducted.\nAfter surgery, no pulse deficit was observed in the left limb. The patient recovered in the intensive care unit and hydration was administered for deficient blood urine nitrogen and creatinine levels (due to the patient’s nephrectomy history). She was discharged on the fourth day after surgery. |
A 30-year-old male of asthenic build presented with the complaint that fecal matter was coming out through an opening in the right iliac fossa region of his abdomen for the past 6 months. Initially, he had developed a swelling in the skin of the right iliac fossa over the scar of an appendectomy done 14 years back. The swelling had ruptured after 7-8 days, to discharge fecal matter mixed with pus. Within a week, the opening healed, but the swelling reappeared 7-8 days later, only to rupture again after a few days. This cycle of swelling followed by rupture was associated with evening rise of temperature. The fever was of moderate degree and was associated with the swelling stage only. His appetite was adequate and he was passing stools and urine normally.\nOn examination, the patient was moderately nourished and there were no features of anemia, hypoproteinemia, dehydration, or electrolyte disturbances. A 0.5 cm × 0.5 cm fistulous opening with fecal soiling was seen over the scar of a previous surgery in the right iliac fossa []. No mass could be palpated in the abdomen. The patient had no history of any other surgery or hospitalization. There was no history suggestive of any chronic disease such as TB, diabetes mellitus, or ischemic heart disease. Biopsy of the inflamed appendix and of an enlarged lymph node, taken at the time of the surgery done 14 years back, had not shown caseating granuloma but only nonspecific inflammatory reaction.\nWe took X-rays of the chest and abdomen, both of which were normal. Abdominal sonography revealed bowel-wall thickening in the ileocecal region and enlarged mesenteric lymph nodes, as well as a fistulous tract between the terminal ileum and the skin. A percutaneous fistulogram was obtained, which revealed a narrowing involving the ileocaecal junction and the terminal ileum, and a fistulous tract connecting the terminal ileum to the opening in the skin in the right iliac fossa []. Colonoscopy was performed and showed a normal colon. Tissue specimen taken from the margins of the fistulous opening on the skin was subjected to Ziehl-Nielsen staining for acid-fast bacilli (AFB) but was negative. However, histopathological examination of tissue specimens from the same site showed caseating granuloma, consistent with tuberculosis. The tuberculin test was weakly positive.\nFour-drug antitubercular therapy was started. The patient was sent home with the advice to return for follow-up after 6 weeks. At 6 weeks, the fistula did not show any signs of healing. Although it was a low-output fistula (with an output of about 100 ml/day), in view of the long history and the fact that the edges of fistula had completely epithelized, exploration was planned after complete bowel preparation.\nIntraoperatively, the ileocecal portion was found to be clumped and the site of fistula was seen to be the terminal ileum. The terminal ileum was dilated and its walls and the mesentery were thickened. Fistulectomy with right hemicolectomy was performed along with ileotransverse anastomosis. Histopathologically, the fistulous tract showed transmural dense chronic inflammatory cellular infiltration and histiocytic proliferation with Langhans-type giant cell reaction. Histological examination of the resected specimens revealed ‘casseous granuloma with central necrosis’ in the ileocecal portion and mesenteric lymph nodes, which is typical of tuberculous involvement. TB of the lungs or of any other organ was not demonstrated. The patient's postoperative course was uneventful and four- drug antitubercular therapy was continued for a further 8 months. Follow-up over 2 years has been uneventful and antitubercular therapy has been stopped. |
A 38-year-old woman was referred to the surgical out-patient Department of our Center presenting abdominal discomfort and recurrent episodes of acute abdominal pain during the previous 6 months. Her clinical and surgical history were free, and the patient did not mention any other symptoms. Physical examination only revealed tenderness during palpation of the right upper abdominal quadrant. Laboratory blood examination showed results within normal limits. CT-scan and MRI were ordered, in order to further investigate this condition. These imaging controls revealed the presence of a giant hemangioma (~9.6 cm in diameter), involving segments VI and VII of the liver (Figs and ). Therefore, in this symptomatic patient, considering the location, size and possible future complications of the lesion, a right segmental hepatectomy was decided.\nThe procedure was carried out under general anaesthesia and the patient was monitored by ECG. Peripheral oxygen saturation (SpO2), end-tidal CO2 concentration and body temperature were measured throughout the whole operation. Gastric intubation and bladder catheterization were realized. A central venous line was placed and an intra-arterial catheter was positioned for continuous monitoring of the blood pressure and gas analysis. The patient was placed in supine position with parted legs in 20° Trendelenburg position. Pneumoperitoneum was achieved. A port was placed for the robotic camera and three additional ports were introduced. Cholecystectomy was first performed leading to a better optimal exposure of the hepatic hilum. Using the da Vinci surgical system, a right segmental hepatectomy was performed (Figs and ).\nThrough this method, the procedure was completed with an operating time of 120 min; the intraoperative blood loss was only 450 ml. The patient was transferred to the intensive care unit and then to her ward. No events emerged after the surgery and the patient was discharged on the second postoperative day. Pathologic examination confirmed that the lesion was a cavernous hepatic hemangioma. On follow-up control, three months later, she reported a complete relief of her symptoms and total satisfaction for the aesthetic results. |
The patient is a 16-year-old girl with Edwards syndrome which was diagnosed clinically at birth. The diagnosis was confirmed again by karyotyping seven years later.\nThe child was born to a 30-year-old, P3+0 mother at 37 weeks of gestation after an uneventful pregnancy and good maternal antenatal care. On antenatal scans, liquor was adequate and the fetus was identified as having intrauterine growth retardation. The birth was by a lower segment Caesarean section, with a birth weight of 1.6 kg and poor Apgar scores (6 at one minute and 8 at five minutes). Her mother had completed her immunizations, and all tests of the infant postnatally were negative for any infections. She did not breathe spontaneously at birth and did not cry until four minutes later. She was ventilated for two days at birth after failed nasal intubation due to unilateral choanal atresia. She was also given phototherapy for neonatal jaundice for seven days. She was kept in the neonatal intensive care unit for 35 days. During this time, on examination, a grade two ejection systolic murmur was heard, and an echocardiography revealed mild hypertrophic cardiomyopathy, which improved with time and caused no outflow obstruction. An ultrasound scan of the head on day one revealed a choroid plexus cyst. She passed meconium on day two, and chest and abdominal examinations were normal. She was initially kept nil per os and later switched to oral feeds because she was not sucking well initially. She was breastfed up to the age of six months, after which she continued on formula.\nAt one year of age, she failed the brainstem electric response audiometry screening test for hearing with varying low scores bilaterally. In her early years, she had global developmental delay and had multiple hospital admissions requiring intravenous antibiotics for episodes of broncho and lobar pneumonia.\nAt the age of four, she was diagnosed with neurogenic bladder by a cystourethrogram which showed multiple bladder diverticula and associated trabeculation. The cystourethrogram was done due to the presence of a suprapubic mass and suspected right ureterocele but results suggested neurogenic bladder. She was also diagnosed with a Morgagni diaphragmatic hernia at four years of age when intestinal loops were seen on a chest X-ray done during a hospital admission due to an episode of bronchopneumonia (Figure ). However, no surgical intervention was done and the intestinal loops currently persist in the thoracic cavity.\nAt two years of age, she was diagnosed with epilepsy. After taking the required course of phenobarbitone and being seizure-free for two years, she discontinued the medication.\nOn a routine visit to the ophthalmology clinic, she was diagnosed with exposure keratitis (at age 14) and was advised conservative treatment. She also has myopia and requires the use of glasses.\nCurrently, the patient attends regular follow-ups with the ear, nose, and throat (ENT) clinic, along with the pediatric ophthalmology clinic, and is often admitted to the hospital due to recurrent bronchopneumonia. Her most recent echocardiogram showed no abnormalities. She has spastic quadriplegia and contractures which require regular physiotherapy and the use of a special wheelchair. She has failure to thrive with growth parameters of height, weight, and head circumference well below the third centile and therefore requires regular monitoring. Her pubertal development is normal, with regular menses. She has no known allergies and is on a soft diet orally as the insertion of a gastrostomy tube was refused by the parents. In the neonatal period, she had trouble swallowing and sucking. The trouble with swallowing is ongoing and may be the cause of her recurrent aspiration pneumonia. She urinates and passes stool spontaneously but wears a diaper. She has had chronic constipation since the age of four and often requires the use of laxatives.\nOn inspection, she has the typical features of Edwards syndrome, namely, prominent occiput, short sternum, low set ears, low hairline, micrognathia, overlapping fingers, and rocker bottom feet.\nHer most recent admission to the hospital was due to fever, abdominal pain, and constipation for the past four days. She also had poor oral intake recently and had not been taking laxatives (Movicol). Computed tomography revealed a fecaloma reaching the intestinal loops posterior to the sternum (Figure ). On examination, she was febrile with a hard, distended abdomen and significantly reduced bowel sounds. She was treated with fleet enema, ceftriaxone, and paracetamol and was discharged within a week.\nShe has no known family history of any genetic disorders, syndromes, or blood-related illnesses. Her parents are non-consanguineous and she has four healthy siblings. |
Our patient was a 46-year-old Caucasian woman when she first noted a mass in her right breast in 2015. A screening mammogram at that time showed scattered fibroglandular densities as well as a skin lesion in the inner right lower quadrant of her right breast, categorized as a BI-RADS 2. No further work up was performed. In October of 2017, our patient noticed a skin tag over her right breast and presented to her primary care provider, who removed it (Fig. ). She subsequently underwent repeat diagnostic breast imaging with mammogram and ultrasound which showed a bulging 5.2 cm suspicious mass in the right lower quadrant composed of mixed cystic and solid components, classified as BI-RADS 4. She underwent an ultrasound-guided core biopsy of the right breast mass. The pathology specimen was initially read as metaplastic carcinoma of the spindle cell type. The report noted the specimen was negative for estrogen and progesterone receptors as well as the HER2-neu receptor. A computed tomography scan of her chest and abdomen noted no metastatic spread and a right axillary ultrasound showed no lymphadenopathy. She was diagnosed as having clinical T4 N0, stage IIIB metaplastic breast cancer. Neoadjuvant chemotherapy was planned and was to be followed by mastectomy. Prior to this diagnosis, our patient had no significant past medical history. Her family history was significant for breast cancer in her paternal grandmother. Her social history was notable for a 30-pack year smoking history and no significant alcohol use.\nDue to the rarity of metaplastic breast cancer, a second opinion was sought at our institution in January of 2018. Her physical examination at that time was notable for a 10.5 cm × 10 cm hard mass in the inferior right breast with associated hyperpigmentation and surrounding erythematous skin lesions. On re-examination of the biopsy specimen, the pathology report noted monomorphic spindle cell growth arranged in fascicles with minimal atypia and focal mitotic activity which was observed to arise from the dermis (Fig. a). These atypical cells infiltrated into the subcutaneous tissue and focally entrapped fat cells. By immunohistochemistry (IHC), the spindle cells stained diffusely positive for CD34 with focal weak p63 immunoreactivity while negative for CK5, CK7, CK19, CD20, pankeratin, CK5/6, CK903, SMSA, desmin, and S100 (Fig. b). Molecular testing revealed the presence of a COL1A1-PDGFB fusion transcript confirming the diagnosis of DFSP. She was subsequently seen by a breast surgeon and a medical oncologist specializing in sarcoma. Breast magnetic resonance imaging (MRI) measured the lesion to be 61 × 64 × 45 mm. Our patient desired breast-conserving therapy; however, with the initial size of the mass, this was not surgically feasible. She was started on neoadjuvant imatinib at 400 mg daily which she tolerated well with only some minor side effects noted which consisted of fluid retention most noticeable in her face and hands. She was monitored monthly; a repeat breast MRI 4 months after starting imatinib showed a 40% reduction in tumor size (Fig. ). She underwent wide local excision in August of 2018. Pathology revealed a 5.4 cm DFSP tumor with negative margins and evidence of treatment effect, with 5% tumor necrosis (Fig. ). No adjuvant therapy was recommended. Throughout the course of her treatment she experienced no other adverse or unexpected events. She continues to follow-up with medical and surgical oncology with annual mammograms and ultrasonography. A timeline of our patient’s clinical course is summarized in Fig. . |
A 65-year-old morbidly obese man underwent mesh hernioplasty for large right inguinal hernia. After removing the urethral catheter, he developed gradually increasing right scrotal swelling with fever. Ultrasound scrotum revealed a septate fluid collection for which incision and drainage was performed. Thereafter, he developed continuous urine leakage from the site of incision and drainage, . The patient was referred to our institute. We carried out a cystogram via the urethral catheter that revealed a fistulous communication between bladder and scrotal skin, . He was kept on urethral catheter drainage for 3 weeks. At 3 weeks, cystoscopy revealed normal anterior and posterior urethra, non-obstructing prostatic lobes and a defect in the anterior bladder wall with no evidence of mesh erosion. Bilateral ureteric openings were normally present and were away from the fistula site. A guidewire was passed through the defect that exited via the scrotal wound. Exploration was performed through a right groin incision extending into the right scrotum. Almost the whole of the bladder was lying in the right scrotum and densely adherent to the right testis and cord structures and mesh. There was a fistulous opening at the dome of the bladder wall well away from the mesh. The bladder was dissected carefully from all around. Our main concerns were inguinal hernia repair and creation of extraperitoneal space to reposition the bladder in the normal position, which was not possible without performing right high inguinal orchiectomy. Hence, we performed right high inguinal orchiectomy and removal of mesh and extraperitoneal space was made to reposition the urinary bladder to its normal position. Fistula opening was repaired in two layers and the bladder was put on continuous drainage via 20 french urethral catheter, . The right inguinal canal was closed in three layers. Scrotoplasty was also performed. An 18 french suction drain was kept in the scrotum. Post-operatively at 2 weeks, there was no urinary leak on cystogram and the urethral catheter was removed and normal voiding was restored. |
The patient reported is a 32-year-old man with a past medical history of seizure disorder and a known psychiatric history of bipolar disorder with multiple inpatient hospitalizations for manic episodes during which he exhibited agitated and violent behavior. The patient has a comorbid use of synthetic cannabinoids. He was admitted to the acute inpatient service for a recurrent episode of agitation and threatening behavior in the community in the context of medication nonadherence and continuous use of synthetic cannabinoids. In the course of index hospitalization, the patient self-reported his coprophagic behavior during his initial psychiatric assessment. The patient stated that he believed that the consumption of feces was preventing him from going to jail and facing the death penalty for a murder that he claimed he committed in the past. He was unable to describe any specifics for his murder but felt that he deserved unusually cruel and harsh punishment from law enforcement that could only be prevented by consuming feces. This feeling of guilt and continued belief that he was deserving of cruel and harsh punishment induced his coprophagic behavior. He did not report any associated command auditory hallucinations but expressed depressed mood as he discussed his reasons for feeling guilty. His affect was however incongruent with his stated depressed mood. He was oriented to person, place, and time. Laboratory work-up was within normal limits and a urine toxicology panel (which does not detect synthetic cannabinoids) was also negative for illicit substances. He declined brain imaging for unclear reasons.\nThe patient was started on a course of risperidone 2 mg twice daily, by mouth, and divalproex sodium 750 mg orally twice a day for mood stabilization. He continued to display mood instability with multiple periods of agitation and aggression, as well as coprophagic behavior. His expressed reasons for coprophagia remained the same. He declined any psychotherapeutic interventions to address his excessive guilt and automatic negative cognitions. Over the course of a few days, risperidone was titrated to 6 mg daily in divided doses, with augmentation of his treatment with 200 mg every eight hours orally for mood stabilization/impulsivity in addition to divalproex sodium. The patient's mood symptoms improved after a few days on the current regimen; he became less irritable and easily redirectable, exhibited less impulsivity, and expressed more logical thinking process. His activities of daily living also improved, as he was less malodorous and exhibited improved grooming and hygiene. The patient's coprophagic behavior, however, did not resolve. He continued to express the same guilt and desire of unusually cruel and harsh punishments for his previous transgressions. He was discharged on the twenty-sixth day of admission with resolution of his acute manic episode. His coprophagic behavior and feelings of guilt did not show any resolution. |
A 48-year-old otherwise healthy woman had suffered epigastric and back pain for 4 weeks. At first, gastroscopy was performed showing no pathological findings. Subsequent contrast-enhanced computed tomography depicted a 3.3 cm lesion in the body of the pancreas with invasion of the celiac axis (). Therefore, the tumor was declared unresectable in an external hospital, and a palliative chemotherapy with gemcitabine and erlotinib was initiated. After 3 cycles of chemotherapy, she presented to our clinic to be reassessed. We carefully reviewed the findings and agreed a surgical approach with her. The patient underwent laparoscopy to exclude hepatic and peritoneal metastasis. Subsequently a selective embolization of the common hepatic was performed. First celiac and superior mesenteric artery angiograms were obtained to confirm the presence of the pancreatoduodenal arcade. Thereafter, a 6F sheath was placed in the common hepatic artery. After the identification of the origin of the gastroduodenal artery an 8 mm Amplatzer vascular plug 4 (AGA Medical Corp., Plymouth, MN USA) was placed in the common hepatic artery (). Completion angiograms of the celiac trunk and the superior mesenteric artery confirmed complete occlusion of the common hepatic artery and retrograde arterial perfusion of the liver and the stomach through the pancreatoduodenal arcade ().\nFifteen days after embolization of the celiac axis, the extended distal pancreatectomy with splenectomy and en bloc resection of the celiac axis was carried out. We favour an upper abdominal transverse incision with extension to the xiphoid to ensure maximum visibility and access to the pancreas. After confirming the absence of hepatic and peritoneal metastasis, the superior mesenteric artery was exposed at its origin from the aorta to exclude tumor infiltration. The patient underwent a prophylactic cholecystectomy to avoid postoperative ischemic cholecystitis. The hepatoduodenal ligament was dissected, and the proper hepatic artery, the common hepatic artery, and the gastroduodenal artery were exposed. After the common hepatic artery was divided proximally to the origin of the gastroduodenal artery, an excellent pulsation of the left and right hepatic artery was still assessed. The pancreas was transected at the level of the portal vein. The dissection of the portal vein revealed a potential tumor infiltration at the splenic vein confluence; thus, a concomitant portal vein wedge resection was performed. Then the origin of the celiac artery was identified above the superior mesenteric artery, and the celiac artery and the left gastric artery were divided. The body and tail of the pancreas, the spleen, the celiac axis, and the surrounding lymph nodes and nerve plexus were removed en bloc (). Finally, the pancreatic duct was ligated separately and the pancreatic head stump was oversewn with nonabsorbable suture material. The operation lasted 195 min, the intraoperative blood loss was 350 mL, and no red blood cell transfusions were required.\nThe postoperative course went uneventful, and the patient was discharged after 20 days stay in the hospital. A postoperative CT scan revealed sufficient arterial flow of the hepatic arteries via the pancreatoduodenal arcades (). A moderate increase of the serum concentrations of alanine aminotransferase and aspartate aminotransferase returned to normal ranges within the hospital stay. A postoperative diarrhoea or ischemic gastropathy did not occur. Additionally, the patient did not develop a pancreoprivic diabetes mellitus. The postsplenectomy vaccine prophylaxis against Streptococcus pneumoniae, Haemophilus influenzae type B, and Neisseria meningitidis was administered during, the hospital stay.\nThe histopathologic findings showed an invasive ductal adenocarcinoma of the pancreas (pT3) with a size of 6 cm and clear histologically surgical margins (R0). The histopathologic grading was G2 (moderately differentiated), and two regional lymph node metastases were detected (2/17). A notable perineural infiltration extending to the celiac plexus was present. On the recommendation of the interdisciplinary gastrointestinal tumor board, a postoperative chemotherapy with gemcitabine (1000 mg/m2 for 3 weeks of a 4-week cycle for 6 months) was carried out. After treatment completion no local recurrence and distant metastasis occurred as evaluated by CT scan. |
A 42-year-old female patient presented to our pain clinic with complaints of diffuse anterior chest pain for the past two years. She complained of pain radiating from the chest to both the upper limbs, in a diffuse distribution up to the wrists. The pain was continuously present and of a throbbing nature. Pain was of moderate intensity with a Visual Analog Scale (VAS) score of 60 to 70 mm, occasionally rising up to 100 mm. The pain was not associated with sweating, palpitations, or loss of consciousness. She stated that the pain was aggravated at times while performing household work, and was relieved by lying down or sleeping. She complained of a mild tingling sensation at times. She had no other comorbidities.\nThe patient was referred to the pain clinic by cardiologists in our institute. She previously had complaints of palpitations and an episode of syncopal attack about 4 years earlier; following which she was diagnosed as having supraventricular tachycardia secondary to an aberrant left postero-lateral conduction pathway. This bundle caused pre–excitation of the ventricles, commonly labeled as Wolff Parkinson White syndrome. The aberrant conduction bundle had been ablated using radiofrequency technology. Her postprocedural course was uneventful and she had no further symptoms and did not require regular medications.\nHowever after two years, she started feeling pain in the anterior chest diffusely, which radiated to both upper limbs up to the wrists. The pain was bothersome and she complained of loss of appetite and decreased sleep associated with the pain. She had been on regular follow up with the cardiology department for these complaints and had been thoroughly worked up to rule out resurgence of WPW syndrome or other cardiac pathology. She underwent stress echo testing which revealed that she had good effort tolerance with 10 metabolic equivalents (MET). A coronary angiogram was done to rule out ischemic pathology, and she was found to have a normal coronary system with good flow in all the arteries. A 24 hour Holter monitoring was repeated to look for episodes of supraventricular tachycardia. Holter monitoring only revealed a few occasional ventricular ectopics, which were not significant and did not explain her symptoms. After a thorough cardiology work up with negative results, she was referred to us for control of her pain.\nAt the pain clinic, she was examined to look for any local pathology and tender points. There were no significant findings on examination. Breast tissue examination was also normal.\nIn our institute, it is customary for all patients to fill out a questionnaire regarding their pain and associated features, before meeting the pain physician. The questionnaire includes the Patient Health Questionnaire-9 (PHQ-9), derived from the Diagnostic and Statistical Manual of mental disorders IV (DSM–IV) axis for depressive disorders. Our patient had a score of 21 on a maximum of 27 on the PHQ-9 scale, which falls under the category of severe depression. On asking her, she revealed that she felt depressed and had a lack of motivation to do daily activities. There were no obvious stressors to which her mood could be attributed.\nKeeping in mind her high PHQ-9 score, and due to the absence of any other obvious causes of pain, we started her on oral duloxetine with a single dose of 30 mg in the morning. She was asked to report after continuing the medication for a period of 3 weeks. The patient and her family was counseled and reassured regarding the benign nature of her pain, and were assured that it would soon resolve following treatment with the prescribed medications.\nOn her subsequent follow up, the patient experienced a 50% reduction in pain. Her VAS score had reduced to 30-40 mm. She mentioned that the continuous pain was substantially lower and the pain only occurred as episodes. There were no complaints of palpitations or fatigue. At this visit, the dose of duloxetine was escalated to 60 mg once a day. By the time of the next visit after 6 weeks, she was almost pain free. She had 1 to 2 episodes of pain per day with a VAS of 10 mm which was not bothersome. Her mood was considerably better, and she was also able to fall asleep easily. The PHQ-9 score was re–evaluated and considerable improvement was noted with a score of 12 of 27. She is on regular follow up with us now for the past 4 months and is free from chest pain. The future plan consists of slowly tapering oral duloxetine and eventually weaning her off medications based on clinical judgment with regular follow-ups. |
A 51-year-old female with a history of stage 1 hypertension was admitted to the hospital post cardiac arrest. She had a significant family history of premature coronary artery disease. Her father had a coronary artery disease diagnosed in his 40’s, her mother had a pacemaker inserted, and she had two first degree relatives diagnosed with an early cardiac disease. She is a nonsmoker, nonalcoholic drinker and denied recreational drug use. She was in her usual state of health and functionally active until her husband noticed her moaning and eventually unresponsive in the middle of the night. Prior to this, the patient did not complain of any chest pain, dyspnea, dizziness, lightheadedness or any other constitutional symptoms. Her husband started cardiopulmonary resuscitation and was taken over by the emergency medical staff (EMS). She was intubated and defibrillated five times by EMS for ventricular fibrillation then was given a bolus of amiodarone. She eventually had a return of spontaneous circulation and was transported to the hospital in which she was noted to have decorticate posturing with no purposeful movements. Electrocardiogram (ECG) during this time showed sinus rhythm (Figure ). She was admitted to the coronary care unit and was placed on targeted temperature management and was maintained on amiodarone drip. Transthoracic echocardiogram showed no valvular abnormalities, normal left atrium and left ventricular cavity size but with borderline concentric left ventricular hypertrophy with an ejection fraction of 56% and note of subtle regional wall motion abnormalities. About 12 hours after initiating the targeted temperature management, the patient was in severe bradycardia in the mid 30’s and a decision was made to hold the amiodarone at that point. The patient completed the targeted temperature management protocol and eventually had a full neurologic recovery thereafter.\nShe was extubated and subsequently transferred to the floor. She was scheduled for cardiac catheterization; however, she had witnessed ventricular fibrillation arrest on the floor (Figure ). Advanced cardiopulmonary resuscitation was initiated and she was revived immediately after defibrillation. A total of three minutes of resuscitation was performed and she was following commands afterwards. She was also given an amiodarone bolus and transferred back to coronary care unit. ECG did not show any ST elevations at this time. Subsequently, she had 23 more episodes of ventricular fibrillation arrest (Figure ) over a six-hour period which were intervened by timely defibrillation and administration of antiarrhythmic medications and inotropic agents. The antiarrhythmic medications administered were amiodarone and lidocaine boluses and drips. Intravenous magnesium was also given during the resuscitation course. Emergent cardiac catheterization showed minimal coronary artery disease. A transvenous pacemaker wire was placed due to bradycardia during a code in the catheterization laboratory, although the bradycardia did not initiate the arrest. An intra-aortic balloon pump was placed. Subsequently the balloon pump and temporary pacemaker wire were removed after she eventually became more hemodynamically stable. She once again had a full neurologic recovery and underwent an uneventful implantation of a dual-chamber implantable cardiac defibrillator (ICD). She was discharged from the hospital on amiodarone therapy and was closely followed up in the cardiology outpatient clinic. Cardiac magnetic resonance imaging was done which showed mild biventricular enlargement but with normal regional systolic function, no late gadolinium enhancement consistent with the absence of myocardial inflammation, infiltration or infarction and with no imaging features of ARVC. She eventually had a targeted genetic testing for idiopathic ventricular fibrillation and was found to have a mutation in the desmoglein-2 (DSG2) gene (c.338T > C, pVal1113Ala), which is a gene typically involved in ARVC.\nShe had been readmitted several times to the hospital due to palpitations, dizziness, lightheadedness, and defibrillator shocks. Her defibrillator had been interrogated as well as her antiarrhythmic medication had been adjusted which improved her symptoms and reduced defibrillator discharge. Because of intermittent episodes of defibrillator firing due to ventricular fibrillation despite antiarrhythmic medications and intracardiac device, a ventricular fibrillation radiofrequency catheter ablation was performed successfully. Thereafter, she had no repeated episodes of palpitations, dizziness, lightheadedness, and defibrillator shocks. |
This case is of a 55-year-old female who initially presented with a palpable left neck mass. The mass had been noted by the patient 8 years ago and had progressively grown in size. A computed tomography (CT) of the neck was obtained, which demonstrated a 3.5 × 2.3 × 4.6 cm lesion deep to the left sternocleidomastoid (SCM) muscle in addition to a smaller 1 × 1 × 1.8 cm left posterior neck-enhancing mass. A fine needle aspiration (FNA) had been performed 2 years prior at an outside hospital with pathology indicating a low-grade spindle cell proliferation. She was thus referred to our institution for further evaluation. At the time of initial presentation to our group, the patient had noted dysphagia, left ear ache and tinnitus, and neck pain localized to the two masses. A positron emission tomography (PET) CT was performed, which demonstrated increased fluoro-2-deoxy-\nd\n-glucose uptake in the high cervical mass and, to a lesser degree, in the mass located within the posterior triangle of the neck (\n). The outside tissue blocks were reviewed at our institution and felt to be consistent with a peripheral nerve sheath tumor compatible with schwannoma from both lesions. Because of the patient's ongoing symptoms and because malignancy could not be completely excluded based on the results of the prior FNA, the decision was made to proceed with surgical excision of both lesions. Prior to proceeding, a magnetic resonance neurogram was obtained (\n). Tractography demonstrated that the two lesions appeared to originate from the spinal accessory nerve and that apparent diffusion coefficient values were elevated in both masses, supporting the diagnosis of a less aggressive tumor (\n).\nThe patient was taken to the operating room for surgical resection. The patient was positioned with her head turned slightly to the right with the neck extended (\n). The smaller lesion was approached first through the posterior triangle of the neck. Stimulation mapping of the tumor was conducted. The tumor was found to be located on the distal spinal accessory (cranial nerve XI) nerve, with evidence of trapezius activation with nerve stimulation (\n). After identifying no overlying nerve fibers, the tumor was removed en bloc. A separate incision was made in the upper cervical region to approach the larger second mass located lateral and deep to the SCM muscle, which was reflected medially (\n). During dissection of the tumor away from the nerve, motor evoked potentials (MEPs) to the trapezius were lost. As the tumor was of significant size, view of the proximal aspect of the afferent nerve was initially obstructed. Distally, the tumor was mapped, and the fascicle of origin was identified, which appeared to activate the SCM. After significant debulking of the mass, the proximal fascicle of origin was identified but did not provide any muscle activation after stimulation. The tumor was therefore removed in its entirety. All parameters for brachial plexus monitoring remained stable.\nPathology for both lesions was consistent with schwannoma without malignant features. Next-generation sequencing analyzing the coding regions of 479 cancer genes as well as select introns of 47 genes using the UCSF 500 Cancer Gene Test revealed a small in-frame insertion at codon p.R177 of the Sox 10 gene. There were no identifiable alterations in NF1, NF2, LZTR1, SMARCB1, and TRAF7 genes. Despite the change in MEPs, the patient was noted to be full strength in all muscle groups in the left upper extremity including shoulder shrug and head turning immediately postoperatively. At follow-up, her neck pain and prior dysphagia had improved significantly. |
The patient is a 62-year-old right-handed woman with a past medical history of significant tobacco use with chronic obstructive pulmonary disorder, hyperlipidemia, and prior remote meningioma resection at 42 years of age with residual seizures on levetiracetam. She was diagnosed with stage IIB pT1aN1 left lung adenocarcinoma at 51 years of age and underwent left upper lobectomy with mediastinal lymph node dissection followed by adjuvant chemotherapy. Six months after her initial diagnosis, she was found to have brain metastases and underwent four Gamma Knife radiosurgeries to a total of 17 lesions over the course of 12 months. She did well over the next five years but then had progression of a right posterior temporal mass that was in the immediate area of a small lesion previously treated with Gamma Knife. She underwent a right-sided craniotomy and tumor resection, followed by adjuvant stereotactic body radiotherapy (SBRT) with 30 Gy in five fractions to the resection cavity. Two years later, she developed seizures and headaches, and she was found to have a growing focus of enhancement seen using MRI at the site of her previous surgery consistent with disease recurrence. Four months after this finding, she was taken back to the operating room for a redo right craniotomy for tumor resection.\nThe patient unfortunately once again developed local recurrence at the surgical site seen on fluorodeoxyglucose (FDG) positron emission tomography (PET) and gadolinium-enhanced T1 weighted MRI four months post operation. She was initially considered for laser interstitial thermal therapy (LITT) but was thought to have a tumor configuration and morphology that is not amenable for treatment using this modality. After multi-disciplinary tumor board discussion, she was ultimately recommended repeat resection and GT. Accordingly, the patient underwent a third right craniotomy with tumor resection and placement of GTs in the tumor bed, and titanium mesh cranioplasty overlying the surgical site. The patient tolerated the procedure well.\nA target was contoured on the preoperative MRI by the radiation oncologist. The prescription was to treat 60 Gy to 5 mm beyond the surgical cavity. The number of tiles needed was estimated by evaluating the circumference of the target in the axial, coronal, and sagittal views. Five tiles were estimated by a team consisting of the neurosurgeon, radiation oncologist, and medical physicist for the procedure. An extra sixth tile was ordered to account for any changes in the cavity on the day of the surgery. Three full and three half tiles were implanted, resulting in a total of 18 seeds with 3.51 U (5.51 mCi) per seed.\nThe patient had a thin slice (1 mm) CT of the head on postoperative day zero, which was unremarkable (i.e., no acute hemorrhage); MRI on postoperative day two showed a near-total resection. She also had a two-week follow-up wound check and was doing well at that time. The CT and MRI were fused, the postoperative cavity was contoured, and the 131Cs seeds were identified. A 5-mm expansion from the cavity (excluding bone) was used as a clinical target volume (CTV). The postoperative cavity volume was 14.36 cm3. Figure shows preoperative and postoperative MRI with cavity contours.\nTo model the GT dosimetry on a phantom CT, we created a spherical cap and a cube with the same volume as the postoperative cavity, expanded 5 mm (in all directions except for one flat surface), and acquired the CTV. Four and a half tiles (18 seeds in total) were each modeled in the spherical cap and the cube according to the tile configuration. The seeds were 3 mm from the cavity wall and 1 cm from each other. Figure shows the three-dimensional representation of the 18 seeds in the postoperative cavity for the patient and for the cube and spherical cap models.\nPostoperative plans for the patient and the two models were planned in MIM Symphony™ (MIM Software Inc., Cleveland, Ohio). Dose volume histograms (DVH) were compared for all three plans (Figure ). The cube model resembled the patient DVH better than the spherical cap model. Table shows the dosimetry parameters of the patient and the two models. Volume of the contours, mean dose, CTV volume receiving the prescription dose (V60Gy), and dose to 90% of the CTV (D90%) are reported. |
We present the case of a 24-year-old woman who was brought to the emergency department 30 minutes after a fall from height. She had an accidental fall from her home roof of three stories. She presented with severe back pain that was associated with the inability to move both of her lower limbs with absent sensation. Further, the patient had complete urinary and bowel incontinence.\nOn examination, the patient appeared in significant pain and distress. She had extensive abrasions over her lower limbs. Her vital signs were a pulse rate of 130 bpm, blood pressure of 125/74 mmHg, respiratory rate of 15 bpm, and temperature of 37.2℃. The patient was alert, conscious, and oriented. His Glasgow Coma Scale was 15/15. Neurological examination revealed hypotonia and power of 0/5 in both proximal and distal muscle groups of the lower limb. The patient had lost touch and pain sensation in both lower limbs. Palpation of the lumbar spine revealed severe tenderness on palpation with step-like deformity. The knee and ankle jerk reflexes were absent. The anal tone was absent. Initial laboratory investigations were within the normal limits (Table ).\nThe patient underwent a computed tomography scan of the abdomen and pelvis. It demonstrated a complete fracture-dislocation of the second lumbar vertebra relative to the first lumbar vertebra causing shortening of the vertebral column. The second lumbar vertebra had a complete lateral dislocation and appeared in the same axial plane as the first lumbar vertebra, giving the appearance of a “double vertebrae” sign. The posterior elements of both the first and second vertebrae were fractured and were separated from their vertebral bodies resulting in widening of the vertebral canal (Figures , ). There was no evidence of hemoperitoneum. The internal organs appeared unremarkable with no injuries. No other fractures were observed.\nSince the patient was hemodynamically stable, she was prepared for emergency open reduction internal fixation with a posterior surgical approach. The operation was done under general anesthesia with the use of sensory-evoked potential responses to avoid any neurological injury. A midline thoracolumbar incision was made. The paravertebral muscles were severely injured. Laminectomy was performed for the second lumbar vertebra. The dura was found intact. Vertebral column restoration was performed by long segment fixation. Good realignment of the thoracolumbar spine was achieved. The patient tolerated the procedure well with no neurological injury noted by the monitor. She underwent an intensive physiotherapy rehabilitation program. Six months later, the patient was asymptomatic and resumed her regular activities. She had full power of both lower limbs with urinary and bowel control. |
An 18-year-old girl presented with a complaint of “excessive gum visibility while smiling as well as in resting lip position.” The central portion of her upper lip was raised exposing good amount of gingiva, and her lower lips were hiding upper front teeth while smiling (). She had severe anterior deep bite and several posterior teeth had severe attrition and were malposition. Gingiva in the anterior sextant showed reduced stippling, rolled out crest with flat architecture.\nShe had undergone orthodontic therapy with myofunctional and fixed appliances for 2 years to upright her inclined teeth. The orthodontic treatment had to be called off in between because of poor response to treatment. She had then consulted for the possibilities of orthognathic surgery to correct the gingival appearance and deep bite, but lack of proper posterior teeth occlusion, compound gingival pockets and high expectation of treatment outcome challenged the option of maxillary osteotomy surgery. The surgical option that would require removal of two premolars was not preferred in the present condition. Anyway, she was referred for periodontal management of deep pockets before its further planning.\nOn examination of gingiva, pockets ranging from 3 to 5 mm were found on all aspects of teeth without attachment loss indicating the unusual anatomical crown length of anterior teeth. Trans gingival probing indicated the presence of alveolar bone at very higher level than the present gingival margin (). Gingivectomy, to expose anterior teeth till the cementoenamel junction (CEJ) and to obtain more physiologic gingival contour was proposed to eliminate the gingival pocket.\nBecause she had undergone orthodontic therapy for 2 years period to upright the front teeth without much improvement and the treatment had to be stopped in between, a thorough periodontal assessment was crucial. Clinical and radiographical assessment of the concerned area was carried out to rule out any major pathology of the region. Thorough oral prophylaxis was performed to make the pocket area and the gingival margin firm. Since there was no attachment loss of gingiva and the future gingival level has to be positioned in CEJ, gingivectomy surgery was preferred over flap surgery., Owing to the extended bevelling required to shape the gingival margin and the thickness of gingiva under consideration, scalpel surgery was decided.-\nGingivectomy surgery bevelling the gingiva upto a level 3 mm coronal to the alveolar bone crest was done, preserving adequate biologic width of dento gingival unit., The CEJ was barely visible and sufficient exposure of crown was achieved to rectify the unaesthetic gingival exposure in resting lip position.\nThe case was reviewed after 1 month; though the gingival visibility became normal, and gingival margin was healthy, the inversely inclined position of teeth was more visible (). A treatment plan of prosthetic crowns on four incisor teeth with a straightened profile was suggested to correct the inclined profile of teeth, and she was ready for the procedure. Since the crown preparation was planned in an esthetic zone and subgingival restorations were to be placed, patient was recalled after 6 months for prosthetic treatment.\nThe four upper incisors were prepared with labial angulations to receive crowns following the principles of subgingival crown preparation to favour gingival health (). Porcelain fused to metal crowns was fabricated with required lip fullness and the labial inclination towards incisor tip. The crowns were tried for its marginal fit, aesthetics and patient judgment, and upon finding completely satisfied, they were permanently cemented ().\nThe marginal gingiva - crown relationship was found satisfactory, and crown contours were favourable for gingival maintenance. Her complaint of gingival visibility during resting lip position and the inclined teeth profile were well attended with absolute satisfaction ().\nThe patient was given post treatment instructions highlighting the importance of maintaining gingival health and the importance of periodic review. She was reviewed after a month, and 3 years follow-up of the case has been completed, and she is found satisfied with the prosthesis and also maintains good gingival health. |
We present a case of a 65-year-old female patient admitted to the Cardiac Surgery Department in Wroclaw in January 2018 with severe mitral regurgitation (MR) and the history of ischemic heart disease, after elective percutaneous coronary intervention of the circumflex branch of left coronary artery with two drug-eluting stents (DES) implantation 4 years earlier. Furthermore, the patient diagnosed with many chronic conditions, such as metabolic syndrome, obesity with BMI 33 and gastroesophageal reflux disease. Currently, with an exercise dyspnoea for about 2 years, intensifying in recent weeks, she was hospitalized in the Cardiology Department for further diagnostics. The transthoracic echocardiography (TTE) revealed non dilated left ventricle with a normal systolic ejection fraction of 60%, and no evidence of segmental wall motion abnormalities, severe MR with the prolapse of the A2 segment and systolic restriction of the posterior leaflet. Colour Doppler showed a highly distinctive eccentric turbulent jet directed towards the lateral wall and the base of the left atrium with ERO 0.6cm2 and regurgitant volume of 60 ml. Additionally, in the performed coronary angiography, hemodynamically significant narrowing was found in the area of the previously implanted DES. The patient was then consulted by the cardiac surgeon and qualified for surgery. After admission to the Cardiac Surgery Department, as part of the pre-operative preparation, TTE was again performed, in which the severe MR was confirmed and no pathological structures in the right atrium were described. Due to the unclear image in the right pulmonary field, described by the radiologist in the chest X-ray (Fig. ), diagnostics was extended by performing a computed tomography of the chest, which excluded the presence of pathological shadow in the lung parenchyma. There was no referral to the atrial septum in the CT report. The patient was scheduled for mitral valve repair surgery and coronary artery bypass grafting (CABG) with the use of saphenous vein graft to the circumflex artery. During the standard procedure of commencing the cardiopulmonary bypass (CPB) and bicaval cannulation, it was found difficult to insert the cannulas from the atrium into both vena cavas. Therefore the cannulation was performed using the smaller cannula sizes, which eventually allowed to go on bypass. On the free wall of the atrial septum, there was a thickening and an excess of adipose tissue with a firm consistency and the size of a walnut, significantly impeding access to the operated mitral valve through the left atrium, and probably completely preventing surgery by the transseptal approach. In the transesophageal echocardiography (TEE), a characteristic image of LHAS was confirmed by the presence of hypertrophy of the septum, up to 2.7 cm, an hourglass shape with a characteristic indentation at the place of the fossa ovalis (Figs. and ). Based on the intra-operative TEE, as well as by analyzing the chest X-ray and CT scans, the diagnosis of LHAS was made. Due to the asymptomatic course of the LHAS and the complexity of the scheduled operation, the decision was made to leave the change intact. The mitral valve was replaced through the left atrial approach. The surgery was completed in a standard manner and the weaning from the CBP went uneventfully. The patient’s early postoperative period was a routine. |
This is a 53-year-old woman with a history of hypertension, hypothyroidism, and hypercholesterolemia. She presented to the orthopedic outpatient department complaining of chronic progressive bilateral knee pain for more than ten years. The pain was worse on the right side. This pain was relieved by daily analgesics and aggravated by activity. There was no evidence of neurological, gastrointestinal, immunological, or hematological dysfunction, or underlying malignancy on systematic review. Her surgical history was remarkable as she had undergone two cesarean sections, tonsillectomy, colonoscopy, and a gastric bypass surgery.\nGeneral physical examination showed her body mass index as 33.2 kg/m2, weight as 84 kg and her height was 160 cm. Further examination showed a mild varus deformity of both knees, with no flexion contracture. Her gait was normal and no varus thrust was noted. The active and passive range of motion of her right knee was from 0 to 100 degrees and her ligamentous exam was stable. Radiological studies demonstrated bilateral tricompartmental osteoarthritis (). Routine preoperative laboratory tests, including complete blood count, electrolytes, and coagulation profile were normal.\nThe patient underwent uncomplicated elective right total knee replacement (TKR) (), which was performed under epidural anesthesia. The perioperative antibiotic regimen included administration of 1 g of intravenous cefazolin before incision and 1 g continued every eight hours over the next 24 hours. Postoperative medications, in addition to the antibiotics included analgesics, an anticoagulant, and her regular medications. The patient was started on a liquid diet four hours postoperatively and, because of her poor appetite, she continued on a soft diet for one week before resuming with solid food. From the second postoperative day, she started complaining of an intermittent mild right lower quadrant back pain which was radiating to her right flank. This pain was relieved by analgesics. Her postoperative physiotherapy progressed uneventfully. She was discharged on the sixth postoperative day.\nOn day 13 postoperatively, the patient complained of severe colicky right upper quadrant pain that radiated to the back. It was associated with fever, nausea, and vomiting. The pain was not relieved by conservative treatment. The patient reported no history of a similar attack previously or gallbladder disease. The patient was admitted to the hospital through the emergency department and her examination showed that she was febrile, with no knee findings, and her laboratory investigations yielded these results: white blood cell (WBC) count 16.19 × 109/L (reference range 4.5–11x109/L); total bilirubin 22 μmol/L (reference range 3–17 μmol/L); direct bilirubin 12 μmol/L (reference range 0–5 μmol/L); albumin 23 g/L (reference range 35–50 g/L); total protein 61 g/L (reference range 60–80 g/L); aspartate aminotransferase 12 IU/L (reference range 3–30 IU/L); ALP 120 IU/L (reference range 50–163 IU/L);and amylase 8 IU/L (reference range 25–115 IU/L) while other liver enzymes were within normal ranges. An abdominal ultrasound was consistent with an acute cholecystitis. Her gallbladder was markedly distended, with a thick and edematous wall measuring 1.15 cm. Ultrasound also showed two mobile stones, with the largest measuring 0.9 cm. The common bile duct (CBD) was dilated and measured 0.7 cm, with a thickened wall.\nThe patient underwent laparoscopic cholecystectomy and her intraoperative findings revealed an acute gangrenous gallbladder and suppuration in the abdomen; which necessitated a drain insertion. The infectious disease team was consulted immediately after admission, and they started her on intravenous piperacillin/tazobactam 4,500 mg every six hours, which had to be maintained for seven days until culture results were available, the drain was clear, and no collection was identified by ultrasound. Cultures were positive for gram-positive cocci and gram-negative bacilli (Enterococcus species, extended spectrum beta-lactamase-producing Enterobacteriaceae, and Escherichia coli), and piperacillin/tazobactam was consequently substituted for intravenous imipenem 500 mg every six hours plus intravenous ampicillin 500 mg every six hours based on the sensitivity results. The drain was removed on the second postoperative day, as it only had a small amount of serous fluid. On the fourth postoperative day, the patient started complaining of generalized abdominal pain, which was associated with an increase in WBC. Computed tomography (CT) of the abdomen and pelvis was done to rule out intestinal obstruction, and it showed no signs of intestinal perforation, no leak of oral contrast or collection. On day 10 post-op, the patient was discharged on oral antibiotics (oral ciprofloxacin 500 mg every eight hours for seven days). She was followed in the outpatient clinic after discharge and, on her last visit to the clinic (four weeks post-laparoscopic cholecystectomy), she was free of abdominal symptoms. The patient did not have any new complaints in regard to her TKR. |
A 64-year-old man underwent repair of partial right-sided anomalous pulmonary venous drainage (PAPVD) with intact interatrial septum. At this operation, the atrial septum was excised to create a pericardial baffle for the pulmonary veins to drain into the left atrium. The anomalous pulmonary veins entered the superior vena cava high above the level of the right pulmonary artery; therefore, the superior vena cava transection and relocation approach had been chosen to correct the defect. Over time, the atrial septum had grown back, therefore causing pulmonary venous stenosis. As a consequence, the patient presented with NYHA class III dyspnea, right ventricular failure, and pulmonary hypertension.\nThe preoperative echocardiogram showed a severely dilated right heart with a tricuspid annulus of 58 mm, severe pulmonary hypertension suggested by a tricuspid regurgitation jet velocity of 4 m/s, and normal left ventricular function. A preoperative computed tomography scan was performed, and it demonstrated a severely dilated right ventricle (end-diastolic diameter of 68 mm) and a severe stenosis at the distal end of the baffle (Figure A). Mild coronary artery disease was also present, and it was confirmed by the preoperative coronary angiogram.\nThe patient had several comorbidities, including type II diabetes mellitus and paroxysmal atrial fibrillation on Warfarin; furthermore, he had undergone a left pneumonectomy at the age of 4 due to rubella, and he had subsequently experienced recurrent low respiratory tract infections on his right lung.\nHaving considered the comorbidities and the high risk for complications on the right lung that a conventional open heart reoperation would have carried, it was decided to address the pericardial baffle stenosis through an interventional hybrid approach. In order to gain access to the pulmonary venous system, a redo longitudinal median sternotomy was performed, and after dissection of the adhesions a purse string suture was placed on the stump of the native superior vena cava. A 14 F sheath was then inserted and a 16 mm × 45 mm Premounted CP Stent (PFM Medical, Koln, Germany) was implanted within the pericardial baffle on the beating heart and avoiding CPB. The stent was then post-dilated with a 20-mm Cristal balloon (BALT Extrusion, Montmorency, France). Transesophageal echo and angiography were used to confirm the good result; no gradient across the stent was measured. The final result is shown in Figure B.\nThe early postoperative course was uneventful. The patient was electively readmitted to our institution 6 months after the procedure to have a cardiac catheterization as part of the workup for his pulmonary hypertension. This demonstrated pulmonary hypertension secondary to lung disease and confirmed the baffle stent to be patent, showing half-systemic pulmonary artery pressure with a calculated pulmonary vascular resistance of 4 Wood Units, a pulmonary capillary wedge pressure of 13 mmHg, and a mean gradient across the stent (between pulmonary capillary wedge pressure and left ventricular end-diastolic pressure) of 5 mmHg. |
A twenty-six-year-old female, gravida two para one, was transferred to our tertiary gynaecological facility from the nearby general hospital with a presumptive diagnosis of a ruptured ectopic pregnancy. The patient presented in the early hours of the morning with sudden onset left iliac fossa pain, which woke her from sleep four hours prior. The patient was unsure of the date of her last menstrual period (LMP) but estimated that it was eight weeks prior and reported no per vaginal bleeding currently. In the time since initial presentation at the general hospital to her evaluation at our centre her haemoglobin had dropped 24 points from 132 g/L to 108 g/L and a bedside transabdominal ultrasound scan revealed approximately one litre of free fluid in the abdomen. An initial quantitative b-HCG was 14 IU/L and given the possibility of the patient's LMP being eight weeks prior a ruptured ectopic pregnancy was highly expected and the patient underwent an urgent laparoscopy. Apart from some recent illicit drug use in the form of methamphetamines and a normal vaginal delivery seven years previously the patient's medical history was unremarkable. She was not currently using any medical form of contraception.\nAt laparoscopy one litre of blood was drained from the abdomen but no sign could be found of an ectopic pregnancy (see ). The uterus was of normal size, the fallopian tubes were unremarkable, and a small active bleeding source was identified on the right ovary but without an obvious cyst seen (see ). A limited amount of diathermy to this area controlled the bleeding. The upper abdomen inspection was normal. There was no hysteroscopy performed or handling of endometrium at laparoscopy. After removal of blood and organised clots from the abdomen laparoscopically the operation was concluded and the patient returned to the ward.\nThe next day the patient was discharged home as she was haemodynamically stable but two days later at a scheduled follow-up the quantitative b-HCG level was 38 IU/L, a clear doubling. The patient also reported mild pelvic pain and a small amount of per vaginal bleeding. In her next follow-up visit quantitative b-HCG level was 725 IU/L and the patient had ongoing per vaginal bleeding but no identifiable pregnancy on transvaginal ultrasound scan. She was advised that the possibility of a viable intrauterine pregnancy could not be excluded. On her final documented follow-up two days later the quantitative b-HCG level dropped to 218 IU/L and she had a small amount of ongoing vaginal bleeding. The final diagnosis in this case was of haemoperitoneum secondary to a presumed ruptured right corpus luteal cyst and failed pregnancy of unknown location, likely intrauterine (see ). |
A 16-yr-old female was referred to the Department Neurology Emergency, Qaem Hospital, Mashhad, Iran in 2013, because of left- sided weakness since two weeks ago. Informed consent was taken from the patient. She had a history of an upper respiratory tract infection one week before beginning her symptoms. She had no history for a specific disease and drug use, except some antibiotics for infection mentioned above, including azithromycin 500 mg first and then 250 mg daily up to 5 days. There was not any history of previous trauma. Her symptoms were started and progressed step by step. First, she had left arm pain with developing numbness. The weakness of the left arm and leg was appeared as well as the weakness of the ipsilateral face.\nOn neurological examination, she was conscious and oriented. The speech was fluent. Left central facial palsy was seen on her face. Her gait was hemiplegic, muscle tone was increased on the left side of the body, muscle power was 3/5 over left upper and lower extremities, deep tendon reflexes were exaggerated and plantar reflex was upward on the left side. There was no abnormality on her systemic examination including cardiac and pulmonary exam, no fever and no meningeal signs. So, after admission on our hospital, brain MRI was performed. According to the previous history of her upper respiratory tract infection (URTI), the course of onset the symptoms and brain MRI which showed right deep white matter lesions, it was not in accordance with the known vascular territories (). Thereby we initially suspected of a demyelinating process, and she received pulse methylprednisolone 1g/daily for 5 consecutive days. However, other diagnostic measures was carried out at the same time. The laboratory tests consisted of immunologic tests for vasculitis, hematologic (Hyper coagulative tests), and biochemistry tests which were all within normal ranges.\nDuring her hospitalization, our patient gradually deteriorated. She experienced increasing difficulties with walking and speech as well as progressive hemiparesis to hemiplegia, so additional evaluations were performed. Cerebrospinal fluid analysis was normal. A repeated brain MRI revealed further lesions fitting more on the arterial territories at this time, rather than purely white matter involvement (). For more evaluation of the vascular structure of the brain, brain and cervical CT angiography was done. CT angiography showed bilateral internal carotid stenosis with "puff of smoke" collateralization arising from the circle of Willis, therefore a diagnosis of Moyamoya disease was raised ().\nThe deteriorating clinical condition and complications of the long-term hospitalization and immobilization, including infections and deep venous thrombosis accompanied with an episode of pulmonary thromboembolism, prevented further evaluation of the patient, including brain angiography. Our patients eventually died as a consequence of the above-mentioned conditions. |
The patient is a 24 year-old African-American female with SCT who presented with a one month history of gross hematuria and intermittent right flank pain in December 2014. Computed tomography (CT) imaging revealed a well-defined solid mass with a central necrosis within the upper pole of the right kidney measuring 6.0 cm × 3.9 cm × 5.0 cm and two pulmonary nodules (3 and 2 mm in size) in the left lower lobe concerning for metastatic disease. Thus in January 2015, right radical nephrectomy was performed. Histological and Immunohistochemical (IHC) analysis revealed RMC. Further genetic study revealed SMARCB1 mutation as previously reported in RMC []. Follow up imaging in February 2015 showed an interval increase in the size of the aforementioned pulmonary nodules to 7 mm and a new lesion in the upper left lobe. Unfortunately, two new nodules of 1 cm each were also detected in the post-surgical bed. Subsequently in March 2015, she was started on palliative chemotherapy consisting of cisplatin, paclitaxel and gemcitabine. Due to a grade 3 neutropenia she experienced after the first cycle, she only received cisplatin and gemcitabine for the remainder of the treatment. After the third cycle of the cisplatin and gemcitabine, a complete response was observed and at the end of the sixth cycle in July 2015, she remained free of disease. A follow up CT abdomen and pelvis performed in October 2015 showed an increased in disease burden with two pulmonary nodules in the left lower lobe and a 1.6 × 1.6 cm nodule within the right nephrectomy bed. IHC staining performed on the initial nephrectomy specimen revealed the expression of PD-L1 on 25% of tumor cells (Fig. ). As such, she was started on nivolumab 3 mg/kg every 2 weeks on October 19th 2015. She tolerated well the infusion of nivolumab except for mild nausea controlled with ondansetron. After completing 4 cycles of nivolumab, she presented at an outside hospital in December 2015 for UTI and was found to be 6 weeks pregnant prompting the nivolumab to be stopped. She elected to undergo a dilation and curettage in January 2016. From November 30th 2015 (date of her 4th cycle), to February 12th 2016 (5th cycle of nivolumab), she did not receive nivolumab. Prior to resuming the nivolumab, a CT scan showed a stable disease in the right nephrectomy bed and improved metastatic lung disease (Fig. ). She received 8 additional cycles of nivolumab before undergoing another PET scan in May 2016 which showed a slight increase in the recurrent lesion in the surgical bed and a persistent resolution in metastatic lesions to the left lower lung lobe. Despite the interval progression in the surgical bed, she remained asymptomatic. In July 2016 she presented to the emergency room with nausea, diarrhea and abdominal pain suggestive of colitis. An improvement was noted when with a course of prednisone. While she was recovering from the colitis, the nivolumab was stopped. She underwent a surgical resection of the recurrent lesion and resumed the nivolumab on July 21st 2016 (Cycle#13). After receiving 5 additional cycles, a PET scan in October 2016 revealed an increase in the disease burden. She received palliative radiation to the left mediastinal disease with 4400 cGy in 200 cGy fraction and to the left upper lung lobe lesion with 6000 cGy in 200 cGy fraction via intensity-modulated radiation therapy (IMRT) while continuing nivolumab. She completed the radiation therapy in December 2016 and continued to receive nivolumab. Since the initiation of nivolumab, the patient has completed 28 cycles and a follow up PET scan in February 2017 showed a decreased in tumor burden in the mediastinum but further progression outside of the radiation field with new lung and liver lesions and recurrence in the right nephrectomy bed (Fig. ). In light of disease progression, the decision was made to add ipilimumab to the nivolumab. At time of this submission, response assessment from this therapy is awaited (See Table for a summary of chemotherapy and immunotherapy agents administered). |
A 16-year-old male patient was referred to our institution with the chief complaint of pain in the right temporomandibular joint (TMJ) region and difficulty in mastication and mouth opening. He had sustained a trauma to the right temporal region because of a traffic accident. Patient did not present an initial loss of conscience or anterograde amnesia. Clinical examination showed little swelling over the right TMJ region with an inadequate mouth opening. No facial palsy was observed. Intraoral examination showed abnormal occlusion, restricted mouth opening, deviation from the midline to the right side, protrusion, absence of lateral movement to the left side and on palpitation, tenderness and pain over right ascending ramus and right temporal region. A computed tomography (CT) scan showed unilateral fracture of the right coronoid and condylar processes []. There was no evidence of direct trauma or fracture to the zygomatic region or mandibular symphyseal and corpus area. Radiological examination revealed a well-defined radiolucent line at the base of both condylar and coronoid processes []. Under nasal intubation, arch bar was fixed in the maxillary and mandibular dental arches and the inter-maxillary fixation was done with elastic bands. The fractures were corrected using the help of the patients’ occlusion as a guide. Pre-accident occlusion was achieved. Local analgesic with epinephrine (adrenaline) was infiltrated into the submandibular incision site. A mini submandibular incision was made 2 cm below the angle and inferior border of the mandible. Sharp dissection was performed down through the platysma, and then blunt dissection was begun to minimize risk to the marginal mandibular branch of the facial nerve. Periosteum and attachments for the masseter were completely stripped off the lateral cortex of the vertical ramus up to the level of the sigmoid notch. The subcondylar and coronoid region was exposed and fracture lines were seen. Because of insufficient vision and inability to fix the screws to the plaque in correct directions, a small incision was made to the skin and a trocar was placed for fixing the screws. A silk suture was used at this stage in the lower part of the plates for positioning of the plates in the correct position []. Fractured fragments were fixed with mini plates. To avoid hematoma formation, a small penrose drain was placed in the operation area. The patient was followed-up postoperatively without any esthetic or functional problems []. |
A previously healthy 44-year-old male was admitted to a health institution complaining of severe colicky abdominal pain for 2 days. It started suddenly and was continuous and diffuse; however, it was mainly in the right lower quadrant and was increasing in severity. He had never experienced similar pain in the past. The pain was associated with nausea, vomiting, hiccoughs, anorexia and bloating sensation. He had passed his last stool and flatus in the morning on the same day of admission.\nOn admission, he was alert and his vital signs, chest and cardiovascular examination were normal. The abdomen was distended, free of scars, and tender all over mainly in the right iliac fossa with guarding and a palpable mass with absence of bowel sounds. The hernia orifices were intact. The rectum was empty on digital rectal examination. His complete blood count and basic biochemical parameters were also normal. Contrast enhanced abdominal computed tomography scan showed an intra-abdominal mass associated with small bowel obstruction and a small amount of intra-peritoneal free fluid. Following a brief period of resuscitation, the patient was taken to the operating room for exploration. Upon examination, a 4 × 4 cm mass was detected in the small bowel mesentery with multiple small bowel loops adherent to it, together with their mesentery. There was no evidence of liver or peritoneal metastasis. Approximately, 1 m of small bowel had to be resected to completely excise the mesenteric mass with clear margins together with the adherent bowel and mesentery ( and ). Then, primary anastomosis was performed. The final pathology result was a grade 2 gastrointestinal stromal tumor with clear margins, mitotic index less than 2/10 high power field, maximum tumor size of 4 cm, and tumor cells were positive for CD117 (C-kit) and CD34. Hence, the final diagnosis was primary gastrointestinal stromal tumor of the small bowel mesentery. The patient had an uneventful postoperative course and was discharged on the second postoperative day in a good condition. |
A 65-year-old man with continuous irritable cough over 15 days was admitted to our department for a mass lesion in the right hilum. He presented with no face swelling and superficial varicose veins. He was a former smoker, and medical history included hypertension and diabetes mellitus. At bronchoscopy, the tumor was found occluding the right upper lobar bronchus and infiltrating the bronchus intermedius. It was diagnosed as squamous cell carcinoma by transbronchial tumor biopsy. Chest contrast computed tomography (CT) revealed that the mass of 80 mm in the maximum diameter located in the right upper lobe. The tumor directly invaded the arch of the right PA and a wide range of SVC. It was discovered that the left brachiocephalic vein (BCV) along the left margin of mediastinum walking down the line directly into the coronary sinus (). A venous flow was detected in the anterolateral descending aorta with dilatation of the coronary sinus (diameter of 21 mm) without other heart abnormalities by transthoracic echocardiography. Based on these findings, the diagnosis of PLSVC was made. There were no detectable metastases in other organs through brain magnetic resonance imaging (MRI), upper abdomen CT scan and bone nuclear scan.\nThe right posterolateral thoracotomy was performed on October 31, 2006. As expected, the tumor invaded the wall of SVC and no left brachial cephalic vein bifurcation was observed. The SVC was separated, clamped and resected following intravenous injection of 5, 000 IU of sodium heparin. The ringed PTFE graft of 12 mm in diameter was interpositioned between the right brachial cephalic vein and the origin of SVC by running sutures with 4-0 polypropylene. The tumor resection was completed by double sleeve lobectomy of right upper and middle lobes. The anastomosis between the right main bronchus and the cut end of the lower lobe bronchus was performed by interrupted suture using 3-0 Vicryl (Ethicon, USA). Right PA trunk and right lower PA reconstruction was completed by the running suture with 4-0 polypropylene (). And systematic mediastinal lymph node dissection was performed. All excised margins were tested microscopically negative for malignancy. As anticoagulation treatment, we began with low molecular Heparin on the operative day, then switched and continued to warfarin when thoracic drains removed. The postoperative course was uneventful, and the patient was discharged from the hospital on the 12th postoperative day. Final pathological diagnosis was poorly differentiated squamous cell carcinoma invaded the right SVC, right PA and mediastinal nodes were positive for malignancy.\nAfter 4 cycles of chemotherapy (Gemzar and cisplatin) and 1 cycle of radiotherapy, Chest MRI showed patency of the graft and PLSVC (). He died 21 months after operation because of remote metastasis. |
Patient is a 33-year-old female with a past medical history of systemic lupus erythematosus (SLE) and lower extremity deep venous thrombosis (DVT). She has been treated with oral anticoagulants for her lower extremity DVT with complete resolution of thrombus in 6 months venous duplex ultrasound follow up. Her past surgical history is significant for a left lower extremity angiogram with a stent placed in distal superficial femoral artery (SFA). It is unclear what prompted her lower extremity intervention, as patient had no stigmata of peripheral arterial disease clinically or in arterial duplex studies. Her only complain at that time was calf pain; most likely related to her DVT in left popliteal vein. She was seen in clinic with left calf pain, mild swelling of left lower extremity and three blocks claudication of the ipsilateral leg. Her clinical exam did not show evidence of tissue loss, there was swelling of left calf, a dopplerable dorsalis pedis (DP) pulse and a palpable posterior tibial (PT) pulse with sluggish capillary refill and a warm left foot. Patient subsequently underwent arterial duplex studies which showed ABI of 1.13 on the left, presence of high output ATA to anterior tibial vein (ATV) fistula, biphasic flow in the left femoral vein, patent SFA, popliteal artery (PA), with three vessel run off to the foot. In comparison right lower extremity had unremarkable arterial duplex studies as one should suspect in a young, non-smoker female.\nPatient underwent an open ligation of AVF ~6 months since her diagnosis. ATA was ligated proximally at the take off by a medial surgical approach. Routine postoperative surveillance was significant for persistent claudication and left calf swelling. A repeat arterial duplex study revealed yet again the presence of AVF.\nSurgical options were discussed with patients including another open repair vs. retrograde embolization. We decided that embolization would delineate the AVF site much clearer and efficient embolization of the entire fistulous tract could be achieved as live images are visualized under fluoroscopy. Minimal invasive nature of the procedure made the latter option much more appealing to the patient.\nPatient underwent a left lower extremity angiogram. During the procedure she was found to have patent aorta and bilateral common iliac artery. There was no disease visualized in the left external iliac artery, common femoral artery (CFA), SFA, patent previously placed stent, patent above and below the knee PA and two vessel run off to the foot (Fig. ). Upon delayed fluoroscopic images we identified contrast filling the deep venous system rather quickly suggesting a high volume fistula (Fig. ). We then identified the fistula between ATA and ATV. Although ATA was previously ligated there was retrograde filling of the artery from unnamed branches, which communicated with the venous system as shown in. In the midcalf (ATA) was cannulated with a micropuncture needle. Micropuncture wire was inserted and a 5-Fr sheath was inserted after exchanging over wire. At this point VortX coils (Boston Scientific) were placed from the curve of ATA until the whole AVF was sealed. Anterograde angiogram showed no flow through the fistula (Figs and ). There was a palpable PT and DP pulse at the completion of the procedure. Sheath was removed, pressure dressing applied. Patient was discharged home same day. Recovery has been unremarkable. Three months follow-up claudication symptoms have significantly improved. |
A 13-year-old boy from Terhathum District presented to the out-patient department of our hospital with complaints of not able to close the right eye, increased lacrimation, mouth deviation, and swelling of the right side of the face for 2 days.\nHe was fine before 2 days when he was slapped and punched over the face during a quarrel with a friend. However, he could not recall a particular site of insult. Following the trauma, he noticed that he was unable to close the right eye completely. There was increased tear production from the right eye. He also noticed deviation of the face to the left side. There was swelling on the right side of the face too. During the first visit, the patient did not reveal a history of trauma; later, only the parents reported the history of trauma.\nThere was no fever, loss of consciousness, limb weakness, cough, and ear discharge. He did not complain of decreased hearing or ear pain in the past. There was no difficulty in swallowing or aspiration. He denied a history of any recent viral infection, namely, mumps, measles, or herpes zoster. He did not have any other comorbidities. A history was taken from his mother and she denied of any known illness in other family members. The patient was not taking any medicines in the recent past and did not have any allergies to drugs or other substances.\nOn examination, he was 41 kg. His vital signs were within the normal range. The patient was sitting comfortably in the chair. The angle of the mouth had deviated to the left side (). There was an absent forehead crease on the right side of the face (). He could not close the mouth completely and therefore could not blow the cheek () and snarl. He was not able to close the right eye completely (). On the otoscope examination, the right tympanic membrane had bulged and was erythematous. His clinical hearing assessment showed the sensorineural type of hearing loss with normal Rinne test in both ears and sound better heard in the left ear during the Weber test. Sensory, motor, and reflexes were normal. Other systemic findings were unremarkable. His Sunnybrook Score was 63 (resting symmetry = 15; symmetry of voluntary movement = 48; no synkinesis)\nWe counseled the patient for further evaluation in a higher center with a non-contrast computed tomography (CT) of the head as it was not available in our center. However, because of the long distance to travel and associated cost, the patient party opted to get treatment in our center only, on an empirical basis. Therefore, we made a provisional diagnosis of right-sided traumatic facial nerve palsy with sensorineural hearing loss with right-sided otitis media with effusion. We treated him with oral prednisolone (1 mg/kg/day, that is, 40 mg q24h for 2 weeks, followed by 30 mg q24h for 2 weeks, 20 mg q24h for 2 weeks, 10 mg q24h for 2 weeks, and 5 mg q24h for 2 weeks and stopped), cefpodoxime 100 mg q12h for 7 days, cetirizine 10 mg q24h for 28 days, and carboxymethyl cellulose eyedrop in right eye q6h for 6 weeks. As the patient did not give a history of trauma in the first visit oral, acyclovir 400 mg q6h was given, which we stopped once the patient disclosed the incident. The patient was followed up weekly. His symptoms completely resolved after 6 weeks of the incident (–). After complete resolution of symptoms, we followed him at the third month from the incident during which time he had no symptoms. |
A 35-year-old European first-time mother with an unplanned pregnancy presented late for antenatal care at 35 weeks gestation. In her past medical history, she had asthma for which she was using a salbutamol inhaler. At presentation her weight was 147 kg and her Body Mass Index (BMI) was 54 kg/m2. An oral glucose tolerance test was normal. She was seen regularly in the antenatal clinic where she was found to be normotensive with no proteinuria. At her visits she, however, did complain of dyspnoea on exertion, bilateral leg swelling, and excessive weight gain especially in the third trimester. However, these findings were attributed to her pregnancy.\nAt 40 weeks and three days, she presented with bilateral leg oedema, erythema, and tenderness. She was admitted and had a Doppler ultrasound performed which ruled out a deep venous thrombosis. She was treated with antibiotics and had physiotherapy. An ultrasound scan was performed to assess fetal well-being which estimated the fetal weight to be 4755 g and demonstrated polyhydramnios. At 41 weeks and one day, the patient had prelabour rupture of her membranes and was induced after 24 hours with an oxytocin infusion.\nOn the delivery suite, it was difficult to monitor the fetal heart rate by an external monitor and a fetal scalp electrode was applied. The patient was contracting efficiently on the oxytocin. An epidural was sited, which made it difficult to palpate the contractions. There was no change in the cervical dilation after being six hours on the oxytocin infusion and an emergency caesarean section was carried out.\nThere was abdominal subcutaneous oedema and ascites noted at the caesarean section. Although the procedure was technically difficult due to maternal size, there were no complications. A healthy male was delivered weighing 4750 g. Prophylactic antibiotics and low molecular weight heparin were prescribed postoperatively.\nOn day four, the woman complained of chest tightness and dyspnoea. On examination, she was noted to have a tachycardia. She was normotensive and apyrexial. Her oxygen saturation was 97% on room air and an arterial blood gas was normal. On auscultation of her lungs, there was a bilateral wheeze noted and her heart rate was regular with an S3 gallop. An electrocardiogram showed a normal sinus rhythm. A computed tomography was performed which demonstrated mild inflammatory changes and ruled out a pulmonary embolism. She was commenced on antibiotics, nebulisers, and intravenous hydrocortisone to treat an exacerbation of asthma, possibly due to a chest infection. The following day her dyspnoea increased with no change in her clinical examination. A chest X-ray performed demonstrated cardiomegaly with increased vascular congestion bilaterally (). An arterial blood gas on room air demonstrated hypoxia.\nShe was transferred to the intensive care unit where a transthoracic echocardiogram demonstrated a globally hypokinetic left ventricle, an ejection fraction of less than 30%, and mild/moderate tricuspid regurgitation. The working diagnosis at this point was peripartum cardiomyopathy with a superimposed respiratory tract infection. She was given frusemide and a glyceryl trinitrate infusion for the management of her pulmonary oedema and fluid overload. The antibiotics were continued for the suspected superimposed infection.\nAfter the initial treatment, she was commenced on an ACE inhibitor and beta-blocker for the long-term management of cardiomyopathy and was discharged home on day 14 postpartum. An echocardiogram at five months postpartum showed a left ventricular EF of 50% and the ACE inhibitor and a Beta Blocker were continued. At six months postpartum, a Mirena coil was inserted for contraceptive purposes. |
A one-day-old boy was admitted in our neonatal unit with a midline cervical abnormality. The neonate was born by caesarean section after an uncomplicated pregnancy and weighed 3200 gram having a normal Apgar score. There was no family history of congenital defects and his parents were also in good health. On examination there was a midline vertical mucosal surface in the anterior neck extending from below the mental process of mandible to suprasternal area with no skin covering. The defect was 3 cm in vertical length and 0.5 cm in width. A nipple like projection was observed in the upper part of the defect and there was a sinus in the lower part of the defect above the manubrium. The sinus was examined by a small lacrimal probe and a narrow tract coursing toward the suprasternal notch was observed.\nA fistulogram was not done, but ultrasound scan of the neck showed no evidence of any cervical abnormality (). Auscultation revealed heart murmur caused by small ASD shown in echocardiogram. The chest X-ray was normal.\nThe infant was operated on the fifth day of life by the preference of the parents. A vertical elliptical incision was done around the cervical cleft and the mucosal defect, and then all the pathologic tissues with the underlying fibrotic cord were completely removed. The fibrous cord attached to the manubrium was removed. The skin defect was closed by 2 serial Z-plasties. The 4 resultant flaps were sutured first by vicryl 5-0 for dermal suture and by nylon 6-0 for skin closure ().\nPathologic results showed that the skin cleft was covered by a thin epidermis with irregular papillomatosis, located on a fibrotic dermis devoid of skin appendages. A small skin projection was seen consisting of mature fibro fatty tissue, haphazardly arranged striated muscle bundles, and a normal epidermis as covering.\nAlso a sinus tract lined by parakeratotic stratified squamous epithelium as well as respiratory epithelium was observed. The sinus tract was surrounded by a mildly inflamed fibro vascular stroma enclosing a few striated muscle bundles and mucous secreting glands.\nPost operative course was uneventful and there was no wound infection. In a follow-up examination, 1 and 6 months after operation, there was excellent wound healing of the Z-plasty and no wound contracture in the neck could be seen (). |
A 52-year-old man was admitted to the hospital because of sepsis from a posterior neck abscess. His past medical history included heavy smoking, dyslipidemia, diabetes mellitus type II, and coronary artery bypass graft surgery three years earlier. There was no personal or family history of thyroid disease. Ten days before admission, a furuncle developed on his posterior neck but was not treated. His baseline parameters at admission included a fever of 39.2°C and a WBC of 17,000. There was no evidence of organ failure. A CT scan of his neck with an iodine-containing contrast agent showed an extensive inflammatory process involving the subcutaneous and muscle tissue of the posterior neck, which extended to the paravertebral space at the level of C4-C5, without direct contact with the thyroid gland. The CT also showed a slightly enlarged right lobe and isthmus of the thyroid gland as an incidental finding ().\nTreatment was initiated with broad-spectrum antibiotics (ciprofloxacin, clindamycin, and penicillin), and the patient was taken urgently to the operating room for wide incision, iodine irrigation, and abscess drainage.\nIn spite of antibiotic therapy, recurrent iodine irrigations, and regular changes of dressings the patient showed no clinical improvement. Within a few days he developed severe sepsis with acute respiratory failure, necessitating intubation and mechanical ventilation. At that point the patient was transferred to the intensive care unit (ICU).\nTreatment in the ICU included mechanical ventilation support (at first oral tube and later via tracheostomy), antibiotics as indicated by bacteriological cultures, and extensive surgical debridement of necrotic subcutaneous tissue and muscles through the upper portion of the back ().\nAt each operation the wound field was washed multiple times with iodine-containing solutions. The patient underwent two additional CT scans of the head, neck, and chest with contrast iodine to guide surgical exploration (see for details).\nThe surgical wound started to heal with a decreasing need for debridement. The patient was weaned successfully from ventilatory support but was tachycardic, and his fever persisted as evidence of “uncontrolled sepsis.”\nA review of the patient's laboratory results showed normal T4 (1.3 ng/dL), T3 (2.5 pg/mL), and low TSH (<0.05 uIU/mL) levels 10 days after admission.\nThyroid-function testing was repeated two weeks later. The serum TSH level was still very low (<0.05 uIU/mL), while the serum T3 (8.3 pg/mL), T4 (>12.0 ng/dL), and thyroglobulin (2612.0 ng/mL; normal range <0.3–47.99 ng/mL) levels were significantly elevated. The physical examination at the time was unremarkable.\nNormal sinus tachycardia, without other abnormalities, was observed on the electrocardiogram. In view of significant laboratory findings that were consistent with thyrotoxicosis, treatment was initiated with steroids and PTU (propylthiouracil). Propranolol was prescribed to control sinus tachycardia. All iodine exposure was stopped immediately and subsequently averted. The antiseptic solution used for washing and dressing the wound was changed to sulfamylon.\nLaboratory tests conducted one week later showed significant improvement in serum T3 levels to the normal range (from 8.3 to 2.6 pg/mL), and serum T4 levels (from >12 to 1.3 ng/dL). However, the serum TSH level was still at the very low range (<0.05 uIU/mL). The patient's heart rate decreased to normal and the fever diminished. In view of the quick decrease in the serum T4 level PTU therapy was discontinued (see ). The patient was returned to the ENT department and two weeks later plastic surgery for wound closure was performed successfully. |
The patient is a 66-year-old female with over five years of progressively worsening sacroiliac and coccygeal pain that had become constant and made the patient unable to sit for extended periods of time, and she stated the pain was worse later in the day. She was not able to sit directly on the right buttock. The patient had several falls in the past but the patient described that the pain started after bariatric surgery. She developed pain radiating both into the coccyx, right lower buttock and rarely to the posterior thigh. Magnetic resonance imaging (MRI) of the lumbar spine showed minimal facet joint spondylosis without any signs of herniated disc, nerve root or canal compression. The patient had failed or had short-term relief of less than 30 days from other conventional therapies for coccygeal pain including steroid blocks, ganglion impar blocks and radiofrequency rhizotomy of the sacroiliac joint. Radiographs, bone scan and computerized tomography (CT) scans showed an old coccygeal and possibly osteoporotic sacral fracture to the right. To determine the role the sacral insufficiency fracture played in causing her pain, four years after the pain started, she underwent injection of bone cement in both sacral alae and the coccyx. This resulted in the loss of sacroiliac joint pain but not her coccygeal pain. Six months later, she then had a trial with a thoracic spinal cord lead to T8 to T10 that had no effect on the coccygeal pain. She noted her pain as an eight to nine on visual analog scale (VAS) and was taking Oxycodone 5/325 mg from two to four times daily and gabapentin 300 mg four times daily. It was proposed to the patient to do a lower sacral trial stimulator implant as well as a subcutaneous sacral implant to evaluate if these areas would control her pain.\nThe trial procedure was performed under local anesthesia with 1% lidocaine and a trial eight-point percutaneous lead was positioned in the posterior epidural intra-sacral space through a 14 gauge Tuohy needle. The percutaneous lead (Medtronic, Minneapolis, MN) was positioned in the lower sacrum below the S2 level. In this location when the epidural lead was stimulated, the patient only felt tingling in the right buttock and minimal perineal tingling. There was absolutely no coccygeal stimulation which was the area of her primary pain. This lead was removed since stimulation was not obtained in the lower sacral or coccygeal area. Next, another eight-point lead was placed into the posterior deep sacral fascia from her more symptomatic right side. The lead was passed through a Tuohy needle from the inferior right para-sacral area towards the midline near S1. Trial stimulation gave the patient immediate tingling sensation bilaterally into the sacral and coccyx area exactly where her pain was located. The lead was sutured and taped in place. Further testing in the recovery room demonstrated strong stimulation bilaterally in the lower sacrum, medial buttocks and especially the coccyx. She went home and underwent an initial five-day trial, with daily contact by the office nurse on her progress.\nDuring the initial five-day trial, she continued with the same stimulation which provided coverage to the bilateral paramedian and lower sacrum and coccyx areas with marked pain relief. Her VAS score went from pre-procedure of eight to zero to one, there was a total change in facial expression and she spontaneously stopped all pain medication. When she returned to the office five days post-trial implant, rather than removing the lead, it was decided after discussion with the patient, to continue with the trial for another five days in an effort to ensure the relief continued while the patient resumed full activity. Over a total of 10 days trial period, the patient did her normal activities including taking care of special needs of grandchild and she continued with over 90% pain relief. She returned to the office and the temporary trial lead was then removed. After reviewing information on PNFS with the patient, it was proposed to proceed to do bilateral peripheral field electrode implantation explaining that the two leads would allow for stronger stimulation and coverage of the painful area. She had a scheduled trip so the permanent implant was actually performed almost 30 days after the initial trial terminated. During the 30-day period without the stimulator, she stated her pain completely returned to the pre-trial stimulation level with a VAS between eight and nine and she resumed taking hydrocodone 5/325 mg. She then had permanent implantation of two eight-point leads attached to a rechargeable Medtronic battery (Medtronic). Under fluoroscopic guidance, the permanent leads were placed bilaterally in a para-median direction on both sides of the lower sacrum so they were roughly parallel to each other. Repeat intra-operative testing of each lead demonstrated strong stimulation to the lower sacrum and midline coccyx regions. Both leads were in the deep posterior sacral fascia. Neither lead was adjacent to or making direct contact with bone in the coccyx or lower sacral area (Figure ).\nThe final implantation was performed under local anesthesia with mild sedation. The leads were passed together to the battery. At the time of implantation, both leads were separately tested and the patient felt the stimulation equally into the lower sacrum and coccygeal area (Figure ).\nThe patient was seen at one, four, nine weeks and three months post-implant and remained with a VAS score of one, off all medications and fully active. She stated she was using the stimulator between two and four hours daily, primarily in the late afternoon and early evening. In attempting to better understand the effect of electrode position on peripheral field stimulation, post-implant testing of the different effects of unilateral stimulation and bilateral stimulation with and without inter-communication or 'cross-talk' between the leads was performed starting nine weeks after permanent implantation. At the time of post-implant testing, the patient had absolutely no incisional pain at either the lead or battery site, was very familiar with the stimulator sensation she received so she could concentrate on giving clear feedback on different sensations and the precise location of the stimulation with changes in stimulator settings.\nThe original post-implant settings for the permanent implant were the following which provided excellent pain relief for the initial nine weeks: four electrodes on each side with the following settings of left lead +2 and +3 and -5 and -6 and the right lead +10 and +11 and -13 and -14. At these settings, the patient felt equal stimulation bilaterally in the sacral para-spinal region midway between the iliac crest and down to the coccyx. The positive and negative electrodes were on the same lead and there was no intercommunication between the two parallel electrodes. At the follow-up office visit at nine weeks, each lead was tested separately, first at both the original settings and then making all eight points active with four points positive and four negative. Using only a unilateral lead, the patient felt stimulation primarily unilateral but also into the coccyx. There was minimum difference in tingling using four versus eight stimulation points on one side. Next, the leads were set to communicate and 'cross-talk' right to left, so the positive and negative were opposite each other in the two parallel leads. With this right to left intercommunication setting the patient clearly felt stronger midline and also coccygeal stimulation and was able to get pain relief with lower voltage settings compared to a single lead. She preferred this setting and was sent home to evaluate the difference in pain control. She returned in four weeks, which was 13 weeks after implant and stated that the pain was minimal, with a VAS between zero and one. She preferred the 'cross-talk' between the two leads which gave her greater 'coverage' across the lower midline sacrum. With stimulation, she was able to sit comfortably without the constant right buttock pain which had been making it difficult to sit without keeping her buttock off the chair. |
A 9-year-old boy presented with complaints of excessive duration of sleep, increased appetite, weight gain, excessive daytime sleepiness, loss of interest in sports activities, irritability and snoring since 1.5 years after he was shifted to a residential school.\nBefore attending residential school, he used to follow a regular sleep schedule, with bedtime at 10 p.m. He did not have any issue with sleep onset and used to sleep alone in the bed. According to his father, he used to sleep in supine position and did not show any sign of sleep-related breathing disorder. His mother used to wake him up at 5 a.m. and he usually left the bed within 10 minutes, feeling fresh. He was good in studies till the age of 7 years and had many friends.\nAfter 6 months of shifting to residential school, his father received complaints from the school regarding deterioration in studies and sleeping in the class. His teachers had also noticed that he was gaining weight and losing interest in sports activities. Then, his father took him back home before 1 year.\nAfter bringing him home, since the past 1 year, his parents noticed a gross change in his appetite with an increase in frequency and amount of food. A delay in meeting his demand of food used to result in irritability shown by the child. He continued to put on weight at home. His parents also noticed an increase in the time spent in sleep. For the past 1 year, he started feeling sleepy by 9 p.m. During the night, he would snore and spent most of his sleep in prone position. On some occasions, his father had found saliva on the pillow in the morning. His parents were not able to wake him up till 7 a.m., that too, with a lot of difficulty. After waking up, he took nearly an hour to become active. In addition, he started taking 3–4 hour nap after lunch each day. If he was not allowed to take nap any day, he would fall asleep by 5 p.m to wake up at 7 a.m. in the morning. Any force to avoid nap resulted in irritability. He lost interest in sports activities since then.\nThe child's father also noticed frequent memory lapses resulting in misplacing his belongings. There was no history to suggest childhood depression, frequent rhinitis, tonsillitis, attention deficit hyperactivity disorder, restless leg syndrome, cataplexy, hypnogogic or hypnopompic hallucinations, sleep paralysis or any other parasomnia. There was no evidence of any neurological disorder, epilepsy, head trauma or substance abuse. His birth history and developmental history were noncontributory. Family history was also nonremarkable.\nHis craniofacial examination showed presence of central obesity, dental overjet, Mallampatti grade IV upper airway, submental fat and high arched palate. Epsworth Sleepiness Scale score was 24. His weight was 56 kg and height was 132 cm, leading to body mass index (BMI) of 32.18. His neck circumference was 34 cm.\nMental status examination showed normal psychomotor activity. Child was irritable on occasions following trivial issues and was reluctant to comply with examination procedure. He failed to comply when higher mental functions were being tested.\nHis lateral neck skiagram showed adenoid hypertrophy [] and magnetic resonance imaging (MRI) brain was noncontributory. IQ assessment was also ordered. Since the child did not comply on the first day, the tests were performed after 2 days. At the time of administering tests, child was in better mood and performed all the tests. On developmental screening test, he attained a score of 90; on Vineland Social Maturity scale, his score was 70; on Malin's Intelligence Scale (Indian Adaptation) – Wechsler Intelligence Scale for Children (WICS) (performance test and verbal test), he attained a score of 70 each. Thus, the comprehensive score was 75.\nConsidering the clinical picture, diagnosis of obstructive sleep apnea was made and narcolepsy without cataplexy and KLS were kept as differential diagnoses. Consequently, a level-I video polysomnography followed by multiple sleep latency test (MSLT) was ordered.\nLevel I polysomnography was done with a total recording time of 452 minutes. Total sleep time was 362 minutes. Objectively, the boy had sleep efficiency of 90% (100 × Total Sleep Time (TST)/ Sleep Period Time (SPT)); sleep onset latency of 44 minutes; Rapid Eye Movement (REM) latency of 187minutes and Wake after Sleep Onset (WASO) of 47 minutes. During the study night, N1 was 12%; N2 was 48%; N3 was 18% and REM was 22%. Hypnogram suggested frequent arousals []. He spent most of time in right lateral position [] and Respiratory Disturbance Index (RDI) was 2 (REM=4; Non Rapid Eye Movement (NREM)=1). Respiratory events were position dependent. Average saturation during REM was 97% and during NREM was 98%. Oxygen saturation dropped to 87% during REM and 94% during NREM. Snoring was also observed with an index of 8.9 without any effect of sleep stage.\nMSLT was done the following day. Four naps were recorded as per the standard protocol []. Results of this test are depicted in . |
A 62-year-old Caucasian woman who had previously been in good health, except for a history of treated hypothyroidism, presented to our hospital in November 2009 with sudden onset of severe reflux symptoms and right-sided abdominal pain that radiated around the right flank. An abdominal ultrasound examination was performed. This revealed a large right-sided renal mass. A subsequent computed tomographic (CT) scan confirmed a 13 cm × 9 cm right upper pole renal mass with probable invasion of the right adrenal gland and liver. Tumor extension into the left renal vein and the IVC was also observed. This patient's presentation corresponded to Mayo Clinic level III (Figures and ). Her clinical presentation appeared to be consistent with a large renal carcinoma with renal vein and IVC extension. Metastatic nodules were believed to be present in the right pericardial region; the anterior abdominal soft tissue left pelvis; the left perirenal, abdominal and pelvic regions; and the left adrenal gland. This pattern of metastasis seemed to be atypical of renal cell carcinoma (RCC). Typical renal metastases are found in the lung, periaortic lymph nodes or bone. This contrasted with the extensive intraabdominal spread seen in our patient. In addition, direct tumor extension into the liver is a rare finding in RCC. This tumor showed strong fluorodeoxyglucose uptake on a subsequent positron emission tomographic (PET) scan (Figure ). Upon further questioning, the patient complained of ongoing, mild right flank discomfort, chronic fatigue and rare sweats, but no weight loss or chills. Her physical examination did not reveal a palpable abdominal mass. Baseline complete blood count and blood chemistry testing (including liver and kidney function) were normal, except for an elevated lactate dehydrogenase level of 340IU/L (normal range, 120 to 250IU/L). Because the patient had a superficial abdominal wall mass (Figure ), a needle biopsy was performed to aid in surgical treatment planning. The cytology and core biopsy from this specimen revealed a surprising finding: The malignant cells were thought to represent large-cell, B-cell NHL. This was confirmed by flow cytometry, which identified a κ light chain restricted B-cell population that expressed CD19 and CD20. The patient is currently undergoing cyclophosphamide, doxorubicin, vincristine, prednisone plus rituxumab (R-CHOP) chemotherapy. R-CHOP treatment currently represents the most effective chemotherapy regimen for large-cell NHL in patients over 60 years of age []. This regimen was well tolerated, and upon reimaging with PET and CT scans after three cycles of chemotherapy, she showed an objective partial response in tumor dimensions (Figures and ) with markedly decreased fluorodeoxyglucose uptake (Figure ). The anterior abdominal subcutaneous mass also demonstrated a nearly complete response after three cycles of R-CHOP chemotherapy (Figure ). |
A 40-year-old woman had received regular follow-up for a fatty liver, and inferior vena cava (IVC) tumor, which was initially thought to be a blood thrombus, was incidentally detected by abdominal ultrasonography during a routine medical checkup. She was subsequently referred to our hospital for investigation. Computed tomography (CT) of the chest, abdomen, and pelvis revealed a large tumor in IVC, right iliac and ovarian veins, which was derived from the uterus and extended into the right atrium and ventricle. We identified two extension pathways of intravenous tumor originated from the posterior uterine wall (). Furthermore, the thrombus showed enhancement after administration of contrast material, which was indicative of tumor thrombus or benign metastasizing leiomyomatosis. On pelvic magnetic resonance imaging (MRI), an irregular tumor was identified in the right posterior wall of the uterus, which exhibited heterogeneous high signal intensity on T2-weighted images. Dynamic contrast-enhanced MRI using gadolinium with diethylenetriaminepentaacetate revealed the enhancement of the tumor in IVC (). Because the lesion was located in the right atrium and ventricle, cardiovascular surgery consultation was recommended. A transthoracic echocardiogram was duly performed and revealed the tip of the tumor extended into the right atrium and also into the right ventricle. The multidisciplinary evaluation of the patient indicated that this was a case of cardiac-extending intravenous (IV) leiomyomatosis through the right ovarian and uterine veins arising from the uterine tumor.\nThe operation was performed under general anesthesia (). The heart and IVC were exposed by the right lateral thoracotomy and the midline incision of the abdomen. In case of the tumor adhesion to the hepatic and diaphragmatic IVC, visualization of these IVC enables removing the IV mass and repairing the veins safely. That is why the right thoracotomy approach was chosen. Cardiopulmonary bypass was initiated from the superior vena cava and the right femoral vein/IVC. Inflow was instituted from bilateral femoral arteries to the ascending aorta. A right atriotomy was performed, and a large elastic tumor was found occupying most of the right atrium and extending into the right ventricle and IVC. This intracardiac mass was free floating without invasion of the myocardium and was removed from the right atrium. Thereafter, the right ovarian vein was ligated at the IVC level, and longitudinal venotomies were made in the suprarenal and infrarenal vena cava. Then, the intra-IVC mass was removed easily, because the tumor was capsulated well and there was no adhesion to the IVC and the right atrium. A longitudinal venotomy was also performed in the right common iliac vein, and the tumor in the right internal iliac vein was excised. The common iliac vein and IVC were repaired by continuous sutures with prolene suture line.\nNext, the pelvis was explored. The uterus had enlarged to the size of a goose egg size where no myoma nodule was visible and tumors were found originating from the posterior wall of the uterus and continuing into both the right uterine and ovarian vein. The root of the tumor was attached to the lower posterior uterine serosa, and the stalks of the IV tumor were palpable within both veins continuing into the right internal iliac vein and IVC. The rest of the tumor was completely excised. Therefore, the patient underwent total hysterectomy with bilateral salpingooophorectomy. Complete tumor resection was achieved with an estimated blood loss of 1,045 mL ().\nHistopathological analysis confirmed a diagnosis of LG-ESS. The primary tumor in the uterus comprised short spindle cells resembling the stromal cells of proliferative endometrial tissue infiltrating into the myometrium. These cells were positive for CD10. Also spindle cells with abundant cytoplasm and ellipsoidal nuclei are arranged in an interlacing bundle pattern (α-smooth muscle actin (SMA) positive cells) which were mixed with the tumor cells. Small vessels which are similar to spiral arteries and vessel invasion were noted. In contrast, the intracardiac tumor showed extensive smooth muscle differentiation. Main part was occupied with the spindle cells (α-SMA positive cells). The scattered aggregates of small darker cells (CD10 positive) as seen in prototypical LG-ESS were still recognizable. LG-ESS was histologically confirmed in the right parametria and IV tumor ().\nThe patient experienced deep venous thrombosis in the right common iliac vein during her postoperative course. That was due to venous congestion induced by a stenosis of the repaired common iliac vein and was treated with heparin followed by warfarin. She received 600 mg/day medroxyprogesterone acetate (MPA) for 15 months as a postoperative adjuvant therapy and showed no evidence of disease during a follow-up period of 2 years and 3 months. |
A 55-year-old man first presented with lumps on his mid-lower back and right forehead to his general practitioner who referred him to the General Surgery Department. On further history taking, it was noted to be associated with intermittent constipation, early satiety, and loss of weight of 4 kg over the duration of one month. There were no complains of gross haematuria or abdominal pain. He had no past medical history but had a 40-pack-year history of smoking. The lumps were approximately 4 cm in diameter, mobile, and painless with no surrounding erythema.\nGastroscopy and colonoscopy were performed, to evaluate the cause of the early satiety and constipation in a male aged above 50, which revealed polyps of tubular adenoma histology. Blood tests revealed normal renal function and electrolytes with elevated alanine transaminase and alkaline phosphatase. Ultrasound of the forehead lump was reported as a heterogeneous soft tissue lesion with skull vault destruction, highly vascular and separate from underlying brain parenchyma. The mid-lower back lump was reported as a solid vascular lesion. The radiologist decided to also perform a targeted ultrasound abdomen which located a right renal neoplasm with extension of the likely tumour thrombus into the right main renal vein and to the inferior vena cava. The patient was subsequently referred to the Urology Department which ordered further imaging to stage the tumour.\nA computed tomography showed a 6.4 cm endophytic hypervascular right renal tumour () at the interpolar region with focal invasion into the liver () and seeding into the perinephric space and Gerota's fascia. It also confirmed the tumour thrombus in the renal vein extending into the inferior vena cava () and bilateral pulmonary arteries with pulmonary metastases (). There was no lymphadenopathy noted. The back lump corresponded to the metastatic deposit which replaced the whole L2 spinous process without invading the spinal canal. On the bone scan (), the forehead lump corresponded to the large photopaenic defect at the frontal region with increased osteoblastic activity suspicious of metastasis.\nBased on the abovementioned imaging, the tumour was staged at T4N0M1, clinical stage IV. Consolidation cytoreductive nephrectomy was initially entertained with presurgical course of tyrosine-kinase inhibitors but at the multidisciplinary meeting it was decided that the patient was unlikely to benefit from cytoreductive nephrectomy due to the extensive metastatic burden with poor overall prognosis and the surgical risk was high in view of the bilateral pulmonary arterial thrombi. These options were still conveyed to the patient including the stage of his disease as well as the prognosis. Histological confirmation was also encouraged in the form of a fine-needle aspiration of the forehead or back lump. The patient refused to go ahead with any of the suggested procedures or any form of chemotherapy. He opted for Traditional Chinese Medicine and was subsequently referred for palliative services. The patient passed on 6 months later. |
A 55-year-old, nondiabetic and nonhypertensive female from a rural background was brought for psychiatric consultation for her abnormal behavior, increased irritability, and disturbed sleep for the past 2 years. Three years back, she had an episode of unconsciousness lasting for few hours, which was of sudden onset and associated with weakness of the left upper and lower limbs and deviation of mouth to the right side. She was hospitalized in a private nursing home and diagnosed with ischemic stroke of the right middle cerebral artery territory. She was managed conservatively and discharged after 2 weeks with improvement. Over the next 3 to 4 months, she regained power in her limbs and was able to do her activities of daily living without assistance. However, over the next 6 months, she had multiple episodes of unconsciousness without any motor weakness, which resolved on her own in few hours. Two years back, she had again developed sudden onset unconsciousness with weakness of the right upper and lower limbs, for which she was again hospitalized and managed conservatively. Family members reported that the patient was not able to speak and producing some incomprehensible sound when she regained her consciousness. With treatment, she had shown improvement; power in both the right upper and lower limbs improved to a greater extent that she was able to resume her daily activities. However, her speech remained incomprehensible. In addition, there was an increased irritability. Frequently, she would run away from home overnight. She would roam around her village and come back. Family members also reported that she would laugh loudly clapping her hands for several minutes without any obvious reason. Such behavior was reported 2 to 3 times in a day, which was increased to several times (10–20 times) a day for the last 6 months. In the past 6 months, she would have frequent anger outburst in which she would become assaultive, throw things, shout or run away from home. It caused significant embarrassment for the family members. However, she had never complained of headache, vomiting, or visual impairment. There was no history of head injury in the recent past. Her past and family histories were uneventful. Premorbidly, she was an uneducated homemaker who was capable of doing the regular household chores as well as farming-related work.\nOn general physical examination, no abnormality was detected. Her respiratory and cardiac examination did not reveal any abnormality. Neurological examination revealed upper motor neuron type of facial weakness of the left side.\nShe was prescribed quetiapine 50 mg per day in divided doses, which was later increased up to 100 mg per day. With quetiapine, her sleep cycle became normal. Her frequency of anger episodes and running away from home was decreased. However, inappropriate laughter episodes persisted as before. Hence, she was hospitalized. Her vital parameters (pulse, blood pressure, and temperature) remained within normal limit. Her hematological investigations, including coagulation profile, blood sugar, and lipid profile, were unremarkable.\nNeuroimaging was advised in view of residual weakness. Computed tomography of the brain [Figure and ] showed right dorsolateral frontal, temporal, and posterior parietal gliotic changes. There were also left posterior frontal and parietal gliotic areas. In addition, there was diffuse cerebral atrophy and left hemispheric chronic subdural hematoma. Magnetic resonance imaging [Figure and ] confirmed the same findings. Her clinical features were more suggestive of repeated attacks of stroke, and chronic subdural hematoma per se was not producing any mass effect, the decision was taken to manage her conservatively. Escitalopram was added at a dose of 5 mg/day for her pathological laughter. She had shown improvement in her symptoms and became noncompliant in few days resulting in a relapse of symptoms. Restarting escitalopram improved her symptoms. The patient did not turn in follow-up after first follow-up visit (4 weeks following discharge) []. |
A 31-year-old male presented to the vascular surgery clinic with a history of multiple neck swellings and intermittent fever for six years. Initially, it had been just one swelling on the right lateral neck but over time, the swellings had increased in size and number. They were painful and tender to the touch and the pain intensity had increased over the years. The pain was non-radiating and was not associated with any nausea or vomiting, no difficulty in breathing, or weight loss. However, the swellings were associated with a fever of 101-102 °F. The fever was intermittent in pattern, was not associated with any rigors and chills, any cough, or sore throat. The fever was relieved upon taking acetaminophen, and the patient's workup for other causes responsible for the fever was unremarkable.\nThe patient had woken up one day with a stiff neck and had been unable to rotate his neck with only slight movements possible. He had experienced excruciating pain in the neck swelling, which he described as an 8/10 in intensity. This pain he described had radiated from his throat into his right ear. He had also complained of pain in his right shoulder but no loss of shoulder movement. He had also started experiencing pain in the ipsilateral ear along with tinnitus. Along with the pain, he had experienced dysphagia. There had been no loss of hearing, no fainting spells, or hoarseness of voice. This had been accompanied by a loss of control of his right lower lip.\nFor this episode of neck stiffness, he had gone to the local general practitioner where an ultrasound of his neck had been performed, which had revealed a right carotid body tumor. The general practitioner had prescribed pain medications and referred him to the hospital. He had been referred from one hospital to the other until he had reached a tertiary care hospital where an MRI with contrast of the neck had revealed a mass, measuring 50 x 26 x 28 mm on the right side of the neck extending superiorly up into the base of the skull and inferiorly at the level of the thyroid cartilage, as shown in Figure and Figure . The mass had been posterior to the right carotid bifurcation, external carotid artery, and internal carotid artery. There had been anterior splaying of both the internal and external carotid arteries. Superiorly, the internal carotid artery had been completely encased; while inferiorly, it had been partially encased. These findings had further strengthened the diagnosis of a carotid body tumor.\nThe patient was referred to the vascular surgery department of our institution in June 2021. His case was discussed in a multidisciplinary committee with the radiologists, which recommended surgical excision.\nUnder general anesthesia, an incision was made in front of the anterior border of the sternocleidomastoid muscle, extending to the tragus. We noticed a globular, cystic, non-pulsatile mass adherent to the carotid sheath. Upon opening the sheath, the mass had ramifications between the common carotid artery and internal jugular vein, reaching up into sphenoid recess superiorly and inferiorly; the tumor was found encasing the vagus nerve, as shown in Figure . There was a high carotid bifurcation at the level of mandible angle, which was partially encased by the tumor, as shown in Figure . The vagus nerve was excised along with the tumor and the internal jugular vein was double-ligated, as it was found to be already thrombosed. The tumor was separated from the encased internal carotid artery with careful dissection. The tumor was close to the 12th cranial nerve, which was fixed by the tumor. The facial nerve could not be identified separately from the tumor. The tumor was carefully excised and the patient was then shifted to the surgical intensive care unit.\nOn the immediate postoperative day, the patient developed hoarseness, difficulty in swallowing, and deviation of the angle of the mouth to left. This was attributed to the marginal mandibular nerve being injured during the operation, despite careful dissection. He also lost his ipsilateral nasolabial folds. He was put on nasogastric feeding, which was switched to semisolid food on the third postoperative day. His hoarseness settled on the second postoperative day. On indirect laryngoscopy, ipsilateral vocal cord paralysis was confirmed. His hoarseness was temporary as the damaged vocal cord was compensated for by the adjacent vocal cord. He was discharged home on the fourth postoperative day on a semisolid oral diet. The histopathological report confirmed the suspicion of vagus nerve paraganglioma and indicated an encapsulated tumor with zellballen architecture. The sample stained positive for S100, synaptophysin, and chromogranin. The lymph nodes examined showed no evidence of tuberculosis or malignancy. In conclusion, a paraganglioma of the vagus nerve with reactive hyperplasia of the neck nodes was present. |
A 31-year-old woman with no significant past medical history presented to our emergency department complaining of a constant headache for the previous 4 days. The headache had begun approximately 6 h after receiving epidural anesthesia for labor. The documentation from the anesthesia service that day reported the use of a 17-gauge Touhy needle to enter the subdural space in the lower lumbar spine and the placement of a 19-gauge epidural catheter. No complications were reported with the procedure, and specifically, there was no mention of inadvertent dural puncture. The patient had an unremarkable delivery of a healthy infant at 38-weeks gestation later that day.\nThe patient described the headache as constant and occipital with some radiation to the frontal area. The headache was worse when upright and partially relieved in the supine position. She reported taking acetaminophen/butalbital/caffeine and ibuprofen with little relief. She had no associated vomiting, fever, or changes in her hearing or vision. She denied any photophobia or focal weakness or numbness. She was afebrile on physical exam, with pulse and blood pressure within the normal range. Her exam was notable for a normal neurologic exam including cranial nerves and no neck stiffness. The patient was tentatively diagnosed with a PDPH. After evaluation by the anesthesia service, she was admitted for pain control and possible placement of an epidural blood patch the next day. A computed tomography (CT) scan of her head was obtained prior to admission to evaluate for other possible causes of the headache (Fig. ). This CT identified bilateral parafalcine subdural hematomas measuring 7 mm in thickness on the left and 3 mm thickness on the right. There was no associated mass effect.\nThe patient was admitted to the intensive care unit and started on levetiracetam for seizure prophylaxis. Neurosurgical consultation advised observation, and a repeat CT scan of the head the next day showed no significant change in the hematomas. The patient also received an epidural blood patch the next day with no improvement in the headache. A head CT performed on hospital day 3 showed a decrease in the size of the hematomas, and the patient was discharged on levetiracetam for seizure prophylaxis for 1 week.\nISH occurring after dural puncture is extremely rare. Only sporadic case reports and a few small case series have described this condition [–]. Any procedure that results in spinal dural puncture will theoretically predispose to the development of an ISH. ISH has been described following epidural and spinal anesthesia, as well as lumbar puncture, myelography, epidural steroid injection, and after implantation of an intrathecal drug delivery device and a spinal cord stimulator [–]. The incidence of ISH specifically caused by epidural anesthesia used in obstetric practice has been estimated to be 1:500,000 [].\nThe same mechanism has been postulated for both PDPH and ISH []. The leakage of cerebral spinal fluid (CSF) from the dural puncture site may continue for several weeks, causing reduction in CSF volume []. This results in lower intraspinal and intracranial pressure, leading to relative ventricular collapse and caudal movement of the spinal cord and brain. As a consequence, the dura, pain-sensitive structures, cranial nerves, and subdural bridging veins are stretched. This may ultimately result in a tear of the bridging veins and consequently an ISH. Risk factors associated with ISH after dural puncture include excessive CSF leakage from multiple punctures in large needle use, pregnancy, coagulopathy, cerebral vascular abnormalities, dehydration, brain atrophy, and alcoholism [–].\nThe duration of time from dural puncture to the diagnosis of ISH ranges widely from 4 h to 29 weeks []. In one case series, 37% of cases were diagnosed within 1 week of dural puncture, and 85% were diagnosed within 1 month []. A headache, most often diagnosed as PDPH, is the main presenting symptom [, –]. Other reported symptoms and signs present at the time of diagnosis are listed in Table [, , ]. Reported rates of surgical intervention for ISH after dural puncture vary from 9 to 80% [, , ]. In general, surgical intervention for ISH is indicated if the hematoma thickness exceeds 10 mm, there is a midline shift of greater than 5 mm, or there is neurologic deterioration []. Furthermore, some have advocated for the use of epidural blood patching in the treatment of ISH caused by dural tears resulting in chronic CSF leaks [, ]. A full recovery is reported in over 80% of patients, with death reported in 7–10% of cases [–].\nHeadache in the postpartum period is common, occurring in 39% of women []. The majority of these headaches are benign primary headaches, such as migraine and tension type []. Secondary headaches in the postpartum period are typically due to obstetric or anesthetic complications, or the hypercoagulable state after delivery (Table ). Our patient was initially misdiagnosed as having PDPH, similar to many previous reports of this condition. PDPH is defined as a headache that develops within 5 days of dural puncture that significantly worsens soon after sitting upright or standing and improves after lying horizontally []. PDPH is more likely to occur in young women of low body mass as compared with other patients []. An epidural blood patch is considered the gold standard for treatment of PDPH, with a success rate of 70–90% []. Over 85% of patients report resolution of PDPH within 6 weeks regardless of treatment [].\nThe incidence of ISH after dural puncture is probably underreported since many of these patients are treated as PDPH with the eventual resolution of their symptoms. When to obtain brain imaging studies in the assessment of a likely PDPH is unclear. A reasonable approach would be to consider imaging in patients that (1) have a postural headache lasting more than 1 week, (2) do not improve or have worsening of their headache after an epidural blood patch, (3) report a change in the headache from postural to non-postural, or (4) develop other neurologic signs or symptoms with the headache []. |
Our patient was a 50-year-old male suffering from severe abdominal pain related to pancreatic cancer with multiple liver metastases. His life expectancy was less than six months. He had severe and debilitating abdominal pain that was not relieved with medication management including acetaminophen, NSAIDs, gabapentin, and high-dose opioids. The patient consistently reported a pain level of 10/10 intensity on a numerical rating scale (NRS). Since the patient failed conservative therapy with medication management, he was referred to interventional pain management for a celiac plexus block to better manage his pain.\nAfter reviewing the patient’s medical chart, it was noted that this patient was coagulopathic as his international normalized ratio (INR) had risen from 1.1 to 1.6 over an eight-day period. Since the celiac plexus lies anterior and laterally around the aorta, needles are often placed through the aorta to perform a traditional celiac plexus block. Given the patient’s elevated INR, we decided to target the splanchnic nerves bilaterally instead. Additionally, given that this patient’s INR was continuing to rise, there was concern that he may not be a candidate for future interventions if he continued to become more coagulopathic. For this reason, we decided to perform both radiofrequency ablation (RFA) and alcohol neurolysis of the splanchnic nerves to ensure full effect. The patient was counseled regarding the risks and benefits of these injections and he consented to undergo the procedure.\nThe patient was then taken to a procedure room and placed in the prone position. The thoracolumbar spine was visualized. The skin was anesthetized with 2% lidocaine before introducing the needles used for RFA. The needles were advanced towards the anterolateral aspect of the T11 vertebral body (Figure ). Once the needles were confirmed to be in proper positioning, sensory testing was performed and caused abdominal stimulation in the same region of his abdominal pain. The nerves were then anesthetized with 2 mL of 2% lidocaine before RFA was performed. RFA was performed at 80 degrees Celsius for 90 seconds on both sides. The needles were withdrawn slightly to create additional lesions along the vertebral body using the same technique.\nNext, the needles were placed near anterolateral aspect of the T11 vertebral body where the splanchnic nerves typically are positioned. Nonionic contrast was then used to verify proper needle placement before performing alcohol neurolysis. After confirming proper needle placement, another 3 mL of 2% lidocaine was administered on each side. Finally, 8 mL of 100% ethyl alcohol mixed with 2 mL of contrast was then divided equally and administered with intermittent fluoroscopy to confirm appropriate spread of the solution (Figure ). |
A 35-year-old left-handed man who works as a mechanic and firefighter presented to our institution with hand pain and cold intolerance for 3 months. He was referred by a local vascular surgeon who had made the diagnosis of hypothenar hammer syndrome (HHS) following an angiogram of the right upper extremity showing complete occlusion of the ulnar artery (). On initial examination, Doppler signals of the ulnar artery, superficial palmar arch, and ring/small digital arteries were absent in the right hand. Subsequently, the patient developed an ulcer on his right long fingertip after minor trauma that had not healed over a 4-week period. He suffered ischemic hand pain and severe cold intolerance. After conservative management failed, the decision was made for operative intervention. We proposed using an arterial graft versus a venous graft in order to improve long-term patency. Our plan was to utilize the descending branch of the lateral circumflex femoral artery (DLCFA) as an arterial graft.\nIntraoperatively, Doppler ultrasound revealed a segmental thrombotic defect extending from the superficial palmar arch, across the Guyon canal, extending 10 cm proximally in the forearm. The ulnar artery was resected back to healthy bleeding pulsatile flow, resulting in a segmental defect of 12 cm. Final pathology of the submitted ulnar artery segment revealed organizing thromboembolus in the lumen, which at the time of resection was adherent to the lumen.\nThe DLCFA was harvested, with care taken to prevent injury to the lateral femoral cutaneous nerve. A 12-cm segment of the artery was circumferentially dissected, as well as approximately 2 cm of 2 branching vessels at the distal end of the segment that would be used to reconstruct the superficial palmar arch and common digital arteries with one graft. With the arterial graft completely dissected, it was ligated both distally and proximally.\nThe arterial graft and recipient vessels were examined under the operative microscope. The proximal anastomosis was preformed first. The two branches at the distal end of the arterial graft were anastomosed to the superficial palmar arch stump, and common digital artery to the ring and small finger, respectively. A bolus of 5000 units of heparin was given intravenously before release of the microvascular clamps. Adequate pulsatile flow was achieved though the graft and confirmed with Doppler ultrasound to each finger ( and , Video 1). The hand was then irrigated, and the ulnar nerve was explored to ensure that no injury had taken place. The skin was closed, and the patient's hand was placed into a dorsal splint.\nThe patient remained in the hospital overnight for monitoring and was discharged the following morning on oral aspirin as the only anticoagulant. On follow-up examination 2 days later, strong Doppler signals were elicited at the ulnar artery, palmar arch, and radial and ulnar aspect of each digit. At 1 month, Doppler signals remained strong and the ulcer previously present on the right long fingertip had completely resolved (, Video 2). During this visit he reported to be asymptomatic and has had complete resolution of the cold intolerance and burning pain of his right hand. |
A 56-year-old right-handed Caucasian woman with long-standing MS presented to our facility with intense, intermittent pain in the right side of her tongue and floor of her mouth, which was precipitated by speaking, chewing, and swallowing. The episodic pain was intense, sharp, and with a lancinating character, and lasted from two to six seconds. She developed the pain one year prior to her last visit and later on she also experienced vocal hoarseness. The pain was localized to the posterior part of her tongue and surrounding areas on her right side. On certain occasions the pain radiated to her right ear. The laryngeal pain had an episodic nature and between attacks our patient was free from pain. Initially she had a few attacks of this pain per week, however, the frequency and intensity of the pain gradually increased. At the end of the year she noticed that her voice became significantly hoarse and the attacks were triggered not only by swallowing but also by speaking. She modified and restricted her diet to only soft food and liquids, with only temporary relief. During the last two weeks prior to her visit the throat pain recurred regularly with eating and speaking. She saw her primary care physician, who prescribed hydrocodone with only partial relief.\nShe was diagnosed with MS and migraine 18 years prior to case presentation. At the time of her new head and neck pain she was on interferon-β1a 30 μg intramuscularly once weekly.\nA neurological examination revealed a middle-aged woman with stable vital signs and normal mental status. She was oriented to all cognitive spheres with no aphasia, apraxia, or agnosia. During the assessment she was in pain off and on, and pain was precipitated by speaking. A cranial nerve examination revealed dysphagia, vocal hoarseness, and pain in the distribution of the right glossopharyngeal nerve (posterior third of the tongue, floor of the mouth, and right side of the throat). Her palate was symmetrically moving upon phonation and her gag reflex was present. Our patient had difficulty with differentiating basic tastes, particularly salty tastes on the right side of the posterior of her tongue during the painful attacks. Normal sensation in the distribution of cranial nerve IX was present. She was also right hemiparetic with a wide-based stance and ataxic gait. Her deep tendon reflexes were depressed and her plantar responses were flexor.\nA speech pathologist evaluated our patient and could not determine any sign of aspiration; however, a soft mechanical diet with chopped meat, feeding at 90 degrees, with small boluses and maintained in the sitting position with head elevated to avoid and aspiration for 30 minutes.\nA magnetic resonance imaging (MRI) scan of the brain with and without contrast and a magnetic resonance angiogram of the head and neck vessels were performed. On axial fluid attenuated inversion recovery (FLAIR) MRI scans of the brain, multiple hyperintense lesions were present in the periventricular areas (Figure ), medulla, left side of pons, right middle cerebellar peduncle, the left cerebellar dentate nucleus, and in the prerubral area of the midbrain. On T1-weighted images a number of hypointense lesions consistent with T1-weighted black holes were present. No post-contrast T1-weighted enhancing lesions were present. Corpus callosum atrophy was absent. On high-resolution T2-weighted imaging, vascular contact between the glossopharyngeal nerve and the right posterior inferior cerebellar artery (PICA) was observed (Figures and ). A magnetic resonance angiogram of the head revealed the posterior inferior cerebellar artery originating from the right vertebral artery and that the left vertebral artery was underdeveloped, along with compression of the right glossopharyngeal (GP) nerve by the PICA (Figure ).\nA soft diet with hydration using intravenous 5% dextrose in half amount of normal saline 1000ml intravenously daily at a rate of 60ml/h was initiated. Treatment included methylprednisolone 1g intravenously daily for three days, carbamazepine 200mg orally twice a day, and paracetamol 650mg orally every six hours as needed.\nAt the end of the second day our patient reported a marked improvement in pain with absence of pain at nighttime and during sleep. Her speech improved and she requested to advance her diet from soft to mechanical soft.\nWhile on carbamazepine, she reported reasonable control of her glossopharyngeal pain. She also reported that once she missed a dose of her medications, the pain rapidly recurred. The options for surgical intervention such as microvascular decompression versus Gamma Knife® treatment were discussed with our patient in case medical treatment failed; however, she rejected any further interventions. Our patient was discharged on carbamazepine. |
A 59-year-old man with a history of chronic obstructive pulmonary disease presented to the emergency room with severe sinus bradycardia and hypotension. Due to his history of recurrent dizziness and presyncope, prior work up with Holter cardiac event monitor showed predominant sinus bradycardia with no evidence of chronic incompetence and no clear correlation of his symptoms to the bradycardia episodes. His most recent echocardiogram showed normal left ventricular systolic function and wall motion. Myocardial perfusion study done for recurrent chest pain showed normal myocardial perfusion and normal ejection fraction on Gated SPECT. Tilt table test was markedly abnormal consistent with severe dysautonomia. His baseline blood pressure was 113/70 mmHg, and his HR was 49 beats per minute. Only two minutes into the test, his blood pressure dropped to 60/29 mmHg while his heart rate remained at 47 bpm. The patient was offered pacemaker therapy after his abnormal tilt table test but he refused.\nA few weeks later, the patient presented to the emergency room with syncope. Initial ECG showed severe sinus bradycardia, heart rate in the low 40 s with normal PR interval, and left anterior fascicular block. He was hypotensive and diaphoretic. He denied any chest pain, pressure, or tightness. There were no significant signs of cerebral hypoperfusion. The patient was not on any bradycardia-inducing medications, and initial blood work showed no reversible cause of his severe sinus bradycardia.\nThe patient was started on intravenous fluid hydration, and a bedside echocardiogram was obtained. Echocardiogram showed normal biventricular systolic function and no significant valvular abnormalities. Atropine was given but resulted in minimal, transient improvement in his heart rate to the low 50 s. The systolic blood pressure remained below 80 mmHg. Dopamine followed by norepinephrine drips were administered, and the patient was sent to the electrophysiology laboratory for dual-chamber permanent pacemaker therapy.\nDuring implantation of a permanent pacemaker, the right atrial lead was appropriately placed via the left subclavian vein into the right atrial appendage. However, the ventricular lead was placed via the subclavian artery into the left ventricular cavity. He was admitted to the hospital overnight and a chest X-ray was obtained the next day, showing no evidence of pneumothorax (Figure ). Surprisingly, his ECG showed atrial pacing with long AV delay and intermittent ventricular pacing with the paced ventricular beats showing right bundle branch block morphology contrary to the expected left bundle branch block morphology seen when the lead is inside the right ventricular cavity (Figure ). A limited bedside Echo was obtained that showed the ventricular lead clearly crossing the aortic valve to the left ventricular cavity and fixed into the inferolateral wall of the left ventricle (Figure ). Early recognition of this serious pacemaker implantation complication led to pacemaker revision the same day with extraction of the arterial lead and reinsertion of the ventricular lead via the left subclavian vein into the right ventricular cavity (Figure ). The pacemaker revision went uncomplicated. |
A 59-year-old postmenopausal woman presented with an illdefined, hard mass in her left breast for a year. Radiologic evaluations demonstrated a hypo-echoic mass (14 mm) with a speculated margin in ultrasonography and a focal asymmetric density in the upper medial portion by mammography. Fine needle aspiration followed by needle biopsy was performed with the diagnosis of MC and invasive carcinoma with abundant mucin pool formation, respectively. She underwent partial mastectomy with sentinel lymph node biopsy. The sentinel lymph node was free from metastasis. She has been followed for 3 months with chemotherapy for adjuvant treatment and remains disease-free.\nThe aspiration showed a few scattered, variably sized, irregular clusters of columnar cells in the greenish blue mucinous background with necrotic debris. The columnar cells contained abundant mucin vacuoles in cytoplasm which had displaced their nuclei. The nuclear membrane was irregular and sharply angulated and the nuclei revealed a coarse chromatin pattern with prominent nucleoli ().\nOn gross examination, the cut surface showed an irregular, white to tan, solid and firm mass (9×7 mm), with a glistening appearance. Most MCAs in previous reports have demonstrated grossly cystic cut surfaces, except one case of MCA reported from Koenig and Tavassoli in 1998.,- The case in 1998 was small in size (8 mm) and had a grossly solid appearance. The present case was 9 mm in maximal diameter and did not contain a macroscopic cyst. It is assumed that the small size of the MCA meant that it did not produce enough mucin to fill out and dilate cysts and ductal structures. The microscopic findings revealed a few cysts and ductal carcinoma in situ, adjacent to the invasive carcinoma. The cysts were lined by a single layer of tall columnar mucinous cells with focal areas of micropapillary structures, resembling those of the uterine endocervix. The luminal space contained mucin (). The invasive cancer area contained an abundant mucin pool in stroma with floating cell clusters. The floating cells contained mucin vacuoles in cytoplasm displacing atypical nuclei to the periphery.\nSpecial staining and IHC on paraffin embedded tissue were performed. Both intracytoplasmic and extracytoplasmic mucin were stained by periodic acid-Schiff with diastase, alcian blue and mucicarcine, representing the acidic and neutral nature of mucin. The tumor cells were positive for CK7 and negative for CK20 and CDX-2. The IHC for MUC proteins was performed. The cancer cells were positive for MUC5 and MUC1 and negative for MUC2 and MUC6. Mucin of the intracytoplasm and stroma revealed positivity for MUC5 and negativity for the other MUC proteins (MUC1, MUC2, and MUC6). The hormone receptors, ER and PR, were negative. The c-erbB2 was 2-positive (), but silver in situ hybridization demonstrated no amplification of the HER-2 gene. |
A 4-year-old boy with a history of recurrent wheezing was admitted to the inpatient department of our hospital for evaluation of acute exacerbation of severe asthma. On admission, the patient complained of recurrent wheezing and intermittent cough, with shortness of breath, general fatigue, orthopnea, being pale, and sweating. All his symptoms can worsen at night. The child's parents denied any foreign body aspiration, weight loss, and trauma. Physical examination revealed tachypnea, cyanosis, suprasternal retraction, and a prolonged expiratory crackles at lungs. His saturation level of oxygen in hemoglobin was 91% with an oxygen supplemental therapy. In laboratory tests, both his skin prick test and serum dosage of IgE were negative, and his lung function test showed airway hyperresponsiveness, in which his V75 and V50 were in mild reduction, PF was in moderate reduction, and the bronchial dilation was negative.\nThe child has a history of recurrent respiratory infection (4 times), which is characterized by cough, fever, and wheeze. One was at the age of 10 months and three were all after 3 years. He was diagnosed as asthma because of a history of recurrent wheezing episodes and airway hyperresponsiveness suggested by lung function test. But his skin prick test and serum dosage of IgE did not support this diagnosis. However, the patient did not do regular follow-up. During the age of 10 months to 3 years, there was no wheezing. His wheezing could partially be improved by bronchodilator and systemic corticosteroid. Each episode of wheezing had lasted more than 20 days when the patient was treated in outpatient department. It seemed that the patient is not so sensitive to the steroid treatment.\nConsidering the patient's history, this was his fourth asthmatic attack, but this time when he was referred to the inpatient department, he was not relieved of the symptoms by routine treatment. Should we need to consider other diseases rather than asthma? After admission, we did the bronchoscopy to examine his airway. The bronchoscopy revealed tracheal narrowing at the lower end of the trachea without any internal pathology. The normal CT scan of lung showed no parenchymal disease. Then we advised the patient to do an enhanced computed tomography angiogram to get more information about the airway and the vessel. Considering some other social factors and radiation, the parents refused to do so. After 8 days of treatment the patient's parents asked for discharging from the hospital.\nWhen we did follow-up two weeks later, the patient's parents finally agreed to do an enhanced computed tomography angiogram on the lung. The diagnosis of DAA was confirmed. CT angiogram revealed a vascular ring, consisting of a double aortic arch, around the trachea, and the right side was dominant which was compressing the trachea. The medical images of this patient are shown in Figures and . The echocardiogram confirmed the presence of the vascular ring. The inner diameter of right and left aortic arch is 9.3 and 9.9 mm. The double aortic arch gives off the common carotid artery and subclavian artery to the left descending aorta.\nThree months later, the child was scheduled for surgery to correct the vascular ring. After oxygenation with 100% oxygen, anesthesia was induced, the triangle of ductus arteriosus was exposed by pulling the left lung, then an incision was made on the mediastinal pleura at the surface of descending aorta to make the left and right aortic arch free, and finally the right aortic arch was divided at the joint to relieve the compression to trachea and esophagus. The child was ventilated mechanically for 12 hours in Intensive Care Unit. He was relieved of symptoms without any stridor. Then we reperformed a CT scan and airway remodeling on the chest, which showed a slighter narrowing in the lower trachea. But the child refused to do a follow-up check on his lung function test. Then he was discharged on his seventh postoperative day. At follow-up evaluation performed six months after surgery, the patient was free of respiratory problems and pulmonary infections. |
A fifty-one-year-old obese lady with a body mass index of 45.3, known to have hypertension and dyslipidemia, was referred to our institute as a case of a right frontal mass lesion associated with hydrocephalus (). Her symptoms started three years ago when she gradually developed gait imbalance with frequent falling, urinary incontinence, headache, dizziness, confusion, and personality changes. Two years later, she had a progressive bilateral visual acuity deterioration that initially started as photophobia, progressing to blurred vision, then to hand motion perception only. ().\nPhysical examination showed a fully awake patient with a nonreactive right pupil of 5 millimeters and a sluggish left pupil of 6 millimeters in size; ophthalmoscope examination showed bilateral pale edematous optic disk, right oculomotor cranial nerve palsy, left abducens cranial nerve palsy, and a decreased sensation of the entire right side of the face to pinprick. Upper and lower limb sensory and motor examinations were normal. The patient was investigated with an MRI, magnetic resonance venography, and magnetic resonance perfusion that showed patent venous sinuses (). The MRI showed a large right frontal periventricular heterogeneous enhancing frontal tumor with a ventriculomegaly and dilated CSF spaces.\nThe patient underwent a right frontal craniotomy for tumor resection and insertion of an external ventricular drain as her intracranial pressure (ICP) was very high intraoperatively ranging between 28 and 30 cmH2O; initially, the drained CSF was xanthochromic then started to clear gradually. The physical exam postoperatively shows normal movement of all her limbs with reactive bilateral pupils. Histopathological assessment was evident for large areas of necrosis without a nuclear pseudopalisading or significant mitotic activity, which is consistent with the features of grade II WHO PXA.\nSerial postoperative examinations showed remarkable improvement in her presenting symptoms, including visual acuity, gait, and urinary incontinence. Postoperative MRI showed complete resection of the tumor (). The patient underwent a right VP shunt insertion and EVD removal two weeks after her initial surgery and was discharged in a better condition. Her outpatient exam three-month postoperation revealed no abnormal gait disturbance or visual deficits. |
In June 2002, a 36-year-old woman presented to her primary health care doctor with a history of flushing, diarrhoea, night sweats, and a clinically detectable mass in her left medial supraclavicular fossa. Her past medical history consisted only of essential hypertension for which she did not require prescribed therapy. Her family history included a brother with a diagnosis of sarcoma and two other non-first-degree relatives with primary brain malignancies.\nFine needle aspiration confirmed the diagnosis of medullary thyroid cancer, and in July 2002, she underwent total thyroidectomy with left-sided modified radical neck dissection and central compartment clearance. At this point, concerns were raised regarding optimal cytoreduction as the appearances of the central compartment, level 4 and level 5 nodes, were that of extensive disease. In order to maximise local disease control, she received adjuvant radical radiotherapy delivering 60 Gy to the thyroid bed.\nTwo years after completion of treatment, in February 2004, follow-up repeat imaging reported a recurrent nodule at level 4 of her neck. Subsequent resection confirmed this to be recurrent medullary thyroid cancer with no evidence of distant spread at the time. She continued to be monitored at the oncology clinic and remained disease-free until four years later, in May 2008, when computer tomography (CT) imaging revealed new pulmonary parenchymal metastases. These were closely monitored for the next 2 years with repeat imaging and measurement of calcitonin levels. In May 2010, it was decided that the patient should embark on systemic anticancer treatment.\nShe was offered participation in a phase 2 clinical trial with the agent lenvatinib (E7080) and she commenced treatment with 24 mg once daily in May 2010. One week into therapy, it was noted that she was marginally hypertensive with a blood pressure of 140/100 mmHg. No proteinuria was identified at this point, but she was commenced on 5 mg of amlodipine to manage hypertension. Monitoring of blood pressure and urinalysis continued as per study protocol.\nAfter two completed cycles of lenvatinib, CT imaging reported a reduction in size of all lesions. Further tumour assessment after 4 months confirmed a partial response to treatment with a 50% reduction of the sum of the long diameters of target lesions. She was experiencing various grade 1 toxicities throughout this time but was keen to maintain treatment given the good response. Due to the multiple low-grade toxicities, the dose of lenvatinib was initially reduced to 20 mg and thereafter to 14 mg.\nIn December 2011, 19 months after starting lenvatinib, she developed mild ankle oedema. Urinalysis carried out at the time identified proteinuria. A subsequent 24-hour urine collection identified 3.1 g/litre of proteinuria, equating to a urinary protein creatinine ratio (UPCR) of 625. The patient had not started any other medications and the incidence of proteinuria was felt to be lenvatinib related. Treatment with lenvatinib was ceased; however, due to concerns regarding possible intrinsic renal disease, she underwent screening for glomerulonephritis which was negative.\nA subsequent renal biopsy showed focal segmental glomerulosclerosis (FSGS) in two of twelve viable glomeruli, with tuft-capsule adhesion, hyalinosis, segmental intracapillary hypercellularity, and segmental splitting of capillary walls, predominantly in regions of segmental sclerosis. There was mild tubular atrophy, interstitial fibrosis, mild/moderate arterial intimal fibroelastic thickening, and mild arteriolosclerosis. Immunofluorescence showed no staining in glomeruli. Electron microscopy showed mild patchy reduplication of the basement membrane and effacement of only 20% of podocyte foot processes. There were no widespread electron dense deposits and no endothelial cell tubule-reticular inclusions. The endothelial cells showed no evidence of activation or damage. The appearances were consistent with a diagnosis of focal segmental glomerulosclerosis (FSGS). The lack of widespread podocyte foot process effacement suggests a secondary form of FSGS, which in the context of anti-VEGF treatment, could be mediated by microangiopathy. Whilst there was no histological evidence of acute thrombotic microangiopathy, it is possible that some of the pathological changes seen (splitting of glomerular capillary walls and mild arteriosclerosis) could be related to chronic low-grade endothelial cell damage. Based on histology, it is not possible to be certain whether the FSGS was caused by direct podocyte injury or whether it was related to endothelial cell injury. Histological slides are illustrated below in Figures , , and .\nThroughout this time, excretory function remained stable. Treatment with an ACE-inhibitor (ACEi) was introduced but due to poor tolerance and the quick improvement of the proteinuria after cessation of lenvatinib, the ACEi was stopped and patient's blood pressure was monitored closely.\nWithdrawal of lenvatinib had a marked effect on the levels of proteinuria, as illustrated in .\nThe patient continued follow-up at the renal clinic on a regular basis until July 2013, when she was discharged with no evidence of proteinuria, normotensive and with normal excretory renal function.\nAfter her discharge from the renal clinic and between 2013 and 2017, the patient was treated with vandetanib, nintedanib, and cabozantinib with no evidence of recurrent renal disease.\nE7080, also known as lenvatinib, is a potent inhibitor of the receptor protein kinases VEGFR-2 and VEGFR-3 but also displays inhibitory binding properties against VGFR-1, FGFR-1, and PDGFRα/β, albeit at significantly higher IC50 (half maximal inhibitory concentration, IC50). Its ability to restrain angiogenesis was shown on human umbilical vein endothelial cells (HUVEC) where E7080 inhibited VEGFR-2 phosphorylation and thereby capillary tube formation []. Apart from angiogenesis, E7080 decreased lymphangiogenesis in both the primary tumour of human breast adenocarcinoma cells in xenografts as well as in metastatic nodules in the lymph nodes of nude mice bearing these tumours []. Glen et al. showed in preclinical experiments that abrogation of FGFR and PDGFR signalling by E7080 inhibited invasion and migration of human melanoma cells lines (DX3) and human osteosarcoma epithelial cells (U2OS) []. Its potency against FGFR-1 differentiates E7080 from other currently approved tyrosine kinase inhibitors with antiangiogenesis properties [, ].\nThe preclinical data above were confirmed in several early phase human trials with E7080 in 2011 and 2012 in US, Europe, and Japan. Whilst establishing pharmacokinetic and pharmacodynamic properties of the drug, safety and preliminary efficacy was also well described. Lenvatinib was well tolerated at doses from 10 mg BID to 25 mg OD [–] and was associated with a reduction in disease activity biomarkers [], partial response, and stable disease according to response evaluation criteria in solid tumours []. These findings were further established in phase 2 trials and notably responses were demonstrated in thyroid cancer [–].\nApproval in thyroid cancer was granted in light of significant improvement in progression-free survival (PFS) compared with placebo in patients with radioiodine-refractory differentiated thyroid cancer in a phase 3 study (SELECT study) []. Lenvatinib improved median PFS over placebo by almost 15 months (HR 0.21; p < 0.01) and induced an objective response rate of 64.8%. The median survival results were diluted due to crossover of the patients from the placebo arm to the treatment arm; nevertheless, a subgroup analysis on patients stratified by age showed that older patients (>71 years old) had a survival advantage when treated with lenvatinib compared to placebo (HR, 0.53; p = 0.02), and the younger subgroup achieved a PFS of 20.2 months versus 3.7 m (p < 0.001) [].\nProteinuria and hypertension are the two most commonly reported side-effects of VEGF inhibitors and frequently the cause for therapy discontinuation. Proteinuria is used as a surrogate marker for glomerular damage and hypertension often accompanies and aggravates this.\nThe pathophysiology of proteinuria and glomerular damage in anti-VEGF therapy remains complex and far from well understood. Biopsy-proven cases of glomerular disease in anti-VEGF therapy are few; however, most have demonstrated changes in keeping with glomerular thrombotic microangiopathy (TMA) histology, with predominant endotheliosis and membranoproliferative changes [, ]. Other histological changes documented include cryoglobulinaemic glomerulonephritis, acute interstitial nephritis, collapsing and crescentic glomerulonephropathies, and FSGS plus TMA [–].\nIt has been theorized that hypertension is caused by decreased vascular production of nitrous oxide induced by inhibiting VEGF. This leads to renal haemodynamic compromise and subsequent proteinuria (much akin to exercise-related proteinuria) []. However, a mouse model study showed that glomerular injury preceded hypertension [] and many cases document glomerular injury in the absence of hypertension [], indicating that it cannot be the only trigger for proteinuria in anti-VEGF treated patients.\nInhibition of VEGF in podocytes (by injection of anti-VEGF antibodies or VEGF gene deletion) results in loss of endothelial fenestrations in glomerular capillaries, proliferation of glomerular endothelial cells, loss of podocytes, and proteinuria in mice [, ]. VEGF appears to be a crucial endothelial survival factor and its inhibition often manifests as TMA, a histology strikingly similar to that of severe preeclampsia—as placenta overproduces a soluble VEGF receptor (fms-like tyrosine kinase 1) that acts as a VEGF antagonist.\nIzzedine et al.'s 8-year follow-up study results from 2014 shed great light in anti-VEGF-related renal injury. It showed that in 100 patients who developed renal disease whilst on anti-VEGF treatment, the main histology associated with TKIs was minimal change disease and/or collapsing-like focal segmental glomerulosclerosis (MCN/cFSGS), a FSGC variant which is considered a separate entity to FSGS. In the same analysis, TMA histology was most frequently associated with VEGF-ligand targeted therapy (such as bevacizumab and aflibercept) suggesting two, possibly distinct pathophysiologies [, ] between renal damage caused by targeting the VEGF ligand as opposed to targeting the VEGFR tyrosine kinase domain. This could potentially be explained by considering the associations and signal transduction pathways between podocytes, endothelial cells, and VEGF. Podocytes produce vascular endothelial growth factor (VEGF), whereas VEGF receptor tyrosine kinases (RTKs) are expressed by both podocytes and glomerular endothelial cells.\nOur case demonstrates a secondary form of FSGS pathology which cannot confidently be attributed to TMA but could potentially represent the end result of chronic low-grade endothelial cell damage. The moderate histological findings were in keeping with a less-severe clinical course of the FSGS, with fast resolution of proteinuria and hypertension. More significantly, rechallenging the patient with additional three agents blocking the VEGF axis did not result in recurrence of the renal damage. |
A 28-year-old graduate male was brought to the casualty department of our tertiary care hospital by his family members with the complaints of being unresponsive to external stimuli, poor oral intake and decreased personal care since 1 week following the death of his wife due to obstetric complications during delivery of their second child. He was reportedly expressing ideas of hopelessness, voiced self-harm ideations at times and would repeatedly call out his wife's name before presenting to us. However, there were no suicidal attempts. There was no family history of depressive disorders and the patient did not have any history of substance abuse.\nOn examination he was found to be dishevelled, with gross psychomotor retardation and speaking in whispering voice. His vitals were stable and systemic examination was unremarkable. At times he would remain mute with vacant expression on his face and other times he would burst out into crying spells. He was provisionally diagnosed to be having complicated grief reaction and was started on Escitalopram upto 10 mg/day along with lorazepam at doses of 2 mg/day for his catatonic symptoms. But the patient was poorly compliant and continued to be symptomatic. The treating team considered ECT because of presence of catatonic symptoms, poor oral intake, self-harm ideations and severe guilt. The family members were not willing for ECT for some reasons and hence an alternative option of starting the patient on ketamine was suggested to them. The informed consent was taken from immediate family members explaining the rationale, off-label status of drug and possible adverse effects. The same was communicated to the patient who was by then had started reporting of the distress and wish to come out of the current state following which he too consented for the procedure. This also helped in gaining his cooperation and establishing better rapport so essential for such procedures. Detailed physical examination was done and routine investigations of blood and urine were carried out to rule out physical contraindications for the use of ketamine. He was given an IV infusion of ketamine 0.5 mg/kg/hour which lasted around 40 minutes. His pulse rate, BP, O2 saturation and temperature were monitored every 5 minutes.\nWithin few minutes of administration, the therapist initiated the conversation with the patient with open-ended questions pertaining to his feelings, emotions and distress. Later as the patient became verbally communicative and suggestible, he was encouraged to vent out his distress, guilt and unresolved conflicts. Once the patient developed sedation to the extent he stopped conversing, the procedure was terminated. The patient narrated later his subjective experience of trance-like state during the administration of the drug as below:\n“I was picked up by a flying chariot which travelled at a rocket speed and landed on what seemed to be like heaven. There was heavenly peace and pleasant sight all around. Surprisingly everybody were dressed alike. I eagerly looked for my wife amongst many people offering prayers to Lord Shiva. I noticed in some time my wife standing at a shouting distance offering prayers to the Lord who wore snake around his neck. I was overwhelmed seeing my wife. I could not control the emotions and I approached her and interrupted her prayers to speak to her. In return she said she no longer belong to me. Instead she belonged to that heaven. She went on to say that she had no husband and there are no ‘earthly’ relationships there in that world. She even suggested me to go back to earth as my time was not up yet. I felt relieved that finally I could meet my wife for one last time and realized I still had responsibilities of taking care of my children. The same chariot which brought me there came back to earth to drop me down”.\nFollowing the administration of the drug ketamine, the patient had brief period of giddiness which subsided gradually. Within few minutes patient was communicative, cheerful and started taking food orally. He later shared his unique phenomenological experience with many of his family members and other inmates. The antidepressants were continued while the lorazepam was tapered and stopped. The patient insisted on one more injection of the similar kind but was not given explaining him the addiction potential of the medication. He was maintaining well even after 3 months of the treatment during the regular follow-ups. |
An 81-year-old Caucasian female underwent a CTC for colorectal cancer screening. She tested positive to a faecal occult blood stool test, and had experienced longstanding, infrequent, minimal and painless bright red rectal bleeding for a period of over twenty years. She gave no history of loss of weight, change in bowel habits, or family history of colorectal cancer. Past medical history was significant for atrial fibrillation requiring anticoagulation, diverticular disease and a hysterectomy 20 years prior with subsequent radiotherapy to the pelvis as histology had confirmed uterine cancer. In the last twenty years, four screening colonoscopies had been carried out, the last five years prior, and whilst clear of polyps, was significant for an asymptomatic narrowed segment of distal sigmoid which had to be traversed with a paediatric colonoscope. The patient underwent a CTC as, she had, earlier in the year, undergone a failed optical colonoscopy. CTC was carried out with manual air insufflation via a rectal soft tip Foley catheter until the patient felt slight discomfort, at which point a scout AP film was taken to ensure adequate bowel distension. It proceeded without complications. The official report noted a localized contained perforation demonstrated around the rectum (\n,\n). There was extensive diverticular disease demonstrated throughout the sigmoid colon, which was markedly narrowed throughout in keeping with stricture formation, which would be consistent with previous diverticulitis or radiation treatment (\n). Further assessment of that region was difficult. The remainder of the large bowel had achieved excellent distension.\nThe patient was admitted to the ward and commenced on prophylactic antibiotics (Tazocin 4.5g tds), and bowel rest. She complained of mild discomfort in the lower abdominal region, and had a soft abdomen with no evidence of peritonism. During the first two days of admission, she passed a small amount of blood clot per rectum. She was discharged on day five of admission, with no abdominal signs, having undergone an MRI which confirmed no peri-rectal collection and identified no obvious perforation. A further optical colonoscopy was attempted two months later, which failed to enter the sigmoid colon, citing severe diverticular disease as the reason. The patient currently remains asymptomatic. |
A 45-year-old male presented to our hospital with diaphoresis, chills, and worsening right flank pain. He had a past medical history of cirrhosis due to alcoholism and portal hypertension with esophageal varices managed with prior transjugular intrahepatic portosystemic shunt (TIPS) procedure. He had a history of hepatic encephalopathy, hypertension, and type II diabetes. He had a recent admission to another institution for septic shock secondary to recurrent cholecystitis and had recently had an LC fifty-eight days prior. The patient was admitted to the outside hospital fifty-nine days prior to admission to our institution for subacute cholecystitis, which had initially required a cholecystostomy drain on prior admissions, and then ultimately an LC at that hospital. The gallbladder was not noted to be perforated, but the procedure was complicated by dropped gallstones, some of which were retrieved as stated in the operative report. On the current admission, the patient was hypotensive requiring vasopressors and was anemic and thrombocytopenic, requiring blood and platelet transfusions.\nA CT scan of the abdomen and pelvis with IV contrast was performed on admission demonstrating residual gallstones in the gallbladder fossa and/or cystic duct remnant and multiple small fluid collections and/or forming granulomatous masses surrounding additional retained/dropped gallstones in the surgical tracts and vicinity. A fistula with gallstones was also seen extending through the posterior and inferior wall of the second portion of the duodenum, to the anterior and superior aspect of a right renal cyst, which measured 3.4 cm x 3.0 cm and which contained gas in its superior aspect (Figures and ). A previous CT of the abdomen and pelvis performed with IV contrast sixty-five days prior to admission and seven days prior to the LC demonstrated a simple right renal cyst in the location of the now complex and infected cyst, measuring 2.8 cm x 2.5 cm ().\nDrainage of the infected renal cyst seen on the initial CT was considered; however, the cyst was relatively small and inaccessible. A repeat CT of the abdomen and pelvis with oral and IV contrast was performed four days after admission due to concern for abscess formation as the patient continued to have right flank pain. The CT demonstrated an unchanged superinfected cyst in the right kidney, with the fistula tract still visible, and heterogeneous retention of IV contrast in the right kidney, which was consistent with associated pyelonephritis (Figures and ).\nOn initial presentation, the patient was noted to have a history of Klebsiella pneumoniae and vancomycin-resistant enterococcus (VRE) in the cholecystectomy drain and was treated for the gallstone abscess and fistula accordingly with meropenem as there was no other source of infection. Blood cultures were later positive for K. pneumoniae and VRE. The patient was ultimately treated with linezolid and meropenem was deescalated to ceftriaxone.\nDiscussion between the patient's outside hepatologist, the abdominal radiologist, the interventional radiologist, and the gastroenterologists determined that the most likely etiology of the initial sepsis was an infected renal cyst secondary to an infected dropped gallstone. The patient was ultimately transferred to the outside hospital where he previously had his cholecystectomy for surgical follow-up. |
A 65-year-old Japanese woman, who was known to be infected with hepatitis C virus (HCV), underwent rectal resection for advanced rectal cancer, which represented serosal invasion and lymph node metastasis. In spite of HCV infection, the results of her liver function test were normal and she did not hope to receive antiviral therapy because of socioeconomic reasons. At the age of 68, she developed two CRLMs located in segments 4 and 6. She received neoadjuvant chemotherapy, including oxaliplatin, leucovorin, and fluorouracil, and subsequently underwent surgery composed of microwave tissue coagulator (MCT) ablation for segment 4 tumor and segmentectomy 6. The segment 4 tumor was considerably small with a diameter of 1 cm and it was exposed to the liver surface; thus, we performed MCT ablation. A histological examination of the resected S6 tumor revealed adenocarcinoma and was confirmed to be CRLM but not intrahepatic cholangiocarcinoma with negative cytokeratin 7 and positive cytokeratin 20 in immunohistochemistry. Although she was diagnosed histologically as having a cirrhotic liver, by a microscopic examination for background non-tumoral liver, antiviral treatment was again not conducted due to socioeconomic reasons at that time. At the age of 71, abdominal computed tomography (CT) showed a tumor that was adjacent to the previously ablated lesion in segment 4 of her liver (Fig. ). The tumor was irregularly shaped and in contact with her middle hepatic vein (MHV). Based on a three-phase CT study, the tumor showed neither early enhancement nor washout and was less enhanced compared to non-tumoral background liver through all phases. Furthermore, no other tumor was detected in any phases of the three-phase study. Based on these findings and her previous history of rectal cancer liver metastases, we considered the segment 4 tumor to be a recurrent liver metastasis originating from rectal cancer. Because her liver function was well preserved and corresponded to Child-Pugh A (5 points), surgery was undertaken. During the surgery, a small tumor was identified in segment 8, which could not be diagnosed preoperatively. Taking her history into account, we considered that the segment 8 tumor was also a recurrent CRLM. The segment 4 tumor was broadly in contact with her MHV and less distinguishable from the lesion previously treated by MCT. Thus we performed segmentectomy 4 including MHV for the segment 4 tumor. With regard to the segment 8 tumor, it was exposed to the liver surface and well recognizable. Hence, we considered applying MCT ablation to the segment 8 tumor. However, the segment 4 tumor, which was resected at that time, might have been a local recurrence of the segment 4 tumor previously treated by MCT ablation. In other words, MCT ablation might not have been effective enough or might have induced tumor implantation into her liver even if the tumor was small and exposed to the liver surface. Thus we performed partial hepatectomy for segment 8 tumor; methods of hepatectomy were described according to the Brisbane 2000 system of nomenclature of liver anatomy and resections [].\nHistological findings showed a moderately differentiated adenocarcinoma in the segment 4 tumor, confirming that the segment 4 tumor was a recurrent rectal cancer liver metastasis as diagnosed preoperatively (Fig. ). The diagnosis of recurrent CRLM was corroborated by the immunohistochemistry that showed negative for cytokeratin 7 and positive for cytokeratin 20, indicating that the segment 4 tumor was CRLM but not intrahepatic cholangiocarcinoma that often develops in cirrhotic liver. However, histology of the segment 8 tumor showed findings of well-differentiated HCC (Fig. ). According to the Metavir system [], we determined the inflammation and fibrosis status of the non-tumoral background liver to be A1F4 (Fig. ). As to the HCC, it was classified as early stage (A) of the Barcelona Clinic Liver Cancer staging system because the tumor was solitary and 1 cm in size, the liver function of the patient corresponded to Child-Pugh A, and performance status corresponded to 0 [].\nAlthough she had a cut surface abscess postoperatively, she was discharged from hospital 21 days after the surgery and is currently doing well 18 months after the second hepatectomy (Fig. ). She is currently receiving interferon and ribavirin therapy to eliminate HCV (Fig. ). |
This 36-year-old Indian male presented to Sawai Man Singh Hospital Emergency Room with the chief complaint of hemoptysis, having coughed up approximately 500 mL of bright red blood in the previous 12 hours. The patient was an active smoker, with a smoking habit of 45 pack-year. The patient had history of pulmonary tuberculosis 5 years back for which he had taken complete treatment. During these 5 years, the patient had few episodes of hemoptysis amongst which 2 were major episodes, for which he was admitted to the hospital and bronchial artery embolization was done for 2 times, the first one 2 years back and the second one 6 months back from this admission. On presentation, the patient had hypoxemia, tachycardia, and low blood pressure. The patient had pallor and anemia. He was admitted to the intensive care unit for close monitoring and treatment. The patient received blood transfusions because of a rapid fall in hemoglobin levels (the patient had a hemoglobin level of 12.5% on admission, which had dropped to 9.6% in a single day) and severe hemodynamic instability. Chest X-ray showed multiple cavitary lesions of left upper lobe, with fibrosis of mediastinum causing deviation of trachea to left side and tenting of left lobe of diaphragm (). The CT scan of patient showed multiple cavitary lesions in left lung predominantly in upper lobe, with hemorrhagic debris in the cavities. Bronchiectatic changes were also noted in right lung but to a milder level of affection (). He underwent emergency bronchoscopy to identify the site of bleeding. The bronchoscopy revealed that he bled massively from the left tracheobronchial tree. Since a definite lateralization of the bleeding source had been established, a right sided double lumen endotracheal tube was inserted to protect the right lung. The patient was nursed in the intensive care unit. Bronchial lavage cytology was negative for malignancy. The very next day the patient again bled profusely from the left lung, and at this point surgical management was deemed necessary because the exact bleeding side had been identified and the patient was in severe hemodynamic compromise. The patient was taken for emergency surgical resection of the upper lobe of left lung, and one large bronchial artery was identified and ligated (), but, due to dense difficult adhesions all over the parietal surface of left lung, decision of pneumonectomy was made as the time to lyse the adhesions and the generalized oozing that would occur would have compromised the patient more. The stump of the bronchus was not reinforced in this case as there were strong adhesions and surrounding tissue was edematous, and there was urgency to complete the surgery due to hemodynamic instability. After resection, the left lung was cut open for inspection that revealed multiple cavities in left lung in upper lobe that extended up to the lingular lobe and also some area of destroyed lung was found in left lower lobe (). Histopathological examination confirmed the bronchiectatic changes and culture for tuberculosis bacteria was positive. |
A 16-year-old female patient presented to the radiotherapy department in July 2008 with complaint of unrelenting hoarseness of voice for the last 1 year. She developed it gradually over a period of time. Initial treatment was directed to common cold but was of no help. There was no associated sore throat, otalgia, localized pain or tenderness over thyroid cartilage, dysphagia or features of airway obstruction. The patient is a nonsmoker and is nonalcoholic and without any history of past or present marijuana smoking. There was no history of prior neck irradiation. An incidence of cancer in the family could not be elicited from a detailed history. An indirect laryngoscopy was performed, which showed both vocal cords to be mobile. There was no visible growth over any of the vocal cords. Contrast-enhanced CT scan of the neck was performed, and it showed a small irregular lesion over the left vocal cord at the junction of anterior one third and posterior two thirds []. Thickening of left true vocal cord with loss of paraglottic fat is seen, suggestive of a neoplastic lesion. The normal paraglottic fat on the right side is seen as black area just deep to the thyroid cartilage (seen as white). The airway is seen as black oval structure. There is no visible neck node. Fiber-optic laryngoscopy confirmed the CT scan finding, which also showed irregular lesions along the medial margin of the left vocal cord []. Both CT scan and fiber-optic laryngoscopy showed an otherwise normal picture. Microlaryngoscopic removal of the mass was performed. Histopathological examination under low-power and high-power fields showed severe dysplasia and full-thickness replacement of epithelium with dysplastic cells and diagnosed it as carcinoma in situ [Figures and ]. However, the risk of a micro-invasive carcinoma could not be ruled out. A second biopsy was advised to obtain deeper tissue, but it was refused by the patient. So from the available documents, we considered it carcinoma in situ of the vocal cord.\nA detailed physical examination was performed, which was unremarkable. There was no palpable lymph node in neck and other sites. Complete hemogram, liver function test, chest X-ray, renal function test and ultrasonography of the whole abdomen were performed as part of routine investigations before commencing therapy, all of which were unremarkable. The stage of the disease was Tis N0 M0.\nAfter reaching at a specific diagnosis and doing all pretreatment workup, specific anticancer therapy was started with radical radiotherapy (60 Gy in 30 fractions, 2 Gy per fraction, 5 days a week for 6 weeks) using Telecobalt (Theratron 780 C) machine. Radiotherapy was started with two lateral opposed fields to neck to include the vocal cord (a 5 × 5 cm field) extending from superior border of thyroid cartilage superiorly to lower border of cricoid cartilage inferiorly sparing the spinal cord and other organs at risk. A CT-based treatment planning was done to achieve maximum dose to vocal cord, sparing normal tissue as much as possible. |
A five-year-old boy was brought to our dermatology department for recurrent ulcers in the lower limbs for the past 2 years. He was the only child born to second-degree consanguineous parents. He was born by normal vaginal delivery after an uneventful antenatal period and showed no abnormality at birth. But, during infancy he was repeatedly hospitalized for recurrent unexplained fever from which he used to recover normally. His mother also noticed delay in milestones in his infancy. His parents noted that he did not respond to painful stimuli like injection of vaccines or any other accidental trauma. They also noticed self- mutilating behavior like biting of his tongue, chewing his fingers, bruising, and manipulation the wounds. There was no history of similar illness in the family. On general examination pallor was present. His systemic examination was normal. Cutaneous examination revealed generalized xerosis with areas of lichenification over his ankles and dorsa of feet []. Palms showed thickening while there was fissuring of both the soles. Both the heels showed deep ulcers, measuring 3 × 3 cm on the left and 2.5 × 2 cm on the right side []. There was another ulcer of 4 × 3 cm in size over the right knee. There was scarring of left little finger and right index finger (healed ulcers due to biting) []. Oral cavity showed loss of tip of the tongue, many missing teeth and angular cheilitis []. Sensory examination showed complete loss of pain and temperature sensations, but fine touch and vibration were preserved. Deep tendon reflexes were normal. Lacrimation was normal. There were no hypopigmented patches or thickened, palpable nerves. Complete hemogram revealed iron deficiency anemia. Other hematological and biochemical investigations including serum uric acid levels were normal. An intradermal injection of histamine did not show any flare response. Histological examination of the skin showed normal epidermis and dermal structures including presence of normal sweat glands. Based on the clinical and histological features the diagnosis of CIPA was made. The ulcers were treated with appropriate antibiotics and daily dressings. The xerosis was managed with emollients. The parents were counseled about appropriate care of the child. |
A 32-year-old lady fell from height and sustained deep laceration wound on the chin compounded with extensively comminuted mandible fracture. The fracture extends to the mandibular angle bilaterally causing severely deranged occlusion. She also sustained a medially displaced left condylar head fracture and an undisplaced left Le Fort II fracture.\nAn open reduction and internal fixation (ORIF) was planned for her comminuted mandible fracture by using a prebent reconstruction plate through the existing wound. ORIF for both left Le Fort II and condylar fracture was also planned. The aim was first to establish the proper vertical dimension and posterior mandibular height prior to the fixation of the comminuted mandible. However, adding to the difficulties in the execution of the surgical plan, our patient refused for both ORIF of the maxilla and condylar fracture, as it requires additional transcutaneous surgical access. She previously experienced complicated emergency procedure for open fracture of the left supracondylar femur and requested fixation only for her mandible fracture. We therefore have to revise the protocol required for her surgery and decided to use the CPD as a measure to avoid rotational movements of proximal segments of the mandible, as these were the only surgical reference left.\nTo begin with, we applied an additive manufacturing (AM) concept to produce an acrylonitrile butadiene styrene (ABS) three-dimensional (3D) life-size surgical model by using her DICOM computed tomography (CT) data (). From the clinical assessment and having the printed model in hand, we could confirm that we were unable to find any intact anatomical references needed for fracture reduction and fixation nor that we possess the preoperative landmark identification or an intraoperative tool by using a navigation system or imaging to check for the exact and adequacy of bony reduction and position.\nDental occlusion as the most important reference was also lost. Clinically, our patient had 30 mm anterior open bite (AOB) with only the last remaining molar in the occlusion. Loss of left posterior facial height was also observed secondary to condylar neck fracture. Hence, we decided to use the only remaining occlusion on the molar teeth and construct the CPD to make sure that these two remaining references will not change during the surgical procedure that could risk the surgical outcome. Without the use of any feasible technique to gain control of the proximal segment, there is a high risk of malocclusion and proximal fragment positional change. This could lead to more surgical complications such as temporomandibular joint (TMJ) pain, TMJ compression, condylar resorption, and the need for reoperative procedure.\nBy using the ABS model, we adapted the Luhr [] technique of CPD bilaterally by using 2.0 rigid fixation plate in the centric condylar position. We then simulated the osteotomy located at the most posterior aspect of the mandibular fracture with both condyles left undisturbed within the glenoid fossae. Next, fracture reduction and correction of occlusion were carried out, followed by waxing-up of the multiple defect areas to allow good bending and adaptation of the reconstruction plate ().\nIn the operating theatre, general anaesthesia was delivered via fibreoptic nasal intubation to minimize manipulation of the mandible. Once under general anaesthesia, vestibular incision was first made on the posterior maxilla and mandible bilaterally for CPD adaptation and fixation. This was the first step undertaken to prevent any unnecessary change in the condylar position before carrying out further surgical procedures ().\nFor access to fracture sites and for reduction and internal fixation, the existing chin laceration wound was used and extended posteriorly reaching the now-stabilized proximal segments. This followed by manipulation and reduction of the comminuted fragments. Fixations were completed with the prebent locking mandibular reconstruction plate. Small fragments that were amenable for fixation were simplified using microplates and screws. The CPD was temporarily relieved, allowing us to examine the final occlusion and jaw motions following completion of fixations. We found that the CPD encouraged appreciable, undisturbed condylar position as the desired occlusion, jaw opening, and excursion were achieved. The CPD was removed, and the incision closed in the usual manner. Postoperative follow-up and orthopantomogram showed good healing with restoration of her preinjury states of occlusion (). Clinically, good mouth opening was achieved with no deviation during mouth opening. |
The first patient is a 58-year-old man who was a previous smoker who presented to an outside hospital with abdominal pain and was diagnosed with gallstone pancreatitis. However, despite having a laparoscopic cholecystectomy, he continued to have episodes of pancreatitis yearly for the next 3 years. Computed tomography (CT) scan demonstrated a pancreatic duct dilated throughout its course to a maximum size of 8 mm without a discrete pancreatic mass in the head of the pancreas. Endoscopic retrograde cholangiopancreatography confirmed a dilated pancreatic duct with a suspected filling defect of the common bile duct, after which the patient underwent sphincterotomy and stent placement.\nHe then presented to our institution for consultation. Further workup with endoscopic ultrasound (EUS) was performed, which also confirmed the dilated pancreatic duct, but noted irregular contour of the duct. In addition, free mucin was seen exiting the main papilla, and there was a papillary growth noted in the pancreatic duct in the head of the pancreas, all of which was consistent with a main duct IPMN (). Preoperative laboratory values, including CA19-9, were within normal limits. Of note, it was unclear if the dilatation of the pancreatic duct was secondary to obstruction from the mass in the pancreatic head or due to main duct IPMN involving the entirety of the duct.\nHe was scheduled for surgical resection after the stated workup. He was planned for a Whipple procedure (pancreaticoduodenectomy) with possible total pancreatectomy if intraoperatively the main duct was found to be involved by IPMN with high-grade dysplasia. Transection of the pancreatic neck identified cells of high-grade dysplasia free floating near the margin (although exclusive of the margin). In addition, upon probing the pancreatic duct in the tail of the pancreas, the probe did not slide smoothly, suggestive of additional growths in the duct. Due to his young age and high risk of developing pancreatic cancer, a completion pancreatectomy and splenectomy were performed. He was reconstructed in a Roux-en-Y manner. He recovered well from surgery without any postoperative complications.\nHis final pathology demonstrated a 1.4 cm moderately differentiated colloid carcinoma arising within main duct IPMN (intestinal type) with high-grade dysplasia in addition to multiple foci of pancreatic intraepithelial neoplasia. Staining of the tumor was strongly positive for MUC2 and weakly positive for MUC1 and MUC5. The tumor involved the peripancreatic soft tissue, but all margins were negative. 0 of 39 lymph nodes was involved with tumor. His final pathologic staging was pT3N0Mx. He was, therefore, referred for medical oncology consultation. He was recommended for adjuvant chemoradiation with gemcitabine before and after fluorouracil-based chemoradiation, which he is currently receiving and tolerating well. |
A 10-year-old boy was referred to a local hospital with hoarseness for 1 year in July 2009. Clinicians saw a tumor of the vocal cord, which subsequently was resected by using direct microlaryngoscopy with a carbon dioxide (CO2) laser. Respiratory papilloma was diagnosed after pathologic analysis. After the procedure, the symptom of hoarseness actually worsened and the patient also gradually developed a foreign body sensation. He then presented to our hospital in May 2010. Fibrolaryngoscopy demonstrated exophytic masses involving the whole length of the right vocal cord and extending to the anterior commissure and anterior third of the left vocal cord (Figure ). The cords were mobile, and no cervical lymphadenopathy was appreciated on palpation. In addition, there was no recent history of fever or weight loss, and laboratory findings were normal. The tumor was resected by using direct microlaryngoscopy with a CO2 laser, and histological examination was performed (see below).\nSix months after the second operation, the patient was referred again for hoarseness in addition to shortness of breath. Via laryngoscopy, similar exophytic masses were observed at the same location, and these masses were then resected. However, the lesion returned 5 months later and was removed again, only to reappear a fourth time after 2 months. The tumor from the fourth operation showed a proliferation of spindle cells in the subepithelial stroma. A diagnosis of IMT was made after reviewing the histopathology, combined with the results of immunohistochemical staining and testing for human papillomavirus (HPV) and ALK. During a clinical and pathological discussion, it was proposed that incomplete resection was the cause of tumor relapse. A fifth operation was performed, and frozen sections of the surgical margins sent at the time of the procedure were all negative for IMT. The child’s voice improved after 3 months, and the tumor failed to recur during 2 years of regular follow-up. This report was approved by the hospital ethics committee, and written informed consent for publication of the clinical details and clinical image was obtained from the patient’s guardian.\nAll histologic sections from the five operations were reviewed. Because the primary lesion and the recurrences had similar histological and immunohistochemical features, they are described together. Microscopically, the lesions displayed an exophytic, polypoid, and papillary appearance under low power (Figure A) with a hyperplastic squamous epithelial lining. In this squamous lining, there appeared to be koilocyte-like cells. The subepithelium consisted of fibrovascular cores. Most of the cores were large and compact with obvious hyperplasia of spindle cells (Figure B), unlike the fibrovascular cores of squamous papillomas, which are thin and loose. The hyperplasia of stromal cells was more obvious in the last two specimens because the sections from the first three operations were relatively superficial. The spindle cells were arranged in both a fascicular and storiform pattern with lymphocytes and plasma cells in the background (Figure C). The spindle cells contained pale, eosinophilic cytoplasm and nuclei that were elongated and slightly pleomorphic with one or more small nucleoli (Figure D). Occasional regular mitoses (about 0.8 per 10 high-powered fields) and some ganglion-like cells could be seen, and necrosis was absent. Immunohistochemically, the squamous epithelium was negative for P16. The spindle cells were positive for vimentin, smooth muscle actin, and ALK-1 (Figures A-C) but negative for desmin, muscle-specific action, S-100, Bcl-2, CD34, cytokeratin, and p53. The Ki-67 proliferation index was about 3%.\nWe applied in situ hybridization as well as polymerase chain reaction analysis to test for the HPV genotype. The two analyses included HPV types 6, 11, 16, 18, 31, 33, 35, 39, 42, 43, 44, 45, 51, 52, 53, 58, 59, 66, 68, and CP8304. The viral tests did not demonstrate any of the above-mentioned HPV types. We performed FISH with Vysis LSI ALK Dual-Color, Break-Apart Rearrangement Probe (Abbott Molecular, Abbott Park, IL, USA) on the spindle cells and found translocations involving the ALK locus (Figure D). On the basis of the histopathological and immunohistochemical features and the result of the FISH test, a diagnosis of IMT was rendered. |
A 26-year-old African American female not known to have ever been pregnant arrived to the emergency department (ED) via ambulance complaining of a one-day history of progressively worsening vaginal bleeding and crampy lower abdominal pain. Prior to her arrival to the ED, the patient stated that she began passing clots, and while sitting down on the toilet, she attempted to pull out a thick vaginal blood clot. Instead, she reportedly grabbed hold of a pair of legs and pulled out a fetus that was missing its head. She dropped the body of the fetus into the toilet and called EMS, who brought her and a decapitated fetus to the hospital ().\nThe last menstrual period was estimated to be at 2 months prior to the date of presentation. The patient reported taking a home pregnancy test about 1 to 2 weeks before her ED visit that was negative. She had a medical history significant for chronic myeloid leukemia, for which she reports taking Tasigna (nilotinib), a tyrosine kinase inhibitor which is FDA pregnancy category D (positive evidence of risk). Her surgical history is significant for ovarian cystectomy and left oophorectomy.\nOn examination, the patient appeared well and in no distress. Her vitals were stable and physical exam was unremarkable except for mild suprapubic tenderness to palpation. A transvaginal ultrasound was performed, revealing no intrauterine gestation and a markedly thickened endometrium of up to 3.6 cm. Questionable hyperemia of the anterior myometrium and a probable corpus luteal cyst in the right ovary were also noted. A pelvic exam showed a moderate amount of blood in the vaginal vault, along with the fetal head located at the external cervical os. The fetal head was removed using ring forceps, after which the patient was transferred from the ED to the operating room for dilation and curettage and had an uneventful postoperative course.\nThe pathology report on the fetus reports a 182-gram phenotypic male with the intestinal organs outside of the abdomen cavity through an omphalocele. There was mild ecchymosis, a laceration in the right groin, and the head was disconnected from the body. |
Case 2 was a 59-year-old male physician who had spent nearly 30 years as a member of a group medical practice. He had suffered a head injury while skiing (wearing a helmet) approximately 10 months before the evaluation. Specifically, he fell off a ski lift with head impact on concrete. He later had a fall on the slopes. He denied loss of consciousness (his family noted transient loss of awareness), but reported persistent headache for a few weeks following the head injury. He then began to experience intermittent numbness of his right ear and numbness and weakness in his right hand, for which he sought neurological evaluation approximately 3 months post injury near his residence in another part of the country. MRI revealed recent and chronic bleeding over the left hemispheric convexity, and he underwent a craniotomy to evacuate a left frontal subdural hematoma. The site of the injury was right occipital, thus the subdural hematoma appeared to be a result of a contrecoup injury. Following craniotomy, his headaches persisted as did the intermittent numbness and weakness in his right hand, for which he was begun on levetiracetam.\nDetailed history revealed that for many months before the accident his family had noted a change in his personality, episodic increased agitation and altered cognition (word retrieval, short-term memory). They reported that the patient had difficulty recalling recent events but that recall of childhood information remained generally intact. He forgot recent conversations and began having difficulty recognizing the faces of acquaintances, patients and old friends, as well as pictures of famous people. He struggled with abstract reasoning (for example, how people and things related to one another) and had trouble following directions. He would ask the same questions repeatedly and told the same stories multiple times without recalling he had done so. He was unable to maintain attention and concentration when a story became increasingly complicated, and would become agitated and irritable as a result of not being able to follow the story line. When confronted about his cognitive difficulties he would become angry and agitated, and would ‘act out' in the presence of his family. According to the family, the patient would experience ‘manic highs and depressed lows'. When in a good mood, he enjoyed being with others and was largely socially appropriate. His mood could change rapidly, and he would become withdrawn or belligerent. When in a depressed state he expressed suicidal ideation. According to the family, the patient was less emotionally available for things that had been important to him (for example, family relationships). For months before his fall, he had become apathetic and lost interest in hobbies that he previously enjoyed. Approximately 4 years ago (3 years before the fall), the patient had experienced a depressive episode. He had attributed his depression to work-related stress and was treated with sertraline. He had no history of psychiatric hospitalization or psychotherapy. At the time of his evaluation at Mount Sinai Medical Center he was not taking antidepressants.\nThe patient was referred for a comprehensive neuropsychological assessment (JM) and neurological evaluation (SG, AA) at the Mount Sinai Alzheimer's Disease Research Center, as well as appropriate laboratory studies. CT and MRI revealed atrophy of the anterior poles of both temporal and frontal lobes (). Molecular imaging included both [18F]-fluorodeoxyglucose (FDG) PET and [18F]-Florbetapir scans. At the time of the evaluation, his medications were atorvastatin 20 mg daily and acetaminophen PRN. He had had a previous neuropsychological evaluation at another facility, 3 months before our assessment. The findings on the prior examination were consistent with the current evaluation. |
A 48-year-old female patient presented to the emergency department (ED) after CPR due to VF that occurred after a seizure. Four years prior, she presented to other hospital ED and discharged after observation for a condition where her arms were raised and her limbs were stretched continually for approximately 1 min while sleeping after an episode of alcohol intake. She did not undergo any particular tests and neither was she administered any drugs, under the assumption that these symptoms were temporary and had been caused by alcohol intake. Three years prior, a similar episode occurred during sleep, for which some medical tests were conducted such as electrocardiogram (ECG), brain MRI, and electroencephalogram (EEG), but no conclusive results were reported. Drugs were also prescribed but not taken by the patient. One and a half year prior, the patient experienced another relapse again during sleep at dawn. According to the statement given by her husband who witnessed the episode at the time, for approximately 1 min while sleeping, the patient groaned with her mouth shut tightly, her arms raised, limbs stretched, and did not respond when called. Subsequently, her body collapsed, she stopped breathing for approximately 30–40 s, and then she breathed in a concentrated manner. Urination and post-ictal confusion were also noted. The patient had no family history of epilepsy or no medical history of febrile convulsion in the past, and only reported of the inability to sleep well and waking up frequently due to a burning sensation in the foot. In the previous hospital, the patient underwent video-EEG and polysomnography in an epilepsy monitoring unit (EMU) for two days to investigate the cause under the putative diagnosis of restless legs syndrome and nocturnal bilateral tonic seizure – frontal lobe epilepsy. Based on EEG findings, there was no inter-ictal epileptiform discharge and an intermittent slow wave was observed in the right frontotemporal area, but it was equivocal, and therefore, no clear abnormality was reported. Subsequently, administration of pramipexole and pregabalin was started to control the rare nocturnal seizures and symptoms of the restless legs syndrome under the putative diagnosis of restless legs syndrome and unknown etiologic epilepsy – bilateral tonic seizure.\nOn the day before current admission, her epileptic seizure relapsed during sleep but she recovered consciousness immediately; thus, she did not come to the hospital. In the evening of the admission day, while conversing with her children, she suddenly had a general tonic-clonic type seizure for 2–3 mins and emergency medical services (EMS) were informed as she did not recover consciousness immediately after the seizure like in previous episodes and her breathing was almost negligible. Following the instructions of the EMS center, her family started CPR. After the paramedics arrived, spontaneous circulation was restored after CPR and 2 times of defibrillation as VF was indicated by the AED findings (). At the time of ED arrival, the patient was stupor and her vital signs and oxygen saturation were normal with a blood pressure of 121/82 mmHg, pulse rate of 80 beats per minute, respiratory rate of 16 breathes per minute, and body temperature of 36.2°C. ECG performed immediately after arrival showed a sinus rhythm with a heart rate of 78 beats per minute and QTc duration of 401 ms (). The initial arterial blood gas analysis (ABGA) showed a pH of 7.35, PCO2 21 mmHg, PO2 134 mmHg, HCO3 11.6 mEq/L, and a lactate level of 6.1 mg/dL. Approximately 10 min after arrival, she regained consciousness but Todd’s paralysis in the right limbs and global aphasia were shown. The repeat ABGA showed that all values had recovered to normal levels. All other blood test results were normal, including electrolyte and myocardial enzyme levels. Levetiracetam was administered intravenously in the ED. Brain diffusion MRI showed no abnormality. Thereafter, the patient was admitted to the cardiology intensive care unit (ICU) and received a full cardiologic work-up. After admission to the ICU, her consciousness was maintained in an alert state, the paralysis in the right limbs and aphasia resolved completely, and the EEG showed normal results. Transthoracic echocardiography performed on the day after admission showed that the left ventricle ejection fraction (LVEF) was reduced to 44%, and left ventricle global hypokinesia and moderate systolic dysfunction were observed. After that, consecutively performed coronary angiography (CAG), spasm provocation test, cardiac MRI, and 24-hr Holter monitoring showed normal results. For the purpose of preventing sudden cardiac death (SCD), the insertion of an implantable cardiac defibrillator (ICD) was recommended. The patient was discharged without any complications 6 days after the procedure. Currently, levetiracetam administration is being maintained by the neurology department and her condition is being periodically followed by the cardiology department under the diagnosis of aborted SCD due to VF and heart failure with mid-range ejection fraction. |
A 61-year-old female with past medical history significant for type 2 diabetes mellitus, hypertension, and dyslipidemia and family history of breast cancer in her brother and maternal aunt presented to the emergency room for recurrent chest pain. She also reported fatigue for the past few months and was being evaluated for worsening anemia by her primary care physician. Her hemoglobin was 6 gm/dl with iron panel consistent with anemia of chronic disease. Her electrocardiogram (ECG) at arrival showed an “RSR pattern” in V1 and V2 leads but was otherwise normal. She underwent a stress myocardial perfusion imaging (MPI) which was negative for cardiac ischemia. A transthoracic echocardiogram revealed a 1.6 cm × 1.5 cm atrial mass attached to the anterior wall of the left atrium, which appeared to cross the mitral valve in ventricular diastole. The left atrial mass was further characterized by a transesophageal echocardiogram (TEE) as a solid, irregularly shaped, partly mobile mass attached to the atrial septum and extending to the anterior mitral valve leaflet (). The segment attached to the septum measured 2.6 cm × 1.43 cm, and the segment attached to the valve measured 1.4 cm × 2.22 cm. In addition, MRI of the heart with gadolinium was done preoperatively which confirmed the circumscribed hypodense mass with speckled appearance which originated at the atrial septum and extended along the anterior mitral valve leaflet (). The mitral valve flow was normal with no evidence of obstruction, stenosis, or regurgitation. The patient underwent a minimal incision valve surgery for resection of the mass which was presumed to be myxoma due to its location. Intraoperatively, on open examination of the left atrium, it was noted that the mass originated from the fossa ovalis region of the interatrial septum and infiltrated the atrial wall down onto the entire anterior leaflet of the mitral valve. A fibrotic density that surrounded the tumor was also noted. The mass along with a portion of the interatrial septum and the mitral valve was resected. The mitral valve was replaced using a 27 mm Hancock II bioprosthetic valve. The atrial septal defect caused by the resection was repaired with a bovine pericardial patch. A postoperative TEE was performed which confirmed the successful placement of the bioprosthetic valve with no paravalvular leak. The patient had an otherwise unremarkable postoperative recovery and was discharged home after fourteen days of hospital stay.\nTwo weeks following discharge, the patient presented to the emergency with cough, diaphoresis, and palpitations. She was noted to have jugular venous distention and bibasilar crackles on auscultation of the lungs and was found to be in acute heart failure. An urgent transesophageal echocardiography demonstrated severe mitral regurgitation with paravalvular leak (Figures , , , and ). At the same time, the histopathologic examination of the atrial mass showed a high-grade sarcoma consistent with dedifferentiated liposarcoma. The tissue exhibited spindle cells with pleomorphism, multinucleated giant cells, and inflammatory cells. Immunohistochemical stains demonstrated that the neoplastic cells were positive for vimentin, focally positive for S-100, and weakly positive for CDK4 and negative for p53 (). FISH studies performed showed an MDM-2 gene amplification in 95–200 nuclei examined. The patient was aggressively treated with intravenous diuretics and afterload reduction using furosemide and nicardipine infusion. Blood cultures were obtained with suspicion of postsurgical infective endocarditis causing valvular dehiscence. However, cultures did not grow any bacteria. The patient clinically deteriorated due to new-onset atrial fibrillation and worsening heart failure despite medical treatment in CCU. CT chest obtained showed a 1.3 cm lytic iliac bone lesion and 3.1 cm × 2.5 cm right upper mediastinal soft tissue density. Due to high suspicion of metastatic disease, MVR and cardiac transplant were not offered until further evaluation for metastasis. Unfortunately, due to rapid clinical decline with a new diagnosis of high-grade cardiac tumor with possible metastases, the patient opted for hospice care. PET study was not obtained. |
A previously healthy 20-year-old female presented to the clinic with a 2-month history of an enlarging submental neck mass. She originally noticed the mass while pregnant. Four weeks after giving birth, however, the area became increasingly more painful and firm to palpation. She began to have pain with swallowing. Physical examination revealed submental and floor of mouth fullness that was exquisitely tender to palpation, limiting the ability to examine her.\nA neck ultrasound and computed tomography (CT) scan were obtained and showed a multiloculated cystic lesion with calcifications and a central solid component present in the submental space extending down towards the hyoid (). There was no radiologic or clinical evidence of thyroid gland disease or neck lymphadenopathy.\nA clinical fine-needle aspiration (FNA) was performed to further evaluate the mass and showed mixed inflammatory cells with histiocytes in a background of proteinaceous fluid, consistent with a cyst. Thyroglobulin was significantly elevated within the cystic contents, raising suspicion for malignancy. A repeat FNA with ultrasound guidance was then performed of the solid component of the mass and cytologic preparations demonstrated nuclear overlapping and nuclear grooves with pseudonuclear inclusions, consistent with papillary thyroid carcinoma.\nThe patient underwent a Sistrunk procedure along with total thyroidectomy. A total thyroidectomy was performed due to the large size of the TGDC carcinoma, the patient's young age, and the likely need for postoperative radioactive iodine therapy. There was no radiographic evidence of lymphadenopathy, and therefore, no neck dissection was planned; however, given the size and location of the mass, contents of level IA and the superior part of level VI were taken with the specimen. A formal level VI neck dissection was not performed due to the negative preoperative imaging.\nIntraoperative findings included a large cystic mass consistent with a TGDC and a small palpable nodule within the thyroid gland (). The final surgical pathology was consistent with a pathologic T3aN1a papillary thyroid carcinoma. There was a 6 cm focus of papillary thyroid carcinoma within the TGDC, metastatic papillary thyroid carcinoma in three of six regional nodes with the largest node being 0.2 cm in diameter, and a 0.6 cm focus of papillary thyroid carcinoma within the thyroid gland itself. Her postoperative course was uneventful. She is currently being considered for postoperative radioactive iodine ablation. |
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