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A 29 years old pregnant patient with singleton pregnancy at 38th gestational week (GW) was admitted to our obstetric emergency room for membrane rupture. She referred to be affected by a giant, congenital AVM of the left chest wall, extending from the third to the seventh thoracic interspace, involving the overlying thoracic muscles and the correspondent thoracic nerves, the rib cage, the parietal pleura, the transverse process of seventh thoracic vertebra and the scapular girdle, with severe neuropathic pain and reduced mobility in her left arm. She underwent to multiple embolizations to treat the lesion, with no or mild improvement of her pain despite she was taking 60 mg Oral Morphine Equivalents (OME) per day and pregabalin 300 mg twice daily. To treat her medically refractory neuropathic pain she was therefore referred for SCS system implant. A Model SC-1200 Precision™ Montage™ Magnetic Resonance Imaging (Boston Scientific) was successfully implanted, with electrodes at thoracic level and Implantable Pulse Generator (IPG) in the left buttock. The procedure led to a dramatic improvement of the symptoms and withdrawal of opioids and antiepileptic drugs in 8 weeks. One year later she obtained a spontaneous pregnancy. During pregnancy her pain control improved, leading to SCS deactivation through the period. Despite the absence of stimulation she reported her neuropathic pain “go into remission”. So she needed no other medications. Her pregnancy progressed normally until 38th GW. At admission just a pre-SCS implant Magnetic Resonance Imaging (MRI) was available (Fig. , axial view; Fig. , coronal view) thus making unpredictable the location of her SCS leads, extensions, and IPG. An urgent cesarean section was planned because of the membrane rupture and the risk of severe bleeding due to a possible AVM rupture in case of vaginal birth, due to the increasing of thoracic pressure during pushing (urgent CS type 3 according to NICE classification) []. To rule out a possible lesion to SCS system and to identify a safe lumbar interspace, a US assisted spinal anesthesia has been performed. No drugs have been administered before surgery. During anesthesiologic and surgical procedure standard hemodynamic monitoring has been provided for pregnant [Continuous Electrocardiographic monitoring (ECG), Pulse oximetry (SpO2), Non-invasive Blood Pressure (NIBP)]; fetal wellbeing has been registered by cardiotocographic monitoring. Supplemental oxygen has been provided by Venturi mask. In sitting position, after skin disinfection with surgical solution (ChloraPrep®, Carefusion, 244 LTD, UK) and using a broadband (5–8 MHz) convex probe, a left US paramedian sagittal oblique view has been obtained, starting at the sacrum and moving cephalad, to identify the L4-L5 lumbar interspaces (Fig. , paramedian sagittal oblique view). To identify the neuraxial midline, after rotating the probe 90° into a transverse orientation, a transverse interlaminar view has been obtained (Fig. , transverse interlaminar view). No electrodes have been reported in that interspace. After local anesthesia with lidocaine 2% (5 mL) a 25-gauge Whitacre spinal needle has been used to perform a spinal anesthesia using hyperbaric bupivacaine 0.5% 10 mg plus sufentanil 5 mcg and morphine 100 mcg intrathecally administered. Surgical procedure started when T4 level has been reached. An uneventful cesarean section has been performed. A healthy 3395-g female baby was born (Apgar scores of 9–10 at both 1 and 5 min). A postoperative thoracic X-Ray confirmed the right placement of thoracic electrodes but with entry point of SCS leads at L2-L3 interspace (Fig. , antero-posterior X ray; Fig. , lateral X ray). At postoperative day 3 patient has been discharged home. |
A 55-year-old woman with a medical history of chronic viral hepatitis presented to our clinic after sudden onset of headache with vomiting. We did not find any neurologic deficit and her mentality was drowsy. Her CT scan showed a diffuse SAH and mild hydrocephalus (). CTA confirmed an upper basilar abnormality (). Cerebral angiography obtained two hours later failed to disclose a vascular abnormality (). The patient received an external ventricular drain (EVD) for symptomatic hydrocephalus on posthemorrhage day 2. The patient's clinical condition improved and her level of consciousness improved to alert immediate after EVD. On the second day after EVD, her mentality suddenly deteriorated to semicoma. Immediate CT scan showed an increased amount of SAH, which suggested rebleeding. A repeat CTA after rebleeding did not show an abnormal vascular lesion. However, cerebral angiography at the same time confirmed a small cylinder like contrast stagnation from the upper basilar artery at late arterial phase, indicating pseudoaneurysm formation (). We inserted two Solitaire stents (4 × 20 mm, ev3, Irvine, CA, USA) for flow diversion and anticipating thrombus formation of the pseudoaneurysm. The patient's clinical course was uneventful, however, one-month follow up CTA still showed a saccular lesion from the upper basilar artery. Therefore, we decided to perform DSA and intervention. DSA still showed contrast stagnation at late arterial phase on the upper basilar artery and superselective microcatheter angiography confirmed a pseudoaneurysm arising from a small perforating artery of the superior cerebellar artery (SCA) (). Due to concern about cerebellar infarction, we could not sacrifice the SCA. To facilitate thrombus formation of the pseudoaneurysm, we performed repeated temporary balloon occlusion using a Sceptor XC balloon (4 × 10 mm, Microvention, Tustin, CA, USA) and achieved decreased contrast inflow to the pseudoaneurysm (). On follow-up at 11 months, the patient had no neurologic deficit, and she was able to live independently. Follow up DSA performed 11 months after hemorrhage confirmed no contrast stagnation and abnormal vascular lesion (). |
A 63-year-old male patient, a teacher, presented with swallowing difficulty lasting for a week and had a history of anterior neck trauma caused by bamboo penetration 6 mo before.\nThe patient had undergone debridement and extraction of a bamboo section in a primary care hospital. The wound healed without auxiliary examination and treatment (Figure ), and the patient did not complain of subsequent discomfort. A month ago, he had abnormal sensations in his neck and heel, but no dysphagia. The laryngoscopy performed at the local county hospital did not show any abnormalities, so no other auxiliary examinations or treatments were performed.\nA week ago, the patient presented with marked dysphagia and could only ingest liquid food. Gastrointestinal (GI) endoscopy revealed a dark-brown foreign body in the lateral wall of the esophagus. In the primary hospitals, attempts to remove the foreign body with a flexible GI endoscope were unsuccessful because the foreign body was embedded in the lateral wall of the esophageal mucosa, which could cause further trauma if the foreign body was forcibly removed.\nOn admission, we performed neck and chest computed tomography (CT) and found that two foreign bodies had penetrated the esophagus from the superior mediastinum. In addition, each layer of the esophagus was penetrated diagonally, and the embedded foreign bodies were adjacent to the left common carotid and left subclavian arteries above the aortic arch (Figure ). The laboratory data were judged as normal after multidisciplinary discussion among the anesthesiologist, gastroenterologist, and thoracic surgeon. Rigid esophagoscopy combined with angle endoscopic surgery was performed under general anesthesia (Figure ).\nA rigid esophagoscope was inserted in the esophagus. However, because the rigid esophagoscope was significantly long, an angle ureteroscope was inserted in the rigid esophagoscope to reveal the foreign body impaction in the lateral wall of the esophagus from the esophageal mucosa 23 cm from the incisor (Figure ). |
A 48-year-old female (P2L2) was referred to our hospital for a CT scan after an ultrasound scan elsewhere, which revealed a cystic mass in the right adnexa with septations and solid components. She initially came to the hospital with complaints of loose watery stools for 10 days and lower abdominal pain for seven days. She was on day 10 of her menstrual cycle. She admitted to having regular cycles with normal flow. She was nondiabetic and normotensive. She had low-grade fever however no chills, no lower urinary tract symptoms or previous episodes of loose stools lasting for over three days. On examination, she had pallor and seemed lethargic, but had no icterus or lymphadenopathy. There was mild tachycardia (105/min) while the rest of her observations were stable. On abdominal examination, slight discomfort in the lower abdomen and pelvis region was noted; however, there was no obvious palpable mass. Ultrasound scan done elsewhere showed possible tubo-ovarian mass/abscess which could be infective or neoplastic. Apart from an elevated Ca-125 level (68U/L), all other blood investigations including thyroid profile came back normal. We initially considered a possibility of pelvic TB, but Mantoux test was negative. CT scan revealed lobulated heterogeneously enhancing complex cystic mass measuring approximately 9.2 x 6.8 x 4.3 cm in the right adnexa extending up to the pelvic brim (Figure ). The right ovary could not be visualised separately from the lesion while the left ovary appeared normal. There were no areas of calcification or fat density noted. No free fluid could be visualised in the pelvis to suggest ascites (Figure ). No significantly enlarged pelvic retroperitoneal lymph nodes were seen. The adrenal glands appeared to be normal and the adjacent bowels loops as well were unremarkable. No focal liver lesions were visible and the visualised lung segments did not contain any significant nodules. There were no destructive bony lesions noted. The scan also did not reveal omental caking or the presence of mesenteric nodules.\nAn ultrasound scan suggested the possibility of a tubo-ovarian mass or an infection, however, infection was unlikely after the CT scan. The absence of areas of calcification or fat density further ruled out the possibility of a mature cystic teratoma, while the presence of soft tissue component in the scan made mucinous cystadenoma a less likely differential. The characteristic feature of a large lobulated multiloculated solid cystic mass showing enhancement made malignant ovarian mass a strong possibility, although the absence of enlarged lymph nodes in the surrounding areas and lack of evidence of metastasis dampened the possibility.\nThe decision was taken for surgical resection of the tumour and confirmation of clinical diagnosis. The resected sample (Figure ) was sent for histopathological examination and it was ultimately found to be follicular variant of papillary thyroid carcinoma in SO (Figure ). Histology slides depict the characteristic ‘Orphan-Annie eye’ nuclei with pale chromatin and areas of nuclear overcrowding. Some nuclei also appear to be coffee-bean shaped due to nuclear membrane infoldings. |
A 55-year-old gentleman was admitted via the primary PCI pathway after presenting with severe, persistent central chest pain following exertion. He had no prior history of coronary artery disease, and his risk factors included tablet-controlled type 2 diabetes mellitus and a current smoking history. His ECG demonstrated significant ST-segment elevation in leads V1-3, though he was clinically stable on arrival with no evidence of decompensating left ventricular failure.\nThe patient also reported a history of haemophilia A (codon 2164 mutation) with no prior major intra-articular or intramuscular haemorrhage. At presentation, he was well controlled on tranexamic acid maintenance therapy. Haematological advice was that he required recombinant factor VIII or desmopressin only prior to major surgery.\nGiven his lack of significant bleeding episodes and his clinical presentation with chest pain and ST-segment elevation, he was transferred for primary percutaneous coronary intervention without further delay.\nCatheterisation was performed via the right radial artery using a 6F JR4 and XB 3.0 guide catheter. The patient was loaded with 300 mg aspirin and 60 mg prasugrel prior to the procedure and was then administered with 5000 units of heparin. A flow-limiting 95% stenosis in the proximal left anterior descending artery was identified, with no significant disease in the left mainstem, circumflex, or right coronary arteries (). Two attempts at thrombus extraction using a Pronto LP device yielded very little, so the decision was made to direct stent the proximal LAD with a bare metal stent (3.5 × 23 mm Vision). The final result was excellent, with TIMI 3 flow down to the distal LAD (). Haemostasis at the puncture site was achieved using a TR band filled with 13 mLs air.\nAfter PCI, the patient was transferred to the High Dependency Unit for observation. His blood revealed an APTT of 54.1, factor VIII level of 60%, and a peak troponin I level of 6.72. He was well after procedure and haemodynamically stable, with no further chest pain and normalisation of his ST-segment elevation. There was no bleeding or haematoma formation at the right radial puncture site. No further haematological intervention was needed.\nThe patient had an unremarkable recovery and after the requisite 72-hour monitoring period he was discharged on 75 mg/day aspirin and 75 mg/day clopidogrel for one month, followed by lifelong 75 mg aspirin thereafter with prophylactic tranexamic acid during clopidogrel therapy. |
A 44-year-old man presented with a sudden onset of lower extremity paresthesias, with an urge to move his legs when he rests in bed or sits for a long time. The patient was questioned regarding the clinical symptoms of RLS based on the IRLSSG criteria. When he rests in bed or sits for a long time, he had unpleasant sensation in the legs and he had an urge to move his legs. His complaints worsened in the evening and especially occur when he lies in bed trying to sleep at night. He had to walk for a while to relieve these complaints. His examination was normal except brisk lower extremity deep tendon reflexes.\nMRI of the brain revealed periventricular and pons plaques. (Figures and ) His cervical () and lumbar MRI was normal. His vitamin B12, vitamin E and D levels, serum iron, iron-binding capacity, and ferritin were all within normal limits and autoantibody tests such as ANA, anti-ds DNA, ANCA, anti-SSA, anti-SSB, and antiphospholipid antibodies were negative. He did not have any drug intake (such as dopamine antagonists, antidepressants, and lithium) associated with RLS. Posterior tibial somatosensory evoked potentials showed prolonged P1 and P2 latencies and central conduction time on the left side. Pramipexole was prescribed and increased to a dose of 0.5 mg/day. Four months after his initial presentation, he developed blurred vision in the right eye. Neurological examination revealed right optic disc edema and diminished visual acuity. Visual evoked potential showed prolonged P100 latency on the right side. 1000 mg methylprednisolone was given for five days and his blurred vision was resolved within 2 weeks. One month later he had right hemiparesis confirming a diagnosis of clinically definite MS. Neurological examination using the manual muscle test revealed a right arm and right motor weakness of 4/5 on the Medical Research Council (MRC) scale, deep tendon reflexes on the right were 3+, and a Babinski response on the right without clonus was present. His control brain MRI demonstrated demyelinating plaques in the supraventricular and periventricular white matter, pons, and both middle cerebellar pedincles (Figures and ) and his control cervical MRI showed demyelinating lesions in C1, C4, and C5-6 intervertebral disc levels and in upper thoracic segments especially placed in posterior and posterolateral cord (). Interferon beta 1a treatment was started. |
A 39-year-old African American male presented to the emergency department with shortness of breath and chest tightness of one day duration after missing hemodialysis (HD) two days prior. His past medical history was significant for hypertension, heart failure with an ejection fraction of 23% with diffuse hypokinesis, pulmonary embolism (PE), end stage renal disease (ESRD), and sarcoidosis with cardiac, hepatic, renal, and central nervous system (CNS) involvement (, ).\nThe patient reported that he was diagnosed with sarcoidosis nine years before by undergoing liver and kidney biopsies at another Institution. At the time of diagnosis, imaging revealed an inoperable 2-millimeter left frontal lobe brain aneurysm. The patient was started on prednisone, methotrexate, and adalimumab. Over the next several years his disease was complicated by worsening hypertension, chronic kidney disease (CKD), and heart failure with a reduced ejection fraction (HFrEF).\nThe patient suffered from three cerebrovascular accidents (CVA) which occurred 5–8 years after the confirmed diagnosis, resulting in left lower extremity weakness and a tonic-clonic seizure disorder. He had also undergone biliary duct stent placement due to obstruction likely provoked by granuloma expansion. The year prior to presentation, the patient developed Staphylococcus aureus bacteremia complicated by sepsis and endocarditis requiring cardiothoracic intervention with the placement of a bioprosthetic heart valve in mitral position. His immunosuppressant medications were stopped at that time. Four months before the current presentation, the patient was admitted for management of hypertensive emergency, a computed tomography (CT) and magnetic resonance angiography (MRA) of the head revealed high T2 and prominent brainstem signaling, suspicious for neuro-sarcoidosis (). Additionally, the imaging noted a partially empty sella and left ophthalmic artery aneurysm (). During the following months, the patient commenced regular HD sessions due worsening of his renal disease. He was also started on azathioprine for management of his sarcoidosis.\nUpon arrival to the emergency department, the patient’s temperature was 97.8 degrees Fahrenheit, blood pressure 185/106, heart rate 61 beats per minute, respiratory rate 18 breaths per minute, and oxygen saturation 100% on ambient air. The patient appeared uncomfortable lying in bed without increased work of breathing or use of accessory muscles. Cardiac, respiratory, abdominal, and extremity exams were unremarkable. During the interview, the patient reported being noncompliant with all of his medications including his immunosuppressors and those for his chronic medical conditions.\nGiven the underlying diagnosis, the patient received a CT angiography of the pulmonary artery, which elucidated an acute PE to the left lower lobe, branching to the anterior medial and posterior lateral basilar segments. Laboratory test results obtained on admission are shown in . He was admitted to the medicine service for management of a sub-massive PE and started on warfarin with a heparin bridge of 1000 units/hour. On hospital day 2, the patient was found to be fluid overloaded likely due to an acute exacerbation of his HFrEF, radiocontrast received and previously missed HD. He was emergently dialyzed, placed on a 1 liter per day fluid restriction, and started on isosorbide mononitrate 60 mg daily and carvedilol 12.5 mg bidaily. For management of his hypertensive emergency, he started clonidine 0.1 mg bidaily and hydralazine 50 mg tridaily. He was continued on phenytoin 50 mg bidaily and levetiracetam 750 mg bidaily for management of his seizure disorder.\nThe patient continued his regularly scheduled HD. During HD on the fifth day of hospitalization, he became hypertensive to 175/120 and acutely dyspneic with decreased mentation. He was intubated for airway protection and placed on mechanical ventilation for two days while being cared in the intensive care unit. This event of dyspnea was likely due to acute pulmonary edema and managed accordingly. After successful extubation, the patient’s international normalized ratio (INR) stabilized to 2.4 and he was discharged on warfarin 5 mg daily. The patient was lost to follow up. |
A 59-year-old Korean man had a medical examination for healthy check-up in December of 2012. An abnormal mass-like lesion in the right hilum was incidentally detected on chest X-ray without any symptom. He was referred to our center for further evaluation.\nThe patient has hypertension, diabetes mellitus and a history of angina. He visited the division of cardiology of our hospital in March of 2010, and was diagnosed with angina and hypertension. A cardiac angio computed tomography (CT) scan performed at that time detected a 2.5-cm diameter, minimally enhanced mass in the right hilar area (). Since then, he failed to return and was lost to follow up. The patient had a 40 pack-year cigarette smoking history and a family history of stomach cancer. On this admission, the physical examination was unremarkable. The blood examination showed eosinophilia (eosinophil 18.5%, 1,750/µL) and the rest of the laboratory tests including serum tumor markers (carcinoembryonic antigen, cancer antigen 19-9, alpha-fetoprotein, and prostate specific antigen), viral markers (hepatitis virus and human immunodeficiency virus), and autoimmune antibody titers were all within normal range. Examination for parasites was also negative.\nA chest CT scan showed a 2.5-cm diameter mass in the right hilum that changed little in terms of size since 2010 (). A primary lesion was not found in the lung parenchyma. The torso fluorodeoxyglucose (FDG) positron emission tomography identified an abnormal FDG uptake in this right hilar mass, but no other pathologic hypermetabolic lesion was seen that may indicate a primary site. Bronchoscopy revealed mild bronchial wall thickening at the right upper lobe and right middle lobe opening but no endobronchial lesion was present. A biopsy of the bronchial wall thickening was done and histologic examination showed chronic nonspecific inflammation with squamous metaplasia. No abnormal finding was seen on the cytologic exam in the bronchial washing specimen. Endobronchial ultrasound guided transbronchial needle aspiration from the right hilar mass was performed. Atypical cells, favor malignancy, was seen in the aspiration cytology. Endoscopy for gastrointestinal tract and head and neck examination also failed to disclose a primary site of cancer.\nRight pneumonectomy and mediastinal lymph node dissection were done. Grossly, several enlarged lymph nodes were found in the hilar and interlobar areas (). On serial gross section of the lung, there was no abnormal lesion at bronchial tree and lung parenchyma except multiple lymph nodes enlargement. Entire bronchus and peribronchial tissue were embedded and evaluated. Microscopically, metastatic squamous cell carcinoma was found at several lymph nodes (8/45) and there was no tumor in the lung parenchyma and bronchial tree (). Although the tumor was very close to the main bronchus, there was no evidence of bronchial invasion microscopically by tumor. The patient was diagnosed with T0N1M0 lung cancer and has received four cycles of adjuvant chemotherapy with cisplatin and vinorelbine. He has been observed at outpatient clinic after treatment without any evidence of recurrence so far. |
A 46-year old female patient, who had been suffering from autoimmune thyroid disease for eight years, presented at our clinic with an acute exacerbation of GO. Clinical examination revealed a convergent strabismus fixus with severe hypotropia of both eyes (Fig. ). The patient complained of increasing loss of eyesight and heavy retrobulbar pain. Visual acuity had deteriorated significantly from 0.6/0.5 to 0.1/0.1 within 3 months. A contrast enhanced orbital MRI scan showed distinct swelling of all extraocular muscles with bilateral compression of the optic nerve (Fig. ). There was no history of comorbidities except nicotine abuse. Laboratory tests showed a euthyroid biochemical status with TSH within the normal range, but elevated levels of Anti-Thyroid Peroxidase Antibody, Anti-Thyroglobulin Antibody and Thyroid Receptor Antibody. The patient’s daily medication comprised of 200 μg L-Thyroxin and 200 μg Selenium. Over many years, the patient had shown only mild to moderate symptoms of GO, but following a thyroidectomy, the symptoms had recently worsened dramatically. Since the disease could not be controlled by high-dose systemic glucocorticoid therapy, bilateral three wall orbital decompression had been performed twice previously. In the first step, the medial orbital wall had been resected via an endonasal approach. Due to ongoing findings as before, two months later partial resection of the orbital floor and fenestration of the lateral orbital wall via a combined transconjunctival/transcaruncular approach with piezosurgery had been performed. In addition, high-dose systemic glucocorticoid therapy was conducted prior to surgery and for the first two months after surgery. Glucocorticoid medication had to be gradually reduced until zero because the patient suffered from an upcoming depression and Cushing syndrome. Orbital radiotherapy for the treatment of thyroid eye disease had been considered as a therapeutic option, but the rapid progress of the disease with the growing risk of dysthyroid optic neuropathy forced us to act more quickly than orbital radiotherapy could perform []. Since the patient increasingly suffered from loss of vision and heavy pain attacks because of medial caudal squinting, we decided to correct the hypo- and esotropia surgically by releasing and repositioning the insertion points of the inferior and medial rectus muscle. Acute surgery was the last remaining treatment option.\nOperations on the left and right eye were performed consecutively with an interval of one week. The eye with the lower vision (left side) was chosen first. A forced duction test showed a complete fixation of the bulb. We opted for a transconjunctival approach (limbal incision) combined with lateral canthotomy to gain access to the dorsal part of the inferior part of the eye bulb. The insertion of the inferior rectus muscle was localized and then circuited with a squint hook (Fig. ). A non-absorbable polyethylene suture (Mersilene 4.0, Ethicon, U.S.) was placed at the anterior rim of the muscle before the muscle was detached from the outer bulb. This release in tension immediately resulted in the spontaneous elevation of the bulb. Lengthening of the muscle with an interposition graft (e.g. fascia lata) was not possible because of the deep retraction of the muscle. Instead, the polyethylene thread loop was directly fixed to the sclera at the former muscle insertion area, placing the muscle 12-15 mm dorsally with regard to its original fixation position. By marking of the original muscle insertion point with a non-absorbable polyethylene suture, an option could be preserved for a more precise muscle readaption at a later stage. Subsequently, the same procedure was carried out with the medial rectus muscle. Less tension allowed direct refixation of the muscle to the sclera without bridging by the polyethylene suture. The bulb was freely movable and remained vertically and horizontally in a primary position (Fig. ). Despite a significant exophthalmos, passive eyelid closure could easily be performed. The significant conjunctival contraction caused by the long-term hypotropia meant that only partial conjunctival wound closure with a polyglactin suture (Vicryl 7.0, Ethicon, U.S.) was possible. No postoperative complications occurred under the postoperative systemic antibiotic medication with Clindamycin of 3x600mg per day over three days supplemented by local application of Neomycin eye ointment for one week. Surgery of the right eye was conducted in the same manner (Fig. ) and under the same perioperative protocol one week later. Additionally, two mucosal grafts of 3.0 × 1.5 cm were harvested bilaterally from the inner cheek to be used for the bilateral reconstruction of the conjunctiva. The intraoral donor sites were closed primarily by using Vicryl 3.0 (Ethicon, U.S.). Starting from extreme eso- and hypotropia, the operation succeeded in repositioning the bulbs into the vertical and horizontal primary position with no restriction of passive movements. Three months postoperatively, the patient was free of pain and had a visual acuity of 0.3/0.6. Visual field testing (Goldmann perimetry) showed only slight concentric bilateral restrictions. Surprisingly, the patient did not suffer from diplopia despite the persistent restriction of active ocular mobility and a moderate bilateral exotropia (Fig. ).\nFollow-up examinations of the patient will be performed at close intervals, including ophthalmological check-ups and the testing of thyroid blood parameters. Contrast enhanced orbital MRI scans will allow the measurement of extraocular muscle volume, as described by Kolk et al. []. Orbital MRI scans were performed preoperatively and three months postoperatively (Fig. ) and the volumes of the extraocular muscles were calculated by using manual segmentation (Osirix Imaging software 5.9) (Table ). During this time interval, the total extraocular muscle volume increased from 24.91cm3 to 29.29cm3. Together with the ongoing high levels of thyroid-specific antibodies (Anti-Thyroid Peroxidase Antibody, Anti-Thyroglobulin Antibody and Thyroid Receptor Antibody), this indicated that the patient was still in an active stage of GO. Volumetric measurements of the extraocular muscles, based on follow-up MRI scans, will help to monitor the course of the disease. Further squint surgery, in terms of a precise readaption of the extraocular muscles, will be postponed to the future, when a more stable stage of the systemic autoimmune disease will be reached. |
An 80-year-old woman with a medical history of hypertension and obesity developed exertional dyspnea in late 2018. An echocardiogram showed a severe aortic stenosis while the coronary angiogram was unremarkable. The Heart Team decided in favor for TAVI and in April 2019, a Medtronic Evolut R 26 mm was implanted. The postoperative course was uneventful. The routine control at the cardiologist 1 month after the TAVI procedure showed satisfying results with an improved performance on the 6-min walk test, and an unremarkable physiological examination.\nIn October 2019, she was referred from the primary care physician due to a 3-week history of cough and fever chills and a C-reactive protein (CRP) of 128 mg/L (reference < 5 mg/L). A systolic murmur raised suspicion of infective endocarditis. The diagnosis was confirmed with transesophageal echocardiography (TEE), which in addition to mitral and prosthetic valve vegetations of up to 20 mm, also showed an abscess in the intervalvular fibrous body (). Blood cultures revealed growth of Cutibacterium spp. After a thorough discussion with the patient, the decision for surgery was taken.\nA full-median sternotomy was performed, and the heart was accessed after opening of the pericardial sac. After bicaval and aortic arch cannulation, the patient was put on cardio-pulmonary bypass and antegrade cardioplegia was delivered to induce asystole. A combination of antegrade and retrograde cardioplegia was iterated every 20 min during the procedure. The ascending aorta was incised above the TAVR prosthesis (), on which small vegetations were found on the stent as well as on the leaflets (). The TAVR prosthesis was removed and after removal of the native aortic valve, an abscess was found at the level of the commissure between the left- and non-coronary cusps. The aortotomy was therefore extended toward this area for better exposure, and the abscess had almost penetrated into the left atrium. The superior vena cava was opened above the sinus node to allow a better exposure to the left atrium. The left atrium was subsequently opened in the roof and the incision was connected to the aortotomy and further down to the anterior mitral leaflet which was excised. Pledgeted sutures were placed in the posterior mitral anulus and sutured to the posterior two-thirds of a 27 mm Perimount Magna, while the anterior one-third of the mitral valve prosthesis was attached to a bovine pericardial patch (). A 21 mm Perimount Magna aortic valve prosthesis was then attached to the aortic anulus and the rest of the bovine pericardial patch with pledgeted sutures (). The left atriotomy was closed using one side of the pericardial patch, and the other side of the pericardial patch was sutured to close the aortic root before continuing with the closure of the aortotomy. Perioperative TEE showed well-functioning biological prosthesis in both mitral and aortic positions without signs of paravalvular leak, and there was no flow across the bovine pericardial patch. The weaning from cardio-pulmonary bypass was relatively uncomplicated although the patient developed a third-degree atrioventricular block without junctional escape rhythm. Epicardial pacemaker electrodes were sutured to the right atrium and right ventricle and tunneled to the left infraclavicular fossa.\nAfter spending 3 days on the thoracic intensive care unit, the patient was transferred to the surgical ward to continue intravenous antibiotic therapy. The postoperative course was uneventful and on the seventh postoperative day, a pacemaker generator was connected to the tunneled electrodes. Routine postoperative echocardiogram confirmed the perioperative findings with well-functioning biological prostheses in aortic and mitral positions. Postoperative chest X-ray also showed ordinary findings (). Cultures from the native mitral valve and the TAVR prosthesis were positive for Cutibacterium spp. Initially, the patient was treated with a combination of Vancomycin and Cefotaxime. Cefotaxime was withdrawn after 2 weeks and Vancomycin in single therapy was continued another 2 weeks, and the patient was discharged to her home after 4 weeks of postoperative intravenous antibiotic therapy. At discharge, she was feeling well with no fever, a CRP concentration of 13 mg/L and a leukocyte count of 5.0 x 109/L (reference 3.5–9.0 x 109/L). |
A 64-year-old woman presents to the Emergency Room with palpebral edema as well as various erythematous plaques in supraciliary and malar areas that appeared and have gradually worsened a couple of days prior to presentation (). Initially the patient did not share having changed her daily facial hygiene, making any diet changes, getting any animal bites, or taking any new medication.\nOn exanimation, the patient had facial swelling, pain on palpation, and flushing.\nA full blood count was performed with no alterations of the blood formula, neither leucocytes nor the eosinophils, and no inflammatory markers or IgE elevated.\nStill suspecting an allergic reaction, she was given treatment with corticosteroids and antihistaminic medication, both intramuscularly and with a unique dose.\nFour days later, the patient returned to the Primary Care consultation due to recurrence of her symptoms and was referred once again to the Emergency Room where she was clinically diagnosed with cellulitis and received antibiotic treatment (amoxicillin with clavulanic acid) along with corticosteroids and antihistaminic medication once again.\nAlthough she referred a short period of improvement of the symptoms, the swelling and rash reappeared when the treatment was suspended along with another two nodules of hard consistency localized on the cheekbone of the right facial side ().\nDuring the fourth Primary Care consultation, the patient was interrogated more thoroughly and only then she mentioned that she was visiting, for about four months, an Esthetic Private Clinic, where she was injected in various sessions with Metacrill, as a facial lift, for beauty purpose. Apparently no other patient suffered the same symptoms.\nDue to suspecting an allergic reaction to the Metacrill and the failure of the initial treatments, she was referred to the dermatologist.\nDuring the dermatology consultation, a biopsy of one of the granulomas was performed, being diagnosed as Chronic Edematous Panniculitis to a foreign body and probably caused by a polymeric material in the context of an iatrogenic infiltration of esthetic filler ().\nThe patient received a new treatment with corticosteroids, in a decreasing dose associated with antibiotics (doxycycline orally).\nWhen trying to lower the dose of corticoid, the patient suffers a new episode of edema and as a consequence the dose of oral corticoid is raised and is associated with a corticoid topic cream.\nDue to the bad evolution, the patient is sent to the autoimmunity consultation at the hospital where she receives an immunosuppressive treatment with Tacrolimus and was not presenting new symptoms ever since. Every three months, the levels of the immune response are being tested to avoid toxicity (). |
An 80-year-old woman (46 kg, 154 cm) was transported to our intensive care unit intubated and ventilated with recovery of spontaneous circulation after cardiopulmonary arrest (no-flow 15 minutes, low-flow 10 minutes). She reported dyspnea and syncope in her toilets following a pushing effort. She had no previous history of respiratory or cardiac disease. She is followed for giant cell arteritis and myelodysplasia without other medical history.\nInitial evolution was rapidly favorable, and the patient regained consciousness without sequelae. Etiologic assessment carried out initially did not explain this episode of cardiopulmonary arrest. CT-chest performed initially when the patient was intubated found a megaesophagus but no tracheal compression ().\nA week later, the patient presented an episode of acute respiratory distress with bradycardia during a meal. Faced by suspicion of inhalation pneumopathy, a bronchoscopy was performed but not finding of foreign bodies. In view of the presence of a pulmonary focus on chest X-ray (), diagnosis of aspiration pneumonia was retained. The patient improved and returned home after few weeks.\nThree months later, the patient was rehospitalized in a state of acute respiratory distress, once again after a meal. At this time, new CT-chest without intubation found megaesophagus, this time with tracheal compression (). Bronchoscopy performed at this time revealed a TM with complete expiratory collapse and 80% inspiratory collapse associated with an inflammatory mucosa ().\nIn view of all these elements, we have retained the diagnosis of acute airway obstruction and cardiopulmonary arrest due to TM caused by megaesophagus compression secondary to achalasia. Achalasia was subsequently confirmed by esophageal manometry. No other cause of TM, in particular, no arguments for systemic infection or collagen vascular disease, was highlighted on various examinations. Please note that the patient's giant cell arteritis was perfectly controlled during these respiratory episodes.\nOn therapeutic plan, in front of patient advanced age and history of myelodysplasia with severe pancytopenia, surgery was not proposed. We proposed intraesophageal botulinum toxin injections under endoscopy to try to treat achalasia. The best treatment for TM is that of the cause. Following this treatment, the patient has not presented any cardiopulmonary arrest but she continued to have repeated lung infections. Unfortunately, she died two years later from a complicated lung infection. |
An 84-year-old Japanese woman with a history of lung cancer treated by surgery was admitted due to gradual worsening dyspnea over the previous month. She had had asymptomatic and unchanging pericardial effusion and a pelvic mass diagnosed 3 and 11 years previously, respectively, and had been followed-up without the need for treatment. The chest radiography and magnetic resonance imaging of the pelvis 4 months before admission are shown in Fig. and Fig. , respectively. The chest X-ray showed a nodular shadow in the right lung field (Fig. ), as well as unchanging pericardial effusion volume revealed by a chest computed tomography (CT) scan (Fig. ). Transbronchial biopsies of the lung nodule were performed, and the pathological specimen disclosed adenocarcinoma. Then, she underwent a right upper lobectomy for localized lung adenocarcinoma with pericardial fenestration, which confirmed that the pericardial effusion included abundant lymphocytes and did not contain any malignant cells. She progressed favorably after the operation and was discharged. However, she began to experience dyspnea on exertion leading to admission. On admission, she was not suffering from fever, night sweats, chest discomfort, or abdominal pain. She had a prior history of pulmonary tuberculosis treated with chemotherapy 20 years before and was treated for diabetes mellitus and hypertension in the hospital. She had no history of smoking, illicit drug use, recent travel abroad, or asbestos exposure.\nThe patient was conscious and afebrile, with a pulse of 108 beats/min, blood pressure of 148/50 mmHg, respiratory rate of 28 breaths/min, and oxygen saturation of 90 % in room air, which improved to 96 % on 3 L/min supplemental oxygen administered intranasally. Pulmonary auscultation revealed a decreased breath sound over the right lung field. She had normal heart sounds without murmurs. A large, hard, non-tender mass was palpable in the lower part of the abdomen. There was no finger clubbing, cyanosis, lymphadenopathy, or edema in her extremities.\nThe complete blood count, blood biochemistry, and autoantibodies were all unremarkable, except for serum carcinoembryonic antigen (CEA) at 6.1 ng/mL (normal, below 5.0 ng/mL) and carbohydrate antigen 125 (CA-125) at 260 U/mL (normal, below 35 U/mL). The chest radiography showed cardiac enlargement and right pleural effusion, as well as blunting of the left costophrenic angle (Fig. ). Her electrocardiogram (ECG) was within normal limits. A transthoracic echocardiogram showed preserved right and left ventricular function with pericardial effusion. A chest, abdominal, and pelvic CT scan showed the presence of pericardial and bilateral pleural effusion and ascites with a left ovarian mass, 20 cm in diameter, without hepatosplenomegaly or lymphadenopathy (Fig. ). Pericardial effusion had decreased compared with that before the lung operation. A thoracentesis revealed clear and yellowish pleural fluid. It was a lymphocyte-predominant exudate; the total protein level in the pleural fluid was 3.1 g/dL (6.2 g/dL in serum) and lactate dehydrogenase (LDH) in pleural fluid was 181 U/L (238 U/L in serum), which fulfilled Light’s criteria for an exudate []. The adenosine deaminase (ADA) and CEA levels in the pleural fluid were 9.7 U/L and 2.4 ng/mL, respectively. The bacterial and mycobacterial cultures of the pleural fluid were negative. Cytological examination of the pleural fluid showed abundant lymphocytes without any evidence of malignant cells, similar to that in the pericardial effusion confirmed during the lung surgery.\nDespite drainage of the pleural fluid, recurrent episodes of pleural fluid accumulation occurred requiring weekly thoracentesis. Despite tube thoracostomy into the right pleural cavity for drainage, the pleural effusion persisted. As the combination of her ascites, pleural effusion, and ovarian mass was suggestive of Meigs’ syndrome, she was referred to the department of gynecology and underwent bilateral salpingo-oophorectomy due to strong adhesions of bilateral ovaries. Histopathological examination of the resected ovarian mass revealed ovarian fibroma (Fig. ). Thereafter, she made an uneventful recovery and the volume of pleural and pericardial effusions, as well as ascites, decreased and subsequently resolved. The follow-up chest X-ray and CT 8 months after removal of the fibroma confirmed no relapse of the pleural and pericardial effusion (Fig. ). |
An 80-year-old male patient with history of hypertension and dyslipidemia presented to our emergency department with new onset of chest pain and a non-ST segment elevation myocardial infarction (NSTEMI) due to a subtotal occlusion of the distal right coronary artery (RCA). Echocardiography showed concentric left ventricular hypertrophy and an inferior wall motion abnormality with mildly impaired left ventricular function (LVEF 44%).\nFor the intervention of the RCA, a guidewire was passed through the severe stenosis of the distal RCA into the posterolateral branch (PLB). Extensive balloon dilatation of the vessel resulted in large dissection planes, which complicated the passage of a second guidewire to protect the posterior descending artery (PDA). Initial wiring of the PDA was considered unnecessary but became crucial after the dissection extended into the distal bifurcation of the PLB and PDA. A dual-lumen microcatheter was not available at that time, and the operator was unable to deliver the second guidewire distally passing the dissection planes. He then came up with a guidewire co-transporter made from a regular 2.0/20 SC balloon catheter that was used to predilate the lesion and was able to successfully place a second guidewire into the PDA.\nPreparation of the guidewire co-transporter (sidecar) is illustrated step-by-step in Figure . Any regular balloon catheter can be used with longer balloons being more practical. Inflate the balloon to 4 atm and puncture it at its proximal end with a 23-gauge needle (radial puncture needles are ideal). Insert the needle gently into the balloon without puncturing the distal end of the balloon. Insert the guidewire through the needle into the balloon and push it until the tip of the balloon. Squeeze all the air and contrast media out of the balloon by sliding your fingers from distal to proximal while holding the second guidewire together with the balloon shaft. Hold both the balloon and the inserted second guidewire between your right thumb and index finger and gently remove the needle with your left hand. Thread the balloon onto the main guidewire and push both the balloon and the second guidewire forward together.\nThe interventional procedure of the sidecar maneuver is demonstrated step-by-step in Figure . Advance the balloon through the lesion as far distal as possible. Retract the second guidewire from the balloon and advance it again outside parallel to the balloon. Then remove the balloon catheter from the coronary artery.\nThe cartoon (Figure ) illustrates the removal of the second guidewire from the sidecar once the distality has been reached. Moving images of both the preparation of the guidewire co-transporter (Video ) and the entire sidecar technique (Video ) are available in the online appendix.\nAs an alternative to the sidecar technique, one can also puncture the distal end of the balloon and let the guidewire exit the balloon at the distal end. This may be needed in certain situations such as reverse wiring or puncture of an occluded side branch (slipstream technique). |
A perimenopausal 56 year old nulliparous woman underwent supracervical laparoscopic hysterectomy and bilateral salpingoophorectomy for pelvic pain, menorrhagia, and large uterine leiomyomas. She had no history of abnormal Pap smears and a preoperative Pap smear was normal. Her family history was negative for any known gynecologic, breast, or gastrointestinal malignancies. Preoperative chest radiograph and endometrial biopsy were not obtained. Due to menorrhagia, an endometrial curettage was performed at the initiation of the procedure. The specimen was sent for frozen section and was negative for malignancy. Intraoperative findings were significant for an 18–20 week size uterus weighing 1518 g. Prolonged mechanical morcellation with a ROTOCUT G1 morcellator (Karl Storz, Germany) was required to remove the uterus and a small amount of superficial endometriosis in the pelvis was seen and cauterized. The fallopian tubes and ovaries were removed intact in an endoscopic bag. The uterus was not morcellated in a bag, and the fragments were subsequently collected from within the abdomen, and withdrawn from a laparoscopic port. Final pathology on six uterine fragments revealed weakly proliferative phase endometrium without atypia, adenomyosis and leiomyoma in the uterus. The ovaries showed fragments of benign ovarian parenchyma and patchy endosalpingiosis. No malignancy was identified in any specimen.\nThe patient's initial recovery was unremarkable until 14 months post-operatively when she presented with left lower quadrant pain and persistent cough with shortness of breath. Examination revealed a firm palpable abdominal mass at the left lower quadrant and a fleshy lesion at the apex of the vagina. The cervix could not be identified. PET–CT revealed multiple hypermetabolic masses in the pelvis (), abdomen, and chest () concerning for metastatic disease. A core needle biopsy from the 9 cm left pelvic mass revealed poorly differentiated malignant neoplasm of epithelioid cells with hyperchromatic nuclei in a background of necrosis. Immunohistochemical stains showed tumor cells positive for cytokeratin, vimentin, and CD138. The tumor cells were negative for SMA, EMA, ER, TLE1, HNB45, desmin, myogenin, CD17, BCL2, inhibin, MDN2, CD4, CA125, WT-1, CD45, calretinin, DOG-1, p16, CEA, CK 20, HPV, and CD99. The vaginal biopsy showed grade 3 endometrioid adenocarcinoma. Pathology re-review of the supracervical hysterectomy and bilateral salpingo-oophorectomy specimens showed no evidence of malignancy. The differential diagnosis included recurrent uterine cancer (unrecognized at time of morcellation) versus primary peritoneal carcinoma that developed from endometriosis.\nThe patient was treated with intravenous carboplatin AUC 6 and paclitaxel 175 mg/m2 every 21 days and had a partial response after two cycles. She completed six cycles of therapy, but unfortunately developed rapidly progressive disease. One month after her sixth cycle of chemotherapy, she presented with a complex fistula involving the pelvic tumor, abdominal wall, and sigmoid colon. She underwent a diverting loop colostomy. She strongly desired further therapy and received one cycle of doxorubicin 60 mg/m2. After admission for febrile neutropenia and pulmonary embolism, she elected to transition her care to hospice and died of her disease two months later. |
A 67-year-old female with reported allergy to clonazepam and bee venom with a past medical history of aortic valve regurgitation, B12 deficiency, congestive heart failure, coronary artery disease, osteoporosis, and multiple fractures (pelvic, clavicle, ankle) presented with diffuse myelopathy and severe back pain secondary to traumatic T11 burst fracture with disruption of posterior elements including pars and facet joint as well as left clavicle fracture (Figure ).\nShe underwent T9-L1 minimally invasive bilateral pedicle screw fixation using a titanium fixation system (Figure ).\nThe patient’s initial post-operative course was uneventful with complete resolution of her back pain. Wounds were healing well without erythema or drainage. One month post operatively, the patient was noted to have a 4.9 kilogram weight loss, anorexia, and fatigue. She was followed by her primary provider and was treated with a high caloric diet, merinol, and followed monthly for weight management. She began to develop pain along the hardware site approximately six months post operatively which was felt to be due to weight loss and thoracic hardware near the skin's surface. The incision sites appeared to be well-healed upon examination in the clinic. Complete blood count with differential was ordered to rule out delayed infectious process and resulted within normal limits. Post-operative three months and six months X-rays demonstrated intact hardware without evidence of lucency (Figures -).\nOf note, the left clavicle was initially treated conservatively. However, due to non-union, a clavicular plate was placed six weeks post spine fusion. She developed a skin rash over the clavicular plate site nine months post operatively and it was removed. At that time, the patient reported a nickel allergy she has had since childhood, which would likely explain the rash over the clavicular plate. Although there were no outward signs of skin rash along the titanium spine hardware, the patient was referred to a dermatologist for possible titanium allergy testing. According to the patient, memory lymphocyte immunostimulation assay (MELISA) testing was not performed and we were unable to obtain her dermatology clinic visit notes. She continued to decline over the next two years, ultimately losing nearly 23 kilograms. Due to the risk of skin erosion over the thoracic hardware, the hardware was removed. No caseous necrosis or metallic debris was noted surrounding the peri-prosthetic tissue during hardware removal. Therefore, no tissue samples were taken. The patient’s anorexia and fatigue improved significantly within one month of hardware removal. She gained 18 kilograms over the next six months and reported significant improvement in stamina. |
We present a case of a 72-year-old Caucasian female who underwent adjuvant chemoradiotherapy for a Grade 2 invasive ductal carcinoma of the left breast. She had no other significant past medical history, including dermatological history, and took no regular medications. She was a non-smoker and did not consume alcohol. Presenting from the national screening programme, she underwent a wide local excision with axillary lymph node clearance. Her tumor was estrogen receptor positive, HER2 negative and had 7/28 lymph nodes positive. Weekly paclitaxel treatments were stopped after four cycles due to anaphylactoid hypersensitivity. She underwent 40 Gy of radiotherapy in 15 fractions over 3 weeks to the left breast and left supraclavicular fossa. Before commencing radiotherapy, she had no noted skin changes.\nUpon routine review 8 weeks post completion of radiotherapy, oedema and erythema with blistering and desquamation of the skin were noted over the left breast and chest wall. There was also an area of desquamation on the patients’ back consistent with a radiotherapy exit field. The patient had been using simple moisturizers as instructed and managing pain with simple analgesia. The skin changes were first apparent 4 weeks after completion of radiotherapy. Initially, the skin lesions were confined to the radiotherapy field. She was afebrile with otherwise normal observations. Differentials included radiation-induced skin changes with superimposed cellulitis, autoimmune bullous disorder or a paraneoplastic process. An admission for further treatment was organized where intravenous antibiotics and antivirals were commenced alongside a regular skin treatment regimen. This consisted of Viscopaste and Tubigrip, Hydromol and Dermovate ointment as required. An initial superficial skin swab showed mixed skin flora only.\nDespite optimal medical management, her skin continued to deteriorate, with a new area of desquamation over the right hip. The patient also developed painful oral erosions. Serum samples for indirect immunofluorescence were requested and the patient was commenced on 40 mg of prednisolone once daily after a punch biopsy was performed. Biopsies revealed a negative direct immunofluorescence, with some increased mitotic activity indicating quick turnover of the epidermis. There was no superficial acantholysis suggestive of pemphigus foliaceus. Indirect immunofluorescence was positive for intercellular IgG antibodies and anti-desmoglein3 antibodies, consistent with a diagnosis of pemphigus ( and ).\nMaintenance prednisolone was continued at 40 mg until review in dermatology outpatients 1 month later. There had been no new skin blistering; however, oral ulceration remained problematic. Steroid mouth washes were added to her regime and the topical treatments were continued. Two months after initial diagnosis, a weaning regime of 5 mg per month of prednisolone was recommended after her oral lesions had completely resolved. Now 6 months after initial presentation, there has been no relapse of oral or skin lesions and corticosteroids continue to be weaned by 5 mg per week. All topical treatments have been discontinued. |
A 7-year-old boy presented to the pediatric surgery clinic with a 6-year history of enlarging neck swelling. On clinical examination, a large nontender mass was present in front of the neck extending laterally to the right sideand the back, covering the entire right side of the neck []. There was skin plethora at neck, shoulder, and upper part of chest along with a swollen right arm []. This swelling had appeared at 6 months of age and had gradually increased with time. Excisional biopsy was done at 3 years of age and a diagnosis of lymphangioma was made, for which he had received two sets of sclerotherapy.\nA differential diagnosis of AVM and enlarged lymph node was made and the patient was advised computed tomography (CT) angiogram, which showed a large cervical AVM with predominant supply from right subclavian and vertebral arteries []. The treatment options were discussed with parents and angioembolization was planned under general anaesthesia.\nIn preanaesthesia evaluation, child had difficulty in breathing aggravated in supine position, cough, and headache. There was pitting edema of lower limbs up to ankles. The arterial pulses in the right upper limb were weaker than those on the left side. A palpable thrill and continuous machinery murmur was heard in right supraclavicular region. Fine crackles were heard bilaterally over the lung bases which were consistent with a diagnosis of heart failure. The airway was Mallampati class III and neck movements (flexion and extension) were mildly restricted.\nAll laboratory investigations were within normal limits except for hypokalemia (serum potassium 2.9 mEq/L), which was probably secondary to chronic frusemide therapy. Child was treated with intravenous 4 mEq/h potassium chloride supplementation for 24 h. Chest radiograph showed a widened mediastinum, prominent superior vena cava shadow, and tracheal shift to the left. Electrocardiography revealed sinus tachycardia and evidence of left ventricular hypertrophy (LVH). Two-dimensional echocardiography showed mild dilatation of all cardiac chambers, mild LVH, and mild biventricular systolic dysfunction with increased flow velocities across all cardiac chambers. The echocardiographic features were suggestive of high-output cardiac failure. The child was managed with oral digoxin 5 mcg/kg q12hrly and intravenous frusemide 1 mg/kg q12hrly. After stabilization of heart failure, the angioembolization was scheduled. Equipment for difficult airway management, resuscitation drugs, and help was specially arranged for the radiology suite. Two units of packed red cells were also arranged.\nThe child was placed in propped up (45°) position. A peripheral intravenous catheter (18 G) was inserted. After application of routine monitors including electrocardiography, pulse-oximetry, end-tidal CO2, and noninvasive blood pressure, patient was preoxygenated for 5 minutes. Inhalational induction of anesthesia was done with sevoflurane. After ensuring adequate mask ventilation, succinylcholine 30 mg was given, and trachea intubated with a 5.5 mm ID cuffed endotracheal tube in the propped up position. Fentanyl 2 mcg/kg was administered intravenously for analgesia. Local anesthetic (lignocaine 1%) 5 ml was injected over the femoral area. Invasive arterial pressure monitoring was done using the sidearm port of femoral sheath as the child had a swollen right arm and both the femoral areas were required by radiologist. anaesthesia was maintained with Isoflurane 1.5–2% in 50:50 oxygen and air. A Foley's urinary catheter was inserted to monitor hourly urine output. Embolization of the AVM with isobutyl-2-cyanoacrylate (IBCA) glue and vaso-occlusive coils was done in a three hour procedure. During the procedure some IBCA glue, used to occlude the feeding vessels, leaked into the pulmonary circulation. Due to anticipated development of pulmonary hypertension (a known effect of IBCA) and worsening of cardiac failure, it was decided to electively ventilate the patient postprocedure.\nIn the intensive care unit (ICU), the chest radiograph showed tiny numerous high density materials in both lung fields especially in basal segments []. Echocardiography revealed severe tricuspid regurgitation, severe pulmonary hypertension, and dilated right heart. He was started on sildenafil citrate (a pulmonary vasodilator) 2 mg/kg q6hrly and it was given through nasogastric tube, which causes marked improvement in symptoms. The child's trachea was successfully extubated after 48 h and he was shifted to the special care unit where he remained hemodynamically stable. The child was discharged from hospital after three days. |
A 19-year-old Chinese girl came to the Department of Periodontology, Oral Center of the Affiliated First Hospital of Second Military Medical University, for the evaluation of localized gingival hyperplasia in the right maxillary incisor area. The patient complained that her gum started to overgrow about a year ago and caused discomfort when brushing. As a nonsmoker, she had no history of systemic diseases and allergies. Clinical examination showed that there was an excessive growth in the purple round gums with a diameter of 11 mm between tooth 11 and 12, covering most of the crowns, but without significant absorption of the alveolar bone in the affected area [Figure and ].\nOn the day of surgery (and after signing the informed consent), the patient received 0.12% chlorhexidine mouthwash. Then, following local anesthesia with 2% articaine and epinephrine at 1:100,000, vascular epulis was completely removed by diode laser without obvious hemorrhage (Pilot™, USA). The vascular epulis was resected by continuous mode wave during the surgery, and the output power was set at 2.0 W and the wavelength at 810 nm. The patient was instructed to brush the surgical area with a soft-haired toothbrush and gargle with 0.12% chlorhexidine mouthwash daily for at least 2 weeks.\nThe wound healed 10 days after laser therapy []. Although chronic gingival inflammation occurred between tooth 11 and 21, there was no significant gingival inflammation at the surgical site 5 years later []. Hematoxylin and eosin staining showed that there were large numbers of thin-walled vascular hyperplasia and dilatation in the gingival lamina propria and vessels of varying sizes in the proliferative fibrous tissue, and large quantities of red blood cells in the blood vessels were lined with flat vascular endothelial cells [Figure and ]. Immunohistochemical staining showed that expression of CD31 was positive and that of CD34 was strongly positive in the vascular endothelial cells [Figure and ]. |
A 5-year-old neutered male Maltese Terrier dog was presented to a referring veterinarian with a 3-day history of depression and anorexia followed by vomiting, seizure clustering and generalized ataxia. The owner noticed a progressive deterioration of the neurologic signs over the preceding 3 days. Symptomatic treatment before the presentation with phenobarbital (2 mg/kg twice a day orally) failed to alleviate the seizures.\nThe dog had been generally healthy until the occurrence of acute seizure episodes. He had been fully vaccinated and dewormed, with no history of trauma or exposure to toxins. Further medical enquiry with the owner revealed that the dog had been fed an almost exclusive diet of boiled sweet potato (Ipomoea batatas) as a primary diet source for 4 weeks with an average consumption of approximately 100–150 g (about one to one and a half small whole sweet potatoes) per day due to his picky eating behaviours (chronic food refusal). Furthermore, this food refusal had gradually worsened over time.\nAt presentation, the seizures were classified as generalized tonic-clonic with vomiting aura. The initial onset of seizures was 3 days before the admission, and the cluster seizures activity was recorded over three sequential days (more than three seizures in a 24-hr period). The average duration of the 3 ranged from 20 to 30 s, and the time interval between consecutive seizures was approximately 10 min. The last seizure episode verified by the owner was at least 6 hr before admission. Except for neurological abnormalities, other abnormalities were not noted on complete physical examination. Mentation was normal. Body condition (body condition score of 4 on a scale from 1 to 9), weight (4.2 kg) and muscle mass were all normal at the time of admission, but the patient had a previous history of obesity. The results of complete blood cell count were unremarkable. Serum biochemical analysis (Catalyst One Chemistry Analyzer, IDEXX Laboratories, Inc., ME, USA) revealed alkaline phosphatase activity of 298 U/L (reference range, 23 to 212 U/L) and alanine aminotransferase activity of 430 U/L (reference range, 10 to 125 U/L). Blood lactate was measure by using the blood gas analysed (pHOx Ultra, Nova biomedical, MA, USA) and the concentration was significantly increased to 12 mmol/L (reference range, 0.5 to 2.5 mmol/L). The slightly elevated levels of hepatic enzyme activities seemed to be attributed to the phenobarbital administration. There was no clinical evidence to support the presence of hypoperfusion or tissue hypoxia. Survey radiographs of the head, thorax and abdomen were categorized as normal.\nNeurological examination identified generalized ataxia with dysmetric movement and an inability to bear full weight on the hind limbs. Conscious proprioception was reduced in all four limbs. An assessment of the cranial nerves showed spontaneous vertical nystagmus in both eyes with an upward fast phase. Segmental spinal cord reflexes and cutaneous trunci reflexes were normal. No other neurological abnormalities were identified. The localization of neuroanatomical lesions was multifocal intracranial, including both the forebrain and brainstem.\nThe intracranial lesions were identified via brain magnetic resonance (MR) imaging using a 0.4 Tesla scanner (Aperto; Hitachi Medical Corporation, Tokyo, Japan) and cerebrospinal fluid (CSF) analysis (collected from the atlanto-occipital cistern tap using a 22-gauge needle). T1-weighted (T1W) images, T2-weighted (T2W) images, fluid-attenuated inversion recovery (FLAIR) images and contrast-enhanced T1-weighted (CET1W) images were obtained from the MRI scan. The CET1W images were obtained after intravenously injecting Omniscan (Gadolinium EDTA; GE-Healthcare, Little Chalfont, United Kingdom) at a dosage of 0.20 mmol/kg body weight. After acquisition of the MRI data and the CSF samples, mannitol (15% D-Mannitol inj., Daihan Pharm., Seoul, Korea) was administered as a bolus of 1 g/kg over 15 min for preventing a rebound of intracranial pressure after the general anaesthesia. MR images (Figure ) revealed bilaterally symmetrical hyperintense lesions in the caudal colliculi, specifically in the T2W and FLAIR images. The lesions appeared iso- to hypointense on T1W images, with no evident contrast enhancement on CET1W images. These abnormal findings on MR images were considered to be a pathologic change in brain metabolic derangement. No abnormalities were found on conventional CSF analysis (total nucleated cell count of 3 cells/μL; reference range from 0 to 5 cells/μL, protein concentration of 15 mg/dl; reference range from 0 to 25 mg/dl). There was no cytological CSF evidence of relatively well-recognized CNS disorders (e.g. inflammatory, neoplastic and infectious causes). Therefore, based on the inadequate diet history, the hyperlactatemic condition (Type B hyperlactatemia) and the abnormalities seen on MRI, a presumptive diagnosis of thiamine deficiency was established in this patient.\nTo confirm our presumptive diagnosis of thiamine deficiency, the whole blood sample was submitted to evaluate the blood thiamine concentration. Thiamine content was measured by the high-pressure liquid chromatography (HPLC) method (Agilent 1,260 Infinity II LC system, Agilent Technologies, CA, USA) with the assistance of a commercial laboratory (Neodin Vetlab, Seoul, Korea). The HPLC is a preferable and reliable method of thiamine analysis in humans and animal studies, which helps by directly measuring the phosphorylated form of thiamine (thiamine pyrophosphate, TPP) within RBCs (Talwar, Davidson, Cooney, & Jo’reilly, ). TPP is the most biologically active form within the body of humans and animals and has a main role in carbohydrate metabolism (National Research Council, ). Following the MRI scan and the blood sampling, a therapeutic trial using thiamine supplementation with dietary intervention was performed on the same day to evaluate the associated response and to rapidly recover the clinical signs. Sweet potato supplementation was immediately stopped prior to starting the treatment. Thiamine supplementation was started with oral vitamin B complex (Beecom-C, Yuhan Corp., Seoul, Korea) at a total thiamine dose of 45 mg once a day for a month. Furthermore, the dog's diet was temporarily changed to a good-quality prescribed dog food containing high levels of protein and fat (Hill's Prescription Diet a/d, Hill's Pet Nutrition Inc., KS, USA).\nA rapid improvement in neurological signs was seen with the initiation of thiamine supplementation and diet change. There were no additional seizures after being discharged from the hospital. Furthermore, the symptoms of anorexia improved along with the normalization of previous metabolic derangement with the treatment, which consequently accelerated the recovery rate of the remaining neurological abnormalities. Nystagmus resolved within the first 24 hr, and ataxia resolved over the following 2 days. The general condition also recovered gradually along with the recovery of neurological signs. Finally, no residual neurological deficits were observed at 3 days after continuous management. In addition, we identified that the blood level of TPP (40.2 ng/ml; reference range, 46.0 to 112.0 ng/ml, provided by the laboratory), which was measured before starting the treatment, was particularly low. Given these findings, the presumptive diagnosis of thiamine deficiency secondary to inadequate dietary intake was confirmed.\nTwo weeks after starting thiamine supplementation and dietary intervention, there were no abnormalities on physical and neurological examination. Repeated blood examination showed that the previously observed biochemical abnormalities had entirely normalized: alkaline phosphatase activity of 98 U/L (reference range, 23 to 212 U/L), alanine aminotransferase activity of 82 U/L (reference range, 10 to 125 U/L) and blood lactate level of 0.9 mmol/L (reference range, 0.5 to 2.5 mmol/L). MR imaging was repeated at the same level as in Figure . The previously observed caudal colliculi lesions on the initial MR images had completely resolved (Figure ). Additionally, re-evaluating the blood TPP levels indicated that its level was significantly above the normal limits (138.5 ng/ml; reference range: 46.0 to 112.0 ng/ml).\nOne month after the initial presentation, thiamine supplementation was discontinued, and the dog's diet was changed to quality well-balanced commercial maintenance dog food. The improvements were well maintained at a 1-year follow-up. |
In January 2021, a 2-year-old boy of white Caucasian origin presented to his local ophthalmologist for acute unilateral sixth nerve palsy and was subsequently transferred to our pediatric emergency department for further evaluation. The patient, generally being fit and well, had developed a sudden dysfunction in lateral movement of his left eye, resulting in a continuous abduction deficit with consecutive fixated turn of the head to the left side. His medical history was unremarkable for trauma, headache, vomiting or fever. He had not received any vaccinations within the last few weeks. Apart from a mild gait instability, there were no concomitant symptoms or other focal neurological deficits on clinical examination. The patient did not suffer from any chronic diseases and did not take any regular medication; his vaccination status was complete according to national recommendations.\nThree weeks prior to onset of symptoms the patient had experienced a respiratory tract infection resulting in an increased respiratory rate, dry cough, intermittent fever and loss of appetite, lasting for 2 weeks. Symptomatic treatment was initiated by his local pediatrician, who attributed the patient's symptoms to a common cold rather than COVID-19. Thus, no oropharyngeal swab for SARS-CoV-2 or other viruses was obtained. At the same time, the patient's father and his uncle developed cough, dyspnea, sore throat and muscle aches; the uncle tested positive for SARS-CoV-2 on PCR from oropharyngeal swab (). The child's uncle does not live in the same household but had been in close contact to the patient 4 days prior to his positive test for several hours due to an indoor-birthday party. The patient's relatives were unvaccinated as at that time the COVID-19 vaccines were still unavailable for the general public.\nOn admission, laboratory inflammatory markers including C-reactive protein were negative. Full blood count showed mild thrombocytopenia (186 109/) but was unremarkable otherwise. Cranial contrast-enhanced magnetic resonance imaging (MRI) showed an hypoplastic left abducens nerve and atrophy of the corresponding left lateral rectus muscle compared to the contralateral side (). There were no signs suggesting any inflammatory intracranial process or elevated intracranial pressure, no papilledema. A lumbar puncture was performed. The cerebrospinal fluid (CSF) opening pressure was 24 cmH2O corresponding to the upper limit of normal range () thus diagnostic lumbar puncture was followed by therapeutic drainage of 8 ml CSF. Routine CSF laboratory parameters yielded a normal result; no oligoclonal bands were detected on CSF/serum. Multiplex-PCR (Filmarray, BioFire, Biomerieux Lyon, France) from CSF was negative for cytomegalovirus (CMV), enterovirus, herpes simplex viruses 1 and 2, human herpesvirus 6, human parechovirus, varicella zoster virus, Cryptococcus neoformans and gattii, E. coli K1, Haemophilus influenzae, Listeria monocytogenes, Neisseria meningitides as well as Streptococcus agalactiae and pneumoniae. An additional multiplex-PCR performed on an oropharyngeal swab sample yielded a negative result for adenovirus, coronaviruses 229E, HKU1, NL63 and OC43, human metapneumovirus, human rhino-/ enterovirus, influenza virus A and B, Middle East Respiratory Syndrome Coronavirus (MERS-CoV), SARS-CoV-2, parainfluenza virus 1–4, respiratory syncytial virus, Bordetella pertussis, Bordetella parapertusssis, Chlamydophila pneumonia and Mycoplasma pneumoniae. Testing for Borrelia burgdorferi showed no antibodies in neither serum nor CSF. An EEG was unremarkable. Repeated ophthalmologic examinations revealed incomitant squint angles due to left-sided sixth nerve palsy and a significant abduction deficit of the left eye, consistent with the diagnosis of left abducens nerve palsy. An underlying retraction syndrome was considered unlikely due to the sudden onset of symptoms and absent globe retraction. Optic nerve examination was unremarkable.\nReal-time reverse transcriptase PCR (rRT-PCR) test for SARS-CoV-2 (oropharyngeal swab sample) was negative on admission, while serology turned out to be positive for SARS-CoV-2 anti-spike IgG (Euroimmune, Germany). Of particular note in this context, SARS-CoV-2 specific IgG was also detected in CSF. Pathogen-specific antibody index as an indicator for potential intrathecal antibody production was negative, suggesting involvement of central nervous system being secondary to systemic infection rather than direct viral infection (). An rRT-PCR for SARS-CoV-2 from CSF was negative.\nGiven the boy's history of recent respiratory tract infection, COVID-19 very likely in his father and proven in his uncle, and detection of SARS-CoV-2-IgG antibodies in the patient's serum and CSF, post-infectious abducens nerve palsy appeared to be the most likely diagnosis. During inpatient stay, symptoms already showed spontaneous mild improvement without therapeutic measures. Following discharge, the boy was regularly seen for ophthalmologic follow-ups. Three months following onset of abducens nerve palsy, the family noticed a distinct improvement in eye movement and the child eventually made a full recovery 2 weeks later. |
A 72-year-old man with underlying dilated cardiomyopathy had an MR conditional cardiac resynchronization therapy defibrillator (CRT-D), Boston Scientific D179 device with lead models 7736 (right atrial), 0692 (RV), 4677 (left ventricular, LV). The system was implanted in 2015 for primary prevention following unexplained syncope with LV ejection fraction of 24% (normal range > 55%), left bundle branch block and New York Heart Association class II.\nThe patient attended for a routine device interrogation in March 2019, which demonstrated satisfactory battery, lead and device settings, consistent with previous follow-up (). On review of the events, there was a single stored atrial tachycardia response (ATR) episode which correlated temporally with him undergoing brain MRI at another centre for investigation of trigeminal neuralgia. Though the device logged this as an atrial arrhythmia episode it shows features on the EGM of the potential effects of MRI on CIED’s.\nOversensing EMI—atrial fibrillation and ventricular fibrillation ()\nshows intermittent oversensing of EMI on the atrial and RV channels from the RF gradients during the MRI scan. There are periods of inhibited pacing and intervals in the ventricular arrhythmia detection zones. No tachycardia therapy is delivered as there are not enough intervals to meet the detection criteria. The device inappropriately detects an ATR episode, mode switches and stores an EGM of the event. There was no evidence of oversensing at any time point remote from the MRI scan (before or after).\nLoss of RV capture ( and )\nEGM demonstrates temporary loss of capture on the endocardial RV lead, however, there is capture on the epicardial (coronary sinus) LV lead. This is proved in , where the loss of evoked response on the RV channel is shown compared to the capture seen in clinic (*). The implications for our patient were minimal due to his continuing pacing from his LV lead and underlying AV conduction.\nProvocation manoeuvres were performed in the clinic with no changes in impedance values and no oversensing seen proving satisfactory lead integrity and performance.\nIn discussion with the local hospital, it was determined that device re-programming was not performed prior to the patient being scanned, although the patient was asymptomatic throughout and the scan was completed without clinical complication.\nAfter a satisfactory device interrogation, the patient was followed-up via remote monitoring, with no subsequent events, battery or lead abnormalities. |
A 32-year-old male with no significant past medical history presented to our facility with a history of worsening pain and swelling in the medial aspect of his proximal left lower extremity over the past several years. Anteroposterior (AP) and “frog leg” lateral radiographs of the left lower extremity revealed a dense lesion consistent with dense matrix, adjacent to the medial aspect of the femur (). An MRI of the left lower extremity revealed that the lesion was of extraskeletal origin, arising from the adductor magnus, without any involvement of the femur (, ). The solid heterogeneous mass was located in the posterior compartment of the proximal-to-mid thigh and measured 9.6 × 7.3 × 13 cm in the transverse, AP and vertical dimensions, respectively. Multiple tiny hypointense foci, which corresponded to the calcific density noted on the prior plain radiographical studies, were noted centrally. The mass was otherwise of intermediate soft-tissue intensity on T\n1 weighted images and moderately hyperintense to muscle on T\n2 weighted images. Following intravenous administration of gadolinium, moderate enhancement of the mass, excluding the central portion, was observed. Superiorly and inferiorly, the margins of the mass were indefinite, and the mass demonstrated peripheral vasculature, which was most prominent at the cephalad and caudad margins. The mass was centred within the adductor magnus muscle fibres that were displaced around the mass. Anterolaterally, the mass was very closely approximated to the posterior cortex of the femur and the fascial margins that separated the quadriceps and the posterior compartments (Supplementary videos). No obvious signal abnormality was noted within the femur to indicate invasion or primary osseous origin. Post-excision pathology confirmed the presence of mesenchymal chondrosarcoma.\nIn the interval, surveillance imaging for metastatic disease with CT scan of the chest, abdomen and pelvis was performed. At 18 months, multiple bilateral non-calcified pulmonary nodules concerning for metastasis were identified (). The most prominent pulmonary nodule was present within the posterior right base, measuring 2.3 cm in the greatest dimension and abutting the pleura (). With regard to the abdominal and pelvic series, a new right adrenal lesion appearing as a hypodense pedunculated mass measuring 1.6 cm in the greatest dimension was noted. There was no evidence of osseous metastases. |
A 43-year-old Hispanic woman who had a past medical history relating to hypertension, obesity, and stroke was first admitted to the hospital in December 2014. There she had undergone hysterectomy for abnormal uterine bleeding, and surgical pathology, and at that time, was confirmed as having uterine leiomyoma.\nTwo years after the hysterectomy, the patient was sent to the ED from the cardiology clinic for dyspnea, dizziness, and multiple episodes of syncope. Physical examination was within normal limits except for the presence of jugular venous distension, and irregular heart rate and rhythm where electrocardiogram (EKG) showed atrial fibrillation and transthoracic echo (TTE) reported nonischemic cardiomyopathy with a left ventricular ejection fraction (LVEF) of 20%-25% and a globular mass measuring 4.0 cm x 3.5 cm, almost filling the entire right atrium. Further workup, including abdominal ultrasound, revealed an enlarged inferior vena cava (IVC) with an intraluminal thrombus and occlusive portal vein thrombus causing absent flow consistent with Budd-Chiari syndrome. CT scan of the abdominal pelvis reported an extensive 5.7 cm x 4.7 cm IVC thrombus extending contiguously from the right mid external iliac vein and the left common iliac vein through the IVC and into the right atrium, in addition to a lobulated 12.0 cm pelvic mass (Figure , B). MRI of the abdomen and pelvis with and without contrast revealed a prominent solid, avidly enhancing portion within the sizeable pelvic mass. The patient underwent right atrial, IVC, and bilateral iliac tumor thrombus resection by a team of cardiothoracic and vascular surgeons. A follow-up CT angiogram of the chest with contrast reported no residual thrombus. Subsequent resection of pelvic mass reported spindle-shaped cells with degeneration consistent with IVL (Figure ).\nA few months later, the patient also underwent an implantable cardioverter-defibrillator (ICD) placement for atrial fibrillation, and the patient's cardiac and functional status further improved, and she was able to get back to work. \nTwo years later, the patient started complaining of an increasing lower extremity swelling, and dyspnea on exertion. A chest CT scan reported new pulmonary nodules (Figure ).\nTransjugular IVC mass biopsy performed, and pathology reported findings consistent with BML given the neoplasm exhibited staining with antibodies directed against S-100 and smooth muscle actin, without significant reactivity for antibodies directed against cytokeratins AE1/AE3 with ER+/PR+ on immunohistochemical stains.\nIn light of her progressive disease, and worsening LVEF to 15%-20% upon preoperative evaluation, a multidisciplinary team recommended medical treatment consideration, and the patient was commenced on a new regimen of leuprolide and letrozole. While on this treatment, her abdominal mass and lung nodules were considered stable by RECIST 1.1 criteria (9.2% decrease in total tumor burden size), and she has had no new symptoms five years after initial diagnosis (Figure ).\nMolecular profiling\nMolecular profiling of the tumor showed amplification of cyclin-dependent kinase 4 (CDK4), JUN, and MCL1, and loss of AT-rich interaction domain 1A (AR1D1A), RB transcriptional corepressor 1 (RB1), SUFU, and hepatocyte nuclear factor 1-alpha (HNF1A) (Figure ). |
An 18-year-old female with history of KD, coronary artery aneurysms, and coronary artery bypass grafting (CABG) presents to the emergency department by EMS reporting abdominal pain starting earlier this morning. The patient describes the abdominal pain as epigastric radiating to her right shoulder and worsened with deep inspiration. The pain has been worsening throughout the day. She also endorses nausea. She denies vaginal bleeding and reports that her last menstrual period was approximately one month ago. She endorses a history of bulimia, and reports forceful vomiting episodes every day for the past year. Otherwise, the patient denies fever, cough, recent illness, trauma or injury. She notably has a history of KD as a child resulting in coronary artery aneurysms and requiring CABG surgery at age 12. She is currently taking warfarin and aspirin.\nPhysical exam is remarkable for blood pressure of 99/69 mmHg and heart rate of 98 beats per minute, remaining vital signs within normal limits. On exam, the patient is in no acute distress. The exam is significant for diffuse abdominal tenderness with voluntary guarding.\nDue to significant abdominal tenderness on initial physical exam, bedside FAST scan was performed, which was found to be positive, demonstrating a significant amount of free fluid in the right upper quadrant, left upper quadrant, and suprapubic views (Figures , ).\nOther workup was remarkable for anemia with a hemoglobin of 8.0 g/dl and negative pregnancy test. Surgery was consulted and computed tomography angiography (CTA) of the chest, abdomen, pelvis were performed emergently due to concern for internal bleeding. CTA imaging demonstrated no acute findings of the aorta, and coronary artery aneurysms at the right coronary artery (RCA) and left anterior descending artery (LAD). Non-contrast CT of the abdomen and pelvis demonstrated free fluid (Figure ).\nProthrombin complex concentrate (PCC) and tranexamic acid (TXA) were given due to concern for bleeding on anticoagulation. Due to ongoing concern for internal bleeding along with the patient's risk factors, the patient was admitted to the surgical intensive care unit (ICU) for further management.\nIn the ICU, the patient was transfused 2 units of packed red blood cells. Serial hemoglobin checks and abdominal exams were performed with a plan for diagnostic laparoscopy if hemoglobin decreased. However, the patient’s hemoglobin remained stable, abdominal pain improved, and the patient was eventually discharged to home the following day with close follow-up. |
A 10 year old white girl with severe (class 3: BMI ≥140% of the 95th percentile for age and sex) obesity and otherwise normal development presented to the Pediatric Weight Management Clinic with her mother. The mother reported that the patient had been at the 75th percentile for height and weight for most of the patient's life but she experienced a “20 to 30 pound” weight gain over the past year. The mother further explained that this recent weight gain coincided with treatment of seasonal allergies with montelukast and she wondered if this may have been the cause of the weight increase. The patient had no prior weight loss attempts.\nThe patient was born full term, weighing 3.18 kg. The mother's pregnancy was uncomplicated, as was the patient's newborn course. Aside from seasonal allergies, the patient was healthy. She had no history of hospitalizations, surgeries, or mental health concerns. She was not taking any medications.\nThe patient was eating regularly-spaced meals consisting primarily of highly processed foods and simple carbohydrates (e.g., pastries for breakfast, potatoes with cheese for dinner). The family was eating fast food three times per week on average. The patient endorsed having a big appetite and feeling hungry all the time. She was eating while watching TV and when bored. She denied binge eating, loss of control eating, emotional eating, sneaking/hiding food, or eating during the night. Her physical activity was limited to gym class at school three times per week.\nThe patient was living with her mother and her mother's partner. The patient's parents divorced when she was very young and the mother's partner had been living with them since the patient was a toddler. The patient saw her biological father rarely. She had no siblings. She was attending fourth grade and enjoyed reading and writing. The mother and her partner worked full-time and the patient was cared for by a baby sitter after school a few times per week. They had no food insecurity. The family history was notable for obesity in both biological parents and type 2 diabetes in the maternal grandmother.\nThe patient's review of systems was negative. She reached menarche several months prior to presentation. On physical examination, her weight was 70.31 kg (155 lbs.), height was 142 cm (4'8”), and BMI was 34 kg/m2 (145% of the 95th percentile). Her blood pressure was 105/65 mmHg and pulse was 74 beats per minute. Her physical examination was normal. The results of her fasting labs were: total cholesterol 176 mg/dL (normal: < 170 mg/dL), HDL-c 49 mg/dL (>45 mg/dL), LDL-c 96 mg/dL (< 110 mg/dL), triglycerides 157 mg/dL (< 90 mg/dL), ALT 27 (< 50 U/L), AST 29 (< 50 U/L), glucose 98 mg/dL (70-99 mg/dL), and HbA1c 5.5% (0-5.6%). Her Pediatric Symptom Checklist (routinely obtained in the Pediatric Weight Management Clinic) score was 8 (> 28 is considered abnormal).\nThe patient and family were started on a program of lifestyle modification therapy and responded particularly well with decreasing fast food consumption and liquid calories. Further, the patient started bringing her lunch to school instead of eating the school fare and was able to keep a food log almost daily. The patient's physical activity, however, continued to be limited. Over the course of 5 months, the patient's BMI decreased 5 units (15%), from 34 kg/m2 to 29 kg/m2 (145% of the 95th percentile to 125% of the 95th percentile).\nAt the end of the 5 month period, coinciding with the end of the school year and beginning of summer vacation, the patient's sleep/wake cycle became irregular. Because she did not like the hot weather, she chose to stay inside all day. Her mother left prepared meals for the patient to encourage healthy eating while mom was at work. Despite this, the patient's BMI began to trend upward from 29 kg/m2 to 31 kg/m2 over the summer months. Upon school resuming in the fall, the patient's sleep/wake cycle normalized and eating behaviors and patterns improved, returning to those of the previous school year. The patient's BMI stabilized for a few months but then increased further. The patient expressed frustration because she believed that she was eating well, which was indeed reflected in her daily food logs. She continued to attend monthly visits with the Pediatric Weight Management Clinic dietician, psychologist, and pediatrician with specialized training in obesity medicine. Yet, the patient's BMI continued to increase such that by 2 years after her initial appointment, the patient's BMI returned to baseline (135% of the 95th percentile) (see Figure ).\nSuspecting that metabolic adaptation was causing the patient's weight rebound, adjunct pharmacotherapy was recommended. Orlistat was considered but not started because of concern about gastrointestinal side effects and lack of insurance coverage. Metformin may have been another reasonable option but the patient's fasting glucose and HbA1c were in the normal range and she did not have acanthosis nigricans on physical examination which would have suggested insulin resistance. She was ultimately started on topiramate 75 mg daily in addition to ongoing LSMT. She and her mother were cautioned that although topiramate is not FDA-approved for the indication of obesity (in children or adults), multiple studies have demonstrated clinically-meaningful weight loss efficacy in adults. Additionally, it was explained that the side effect profile in children is well established stemming from its use for epilepsy treatment.\nAfter 4 months of treatment with topiramate, the patient's BMI trajectory plateaued yet was not decreasing as was desired. Recognizing that the combination of topiramate and phentermine is the most effective weight loss medication currently available for adult obesity, phentermine 15 mg daily was added to the topiramate 75 mg daily. The patient and mother were informed that phentermine is FDA-approved only for individuals older than 16 years and for “short-term use.” With combination treatment for ~22 months, the patient experienced good BMI reduction, from 34.1 to 25.7 kg/m2. Her blood pressure and heart rate were monitored regularly and though her blood pressure did not increase, her heart rate increased slightly from 60 to 70 s, in line with the mechanisms of action of phentermine (stimulant-like effects). Later, the patient reported that she was experiencing some “memory” issues but noted no change in her academic performance. Although it seemed unusual for this type of symptom to emerge 10 months after starting topiramate, the topiramate dose was decreased from 75 to 50 mg daily and the memory issues resolved. Written informed consent was obtained from the parent of the patient for the publication of this case report. |
Figure shows a retrospective CGM summary graph from a 14-year-old female with T1D of 2 years duration, receiving approximately 1 unit/kg/day of short acting insulin via insulin pump. She came to the clinic 3 days prior to her normal appointment and was set up with the clinic’s retrospective CGM. At the appointment her HbA1c level was found to be 7.9%, and her SMBG record revealed scattered high and low blood sugars with no consistent pattern. The patient and her family were reluctant to alter insulin doses due to a history of prior hypoglycemic seizures, and felt her current degree of blood glucose control was satisfactory. However the CGM summary graph of the previous 3 days showed asymptomatic and undetected overnight low blood sugar levels in the 60–80 mg/dL range and low blood sugar levels beginning 1–2 hours after exercise in the evening (Figure ). Using these data from CGM, overnight basal rate on her insulin pump was decreased slightly and the patient was educated on use of the temporary basal feature (decreasing the basal rate on her insulin pump by 25% for the next 6 hours after exercise) to prevent hypoglycemia in the evening following exercise.\nFear of hypoglycemia limits many children from attaining desired HbA1c goals []. For many children with T1D, bedtime blood glucose checks are poor predictors of nocturnal hypoglycemia [,,]. As noted in the 2012 consensus guideline for pediatric CGM, intermittent use of CGM (as demonstrated by the case history above) may detect the dawn phenomenon, postprandial hyperglycemia, asymptomatic daytime and unrecognized nocturnal hypoglycemia, and can aid in evaluating the effects of changes in treatment regimens []. However, initial studies of RT-CGM suggested that its use did not reduce rates of hypoglycemia []. The STAR1 trial of children using SAP therapy identified “failure to respond to high and low alarms, and/or appropriately dose and administer insulin,” as the main contributor to significantly higher rates of hypoglycemia []. The conclusion, therefore, is not that RT-CGM cannot reduce hypoglycemia in patients using insulin pumps, but rather that it needs to be used properly. More recently, multicenter, randomized controlled trials (discussed above, showing that RT-CGM use lowered HbA1c levels in adults and children with good baseline blood sugar control), demonstrated that CGM use was associated with reduced rates of hypoglycemia in both adults and children [,]. Further, in the last year, two industry-sponsored trials have shown significant improvements in HbA1c levels in children while also reducing time spent with blood sugar levels in the hypoglycemia range. The STAR3 trial of children using CGM showed a reduction in blood sugar level variability, favoring SAP use []. In addition, the SWITCH Study Group’s trial of children and adults showed that, in patients using SAP therapy, HbA1c levels, as well as time spent with blood sugar levels in the hypoglycemia range, were reduced. [].\nGreater reductions in frequency and severity of hypoglycemia are likely with further integration of CGM into the insulin pump, as now seen with the Medtronic Paradigm Veo. Retrospective analyses of children and adults using the “low glucose suspend” feature of the Paradigm Veo, which automatically suspends insulin delivery for up to 2 hours after a hypoglycemic event occurs as determined by CGM, have shown both significant reductions in exposure to hypoglycemia and prevention of profound rebound hyperglycemia after the CGM system had automatically suspended insulin delivery for up to 2 hours [,]. |
A 24-year-old white man, a middle-distance runner (800 m) competing at national level (seasonal best/personal best of 1 minute 52 seconds), developed severe left heel pain in the pre-season in March 2013. His maximum perceived pain intensity was 10 cm on a visual analog scale (VAS) that ranged from 0 to 10 cm, with 10 cm expressing the worst perceivable pain; the athlete had to interrupt all running activity, and severe pain was perceived even when walking or standing. He continued training with aqua jogging and cycling. He got personalized hand-crafted orthopedic gel peads. Two months later he was attended by an orthopedic surgeon, who additionally prescribed oral intake of nonsteroidal anti-inflammatory drugs (NSAIDs) for 8 weeks. The athlete could continue his training but was not free from pain. When discontinuing medication in July 2013, pain returned immediately, and perceived pain intensity during walking was 10 cm on a VAS (range 0–10 cm). Eight sessions of ESWT were thus added to his treatment plan, and were conducted at a German Olympic center. He did not feel better after the treatment and reported a high level of frustration. An MRI was performed in January 2014 showing a calcaneal spur, signs of inflammation at the calcaneal tubercle, and structural changes of the plantar fascia, surrounded by a large edema (see Fig. ). In February 2014 he underwent an open plantar fasciotomy. Four weeks later he was allowed to perform the first units of regenerative running. Pain returned after approximately 1 week of training. An X-ray revealed no pathology and he was recommended to continue with soft training sessions. He received a peppering injection that reduced pain for 12 hours, and NSAIDs were re-prescribed. His running performance remained at a remarkably low level in comparison to his non-injured state, despite regular personalized training sessions. He presented himself at our out-patient clinic in July 2014 (for timeline see Fig. ).\nAn examination identified pain to palpation at the medial calcaneal tubercle and along the medial band of the plantar fascia. Thickening and enlargement of the proximal one-third plantar fascia was noted. Full and pain-free range of motion was noted to his ankle and foot. Standing caused moderate (VAS score, 5 cm) pain; walking caused severe (VAS score, 10 cm) pain. Latent myofascial trigger points could be found in the surrounding muscles: gastrocnemius medialis and lateralis, and tibialis posterior. Apart from these symptoms no abnormalities in his medical or family history which may have been relevant to the medical case were reported and he presented himself in a good mental condition. The diagnosis based on these findings was chronic plantar fasciitis (calcaneal spur syndrome).\nHe was treated with neural therapy (that is, injection of < 1 ml procaine 1%, which is a local anesthetic) of the surgical scar and along the surgical puncture channel. He lay in a supine position on a treatment table. Sessions took approximately 5 minutes. In total, three sessions (at baseline, at week 1, and after 4 weeks) were performed.\nAt the first treatment (March 2015), he described a slurping noise, like “if something filled up the pain origin.” Afterwards he could stand pain-free and walking (not running) was subjectively improved. After the third session the pain had been completely eliminated (VAS = 0 cm). He could return to sports at the former level. Since March 2015 no recurrence of the problem could be observed. No adverse events were observed. |
A 24-year-old unmarried female presented to the outpatient clinic with painful swelling of the right sternoclavicular joint of 2-month duration without any discharging sinus (). The swelling was gradually increasing in size and was accompanied with mild pain. The pain was dull, continuous, and limited to the site of the lesion. She also complained of neck stiffness and pain on neck movements. There was no history of any injury. History of cough, weight loss, night cries (severe pain at night), and low grade fever was present for the past 4 months. There was no history of previous tuberculosis or contact with an open case of tuberculosis. She had been prescribed several antibiotics and analgesics at another centre but had no symptomatic improvement. On physical examination the swelling (2 × 3 cm) was present over right sternoclavicular joint and was associated with presence of mild tenderness, erythema, and local rise of temperature. Laboratory tests revealed haemoglobin of 10.4 gm%; total leukocyte count was 10.300/mm3. Her ESR was 34 mm in first hour. She was negative for HIV based on ELISA method. On radiographic evaluation there was destruction with sclerosis on the medial end of the right clavicle along with features of diffuse pulmonary infiltrate (). MRI revealed bilateral upper lung lobe infiltrate with arthritis of right sternoclavicular joint, with regional fluid collection. A destruction of the atlanto-axial junction, D7-8 intervertebral disc space along with a pus collection from D5 to D8 region could also be appreciated (Figures , , and ). An early morning sputum sample was sent for Ziehl-Neelsen (ZN) staining and it came out positive suggesting the diagnosis of pulmonary tuberculosis. Fine needle aspiration of the right sternoclavicular lesion was done using a 22-gauge needle and sent for Gram staining, staining for acid-fast bacilli (AFB), histopathology, and cultures including a tubercular culture. The histologic picture was that of chronic inflammation with a caseating granuloma compatible with tuberculosis. The Ziehl-Neelsen stained smear also showed the presence of acid-fast bacilli (AFB), confirming the diagnosis of tuberculosis. The culture for Mycobacterium tuberculosis came out as negative. Antitubercular chemotherapy with four first line antitubercular drugs (rifampicin, isoniazid, ethambutol, and pyrazinamide) was started. The patient had a good clinical response within 6 weeks and was switched to three drugs (rifampicin, isoniazid, and ethambutol) after 3 months of therapy with four drugs. The clinical, haematological, and radiological parameters showed complete healing of the lesion after 1 year of treatment with ATT, which was further continued for a total duration of 18 months. After successfully completing the therapy for 18 months, the patient was followed up for 2 years and showed no recurrence of symptoms. |
A 51-year-old woman presented with fatigue, nausea, and vomiting for three days. She was diagnosed with metastatic melanoma to pelvic nodes in July 2014 and was treated with ipilimumab (anti-CTLA-4) in combination with nivolumab (PD-1 inhibitor) between September 2014 and April 2015, receiving a total of 4 cycles of combination therapy and 9 cycles of nivolumab monotherapy with an initial mixed response followed by slow progression of disease. In April 2015 she was enrolled on a study of radiation (to pelvic mass) in combination with pembrolizumab (PD-1 inhibitor), receiving a total of 9 cycles of pembrolizumab with no toxicities. In December 2016 she was found to have new brain metastases and in January 2017 pembrolizumab was added. The day following her second dose of pembrolizumab, she developed fatigue and nausea and began having intermittent vomiting and diarrhea. Abdominal CT scan demonstrated diffuse colitis. Infectious studies including C. difficile antigen, stool culture, viral PCR, and ova and parasites exam were all negative. She was started on methylprednisolone 2m/kg/day. Over four days of hospitalization, her abdominal pain worsened and she developed melena, which progressed to bright red blood per rectum. She was given infliximab at 10mg/kg. Repeat imaging performed 48 hours later due to severe abdominal distension showed large amounts of free air with gaseous distention of large and small bowel loops, consistent with perforation in the context of colitis with ileus. She was taken to the operating room for emergent bowel resection and a perforation site was identified at the transverse colon. The resected transverse colon serosa was congested and dusky with site of perforation identified (). The colonic mucosa revealed diffusely edematous folds as well as confluent areas of yellowish exudate and multifocal ulcers (). Histologic sections confirmed the presence of transmural necroinflammation and multifocal ulceration (Figures and ). The findings were of a fulminant colitis with multifocal ulceration and perforation. No evidence of metastatic melanoma to the bowel was identified. |
A 27-year-old female who suffered from EDMD presented for upper endoscopy with planned Botox injection to treat severely symptomatic gastroparesis. Prior to the endoscopy she had lost 15% of her total body weight due to severe regurgitation of undigested food and debilitating chronic nausea. She had undergone a 4-h solid-phase gastric emptying scan and CT scan which were consistent with gastroparesis. She used a gastric tube to infuse supplemental elemental feedings and had failed to respond to all prior medical therapy.\nThe upper endoscopy was performed using monitored sedation with propofol. After ensuring there was no evidence of gastric outlet obstruction, 100 units of Botox (OnabotulinumtoxinA) was injected into the pylorus (25 units into each quadrant). The injections were unremarkable, with no evidence of intra-arterial injection, and she was discharged from the recovery area 1 h after the procedure feeling well. Two hours post-procedure, she experienced weakness in her bilateral lower extremities, which progressed rapidly to her upper extremities. Within 3 h post-procedure, she became unable to walk and was brought to the emergency department by private vehicle 3.5 h after the Botox injection.\nUpon arrival, she was evaluated by the neurology service who noted diffuse muscle weakness (1/5) in all muscles – with only the ability to slightly flex her neck. Her lower extremities were weaker than her upper extremities. She complained of inspiratory weakness but did not require endotracheal intubation.\nBoth systemic botulism and acute inflammatory demyelinating polyneuropathy were considered as potential causes for her symptoms, and a lumbar puncture was performed. Her cerebrospinal fluid was normal without increased protein and given the temporal relationship of her symptoms to the injection, systemic botulism toxicity was felt to be more likely. The Centers for Disease Control and Prevention (CDC) was contacted and recommended administration of the botulism anti-toxin. The anti-toxin was then urgently transported by commercial airline and courier from Atlanta, GA (CDC), to Lebanon, NH (Dartmouth-Hitchcock Medical Center), and given approximately 8 h following the endoscopy.\nThe patient was admitted to the ICU and treated in the ICU and step-down units with aggressive physical and respiratory therapy. She did not require mechanical ventilation. While in the hospital she underwent an EMG, which demonstrated no evidence of demyelination or peripheral neuropathy, but did show myopathic potentials in her right leg. She slowly improved in all her muscle strength and was discharged to a rehabilitation center after a 12-day hospitalization. Six months following the endoscopy, she had recovered all muscle function back to her baseline. |
A 42-year-old man with atopic dermatitis underwent AGV implant surgery with tube insertion through the sulcus at another hospital to treat secondary glaucoma due to chronic iridocyclitis in his left eye. His right eye was blind because of a retinal detachment and secondary glaucoma. Six months after the implant surgery in his left eye, bullous keratopathy developed, and he was referred to our hospital (Fig. ). We performed Descemet stripping automated endothelial keratoplasty in the affected eye. The next day, the IOP increased to over 30 mm Hg. The patient had severe corneal cloudiness, which prevented visualization of ocular interior by slit-lamp microscopy. Using anterior-segment optical coherence tomography (AS-OCT) (CASIA2, Tomey, Nagoya, Japan), we confirmed that the AGV tube was in the superior temporal quadrant and filled with aqueous humor, and no filtering capsule blocked the tube (Fig. ). Although we observed a high-intensity area in the tube lumen under the scleral patch, we could not precisely detect the proximal tube occlusion around the tube tip through the ciliary sulcus because of poor mydriasis and backscatter from the posterior iris pigment (Fig. ).\nWe first performed a bleb needle revision using our method [] to separate the fibrotic tissue around the valve cover from the AGV plate, but the IOP did not decrease. We then used intraocular endoscopy to detect the disrupted outflow from the tube into the AGV. No fibrovascular ingrowth was seen between the valve plate and valve cover, and no intra-valve hemorrhage was seen (Fig. ). When we observed the proximal drainage tube through the intraocular endoscope, we saw that the tube was occluded by the tissue (Fig. ); we used a lens capsule forceps to remove the tissue from the tube (Fig. ; online suppl. Digital Content ; for all online suppl. material, see ). We used hematoxylin and eosin staining to examine the surgically removed tissue embedded in paraffin, but nothing in the bright field microscopy images was stained, and the sample only showed a mass composed of small fragmented fibrous structures (Fig. ).\nThe IOP decreased to about 10 mm Hg with antiglaucoma eye drops and remained stable for 1 year postoperatively. The patient underwent penetrating keratoplasty for recurrent corneal cloudiness. The IOP remained within the normal range for 22 months with antiglaucoma eye drops and self-ocular massage. We confirmed the flow from the tube into the AGV and the encapsulation around the plate of the AGV using AS-OCT 8 months after removing the occluded tissue in the AGV tube (Fig. ).\nThe results showed that the fibrous materials could be Soemmering's ring or Zinn's zonule, which might occlude the AGV tube and block outflow from the tube into the AGV. This case represents a new cause of late-onset tube occlusion of the AGV using intraocular endoscopy. |
A 65-year-old lady referred to our hospital with a complaint of dyspnea. She was not a smoker and her past medical history consisted of hypertension and hyperlipidemia. On admission, physical examinations revealed an elevated jugular vein pressure accompanied by pulmonary rales up to two-thirds from the base of the lung. The patient was tachypnic (respiratory rate = 28) and blood pressure was 180/100 with a normal oral temperature (36.9 °C). Her electrocardiogram showed slight T-wave inversions in the leads V2 through V6 as well as leads I and aVL. Occasional ventricular premature complexes were also present (). The cardiac troponin level was normal. Echocardiogram of the patient showed a LV ejection fraction (LVEF) of 23% without regional wall motion abnormalities with dilated LV and atrium. Presence of moderate amounts of mitral regurgitation and moderate concentric LV hypertrophy was also noticeable.\nSince there was no past medical history of heart failure, we proceed with coronary angiography to exclude ischemic causes as the basis of her disease. Cannulation of the left main coronary artery (LMCA) displayed normal courses of the LMCA, left circumflex (LCX), and LAD. An anomalous RCA as a separate large branch arose from the proximal area of LAD. The abnormally originated RCA had three significant stenoses in the proximal and distal portions (A-C). Attempts to cannulate the RCA with the right Judkins catheter were unsuccessful. Aortography did not show the presence of another origin for a supplementary RCA from another site (D). This was concluded to be a benign anomaly with an atherosclerotic disease. As the amount of LVEF impairment was not concordant with the epicardial coronary abnormalities, the patient was considered as a case of dilated cardiomyopathy and the patient was given long-term medical therapy for her heart failure as well as her atherosclerotic disease. At follow-up, it was observed that the patient had an asymptomatic clinical status. |
A 73-year-old woman referred to our service from the endocrinology department suffering from medullary thyroid cancer with cervical metastases. The patient presented a 3 cm palpable, firm mass on the right lobe of the thyroid gland. Ultrasonography (U/S) and computerized tomography (CT) revealed palpable lymph nodes in the right cervical area at level III and a right paratracheal mass displacing but without invading the carotid artery, the esophagus, and the trachea. Thyroid function tests gave normal results. Fine-needle aspiration biopsy (FNAB) of the nodule was consistent with the diagnosis of medullary carcinoma.\nA total thyroidectomy was performed along with a modified neck dissection type III at the right side and an elective dissection at levels II, III, and IV at the left side of the neck. The thyroid gland was very hard to palpation and strongly attached to the trachea and carotid sheath, without however invading the posterolateral side of the esophagus. Both recurrent laryngeal nerves were identified and respected and their function was appropriately monitored. Intraoperatively, the patient suffered from bradycardia secondary to carotid manipulation and managed with atropine administration.\nPathological examination of the thyroid gland revealed a 4.3 cm well circumscribed, tan-white, indurated lesion with gritty consistency. The rest of the gland was orange-yellow and fleshy, with no evidence of noted nodules. Microscopy revealed the histological features of medullary carcinoma, such as nests or chords of cells penetrating dense pink stroma with a lobular or trabecular growth pattern (). Calcification areas were also noted. On higher power examination, the neoplastic cells were round, relatively uniform with a punctuate chromatin (). On immunohistochemistry tumor cells were positive for calcitonin () and CEA ().\nFour out of thirty-two lymph nodes, found within the tissue removed during neck dissection procedure from the right side, showed metastatic disease.\nIn the first postoperative day, the patient presented right blepharoptosis (Müller muscle) and miosis (stimuli were slower in the affected pupil) typical of Horner's syndrome (). No hematoma, seroma, or infection could be detected as possible cause of the syndrome. The patient exhibited voice weakness (hoarseness) and after laryngeal endoscopy, a palsy of the right vocal cord to the paramedian position was detected due to a potential injury in the communication between the cervical sympathetic chain and the recurrent laryngeal nerve on the right side. No other complication presented in the postoperative period and the patient was discharged from the hospital seven days later. Ptosis and miosis disappeared after four weeks (). Stroboscopic examination of the larynx two months after operation revealed restoration of the laryngeal mobility. |
A 72-year-old male presented to our centre with a 3-month history of slowly growing, painless right breast swelling associated with bloody nipple discharge. On clinical examination, there was a palpable firm right breast lump (2 × 2 cm) in the right retroareolar region with serous discharge from the nipple.\nUltrasound of the right breast showed the presence of a cystic mass with a solid component within the retroareolar region, measuring 1 × 1 × 1.8 cm, which was classified as BIRADS 4. Tissue biopsy of the breast mass demonstrated sheets of mildly pleomorphic tumour cells arranged in a solid papillary architecture with low mitotic activity. p63 immunohistochemistry stain showed positive expression of myoepithelial cells at the periphery of the lesion and absent expression along the papillary fronds. A diagnosis of intrapapillary ductal carcinoma in situ (DCIS) was made. The tumour was also diffusely positive for oestrogen receptor (ER) and progesterone receptor (PR), but negative for human epidermal growth factor receptor 2 (HER2). These are demonstrated in Figure .\nUpon further questioning, the patient denied any risk factors for breast cancer. He has no family history of malignancies or genetic disorders such as Klinefelter syndrome. Apart from hypertension, hyperlipidaemia, and previous cerebrovascular disease from which he completely recovered, he denies any other medical problems, including those which are related to liver disease, thyroid disease, and testicular disease. He is a non-smoker and not particularly overweight.\nSubsequently, we proceeded with a computerised tomography (CT) scan for staging purposes. As shown in Figure , the scan revealed residual lesion in the right breast and unfortunately also an incidental finding of prostatic mass with bladder infiltration and local regional lymphadenopathy. The CT also showed lytic bony lesions at the L2 vertebrae body, pelvic bone, and left proximal femur. The bone scan shown in Figure confirmed these lesions as metastases. His PSA at diagnosis (iPSA) was 430.7 ng/mL.\nA transrectal ultrasound-guided biopsy of the prostatic mass confirmed the diagnosis of prostatic adenocarcinoma with a Gleason score of 8 (4 + 4). The patient agreed to mastectomy of the right breast. The histopathology report of the residual breast mass was similar to the biopsy result of papillary DCIS which was ER/PR positive and HER2 negative. There was no microinvasive or invasive component. The surgical margin was clear. We did not perform axillary clearance or a sentinel node biopsy.\nWe counselled the patient for genetic testing; however, he refused. The patient was started on anastrazole and subcutaneous leuprolide acetate for treatment of his breast tumour and metastatic prostate cancer. His PSA dropped significantly from 430.7 to 31.3 ng/mL within 3 months after starting leuprolide injection. |
A 42-year-old male was admitted in our cardiac center with a history of dry cough, hoarseness of voice, and chest pain for the past 4 months. Chest X-ray showed a widened mediastinum and dilated descending thoracic aorta with elevated left hemidiaphragm []. Computed tomography (CT) angiography revealed a large fusiform aneurysm of the arch of aorta and proximal descending thoracic aorta, distal to the left common carotid artery with involvement of origin of the left subclavian artery. The descending thoracic aorta showed aneurysmal dilatation till D8 level. The saccular part of the aneurysm measured 8.5 cm × 7.0 cm with total craniocaudal span of the aneurysmally dilated segment measuring 16 cm. Compression and narrowing of the trachea was seen with displacement of the trachea and esophagus toward the right. The left major bronchus was mildly displaced anteriorly. Atelectatic changes were seen in the left lower lobe [].\nThe surgical plan in this case was to exclude the thoracic aneurysm and reduce the volume of the aneurysm to reduce compression on the airways. This aneurysm could have been treated by placing a stent graft, but this would not have reduced pressure on the airways for several weeks to months. Thus, the open surgical repair was planned under cardiopulmonary bypass (CPB) through peripheral cannulation.[] Transesophageal echocardiography was performed perioperatively in this case.\nIn preanesthetic assessment, all laboratory investigations were normal, with arterial pressure (blood pressure [BP]) of 110/70 mmHg and heart rate of 72 beats/min. Preinduction monitoring included electrocardiogram, pulse oximetry, bispectral index, and invasive BP in the right radial and left femoral artery. Femoral vein and femoral artery were prepared for emergency femoro-femoral bypass. After induction of anesthesia and endotracheal intubation, FOB was performed to see the extent of tracheal compression. It showed a narrowed and pulsatile trachea at the level of carina. The left main stem bronchus was also narrowed. The right main stem bronchus was normal in caliber [].\nAfter heparinization, CPB was commenced using the right femoral and right axillary arterial cannulation. Interposition dacron graft of 16 mm was used for anastomosis of thoracic aorta. The left subclavian artery was sacrificed. Before chest closure, a repeat FOB was carried out to see the residual tracheal compression and rule out the presence of tracheomalacia. Bronchoscopy showed a normal caliber trachea at the level of carina []. The left main stem bronchus was also normal in caliber. No residual narrowing or tracheomalacia was seen. The patient developed quadriplegia in the postoperative period, which was successfully managed with cerebrospinal fluid drainage. The patient was extubated on postoperative day 6. Postextubation, the patient was stable with no difficulty in breathing and no stridor. On the 10th postoperative day, the patient was discharged from the hospital. |
An 8-month-old female infant had coronary reimplantation at age 3 months for ALCAPA. Postsurgical ejection fraction showed early improvement with subsequent deterioration. A diagnostic left heart catheterization performed as part of a heart transplant evaluation revealed severe ostial stenosis of the LMCA. She was referred for PCI of the left main coronary artery to relieve her heart failure and preempt transplant. Her physical exam was significant for congestive heart failure and failure to thrive. The patient's echocardiogram showed markedly depressed left ventricular function. A cardiac computed tomography angiography (CTA) and initial diagnostic nonselective root aortogram demonstrated stenosis of the LMCA at the site of ALCAPA reimplantation. Selective left coronary angiogram revealed severe kinking of the reimplanted LMCA at the ostium ().\nThe procedure was performed in the Pediatric Cardiology interventional suite after extensive discussion and planning with Pediatric and Adult Interventional Cardiology, Pediatric Cardiac Anesthesia, Pediatric Cardiothoracic Surgery, and Pediatric Cardiac Radiology. General anesthesia and a femoral arterial approach were utilized. An ascending aortic root angiogram was obtained in 2 planes to assess the ostial left main stenosis. Due to the small size of the infant aorta, a 6 Fr JR-4 guide was reshaped to engage the left main coronary artery. A BMW wire was used to cross the lesion. Since the infant left main coronary artery was small but was expected to grow with age, a somewhat larger (3.0 × 8 mm Vision bare metal) stent was carefully implanted in the proximal LMCA at less than nominal pressure to avoid distal dissection. A poststent angiogram showed that the stent had moved during implantation and missed the ostium with residual stenosis of the LMCA origin. Hence, a 3.5 × 8 mm Vision bare metal stent was placed into the LMCA ostium overlapping with the previous stent distally and protruding 1-2 millimeters in the aorta proximally. The ostium and aortic overhanging portion of the stent were postdilated producing proximal flaring. The final angiogram confirmed excellent stent position and normal flow ().\nThe patient tolerated the procedure well and was discharged home on dual antiplatelet therapy (DAPT) with aspirin and clopidogrel one day after PCI. She was followed in Cardiology clinic post-PCI and noted to have no change in her ventricular function. Nevertheless, she continued to do well clinically until six months post-PCI, at which time she demonstrated failure to thrive and required admission for initiation of continuous milrinone infusion. The left main stent was widely patent with normal Doppler flow signals as visualized by echocardiography (). She was listed as status 1A for orthotopic heart transplantation (OHT) and underwent transplantation one year post-PCI, at age 20 months. |
A 45-year-old female came to the surgical OPD with diffuse enlargement of thyroid gland which was more on left side than right. Patient had noticed this swelling for the last two months and there was a history of difficulty in swallowing since one month and an increase in the swelling over the last two weeks. Patient gave a history of hypothyroidism for which she was taking 100 microgram of thyroxine for the last two years. The patient was on a regular endocrinological follow-up. There was no history of pain, loss of weight, palpitations, giddiness, hoarseness of voice or intolerance to heat. There was no history of irradiation to heat and neck region.\nOn examination, there was diffuse enlargement of thyroid. The gland was visible in normal positioning of the neck (WHO –Grade2 enlargement) measuring about 3.5 cms in its greatest diameter on right side and 3 cms on the left side, it was, firm, non-pulsatile and non-tender which moved with deglutination. No nodule or cystic area was palpable. Ultrasound neck showed diffuse enlargement of the thyroid gland with right lobe measuring 30 mm and left lobe measuring 28 mm.\nThe routine laboratory investigations were normal, including thyroid function tests. Her level of antithyroid peroxidase antibody (TPO) was 65 u/mL.\nFine-needle aspiration of the left lobe of thyroid was done because of the increase in the size of the gland on this side as reported by the patient leading possibly to the difficulty in swallowing. It was performed using a 22G needle. The smears showed hurthle cells in sheets with dense polymorphic lymphoid infiltrate comprising of mature lymphocytes, centrocytes, centroblasts , immunoblasts and tingible body macrophages (, ).\nA few thyroid follicular cells were seen and there was a clump of cells with abundant cytoplasm, showing nuclear grooving and large hyperchromatic nuclei with prominent intranuclear cytoplasmic inclusions (). An FNAC diagnosis of papillary carcinoma with Hashimoto’s thyroiditis was suggested, and patient underwent total thyroidectomy.\nGross/histopathology: Total thyroidectomy specimen was grey white in colour, firm in consistency. Right lobe measured 5x3 cm and left lobe measured 5x4 cms with isthmus measuring 1 cm. External surface was rough and nodular. Cut section was grey white and entire thyroid was replaced by multiple nodules measuring a size from 0.5 cm to 1x1 cm (). No normal thyroid parenchyma was identified. The capsule of the gland appeared intact. Random sections were taken from both lobes. Most sections showed polymorphic lymphocytes in varying stages of maturation arranged in follicles and replacing the normal thyroid tissue. Hurthle cells identified by abundant cytoplasm were seen in clusters and sheets. One of the sections from left lobe of thyroid showed two small foci of papillary carcinoma characterized by clear cells arranged in a fibrovascular core and showing a papillary architecture surrounded by large lymphoid follicles, some showing prominent germinal centres. Thus, the final diagnosis was papillary carcinoma of thyroid with Hashimoto’s thyroiditis. |
A 44-year-old phenotypical male patient, not known to have any chronic medical illnesses, presented to the family medicine clinic as a known case of gender dysphoria. The patient also had symptoms of depression and was severely distressed about his life and was actively seeking a solution to his problem. The patient requested that the treating physician addresses him as a female and uses a feminine alias with him as well as feminine proverbs while speaking with him.\nThe patient's birth had been uneventful, and there had been no significant medical issues. The patient grew up in a household with other male and female siblings and was raised as a male but had secretly identified himself as a female since early childhood. The patient liked playing with feminine toys such as Barbie dolls, enjoyed dressing up in female clothes, and enjoyed wearing artificial female hair attachments. The patient studied in an all-male elementary school and often felt isolated with little sense of connectedness. The patient discontinued intermediate school because he could neither fully bring himself to integrate with other males nor fit into to an all-male environment. The patient would have preferred to attend an all-girl school, form friendships with girls, and be involved in feminine hobbies and interests. The patient recalled an incident of physical harassment that occurred during his childhood but denied any incidents of sexual abuse.\nThe patient was faced with a difficult situation in his family, for it was difficult for them to accept his feminine behavior and mannerisms. The patient had had an unsuccessful marriage to a female partner, but it had ended in divorce because of his impotence and sexual dysfunction. The patient had a history of 4–6 suicidal attempts.\nThe occupational history included being a taxi driver and ladies' hairdresser in many different countries. As a hairdresser, he would dress up as a female and take on a female identity. Currently, the patient works in a private company in Saudi Arabia as a male and has an employer who is very understanding of his situation. He now lives alone with no partners or roommates and prefers to keep his interactions with other people to the barest minimum.\nThe patient started taking over-the-counter combined oral contraceptive pills 13 years ago with neither a prescription nor a physician's supervision. He denies using any recreational drugs. However, the patient admitted to smoking a packet of cigarettes a day and to being an occasional drinker.\nThe patient had low mood, had no interest and had symptoms of depression, but denied any history of psychosis, delusions, and hallucinations. He was assessed for depression during each visit to the clinic using the Patient Health Questionnaire-9 depression scale. He was found to have mild-to-moderate depression but no suicidal thoughts during those visits. However, he refused the antidepression medications.\nThe patient's vital signs were within the normal range for his age and sex. He sat comfortably on the bed, was alert, conscious, and not in any apparent pain or respiratory distress. The general appearance was that of a male, but he lacked facial hair and had no hair on the visible parts of his body. Examination of the chest revealed gynecomastia. Auscultation of both lungs and heart was within normal limits, and no added sounds were noticed. His abdomen revealed a lack of normal male pattern of hair distribution. Abdomen was soft, lax, no tenderness, no masses or organomegaly was noticed. Genitalia examination revealed a normal-sized penis, normal scrotum, but the testicles were smaller than normal for his age and sex.\nThe patient's hormonal panel revealed several abnormalities including low levels of testosterone, luteinizing hormone, and follicle-stimulating hormone. Other abnormalities included elevated levels of prolactin. His thyroid-stimulating hormone, T4, and estradiol were all within normal limits. Abdominal and pelvic ultrasound showed that the internal organs were all normal size and consistency. No masses were noted. No uterus, ovaries or rudimentary female reproductive organs were found. Testicular ultrasound revealed bilaterally descended testes. However, atrophy of both testicles and weak peripheral testicular vascularity were noted. Enhanced computed tomography scan with contrast of the abdomen and pelvis revealed severe hepatic steatosis as well as bilateral gynecomastia. Finally, chromosomal analysis revealed a genotypical male with a 46XY chromosomal constitution.\nGender dysphoria with moderate depression was diagnosed by means of a comprehensive history and clinical presentation. |
An 83-year-old Caucasian man with a history of a branch retinal vein occlusion (BRVO) with macular edema (receiving as-needed intravitreal bevacizumab injections in the right eye and advanced open-angle glaucoma with poor vision in the left eye) presented with new vision complaints. Six months earlier at his previous visit, his visual acuity was 20/20 with resolved branch vein occlusion. He described gradual painless loss of a half-moon of central vision in the right eye over a three-month period. Past medical history was pertinent for known recurrent HER2 receptor positive esophageal adenocarcinoma with metastases to the liver and right pleural base as well as poor cardiac and functional status of 24-month duration. He was previously treated with carboplatin and paclitaxel infusions 11 months earlier and completed local radiation to the primary esophageal mass 13 months earlier.\nBest-corrected visual acuity (BCVA) was 20/150 in the right eye and 20/400 in the left eye. Intraocular pressure was 10 mmHg in the right eye and 12 mmHg in the left eye (normal range 10–25 mm hg). Anterior segment slit lamp examination was unremarkable in both eyes. Posterior segment examination of the right eye was notable for a 10.5-sq-mm choroidal lesion centered along the inferior temporal arcade extending into the inferior macula with serous retinal detachment of the macula (, ) with no additional lesions noted in either eye. In view of patient’s medical history, the mass was presumed to be choroidal metastasis secondary to esophageal adenocarcinoma. Local and systemic treatment options including proton beam irradiation and immunotherapy were discussed with the assistance of the oncology team. Ultimately, immunotherapy with pembrolizumab was selected to treat the lesion to preserve vision and maintain superior quality oflife.\nThe patient was initiated on 200-mg intravenous pembrolizumab therapy every 21 days with the first dose administered three weeks after initial ophthalmologic evaluation. The patient was seen for ophthalmology follow-up one day after the second infusion. BCVA had improved to 20/40–1 in the right eye with marked decrease in size of the choroidal lesion and serous retinal detachment (, ). At 15 weeks following initiation of pembrolizumab infusions the patient’s visual acuity improved to 20/20 (). The lesion height regressed completely (4.4–0.7 mm) and became hypopigmented (). |
A 63-year-old male with progressive dysphagia for 15 days was found to have thickened walls in the mid-esophageal region and tumor thrombus formation in the arch of the azygos vein (Fig. a, S) according to contrast-enhanced CT; this was accompanied by mediastinal lymphadenectasis. Gastroscopy confirmed the presence of a neoplasm in the esophageal lumen located 28–35 cm away from the incisor. Biopsy suggested poorly differentiated carcinoma of the esophagus, and immunohistochemistry showed a tendency for basaloid squamous cell carcinoma (Fig. b). The patient had smoked for 44 years, with 20 cigarettes per day, but had quit smoking for 1 month; the patient had no history of alcohol consumption and denied any history of cardiovascular disease, and no abnormalities were found on the cardiac ultrasound. The patient then received four rounds of neoadjuvant chemotherapy, which consisted of 400 mg Abraxane, 120 mg nedaplatin via intravenous drip on d1, Q3W. Twenty-five days after the last round of chemotherapy, enhanced CT of the chest suggested a significant reduction in the tumor volume, mediastinal lymph nodes, and tumor thrombus in the arch of the azygos vein (Fig. c, S), and general PET-CT showed no distant metastasis.\nOne month later, the patient underwent McKeown radical resection. Intraoperative exploration showed a tumor located at the level of the arch of the azygos vein, measuring approximately 3 cm in length and 3 cm in diameter, with the tumor invading into the esophageal outer membrane. Two enlarged lymph nodes measuring approximately 1 cm in maximum diameter were present around the tumor, and the tumor had a relatively clear boundary with the surrounding tissues. The arch of the azygos vein was hard in nature, wherein the embolus could be palpated (Fig. a, b and c). The operative process included entering the posterior-lateral 5th intercostal space of the right chest to expose the esophageal bed and open the mediastinal pleura; the superior vena cava at the arch of the azygos vein was first dissociated, and sidewall forceps were used to clip the converging point of the superior vena cava and azygos vein. This, in turn, prevented tumor thrombus detachment, and then the normal azygous vein was dissociated at the T6 level and blocked. Branches of the intercostal veins were dissociated seriatim and underwent ligation to prevent tumor thrombus detachment countercurrently. Scissors were then used to open the azygos vein, and further exploration showed a clear boundary between the tumor thrombus and the vascular wall of the arch of the azygos vein. There was no invasion or adhesion between the thrombus and the vasculature. After that, blunt and complete dissection of the embolus at the arch of the azygos vein was performed, and the embolus was approximately 1 cm in diameter and 3 cm in length, with no residual embolus observed. Heparin water was used for washing to detach the azygos vein, and then a 5–0 Prolene propene line was used for suturing the azygous vein. We tried to maintain the integrity of the azygos vein during surgery to maintain venous return and to stabilize the gastric tube because of the relative position in the chest. The total length of the esophagus was dissociated. A median incision in the upper abdomen was performed to make a gastric tube, and esophagogastric anastomosis was completed through a right neck incision.\nPostoperatively, enteral combined with intravenous nutritional therapy was given. The patient received a clear and liquid diet on day 8 after surgery and was discharged without any severe complications. Postoperative pathology revealed the proliferation of fibrous tissue at the submucosal and muscular layers of the esophageal tumor bed, and a multinucleated giant cell reaction was partially observed, accompanied by infiltration of a small number of lymphocytes and plasmocytes (Fig. a). There was no evidence of residual cancer tissue, and complete remission was achieved after neoadjuvant chemotherapy. No metastasis was observed at the mediastinal, celiac, or cervical lymph nodes. Most of the emboli in the azygos vein were necrotic tissues, wherein small focal cancer tissues measuring 3 mm in maximum diameter were present (Fig. b). |
A 26-year-old, otherwise healthy male has reported to the restorative clinic at Al-Farabi College, Riyadh for investigation and management of pain affecting the posterior left region of the maxilla. His history of chief complaint showed typical presentation of severe pain due to advanced buccal cervical carious lesion.\nBased on clinical findings, radiographic interpretation, and vitality tests, a diagnosis of acute pulpitis of upper left first molar was made. No periapical diagnosis was identified. Routine endodontic therapy was planned. Local anesthesia using 2% lignocaine with 1:80,000 epinephrine was administered. Standard routine root canal procedure was performed under rubber dam isolation. The carious lesion was removed and replaced by composite material (Filtek™ P90, 3M). The endodontic access opening was prepared. Working length was established. In addition, cleaning and shaping were performed using crown down technique with ProTaper rotary instruments (dentsply). In addition, obturation was done with lateral compaction. A root canal completion radiograph confirmed the correct root canal therapy. A single dose of 600 mg ibuprofen was prescribed to control postendodontic pain. The patient reported to has an increased erectile function and libido after 2 h from taking the ibuprofen tablet. He has reported that has done three sexual intercourses with his wife at that day. On the next day, he was curious, and he repeated taking the ibuprofen tablet. Surprisingly, he had the same experience of his first use of ibuprofen of having increased libido and erectile function that has lasted for at nearly 6 h. He contacted us to question this unusual experience. His medical history was re-reviewed thoroughly with an endocrinologist, and nothing was mentioned. Furthermore, tests for complete blood count, testosterone, thyroid stimulating hormone, cholesterol pre and post ibuprofen intake were assessed, and all results came up as normal. He also reported to have the same previous experience during the test period. A gynecological ultrasound test was also negative for pathological findings. |
This is a case of a 79-year-old man who underwent contrast-enhanced Brain-CT for loss of consciousness. Past medical history included diverticular disease, COPD, hypertension, atrial fibrillation and appendectomy. Of note, he had no prior history of endoscopic procedures or recent trauma. The CT showed a primary cerebral mass at the temporal right site suspected for a glioblastoma. Hence, he went through a contrast-enhanced Chest/Abdomen CT for staging. No secondary lesions were detected but a small amount of free gas was unexpectedly noted in the abdomen secondary to a suspected colonic diverticular perforation (). Therefore, a surgical consultation was granted. At a first observation the patient was totally asymptomatic. Vital signs were in range. He had a healthy appetite, bowel sounds were active and he was opening his bowels normally. On examination the abdomen was soft and nontender with no distension. Laboratory tests were also normal including WCC (4.72 × 103/μl) and CRP (2.05 mg/dl). To confirm the diagnosis we proposed an explorative laparoscopy that the patient refused as he was feeling well. Few weeks later he underwent neurosurgical intervention and the cerebral mass was resected. The postoperative period was uneventful and he was discharged home on postoperative Day 7.\nUnfortunately, once he returned home his temperature started to rise up to 38°C and he was readmitted to the hospital. A new contrast-enhanced Chest/Abdomen CT showed a bilateral pneumonia and a massive amount of intraperitoneal free gas, mainly in the abdominal upper quadrants (). No fat stranding, nondilated bowel, no vascular abnormalities and no free fluid were detected. Although the CT showed an evident worsening, the patient still remained completely asymptomatic on physical examination. Accordingly, he started to be treated with meropenem for pneumonia (Escherichia coli was isolated into the sputum) and he finally agreed to underwent diagnostic laparoscopy. Surprisingly, that was definitely not a case of pneumoperitoneum but an idiopathic PCI as we found multiple intramural air content cyst, about 2–3 cm in diameter, all around the small bowel loops and mesentery (). No intra-abdominal maneuvers were made. The patient had an unremarkable recovery (Clavien–Dindo classification grade was 1) [] and he was discharged home on postoperative Day 15 in well condition, once he had recovered from pneumonia. |
An 80-year-old Japanese man was referred to our hospital for further exploration of the increase in biliary enzymes, immunoglobulins, bile duct wall thickening, and bile duct dilation.\nHe had a history of cerebral infarction and antihypertensive treatment, but no history or symptoms of inflammatory bowel disease and family history were remarkable.\nHe showed no remarkable signs and symptoms, and physical examination upon admission revealed no jaundice or lymphadenopathy and no abnormal findings on his abdomen.\nLaboratory results on the day of admission revealed an increase in the serum levels of aspartate aminotransferase (75 IU/L), alanine aminotransferase (77 IU/L), alkaline phosphatase (1060 IU/L), γ-glutamyl transpeptidase (1160 IU/L), and immunoglobulin G (1850 mg/dL) and its subtype 4 (255 mg/dL). No increase in bilirubin and tumor markers was seen (Table ). CT scans revealed intrahepatic bile duct dilatation, bile duct wall thickening with a mild enhance effect were noted (Figure ). No changes in the pancreatic ducts or parenchymal structure were seen (Figure ). Abdominal US revealed the wall thickening of the common bile duct with narrowing of its diameter (Figure ). EUS also revealed the thickened bile duct wall and narrowing of its diameter with no significant irregularity of the epithelial surface (Figure ). The changes of the bile duct were confirmed with a MRCP showing the stenotic lesions in relatively in the proximal side including the lower common bile duct (Figure ). ERCP revealed multiple stenoses with long stricter in the lower bile duct and only a partial dilatation of the bile duct (Figure ). There were no abnormal findings at the pancreatic duct (Figure and ). Following the ERCP, a biopsy of the lower bile duct was performed, showing the long stenotic lesion (Figure and , blue arrowheads) and the endoscopic nasobiliary drainage tube was placed for drainage.\nHistological analyses revealed the infiltration of inflammatory cells and severe storiform fibrosis in the bile duct tissue (Figure ). These inflammatory cells included the IgG (Figure ) and IgG4 (Figure ) positively-stained plasma cells (more than 10 cells per high power field) and the IgG4/IgG-positive cell ratio was approximately 30% (Figure and ). To differentially diagnose PSC, liver biopsy was performed. The tissue showed mild infiltration of inflammatory cells (Figure ) partly stained with IgG (Figure ) and IgG4 (Figure ), but no onion-skin fibrosis was seen. |
A 56-year-old male without any previous medical history presented to our emergency room (ER) with multiple traumas from a 10 meter fall in a construction field. Physical examination revealed a male patient with a body mass index in the normal range and an acutely ill looking appearance. His right lower leg and ankle were swollen and bruised, and he had a 2 cm laceration wound on the plantar aspect of his right foot. The patient's right ankle had limited range of motion due to pain. The patient had tenderness at the right anterolateral aspect of the mid lower leg and anterior aspect of the ankle. There was grade 1 anterolateral instability of the left ankle. The neurologic examination was normal. Based on the patient's clinical history and physical examination, the orthopedic surgeon suspected a fracture of the right fibular diaphysis and ligament injury of the right anterolateral ankle.\nInitial radiographs of the ankle in the anteroposterior and lateral views showed fractures at the diaphysis at the fibula and anterior lip of the tibial plafond (Fig. ). The patient was not able to undergo ankle Mortise view because of his limited range of motion due to extreme pain. In a subsequent lower extremity computed tomography (CT), the orthopedic surgeon in the ER noticed a segmental fracture of the right fibular shaft and the anterior lip of the tibial plafond.\nTo evaluate the ankle ligaments, a turbo spin-echo (TSE) two-point mDixon technique applied to an ankle MRI (Table ) was performed after procuring written informed consent. In addition to the fractures of the right fibular shaft and tibial plafond, this MRI demonstrated a tiny chip fracture of the lateral talar dome. A tiny wafer-shaped talar dome chip fracture fragment about 7 (anterior–posterior diameter) × 3 (head to toe diameter) mm was clearly delineated only in the sagittal T2-weighted mDixon opposed-phase MRI (Fig. B). In T2-weighted mDixon in-phase imaging, which is considered a conventional T2-weighted image, there was a definite focal wedge-shaped cartilage defect at the corresponding area. However, there was only focal and subtle cortical irregularity and the cortical step-off was not definite (Fig. C). In a T2-weighted mDixon water-only image, which is considered a conventional fat-suppressed T2-weighted imaging, the cartilage lesion and focal cortical irregularity were once again noted, and the subcortical bone marrow edema was additionally confirmed. In these 2 sequences, a fracture was suspected, but the radiologists could not fully delineate the fracture line (Fig. D). In T2-weighted mDixon fat-only imaging, there were dark signal alterations at the subcortical region, but these were not considered fractures (Fig. E). T1-weighted imaging was obtained in the axial plane, and the fracture line was not depicted in this plane (Fig. F). In a CT image reviewed by an experienced musculoskeletal radiologist, there was a lateral talar shoulder cortical fracture at the identical area where the chip fracture was noted (Fig. A) from the T2-weighted mDixon opposed-phase image. In addition, there was a grade 2 injury to the anterior talofibular ligament with severe subcutaneous swelling of the ankle.\nDuring ankle arthroscopy, there was a free floating osteochondral fragment about 4 x 8 mm at the posterolateral talar shoulder, which was removed with basket forceps (Fig. ), and microfractures were performed at the posterolateral talar cortical fracture site.\nThe patient did well after the arthroscopy with recovery of full range of motion after 2 months. |
Our patient, a 66-year-old Caucasian man, was diagnosed with MF 4 years ago. He was a high-risk patient according to the Dynamic International Prognostic Scoring System-plus classification because of a platelet (PLT) count of <100 giga/L, transfusion-dependency, hemoglobin (Hb) <100 g/L, circulating blood blasts >1 %, complex karyotype, leucocyte count <25 giga/L, and no constitutional symptoms []. His medical history was unremarkable except for peripheral artery disease treated with acetylsalicylic acid. He had neither a personal nor a family history of spontaneous bleeding.\nAt diagnosis, for MF-related anemia, we started a therapy with recombinant human erythropoietin that was stopped after 3 months for lack of efficacy. He was not considered fit enough for bone marrow transplantation by a specialized center because of his age and a lack of a suitable donor. He received regular red blood cell transfusions, without chelation therapy. Ruxolitinib was contraindicated due to thrombocytopenia.\nIn order to ameliorate the anemia an indication for splenectomy was given and performed by our regional referral center. The preoperative tests showed a prolonged activated partial thromboplastin time (APTT; 43.8 seconds, normal range 26.8 to 42 seconds). No further investigations were done at that time. Immediately after the intervention he experienced hemorrhagic shock due to bleeding of his phrenic artery which needed a second surgery for hemostasis.\nHe presented significant abdominal pain 35 days after splenectomy that led to a CT scan which showed a hematoma in his pancreatic loge. We referred him to our regional center for a percutaneous drainage. He was finally readmitted to our Department for the postoperatory phase. Repeated laboratory tests found a gradual elongation of APTT: 52 seconds on day 51 and 114 seconds on day 61 from splenectomy (normal range, 26.8 to 42 seconds). A physical examination detected a large ecchymosis on his left arm. The same day we completed coagulation tests that showed a deficiency in FVIII coagulant activity (FVIII: C): <1 % (normal range, 50 to 150 %). Prothrombin time (PT), von Willebrand factor and other clotting factors (II, V, VII, IX, X, and XI) were all normal. FVIII inhibitor (AAFVIII) was positive at a titer of 17.3 Bethesda units (BU). He was diagnosed as AHA based on the above findings (day 0).\nMoreover, hematological examinations showed leukocytosis (white blood cell count 24×109/L) with marked leukoerythroblastosis (blasts cells 25 %), Hb of 113 g/L, and thrombocytopenia (PLT count 57×109/L). These findings were consistent with the diagnosis of leukemic transformation according to the World Health Organization (WHO) criteria []. A bone marrow examination was not performed due to the excessive bleeding risk.\nImmunosuppressive therapy with methylprednisolone 1 mg/kg body weight was promptly started. Due to the apparition of deep muscle hematomas of his arms and legs we added recombinant activated factor VII (rFVIII; NovoSeven®, Novo Nordisk) 90 mcg/kg every 2 hours. On day 4 from AHA diagnosis we associated rituximab (MabThera®) 375 mg/m2 weekly for 4 weeks to the ongoing steroid therapy. Due to a lack of response, on day 6 we stopped the rFVIII and started activated prothrombin complex concentrate (aPCC; FEIBA®) intravenously at a dose of 80 unit/kg every 12 hours. aPCC was subsequently decreased to 80 unit/kg every 24 hours on day 10, then stopped at day 18.\nTo treat the leukemic transformation of the MF that probably was associated to the AHA, on the sixth day our patient started chemotherapy with azacytidine (5-AZA) administered subcutaneously at 75 mg/m2/day for 7 days []. Red blood cell and PLT transfusion were also carried out as supportive therapy. After 10 days of treatment his edema and ecchymosis showed a marked improvement. The AAFVIII rose to 179 BU on day 12 and descended to 25 BU on day 33.\nDuring the post-chemotherapy phase he presented Staphylococcus hominis bacteremia treated with intravenous vancomycin. Subsequently he was healed for a probable Pneumocystis pneumonia by co-trimoxazole and atovaquone. He was referred to our out-patient care department on day 40.\nOn day 57, before his second cycle of 5-AZA, his FVIII: C was elevated to 30 % with no additional bleeding episodes; his APTT was decreased to 72 seconds. AAFVIII was decreased to a titer of 6.6 BU. Evolution of rates of FVIII (VIII: C), rates of AAFVIII, and therapy administered are summarized in Fig. . |
A 17-year-old male presented with dull aching pain and gradually progressive swelling over the left scapular region since five months. Pain was aggravated on shoulder movement and on lying supine. He had no fever. Physical examination revealed a swelling of 3 X 3 cm which was located in the left supraspinous region. The swelling was warm and tender with soft cystic consistency. There was no discharging sinus. Terminal range of motion was restricted with pain especially during the last 15° of abduction.\nHis blood investigations were unremarkable with ESR of 15 mm/hr. Anteroposterior radiograph of left shoulder revealed a well defined, 4 X 3 cm osteolytic lesion at the superomedial aspect of body of scapula, with surrounding sclerosis []. The lateral radiograph showed increased soft tissue shadow in the superior aspect of scapula. His chest radiograph was normal. Since appearance was similar to a bone tumor, differentiation was important and work up on same lines was planned. Whole body bone scan showed abnormal increase in tracer uptake in the body of scapula with the rest of the skeleton being normal. Computer tomography (CT) showed erosions involving the supero-medial aspect of the body of scapula and along the base of the spinous process of scapula [ and ]. Magnetic resonance imaging (MRI) scan revealed a well- defined lobulated lesion []. It showed high signal on T2W and low signal on T1W images. Central T2W hypointensity was also seen. There was a soft tissue component extending posteriorly into the subcutaneous tissue. The left shoulder joint was normal. The differential diagnosis were chronic infection probably tuberculosis, Eosiniphilic granuloma or tumourous conditions. A CT guided biopsy was done Tissue and fluid were sent for cultures and histopathology. Biopsy revealed granulomas composed of epithelioid cells, lymphocytes and a few multinucleate giant cells suggestive of tuberculosis. Culture from Lowenstein Jensen medium showed acid fast bacilli after six weeks.\nHe was started on antitubercular therapy for a period of nine months. He was on two months of intensive therapy and seven months of continuation therapy. The dosage of the drugs given were Cap. Rifampicin 600 mg, Tab. Isoniazid 450 mg, Tab. Ethambutol 800 mg, Tab. Pyrazinamide 1250 mg, Tab. Pyridoxine 10 mg on daily basis. He had complete resolution of symptoms within four months. At one year follow up, he had painfree full range of movements of shoulder. |
A 40-year-old woman was admitted to our institution presenting with slowly progressing dyspnea for the last 6 months. She also exhibited non-specific thoracic pain. The discomfort and subjective shortness of breath were initially attributed to musculoskeletal symptoms. Lung function tests demonstrated no significant restriction of the lung volume. A chest CT scan with an intravenous contrast agent was performed to first rule out interstitial lung disease and further pathology of the chest (128 row multislice scanner, Somatom Definition Flash, Siemens Healthcare, Erlangen, Germany; 70 mL of Iomeron 400 i.v. Bracco, Milano, Italy; 4 mL/s flow; reconstructions with 1 and 5 mm axial slice thickness, 5 mm maximum intensity projection in the axial and coronal planes).\nChest CT revealed a left-sided incomplete occlusion of the left main pulmonary artery and peripheral complete occlusions of the segmental braches on the same side. On the right side, the main pulmonary artery was nearly completely occluded by a hypodense structure ( and ). The filling defect started at the level of the pulmonary valve, across the bifurcation of the pulmonary trunk, and showed progression in the central and segmental pulmonary arteries on both sides. In the absence of any obvious enhancement, the obstruction was first interpreted as an embolus. Because of the inconsistency of the CT findings and the clinical presentation, concerns arose regarding the potential for another entity, such as a primary tumor of the heart. Next, a high resolution PET-CT scan of the chest was performed (tracer 371 MBq F-18-FDG; Swan Isotopen AG, Bern, Switzerland). The luminal lesions exhibited peripheral FDG uptake, which can indicate tumor tissue but also may be due to granulations in the periphery of a thrombus.\nThe patient was submitted to surgery. After resection of the pulmonary trunk, the surgeons delivered a myxoid tumor adherent to the pulmonary valve ( and ). Intraoperative histological evaluation revealed an intimal neoplasia arising from the vessel wall (). During the same procedure, the surgeons removed the affected valve, the pulmonary trunk, and both pulmonary arteries. A valve-bearing conduit connecting the right outflow tract to the distal pulmonary artery on the right was placed. The left lung was then resected. At the 1-year follow-up examination, we found a tumor recurrence along the right cardiophrenic angle and multiple pleural metastases on chest CT (). Despite operative revision, tumor recurrence was evident on a follow-up MRI at 6 months (3 T MRI scanner, Skyra, Siemens Healthcare, Erlangen, Germany; chest and abdomen; 7.5 mL MultiHance i.v., Bracco Suisse SA; protocol consisted of Haste axial/coronal; Trufi axial; Flash 2 axial fat sat axial; T1 Vibe fat sat coronal; Flash 3D dynamic acquisition coronal) with a recurrence of the involvement of the right cardiophrenic angle. An infradiaphragmatic tumor that had spread through the phrenic hiatus along the inferior caval vein into the liver was observed. Furthermore, there was concern of a new pericardial implantation with continuous invasion of the pericardial sac ( and ). Because of the extent of the local tumor and its inoperability at this stage, palliative care was carried out. |
A 67-year-old Chinese man was referred to our hospital with diarrhea for more than 5 mo and for management of early colon cancer discovered by colonoscopy in another hospital.\nThe patient visited a hospital for the first time due to diarrhea of yellow watery stool 8-9 times per day on December 20, 2018. Colonoscopy suggested colon polyps with a pathology of tubular adenoma. No significant improvement was observed in the symptoms after antidiarrheal treatment. The patient underwent colonoscopy in another hospital again, which revealed multiple polyps in the liver curvature of the colon, cecum, and descending colon on April 23, 2019. The biopsy pathology of the polyp (liver curvature) in our hospital’s consultation indicated villous adenoma, and some glands showed moderate-to-severe dysplasia with focal early carcinogenesis, roughly showing in situ adenocarcinoma changes. Therefore, the patient was admitted to our hospital for evaluation and treatment of early colon cancer.\nThe patient underwent right lung nodule resection 1 year prior and lumbar disc herniation surgery 28 years ago. He had no history of other diseases or allergic drugs. He had a smoking history of 30 years and had quit smoking for 8 years. His younger brother died of lung cancer. The patient had no familial history of genetic diseases.\nThe patient’s personal and family history was unremarkable.\nOn physical examination upon admission, the patient’s height and weight were 160 cm and 55 kg, respectively, and he had a blood pressure of 96/58 mmHg and pulse rate of 80 beats/min. There were no other pathognomonic signs during physical examination, except for enhanced bowel sounds at 8 beats/min.\nAfter admission, the patient underwent thorough evaluations including routine blood tests, routine urine tests, routine fecal tests, occult blood tests, blood biochemistry, infection indices, and serum tumor markers. He had hypoproteinemia and hypokalemia with serum albumin and potassium levels of 29.61 g/L and 3.37 mmol/L, respectively. The C-reactive protein level was 7.79 mg/L. His erythrocyte sedimentation rate and white blood cell count were normal. He had a slightly elevated carcinoembryonic antigen level of 5.11 ng/mL. The levels of IgG antibodies to Epstein-Barr virus capsid and nuclear antigen were more than 50.00 and 7.42 AU/mL, respectively. The antinuclear antibody was positive, with a titer of 1:3200. Immunoglobulin E significantly increased with a value of 265.30 IU/mL. The fecal occult blood test results were negative, and stool flora analysis was normal. No other significant abnormal laboratory results were recorded in this patient.\nChest computed tomography (CT) showed a clear lung field (Figure and ). We observed a liver cyst and part of the small intestine that was slightly dilated on abdominal CT (Figure and ). Chest CT on April 14, 2020 revealed new nodules in the lower lobe of both lungs. However, multiple masses in both lungs had progressed more than before excluding tumorous lesions on chest CT on May 31, 2020 (Figure and ). Abdominal CT revealed multiple nodules in the right lobe of the liver and multiple soft tissue nodules in the abdominal cavity with a greater possibility of tumor and ascites, and the colorectal wall was slightly thickened (Figure and ).\nColonoscopy in our hospital revealed a hemispherical polyp with a diameter of approximately 5 mm in the liver curve and a long-pedicle polyp in the descending colon, with a lobular pattern of 10 mm in diameter at the tip. At the same time, it was found that the terminal ileum, ileocecal valve, and entire colonic mucosa were slightly swollen, with pit patterns of types I-II, which were finely grained (Figure ).\nIn addition, gastroscopy showed swelling of the whole gastric mucosa and flaky erosions in the gastric angle and antrum with fine granular mucosa. The mucosa of the descending duodenum was also swollen in the form of fine granules (Figure ).\nAfter admission, we performed endoscopic mucosal resection to remove the two polyps located in the liver curve of the colon and descending colon, respectively. Simultaneously, the entire colonic mucosa was swollen and finely granular. Routine hematoxylin and eosin staining revealed tubular adenoma with diffuse proliferation of atypical lymphocytes in the mucosal layer in the polyp of the liver curve.\nThe polyp of the descending colon was diagnosed as villous adenoma with focal carcinoma–adenocarcinoma (moderately differentiated) limited to the mucosal layer. However, the high-magnification view showed that diffusely proliferated atypical lymphocytes infiltrated mainly in the mucosal epithelium and lamina propria, partially invading the submucosa. The lymphocytes had a relatively simple morphology and medium-sized cell bodies containing few cytoplasm and large oval or irregular nuclei with some visible nucleoli (Figure ). Additionally, immuno-histochemical staining was positive for CD3, CD7, CD8, CD43, and Bcl-2 but negative for CD20, CD4, and CD56, and the Ki-67 index was approximately 80%. In situ hybridization, Epstein-Barr virus-encoded RNA, and IGH, IGK, and IGL rearrangement were negative. |
The patient was a 54-year-old Hispanic male who presented with a one-day history of sudden onset of severe right flank and right lower quadrant abdominal pain with radiation to the right inguinal area accompanied by nausea and vomiting. The patient denied any fever or chills and no significant family history was reported. On examination, the abdomen was soft with minimal tenderness on deep palpation of the right lower quadrant without guarding or any distention. Laboratory workup revealed evidence of elevated creatinine, but leukocyte count was within normal limits. A contrast-enhanced CT of the abdomen and pelvis revealed an obstructing stone at the right ureterovesical junction (Figures -). The appendix was visualized to be a well-circumscribed and partially calcified cystic structure measuring 3.2 x 2.5 x 4.5 cm; there was no radiographic evidence of inflammatory changes.\nThe patient’s clinical picture was initially attributed to ureterolithiasis and was treated accordingly. However, the cystic structure on the appendix was concerning for a mucocele. Consequentially, the decision was made to take the patient to the operating room for a diagnostic laparoscopy with a possible appendectomy. Intraoperative findings were significant for an enlarged appendix (measuring approximately 8 x 1.3 cm) with a thickened partially calcified wall, without evidence of inflammation. An enlarged and elongated cystic structure was noted in the mid-appendix. The rest of the abdominal cavity was unremarkable, without mucin or free fluid noted. A laparoscopic appendectomy was performed in the usual fashion. However, given the intraoperative findings, the decision was made to include a distal segment of the cecum in the resection. No surgical complications were encountered during the procedure.\nThe patient's postoperative course was uneventful and final pathology was obtained three days after surgical intervention (Figures , ; Figures -), reporting a well-differentiated low-grade appendiceal mucinous adenocarcinoma confirmed by the muscularis propria with an estimated size of 1.2 cm, as well as a well-differentiated NET on the distal half of the appendix invading the subserosa without the involvement of the visceral peritoneum or lymphovascular invasion with an estimated size of 1.1 cm and a mitotic rate of less than 2 mitoses per 10 HPF and a KI-67 of less than 3%. The margins were uninvolved by the tumor. Surgical resection was considered appropriate, and no further treatment was required. |
A 3-year-old female child reported with the chief complaint of bleeding from the oral cavity and injury to the upper front teeth following fall from bed 10 h ago. Clinical examination revealed continuous gingival bleeding along with subluxation of 62 []. Medical records and examination revealed that the child was a prediagnosed case of “Glanzman's thrombasthenia” with sickle cell disease “AS” pattern. The patient was diagnosed for sickle cell disease at the age of 1½ years. The patient had a history of blood transfusion at the age of 1 due to decreased level of Hb up to 4.7 g/dl and had a swelling over the forehead. Computed tomography scan was normal. The patient also had spontaneous swelling over the knee that resolved without medication after 5/6 hours. There was a history of prolonged and continued bleeding from the tongue bite while eating and hospitalization for the same at the age of 2. Minor needle pricks at immunization sites also resulted in prolong and excessive bleeding. General physical examination of the patient revealed multiple ecchymotic patches and bruises on skin of the face, trunk, and limbs [Figure and ] that would resolve spontaneously. There was no history of consanguineous marriages in the family. After traumatic dentoalveolar injury, the child received topical application of hemocoagulant (“Botroclot”) at home for control of bleeding. However, blood oozing continued for 10 h during previous night before reporting to medical and dental hospital. Thorough oral clinical examination was done, however, radiographic examination was not possible due to severity of bleeding and child's anxious psychological status. Clinically, oozing was aggravated even by normal tongue movements and frequent contacts of tongue with the injured tooth. In view of patient's medical systemic illness and clinical condition, a treatment regimen was determined. A rigid splint was decided to be given in 61, 62, and 63 region using 21-gauge wire and glass ionomer cement (Xtracem Medicept Dental United states) as a luting agent. After splinting the injured tooth, topical hemocoagulant (Botroclot) was applied with soaked sterile gauze for 15–20 min after which the bleeding was controlled []. The patient was further kept under observation for 1 h to monitor further oozing if any after treatment. There was no evidence of further bleeding after splinting the injured tooth and hence the patient was relieved from the operatory and was advised prophylactic antibiotic and anti-inflammatory drugs. The patient was recalled for check-up after 2 days. Normal healing was evident and also the patient was comfortable and playful. Parents were instructed for home care in terms of soft diet to the child for 15 days, brushing teeth after each meal with a soft brush, and topical application of chlorhexidine gel twice a day for 1 week to prevent infection in the periodontal tissues. The patient was recalled after 10 days and splint was removed []. While taking photograph postoperatively, again bleeding was started in 73 tooth which was controlled by topical botroclot application. Anticipatory guidance was given to parents about oral hygiene maintenance and prevention of further episodes of orofacial trauma. |
A 69-year-old male presented with a history of dysphagia and weight loss. Upper gastrointestinal endoscopy revealed a large ulcerative mass involving the gastric cardia, and its biopsy revealed signet-ring cells, suggestive of adenocarcinoma. A staging computed tomography (CT) scan revealed diffuse thickening of the gastric cardia with no enlarged lymph nodes or distant metastases. In addition, tumor markers (carcinoembryonic antigen and carbohydrate antigen 19–9) were within normal limits.\nBased on these findings, a multidisciplinary oncology team decided to initiate neoadjuvant chemotherapy that aimed to downstage the tumor after evaluation using diagnostic laparoscopy. Because no evidence of metastatic disease was noted, three cycles of chemotherapy with cisplatin and capecitabine were administered. Repeated CT scan revealed disease regression; hence, surgical management was chosen.\nThe surgery started laparoscopically but was converted to open owing to dense gastric adhesions to the pancreatic body, which was suspicious for tumor invasion. On exploration, there was no evidence of peritoneal dissemination or distant metastasis. Because of the high body mass index and difficulty in the surgery, total gastrectomy with D1+ instead of D2 lymph node dissection and Roux-en-Y esophagojejunostomy was performed. Histopathological examination of the obtained specimen revealed negative margins and 36 disease-free lymph nodes. Adjuvant chemotherapy in the form of an XP regimen (six cycles of oral capecitabine [1000 mg/m2] twice daily on days 1–14 and intravenous cisplatin [80 mg/m2] on day 1, every 3 weeks) was initiated, and the patient had regular follow-up at the surgical clinic until 1 year after the surgery, with no evidence of disease recurrence.\nTwo years after the surgery, the patient presented to the emergency department with a 2-day history of abdominal pain, vomiting and constipation, presenting a clinical picture of intestinal obstruction. He reported having similar episodes over the last 2 months, which spontaneously resolved.\nPhysical examination suggested massive ascites. Laboratory test results, including those of tumor markers and acid-fast stain, were within normal limits. An abdominal CT scan revealed dilated small intestinal loops at the center of the abdomen surrounded by a thin membrane and tethered to a peritoneal mass []. Furthermore, massive ascites exerted a mass effect on the intestinal loops and liver with diffuse thickening of the parietal and visceral peritoneum []. Such radiological findings were typical for SEP.\nConservative management with intestinal rest and nasogastric decompression was initiated, which resulted in some improvement. An ultrasound-guided paracentesis was performed, which yielded around 4 L of clear fluid. On analysis, the fluid was negative for any organism, and the cytology results did not reveal abnormalities.\nBecause the findings on paracentesis were unremarkable and there was no evidence of local recurrence in the surgical bed based on the CT scan examination, the patient was prepared for diagnostic laparoscopy. On exploration, massive ascites was observed along with a thick, white membrane covering the entire small intestine, colon and liver. In addition, there were extensive adhesions within the intestinal loops and peritoneal nodules. Multiple biopsies were obtained from the encasing membrane and peritoneal nodules. Histopathological examination of the peritoneal nodules revealed a metastatic adenocarcinoma, and the resected membrane consisted of dense, hypocellular fibrocollagenous tissue. The tumor cells showed immunohistochemistry positivity for PanCK, CK7 and CK20 and partial positivity for epithelial membrane antigen.\nIn view of the advanced disease, no further intervention was planned. Our patient remained on palliative care until he passed away 1 month after his initial presentation to the emergency department. |
A 34-year-old female with recurrent ischaemic stroke, type 1 diabetes mellitus, hypertension, dyslipidaemia and stage 2 chronic kidney disease, presented to the outpatient stroke clinic in June 2020 with a history of recurrent transitory right leg weakness and right hand shaking. These episodes occurred several times per day lasting approximately 30 s whilst mobilising and resolved on sitting. Neurological examination demonstrated recovering dysphasia and mild pyramidal right-sided weakness requiring a walking stick for gait assistance.\nPreviously, in March 2019, she presented with slurred speech, left-sided weakness and visual neglect with confirmed bilateral embolic middle cerebral artery (MCA) territory infarcts on magnetic resonance imaging (MRI). Computer tomography (CT) carotid angiography and Digital subtraction angiogram (DSA) identified severe ICAD with bilateral occlusion of the supraclinoid segment of the internal carotid arteries but no features of moyamoya disease (Fig. ). She had no family history of stroke, a negative thrombophilia screen and no cardioembolic source identified. Secondary prevention was commenced including aggressive lipid lowering with rosuvastatin aiming for low density lipoprotein < 1.8 mmol/L, dual antiplatelet therapy with aspirin and clopidogrel for three months, antihypertensive treatment aiming for blood pressure < 130/80 mmHg and augmentation of her diabetic regime. She recovered to a point of supported independent living but was unable to return to work.\nIn March 2020 she represented to the emergency department with reduced consciousness in a state of diabetic ketoacidosis secondary to a lower respiratory tract infection and episodes of vomiting. On presentation her blood pressure was 70/40 mmHg and she was found to have new right-sided weakness. Diffusion weighted MRI identified multiple acute embolic infarcts in the left MCA territory. Her deficits improved with a period of neurorehabilitation but she had ongoing high-level language and cognitive difficulties, marked post stroke fatigue as well as reduced high-level balance. She was independent in personal care.\nIn neurorehabilitation initial concerns were for post stroke focal motor seizures causing her transient symptoms and she was commenced on levetiracetam. However, these brief episodes spared the face, did not exhibit Jacksonian march and were precipitated by periods of reduced cerebral perfusion such as changing position and dehydration. When reviewed in stroke clinic the leading differential diagnosis was cerebral hypoperfusion syndrome. A 99mTc-ethyl cysteine dimer cerebral perfusion single photon emission CT (SPECT) with CT acetazolamide challenge was performed (Fig. ). Baseline perfusion was reduced in the left frontal, temporal and parietal lobes in keeping with recent infarction as well as a small fixed perfusion defect in the right frontal lobe corresponding to previous established infarct. Although asymptomatic during scanning, worsening hypoperfusion to the left frontal lobe and to a lesser extent the left temporal and anterior parietal lobes, was evident with acetazolamide challenge, indicative of reduced vascular reserve in these territories. Given her ongoing symptoms and declining quality of life a repeat DSA was performed to assess for possible sites of surgical revascularisation. The second DSA demonstrated progressive intracranial stenosis with opacification of the left posterior communicating artery and collateral supply to the left hemisphere predominantly from the posterior choroidal artery and posterior cerebral artery (Fig. ). No feasible site for surgical revascularisation was identified. She was managed on maximal medical therapy including long term dual antiplatelet therapy, consolidated antihypertensive regime, tight glycaemic control and avoidance of dehydration. Over the following six months a systolic blood pressure between 120 and 130/70 mmHg was achieved, with a low-density lipoprotein of 1.0 mmol/L and high-density lipoprotein of 1.2 mmol/L. Glycaemic control remained an ongoing challenge, with a glycosylated haemoglobin level of 9.5% (80 mmol/mol). The patient has recently trialled a continuous infusion pump device. Levetiracetam was ceased. During this period the limb-shaking TIAs settled. |
A 28-year-old male patient presented to our clinic with neck pain. He had neck and right upper extremity pain for two years. Pain was especially on the right shoulder and was extending to the right wrist and hand. His past medical history was unremarkable with no trauma reference. He experienced no benefit from medical treatment. His complaints increased during the last 1.5 months. We admitted the patient to our clinic for further examination and treatment.\nAt physical examination, cervical neck movements were limited and painful in all four directions and there was local tenderness on the right side of C5 and C6 dermatomes. Muscular strengths were normal. There was no sensorial deficit. Deep tendon reflexes were all normal. Hoffman reflex and clonus were bilaterally negative.\nThe patient was referred to the radiology department for further evaluation of the spinal region. Cervical radiographs, computed tomography, and magnetic resonance imaging were performed. No significant lesion was detected in anteroposterior and lateral radiographs. In cervical CT (computerized tomography), there were coarsening of bifid lamina of C5 vertebra and a hyperintense expansile lesion on the lamina (). In cervical spinal MRI (magnetic resonance imaging), there was mild expansion on lamina of C5 vertebra and there was a lesion that was hypointense on both T1 and T2 weighted images and compatible with a sclerotic, osteoblastic lesion like osteoma ().\nThe patient was operated with the primary diagnosis of an expansile sclerotic, osteoblastic lesion on the bifid lamina of C5 vertebra. During the operation, a vertical incision was performed between the levels of spinous processes of C3 and C7 vertebrae. Bifid lamina of C5 vertebra was seen and the expansile lesion on the lamina was removed microscopically following a right-sided hemilaminectomy. The tumoral lesion was reported as osteochondroma in the histopathological examination (). Skeletal scintigraphy demonstrated mild radiotracer uptake only on the right C5 vertebral lamina.\nThe patient had no neurological deficits after surgical treatment. His pain on the right upper extremity disappeared just after the surgery. The patient was then discharged and recommended for clinical control. |
A 72-year-old, right-handed man with a past medical history of urothelial carcinoma of the prostatic urethra presented with left homonymous hemianopsia and headaches. The patient was previously diagnosed with urothelial carcinoma of the prostatic urethra when he was 68 years old. At that time, he was treated with total cystourethrectomy. The pathological diagnosis revealed urothelial carcinoma with no metastasis to the lymph node but invasion into the vein []. At the time of urothelial carcinoma diagnosis, computed tomography (CT) scan of the chest showed a tumor in the upper right lobe of the lung, which was diagnosed as adenocarcinoma with a transbronchial lung biopsy. Three months after total cystourethrectomy, the patient was treated with a left upper lobectomy of the lung and dissection of the lymph node with video-assisted thoracic surgery. The pathological diagnosis was adenocarcinoma of the lung with no metastasis to the lymph node and was considered to have no relationship to urothelial carcinoma.\nA follow-up abdominal CT scan 1 year after cystourethrectomy showed metastasis of urothelial carcinoma to the lateral external iliac of the right lymph node. He was treated with six cycles of gemcitabine and cisplatin chemotherapy. During post-chemotherapy follow-up, other metastases were not found.\nThree years after cystourethrectomy, the patient presented with left homonymous hemianopsia and headaches. Neurological examination upon admission revealed no other neurological deficits. Magnetic resonance imaging (MRI) showed an irregularly-shaped, heterogeneously-enhanced mass with gadolinium enhancement on T1-weighted images [] surrounded by marked edema in the right occipital lobe and the calcarine area. Metastatic tumors were not found elsewhere. The diameter was approximately 30 mm and was considered to have partial attachment to the dura mater. We removed the tumor via a right occipital craniotomy. The tumor had attached to the dura and so we incised it from the edge of attachment. The tumor was soft, yellowish-gray, and fairly well-demarcated from the surrounding brain with no apparent feeding artery. The pathological conclusion was that this was a well-differentiated urothelial carcinoma with necrosis, which was considered to have metastasized from the urothelial carcinoma of the prostatic urethra [].\nPostoperative MRI showed gross total removal of the tumor []. Whole brain radiation therapy, 30 Grays in 10 fractions, was performed following tumor removal. The patient had no other neurological deficits except for left homonymous hemianopsia. |
A 29-year-old male with no known past medical history was referred to the office for lower urinary tract symptoms and hematuria. He described a three-year history of symptoms including decreased force and caliber of his stream, difficulty urinating, and intermittent gross hematuria. He denied any smoking history, fever, dysuria, or flank pain. A urine sample was submitted for urinalysis, which was negative for signs of infection, and urine culture, which was also negative. He was treated with tamsulosin and was advised to return in two weeks for evaluation with in-office cystoscopy if the symptoms persisted.\nThe patient returned to the office two weeks later with persistent lower urinary tract symptoms. A repeat urine sample was submitted for analysis the prior day, which was again negative. He then underwent in-office cystoscopy which revealed an inflammatory-appearing mass at the bladder neck and trigone and base of the prostate gland. The urethra was normal without strictures. A computerized tomography (CT) urogram was ordered to further evaluate for any lesions due to hematuria. Transurethral resection of the bladder tumor was scheduled for the operating room.\nOutpatient CT urogram was significant for posterior wall thickening with low levels of enhancement without any lesions (Figure ). Two weeks after undergoing CT evaluation, the patient returned to the operating room for cystoscopy. Cystoscopy in the operating room showed cystic lesions at the level of the prostate extending to the trigone of the bladder (Figures , ). The mass was partially obstructing the prostatic urethra. Transurethral resection of the tumor was performed, and samples were sent for pathology. Although pathology examination was negative for carcinoma, it demonstrated urothelium with florid cystitis cystica et glandularis with adequate tissue sampling proven by the presence of focal muscularis propria (Figure ).\nDuring his postoperative follow-up in the office, the patient reported resolution of his voiding symptoms. Urinalysis demonstrated microscopic hematuria, likely due to the recent procedure, and no evidence of infection. Due to the posterior wall enhancement seen on the CT urogram, a discussion was held regarding the potential need for re-resection and biopsy. Meanwhile, the patient reported significant alleviation of his voiding symptoms after elimination of the mass at the base of his prostate. |
A two-year-old male presented to our department with a palpable asymptomatic mass in his right palm that was present at birth and had subsequently exhibited a slow and steady pattern of growth (). Imaging showed the typical features of venous malformations []. Doppler ultrasonography indicated the presence of an irregularly shaped space-occupying lesion with multiple chambers and a low level of blood flow between the flexor retinaculum and the external flexor tendons, next to the branch of the nerve innervating the thumb and index finger, and magnetic resonance imaging (MRI) showed a heterogeneous signal, with hyperintensity on T2-weighted imaging and after gadolinium administration, and hypointensity on T1-weighted imaging.\nIt was decided to excise the mass in order to verify the diagnosis of a vascular malformation, and to control the growth of the tumor. Surgery showed a purple mass that was 2.5 cm long (), and interfascicular microdissection of the median nerve was required; nevertheless, neither nerve grafts nor sacrificing any fascicle was necessary. Despite the performance of internal neurolysis, neither sensory nor motor symptoms were observed in the postoperative period.\nHistopathological analysis revealed ectatic channels, surrounded by a thin layer of endothelial cells without hyperplasia (). Immunostaining with GLUT-1, WT-1 and D2-40 was negative. These results were compatible with a venous malformation.\nA year after the intervention, the patient presented to our department with a local relapse. No sensory, motor, or electromyographic deficiencies were found. The mass continued to grow steadily and after 10 years of follow-up, the patient reported local pain and numbness in the thumb, index, middle and ring fingers, and a new operation was planned.\nThe reintervention revealed a bluish irregular mass (), that was 7 cm long and surrounded and infiltrated the median nerve. The histopathology results were the same as those obtained after the first operation; however, this time phleboliths were found (), confirming the diagnosis of venous malformation. Phleboliths and calcifications, pathognomonic of venous malformations, can be found after episodes of local intravascular coagulation, if the venous malformations are medium to large in size []. This may have been the reason why they appeared in the surgical specimen from the second intervention (7.5 cm in length), but not in the surgical specimen from the first operation (2.5 cm).\nAlthough macroscopic complete excision seemed to be achieved, a new local relapse was clinically detected and confirmed by MRI 12 months subsequently (). |
A 24-year-old-male presented to the emergency department (ED) via ambulance for symptomatic hypotension he experienced while running. Approximately ten minutes into his run, he began to experience generalized pruritis, diffuse urticaria, palpitations, and lightheadedness. He sat to rest for several minutes and when he attempted to stand he became more lightheaded and felt like he was going to “black out.” However, he did not lose consciousness. Evaluation by EMS in the field revealed a blood pressure (BP) of 60s over palpable. He received one liter of normal saline (NS) en route to the ED along with 0.3 mg intramuscular epinephrine and 50 mg of intramuscular diphenhydramine. His BP upon arrival to the ED was 113/69 with a heart rate of 87, a respiratory rate of 20, a temperature of 98.1 F oral, and with SpO2 of 98% on room air. He had no wheezing or dyspnea.\nIn the ED, he received one additional liter of NS along with 125 mg intravenous methylprednisolone and 50 mg intravenous ranitidine. He remained stable in the ED, and upon discharge his BP was 124/67, which is near his baseline. He was advised to avoid exercise and was discharged with an epinephrine autoinjector, fexofenadine at 60 mg daily, oral prednisone 60 mg daily for five days, and a referral to allergy for an outpatient evaluation.\nIn the allergy clinic, the patient reported a 3-year history of diffuse pruritus followed by diffuse urticaria during or shortly after running. He experienced approximately 20–25 episodes over the last three years. He had been evaluated in the acute care clinic multiple times and treated with oral or intramuscular diphenhydramine with symptom resolution. On four occasions, he experienced anaphylaxis in addition to urticaria and pruritus. The most recent and serious of which is described earlier occurring three weeks prior to allergy evaluation. Symptoms have not consistently been associated with foods, medications, or environmental conditions. He stated he usually runs in the morning on an empty stomach. He had not had any urticaria or pruritus while weight training; he only experiences symptoms while running. He had minimal benefit from prophylactic H1 antihistamines.\nGiven the unpredictable and potentially lethal nature of EIAn, he was advised to avoid running, carry an epinephrine autoinjector at all times, and exercise with a partner trained in symptom recognition and administration of the epinephrine autoinjector. He was also instructed to avoid food, NSAIDs, and alcohol a minimum of four hours prior to exercise. He was advised to engage in a graded exercise program and at the first indication of flushing, itching, hives, lightheadedness, or shortness of breath to cease exercising, self-administer epinephrine, and seek immediate medical assistance. |
This is a case of a 59-year-old morbidly obese female with situs inversus totalis who presented for a laparoscopic sleeve gastrectomy. Her BMI was 38 (height 4 ft 11.5 inches, weight 188.2 pounds), and she had a lifelong history of morbid obesity and obesity-related comorbidities, including obstructive sleep apnea requiring a continuous positive airway pressure machine, an elevated hemoglobin A1c (5.8) and a fasting blood glucose increasing her risk of developing diabetes mellitus, and degenerative joint disease which significantly impacted her ability to exercise. The main challenges she identified in losing weight involved eating carbohydrate rich foods, overeating during meals and limited activity due to musculoskeletal pain. The patient had made multiple attempts to lose weight through commercial dieting programs but had been unsuccessful. The patient also completed a six-month medically supervised diet through her primary care provider, which also included working closely with a bariatric registered dietician, following a strict diet of about 1800 calories/day and performing modified exercise, about 120 minutes/week. Despite these intense medical weight loss efforts, she was unable to maintain a healthy weight. The patient was motivated to try bariatric surgery after she witnessed the significant weight loss success her daughters had from this intervention. Her greatest hope from the bariatric surgery was to be healthier and to alleviate her obesity-related comorbidities.\nThe patient’s surgical history included cesarean section and evacuation of an ectopic pregnancy. She was a former smoker, quitting over 25 years ago, and has no other history of substance or alcohol use. Family history is positive for obesity, diabetes, hypertension, coronary artery disease and hypercholesterolemia. She did not take any medications, including supplements, except for Ibuprofen 800 mg 1–3/daily for musculoskeletal pain.\nThe patient underwent a comprehensive evaluation and treatment plan prior to the surgery including: psychiatric evaluation and clearance, nutritional consultation with a registered dietician, education about bariatric surgery and pre/post op expectations, routine preoperative labs, UGI, esophagogastroduodenoscopy (EGD), pulmonary function analysis, and a cardiovascular exam. Situs inversus totalis was confirmed with abdominal X-ray and CT, and echocardiogram. Pre-procedural evaluation with UGI revealed mild gastroesophageal reflux observed to the level of the distal one-third esophagus and small sliding-type hiatal hernia. EGD revealed normal esophagus and duodenum. Stomach biopsy was obtained for antral gastritis, and no helicobacter organism was identified. Abdominal ultrasound confirmed fatty liver disease. Pulmonary function analysis including spirometry, lung volumes and diffusion was normal. A pre-procedural cardiovascular exam was performed due to dextrocardia to exclude other structural cardiac abnormalities. 2-D echocardiography with M-mode demonstrated a left ventricular ejection fraction of 60%, no structural abnormalities in the aortic, pulmonic, tricuspid and mitral valve, or right and left atrium and ventricles. Color Doppler and continuous and pulse wave Doppler demonstrated mild pulmonic regurgitation and mild tricuspid regurgitation.\nThe patient discontinued the Ibuprofen more than one month before her surgery and began a low-calorie liquid diet two weeks prior to surgery. On the day of surgery, preoperative antibiotics, Cefazolin, were given within 60 minutes before the first incision. Prior to induction, the patient received 5000 units of heparin subcutaneously and sequential compression device (SCD) boots were placed for deep vein thrombosis (DVT) prophylaxis. No beta-blockers were administered. The patient was placed in the supine position and general anesthesia was induced. A Foley catheter was placed and the patient was supported with positioning devices including: arms on padded arm boards, gel pad under left axilla, footboard with gel pads, hover mat and a bariatric safety belt over patient’s thighs. The abdomen was then prepped and draped in the standard surgical fashion.\nA Veress needle was used in the right upper quadrant to access the abdomen and insufflation was created to 15 mmHg. Veress was removed and replaced with a 5 mm trocar and the scope was placed. Additional trocars were placed in the following position: right and left 5 mm lateral trocars, right and left 12 and 15 mm supra-umbilical trocars. The placement of all retractors and graspers was adjusted accordingly to the mirror image anatomy of the intraabdominal organs. The primary surgeon was positioned on the left side of the patient and the assisting surgeon on right side of the patient. Situs inversus totalis was confirmed with the majority of the liver oriented to the patient’s right, spleen on the right, greater curvature of the stomach on the right and gallbladder on the left.\nThe operating table was placed in the reverse Trendelenburg position. The patient’s right-sided half of the liver was retracted cephalically using a Nathanson retractor to expose the vicinity of the esophageal hiatus. The peritoneum over the cardia was incised using the Ethicon Harmonic scalpel, and the plane between the cardia and the left crus of the diaphragm was opened to expose the right diaphragmatic crus. No hiatal hernia was present. A point 5 cm proximal to the pylorus along the greater curvature of the stomach was marked corresponding to the incisura angularis just proximal to the crow’s foot of Latarjet’s nerve. The vessels along the greater curvature and all the short gastric vessels were sealed and divided using the Ethicon Harmonic scalpel, freeing the greater curvature and the fundus of the stomach. A 42-French bougie was placed and oriented towards the antrum along the lesser curvature (Figure ). The stomach was stapled and divided alongside the tube in a vertical fashion towards the angle of His (Figures -).\nAn Ethicon Echelon Flex triple staple line power stapler with a total of one black, three green, and one gold staple loads was used. All staple loads were 60 millimeters in length with staple line bio-absorbable reinforcement. Hemostasis at the external staple line was achieved (Figure ). The stomach was removed from the abdomen via the left-sided 15 mm trocar (Figures , ). Intraoperative endoscopy was performed using a 5 mm Olympus Ultrathin gastroscope revealing no areas of stenosis, internal staple line bleeding, nor staple malformation or leak seen. Total operation time was 108 minutes. There were no post-operative complications.\nPost-operative care included pain management and DVT prophylaxis with SCD, heparin and early ambulation four hours post-surgery. A UGI was performed on post-op day 2 with normal findings for a post-bariatric surgery evaluation and clear fluids were started. The patient was discharged on post-op day 3 without any complications. Upon discharge, the patient had a treatment plan that was previously discussed that included instructions for diet and medications, including vitamin supplementation and antacids. At her three-month follow-up, the patient was still without complications and very pleased with her weight loss of 46.2 lbs, weighing 142 lbs (BMI 29). |
A 31-year-old nulliparous woman was referred to our clinic for the treatment of severe diffuse adenomyosis. The patient had severe dysmenorrhea, dyspareunia and lower abdominal pain and she had a history of two early spontaneous abortions, although she was trying to get pregnant for the last two years. Previously, she had used progestin and gonadotropin releasing hormone agonist to relieve the symptoms of adenomyosis medically, but they had no effect. A transvaginal ultrasound showed diffusely invaded and whole uterine adenomyosis (). The patient wanted to receive conservative surgical treatment for relief of symptoms and future pregnancy. TOUA laparotomic adenomyomectomy was performed safely.\nThe procedure of TOUA laparotomic adenomyomectomy was performed in the same manner as in our published report []. The operation time was 80 minutes and the estimated blood loss was 400 mL without any intraoperative complications. Final pathology showed uterine adenomyosis. As a postoperative management option, gonadotropin releasing hormone agonist (leuprolide acetate 3.75 mg) was consecutively administered three times for three months. After 20 months later, she visited our clinic for ultrasonographic examintaion to plan for a baby. She had anteverted, normal sized uterus and the endometrium was clear ().\nDue to personal reasons, the woman tried to get pregnant 30 months later. In vitro fertilization was successfully performed and an intrauterine monochorionic twin pregnancy was confirmed ().\nAt 21+0 weeks of gestation, she was admitted for careful monitoring of uterine contractions and any other expected complications. During antenatal care, the myometrium thickness was monitored regularly, and there was no defect in the thickness of the myometrium on ultrasound. At 25 weeks, there was continuous uterine contraction with shortening of cervix. For lung maturation of fetuses, betamethasone 12 mg was injected intramuscularly, twice at 24 hours interval. At 31+6 weeks of gestation, we decided to perform a caesarean delivery to prevent future abrupt fatal complications, which included uterine rupture, monochorionic pregnancy complications in the 3rd trimester. Two male neonates weighing 1,620 and 1,480 g were delivered by caesarean section. There was no gross finding of uterine rupture but the peritoneum and bladder were adherent to the uterus; hence, bladder injury had to be repaired after delivering the baby. The myometrium and bladder were sutured separately and uterine contractions were sufficient and there was no atony or bleeding during the postpartum period. One week later, there was no leakage of urine on cystography and the Foley catheter was removed. The mother was discharged without any other problems and the babies were discharged after three months of prematurity care, without any postnatal complications. |
A 68-year-old woman presented to the Department of Oral and Maxillofacial Surgery at Nagoya Ekisai Hospital (Nagoya, Japan) with a chief complaint of malaise and a 7-month history of swelling of the left buccal mucosa. The patient had no congenital swelling of the left buccal mucosa at birth and no history of systemic disease or relevant family history. The patient had undergone maxillary molar restoration treatment 2 years earlier, after which she reported biting regularly on her buccal mucosa. On most occasions, the wound had healed within a week, so she had not sought medical treatment. An extraoral examination revealed no facial swelling or asymmetry. However, an intraoral examination revealed an area of diffuse swelling on the left buccal mucosa measuring about 15 mm × 30 mm and containing a papillary lesion with multiple red, blue, and clear pebble-like vesicles (Figure ). On palpation, the lesion was nontender and soft. The swelling had not expanded to the veins and was pulsatile. An orthopantomogram confirmed that the adjacent bone was intact. Magnetic resonance imaging revealed a soft tissue mass with a clearly distinguishable outline of the buccinator muscle (Figure ). The lesion was surgically excised under local anesthesia with a margin of 3 mm and a depth of 2 mm via the inside surface of the fascia of the buccinator muscle. The outcome was favorable. Pathologic examination of the specimen revealed expanded lymphatic vessels lined by thin endothelial cells and containing lymphatic fluid. A diagnosis of lymphatic malformation was confirmed on histopathology and immunohistochemical studies. Immunohistochemistry was negative for vascular markers such as CD31 and CD34, and the lymphatics stained specifically for D2-40 (podoplanin) (Figures , , ). On follow-up, the wound was found to have healed with no evidence of trismus or recurrence. The patient continued to be recurrence-free at her 2-year follow-up. Informed consent was obtained from the patient, and the procedures were in accordance with the Helsinki Declaration. |
A 10-year-old boy presented to the Pediatric Clinic, Araraquara Dental School, Univ Estadual Paulista-UNESP with the main complaint of misaligned teeth. An intraoral examination revealed the absence of the left permanent upper lateral incisor (). The patient was submitted to panoramic radiograph that confirmed the hypothesis of the presence of the supernumerary tooth located near of the maxillary left central incisor (). Periapical radiograph was taken to confirm the location of the supernumerary tooth. After mother’s speech, it was verified that the patient presented healthy with positive dental and medical behavior prior and during the routine appointments. The need of the surgical procedure also the remotion of the tooth was explained to both the patient and mother.\nImmediately after the surgical procedure, the region of the extracted supernumerary tooth received an irradiation of the GaAlAs diode low-level laser (Thera Lase, DMC, São Carlos, SP, Brazil) in the invisible wavelength (830 nm) in two different points aiming to increase bone formation. Each point was illuminated for 17 seconds and was delivered 3.4 J of energy with the fluence of 60 J/cm, and the mean output was adjusted to 100 mW (0.1 W). The region that received the sutures was also irradiated into four different points. To these applications, the laser device was adjusted in the red visible light wavelength and the parameters utilized were similar to the anterior case described above, since the reason for the irradiation was increase the quality repair of the soft tissue. This protocol was applied after 24 and 48 hours after the surgical procedure.\nThe pain recorded in the VAS of the patient showed that apart from the period of 12 hours in which the patient marked a number between 2 and 3 thus demonstrating an annoying sensation. The other periods evaluated it was demonstrated a mild to none pain sensation (between 1 and 0). Furthermore, the patient reported no need to take any medication.\nThe patient was recalled after 7 days to remove the sutures and the aspect of the area of intervention presented no signs of hemorrhage and swelling. The soft tissue presented a high level of healing accompanied of satisfaction of the mother with the procedure ().\nA follow-up of 3 months was performed and revealed the health aspect of the gingival contour and the presence of left permanent upper lateral incisor (). |
A 14-year-old South Asian boy from rural Bengal (India), born of a second degree consanguineous marriage, with normal birth and development history, presented with abnormal brief jerky movements involving his trunk and limbs, with recurrent falls for 10 months. The jerks were neither stimulus sensitive nor present during sleep. No loss of consciousness was reported to occur with these jerky movements. Recurrent convulsions involving the left half of his body, without impairment of awareness, was present for 8 months. It was followed by insidious onset of mild weakness of the left half of his body for 7 months. Subsequently he suffered progressive decline in his general ability to maintain average daily activity independently for 5 months. He had to discontinue schooling because of his failing cognitive functions. For 2 months prior to presenting to us, he developed rapid dance-like movements involving all four limbs that flowed from one muscle to the other in a more or less continuous fashion. Occasionally it would become somewhat flinging particularly in his upper limbs. There was no history of similar illness in the family. He received all the scheduled vaccines as was stated by his mother.\nThe height of the boy was 150 cm and he did not have any dysmorphic facial features. A clinical examination revealed generalized choreiform movements as the most obvious finding. These movements intermittently became flinging in nature, resembling ballism. Generalized myoclonic jerks were seen embedded inside the flurry of chorea-ballism. When he was asked to protrude his tongue, besides motor impersistence, oromandibular dystonia was also found. He had severe dysarthria with apparently preserved comprehension. A limited cognitive assessment revealed reduced attention span as well as short-term memory impairment. Rigidity was obvious in all four limbs along with dystonia in both lower limbs. Weakness in the left half of his body along with brisk reflexes and extensor plantar on left side was also detected on motor system evaluation.\nRoutine laboratory parameters revealed impaired fasting glucose (120 mg/dl), mildly raised liver enzymes and creatine phosphokinase (CPK) level of 820 IU/L. Other blood and urine parameters were within normal limits. Screening investigation for Wilson’s disease, storage disorders, and metabolic disorders were all negative. A routine cerebrospinal fluid (CSF) study was unremarkable and anti-measles antibody was negative. Anti-nuclear antibody in blood was also negative. His serum level of lactate was 36 mg/dl (2–19 mg/dl) while CSF lactate was 42 mg/dl. Shortening of PR interval (0.10 second) was found in electrocardiography. Two-dimensional echocardiography was devoid of any abnormality. Serial brain imaging was done at different centers throughout the course of his illness. On studying his MRI brain images sequentially, a relapsing remitting pattern of lesions was detected. On T2/fluid-attenuated inversion recovery sequence (FLAIR) there were hyperintense lesions that mainly involved subcortical white matter in frontoparietal areas (Fig. ). An area of diffusion restriction was found in the right capsule-ganglionic region (Fig. ) that temporally coincided with the onset of left hemiconvulsions and hemiparesis. Magnetic resonance spectroscopy (MRS), done at our center, showed the presence of lactate peak in brain lesions. Brainstem auditory response revealed bilateral prolonged latency. Electromyography (EMG) showed short duration low-amplitude polyphasic motor unit action potential which was suggestive of myopathic pattern. Spike-wave discharges were observed arising from bilateral frontal areas on electroencephalography (Fig. ). A muscle biopsy, which was done from left vastus lateralis, revealed ragged red fibers (Fig. ), suggestive of mitochondrial failure and deposition of abnormal mitochondria below the plasma membrane of muscle fibers.\nAccording to the clinical criteria, MELAS syndrome was the most probable diagnosis in our case and we needed to confirm the diagnosis. As a facility for analysis of respiratory chain enzymes in the muscle was not available, we decided to search for underlying genetic abnormality in mtDNA. A polymerase chain reaction (PCR) method was employed for this purpose. Amplification of DNA in whole blood sample of our patient was performed for detection of mutations 3243A>G, 3271T>C, and 3251A>G in mitochondrial tRNA leucine 1(MT-TL1), by using appropriate wild type and mutant type specific primers for each and a common reverse primer for all. Genetic analysis result was as following: A>G point mutation at position 3251 of MT-TL1 gene of the mtDNA with heteroplasmy of 70%.\nAfter reaching the diagnosis, valproate was taken off and lamotrigine was introduced. He was put on co-enzyme Q supplement and haloperidol for abnormal movements. Six months into follow-up his seizures and abnormal movements were controlled significantly with slight improvement of cognitive abilities. |
The 52-year-old female patient was admitted to our hospital because of repeated black stool for more than 1 wk. There is nothing special about her family history and past history.\nThe patient underwent endoscopy twice due to melena, and metal clip was used to stop bleeding after initially found the ruptured hemorrhage of ectopic varices in duodenal horizontal part. Gastrointestinal hemorrhage recurred later, with suspected submucosal eminence at the bleeding site. Abdominal computed tomography (CT) revealed huge duodenal stromal tumor, which was then surgically removed and confirmed pathologically.\nShe had no gastrointestinal inflammation, ulcer or solid tumor before.\nOther history could cause gastrointestinal hemorrhage, such as medication and alcoholism, were excluded. Her family history has nothing notable.\nShe had an anemic appearance, a flat and soft abdomen without tenderness, rebound pain or muscle tension.\nThe results showed positive stool occult blood test and severe anemia (hemoglobin 45 g/L). Albumin was assessed at 33.3 g/L, fibrinogen was 4.53 g/L, and no significant abnormality was found in tumor markers or other routine examinations.\nThe first endoscopy was performed in the outer hospital, revealed that multiple ectopic varices in the horizontal part of duodenum, with a diameter of about 0.5 cm to 0.8 cm. Rupture accompanied by active hemorrhage could be seen on the surface of varices, and three metal clips were used for hemostasis (Figure ). Gastroscopy in our hospital showed that there was residual metal clip in the horizontal part of duodenum and bleeding was still active locally. We applied two metal clips to stop bleeding. In addition, suspicious submucosal bulges were found in varicose vein areas, which was hard when touched by biopsy forceps and lacked obvious mucosal glide motion (Figure ). Abdominal CT revealed lumpy soft tissue density shadow in the horizontal part of duodenum, with a size of about 7.0 cm × 4.8 cm × 5.7 cm (Figure ).\nPathologically, the postoperative specimens were confirmed to be duodenal horizontal stromal tumors with moderate risk, tumor size was about 7 cm × 7 cm × 5 cm, mitosis had 1/50 high power field (Figure and ), There are curved and dilated blood vessels in submucosa, some of which are congested, which is in accordance with the pathological manifestations of varicose veins, but some of them are blocked by compression (Figure ). The immunohistochemical results are as follows: Cytokeratin (-), vimentin (+), CD34+, diffuse CD117+ and discovered on gastrointestinal stromal tumor-1 (+), S100-, smooth muscle actin (+), desmin+, caldesmin-, Ki-67 less than 1% (Figure ). Gene testing found that C-KIT gene Exon-11 had c.1669_1674 deltggag (p.w557_k558del) mutation, while platelet-derived growth factor receptor alpha and related exons had no mutation (Figure ). |
A 33-year-old male presented with progressive breathlessness and dry cough since the last 5 months and right sided pleuritic chest pain for 10 days. Patient was diagnosed elsewhere as miliary tuberculosis and was under antitubercular treatment (ATT) for four months. He was not a smoker. He worked as a bore-well driller for the past 10 years.\nBlood hemogram and renal and liver functions were normal. Admission chest radiograph showed bilateral, diffuse, well-defined large rounded nodular opacities with right secondary spontaneous pneumothorax ().\nPatient was managed with tube thoracostomy, supplemental oxygen, and analgesics. After three days patient complained of acute onset chest pain on left hemithorax and dyspnoea.\nChest radiograph revealed a new pneumothorax on left side for which immediate chest tube was placed. Subsequently, patient had symptomatic relief. Fibre optic bronchoscopy revealed normal airway. Bronchoalveolar lavage cytology showed only benign bronchial epithelial cells and smear and culture for Mycobacterium tuberculosis were negative. The diagnosis of military tuberculosis was questionable in view of larger size and nonresolution of nodules although four months of ATT and silicosis was more probable, considering his occupation. Hence, percutaneous biopsy of right lung was done that yielded a tiny tissue and showed only lymphoplasmacytic infiltrates. ATT was stopped and he was discharged after bilateral pleurodesis. Six months later he was brought to emergency with signs of severe respiratory failure. His condition deteriorated faster and despite adequate measures, he sustained a cardiopulmonary arrest and succumbed.\nLimited autopsy was done. Postmortem biopsy of bilateral lungs and liver was done. H and E sections of bilateral lungs revealed multiple collagenous nodules, some of which coalesced to form larger nodules (Figures and ). The periphery of the nodules contained dust laden macrophages and inflammatory cells, predominantly lymphocytes (). There were no granulomas; acid fast staining was negative. Under polarized light microscopy, lung tissue showed white spots which represented silica crystals (). |
An 80-year-old Caucasian female with history of hypertension and chronic back pain presented for emergent repair of a 7.2 cm aneurysm of the ascending aorta with Stanford classification type A dissection. Because the ascending aorta was unsuitable for arterial cannulation, the surgeon elected to perform axillary cannulation via the right subclavian artery with side graft anastomosis. The patient arrived to the operating room (OR) with nicardipine and esmolol infusions running through an 18-gauge peripheral intravenous (IV) line in the right antecubital (AC) fossa. Prior to induction of anesthesia, we disconnected the infusions from the right AC and administered medications though an 18-gauge IV in the left forearm. The patient also had a left radial arterial line (AL), and we placed the pulse oximeter and noninvasive blood pressure (NIBP) cuff on the RUE. After intubation, we placed a right radial AL, and the surgeons placed a left femoral AL. All arterial pressures correlated closely. Additionally, a 9-French central line was placed in the right internal jugular vein. The patient was cleansed and draped for surgery with her arms tucked to her sides.\nShortly after the procedure began, the right radial AL tracing went flat, and the pulse oximeter waveform was lost. We attributed this to the surgeon partially clamping the right subclavian artery in preparation for arterial cannulation. We switched the pulse oximeter to the left hand and relied on the left radial and femoral AL for pressure readings. Just prior to arterial cannulation, we noted that the right radial pressure returned, although about 20 points lower than the left radial/femoral. Immediately after initiating CPB, the right radial mean arterial pressure (MAP) increased to 200 mm Hg, and left radial/femoral MAP decreased from 60 to 30 mmHg. The perfusionist alerted the surgeon about the high line pressures and decreased CPB flows. After a brief attempt to troubleshoot and adjust the cannula with little improvement in pressure or flow, the surgeon proceeded with the operation. Over the next few minutes, the left radial/femoral MAP increased to 60 mmHg.\nAs deep hypothermic circulatory arrest (DHCA) was initiated, the right radial MAP decreased to 30 mmHg and left radial/femoral MAP decreased to 10. When CPB was reinstated, right radial MAP again increased to 200 mmHg. After 29 minutes of DHCA and 265 minutes of CPB, the patient was successfully weaned from CPB, and right radial MAP decreased to about 10 points lower than left radial MAP.\nDespite these issues, the surgery was otherwise uneventful. However, upon the surgical drapes being taken down, we noticed that the patient's RUE was swollen with blisters and bullae from the shoulder to the hand; yet, the skin of the upper arm where the NIBP cuff had been placed was normal as seen in figures. The IV in the right AC appeared to be infiltrated and weeping fluid even though we had not used it during the case and did not have any IV fluids attached to it (Figures and ). The IV and right radial AL were removed in the OR, and a Xeroform gauze dressing was applied to the RUE with the surgeons present. The intensive care unit nurses were instructed to elevate the arm and perform hourly neurovascular checks.\nOn postoperative day 1, the patient complained of tenderness and burning in the RUE, but she maintained adequate capillary refill, motor function, and sensation. Plastic Surgery was consulted to rule out compartment syndrome. They were unsure of the diagnosis but recommended nonoperative management and continued neurovascular checks. Eventually Dermatology was also involved, and they performed a punch biopsy of the patient's right dorsal hand. Their initial diagnosis was allergic contact dermatitis (ACD) due to the fact that the area of skin covered by the NIBP cuff was spared. However, the biopsy showed pauci-inflammatory dermal-epidermal blistering, which did not favor ACD. Direct immunofluorescence was also negative, ruling out localized pemphigus. Given the histologic findings, the final diagnosis was hydrostatic edema/bullae correlating with rapid edema during surgery. The patient continued to be managed nonoperatively with 1% triamcinolone ointment and gauze dressings, and within one month the blisters had completely resolved. |
A 61-year-old man presented to the emergency department with hypovolaemic shock following a sudden episode of upper gastrointestinal bleeding associated with melaena, haematemesis, and a temporary loss of consciousness. There was no prior history of gastrointestinal disturbance, and his past medical history included essential hypertension controlled by antihypertensives.\nOn admission to accident and emergency, he was haemodynamically unstable with refractory hypotension (89/60 mmHg) and a sinus tachycardia of 110 bpm. Physical examination revealed melaena on rectal examination and a Glasgow coma score of 14/15 but was otherwise unremarkable. He remained haemodynamically unstable despite aggressive fluid resuscitation with Hartmann's and packed red blood cells. His Rockall score was 3 out of 7, scoring one for age and two for shock.\nInitial blood tests revealed a haemoglobin of 9.0 g/dL, and emergency oesophagogastroduodenoscopy (OGD) demonstrated fresh ongoing bleeding with no identifiable source. His haemoglobin continued to drop despite being transfused a total of five units of blood, and inotropic support was needed to maintain his blood pressure and so arrangements were made for emergency surgery.\nEmergency laparotomy identified a mass lesion on the anterior duodenal wall between the 3rd and 4th part of the duodenum (D3 and D4, resp.) (). Continued haemorrhage was evident from the nasogastric tube, and a bowel clamp applied under the mass with an on-table OGD confirming haemostasis (). A duodenal wedge resection was performed to remove the vascular mass followed by primary transverse closure. Two drains were inserted: one abdominal drain and the second intraluminal proximal to the level of the anastomosis to drain descending gastric and pancreatic fluid. A wedge resection was chosen in preference to a more radical surgical procedure as there was no evidence of extension of the lesion beyond the serosa or the involvement of adjacent tissues. The relatively low risk of intramural spread that is characteristic of GISTs was also considered.\nIn total, he was transfused 12 units of packed red blood cells and 6 units of fresh frozen plasma with one unit of cryoprecipitate and pooled platelets and after surgery he was transferred to intensive therapy unit (ITU). He was extubated after one day and remained haemodynamically stable, not requiring any further transfusions. He was discharged to the ward after 3 days.\nHistopathological analysis of the resected specimen showed a well-demarcated small-bowel tumour measuring 50 × 29 × 28 mm. Clear margins were demonstrated over 2 mm from the stapled resection edge with no mucosal infiltration. Microscopically (), it showed strong positive staining for both CD117 (c-kit) and CD34, all being consistent with a gastrointestinal stromal tumour (GIST).\nStaging computerised tomography (CT) revealed no evidence of metastases and he was discharged from hospital after twelve days with 6 monthly followup. |
A 14-year-old Han nationality male patient came to our clinic for a history of 2 years of right lower lid ectropion without any significant cause, and the symptom deteriorates gradually. The patient has previously been treated with a temporary tarsorrhaphy in the same eyelid in order to solve the exposure keratitis one year ago. However, the procedure could not resolve his symptom effectively after removing the sutures one week later.\nThe boy was the eldest son born at term to healthy nonconsanguineous parents. No history of medication and no exposure to radiation during pregnancy were revealed. There were no other similar cases in their family including his younger sister.\nThe boy had normal intelligence compared with children of his age. His skin was atrophic, dry, with less sweat, with less hair, and with poor flexibility. The surface of the body skin was thin. Parents reported the boy's skin was more fragile and prone to tears and scarring. Subcutaneous blood vessels were pronounced and visible. His height was 1.89 m and weight was 70 kg. His limbs were thin and long. His low limbs were “O” type outward and lame slightly. His fingers, especially the first and second phalange, were shorter and cannot full straighten. His palms and soles were hyperkeratosis. The fingernails and toenails were dystrophy (Figures ~). Front, temporal bone and chin were prominent, while his middle face was depressed with flat nose. The proportion of his lower face was reduced.\nAs his upper alveolar ridge was depressed while lower alveolar ridge was prominent, and there was wrinkle in forehead, eyelid and nasolabial groove region, leading to his aged appearance. His teeth were sparse and irregular. There was part of the permanent teeth absent on the hypoplastic upper alveolar ridge (Figures and ).\nExamination showed that he had normal correct visual acuity and normal IOP. There were sparse eyelashes, multiple rows, or absence of lashes on the upper lids with absence of lash on the lower lids. His prominent eyes looked like golden fish eyes due to subcutaneous tissue and orbital fat atrophic. Eyelid skin was thin and loose. His right eye had hypophysis due to lower eyelid overturned seriously (). Exposed conjunctiva was dry mild conjunctival hyperemia.\nOn the basis of history and the presence of triad of symptoms: hypotrichosis, hypohidrosis, and hypodontia, the patient was diagnosed as having hypohidrotic ectodermal dysplasia (HED). However, multiple congenital anomalies (MCA) pattern consists of finger malformation, skin atrophic, and abnormal tall height; the patient may be a kind of new syndrome.\nThe patient underwent a successful ectropion blepharoplasty surgery on his right lower lid in July 18, 2013. After topical anesthesia and local injection of lidocaine with epinephrine into the portions of lower lid, the lid was split laterally into two lobes at the point of inner and outer 1/3 along the gray line for about 5 × 5 mm with a blade. The tarsus was resected about 3 × 2 mm in V shape and the corresponded lid skin was removed with a blade, respectively. After bleeding was completely stopped, the posterior and anterior lobes and the lid margin were sutured using interrupted 6-0 Vicryl, respectively, which was removed in 7 days. Postoperative eye examination showed that his right lower lid returned to normal position and no ectropion recurred during the 10-month follow-up period (). |
A 14-year-old girl was consulted for psychiatric assessment at the department of paediatric ward of JUTH. She was assessed for a cluster of symptoms including intractable vomiting, inability to eat and walk, and significant loss of weight with emaciation.\nGreatly concerned, her father had taken her to the nearby hospital, a health care facility three hundred kilometres from Jimma town. From there, she was referred to Addis Ababa, which is approximately 650 kilometres from her home. She was then taken to various prestigious public and private health care institutions, where she was examined for a number of possible medical complaints by methods including CT scans and MRI examination of the spinal cord. All results were normal. The family incurred huge expenses from these tests, and yet her condition remained unexplained, and she did not receive the necessary help. In desperation, and losing hope, her father took her back to the first hospital, and from there, she was referred to JUTH. At JUTH, she was first contacted and admitted to the paediatric ward, from which she was finally transferred to the psychiatry clinic through a consultation process.\nThe examining clinician noted sunken eyeballs, generalised body weakness, and significantly decreased overall muscular mass. Slight abdominal tenderness was detected during physical examination and vital signs were within normal range. With support, she was able to sit and stand upright, but was not able to take a single step, or stand for long. At admission, her weight was 22 kg, her height 132 cm, and body mass index 12.6, which was below the 3rd percentile []. Consequently, a nasogastric tube was inserted for feeding and treatment for suspected Peptic Ulcer Disease commenced. It was observed that she was able to move her legs to some degree whenever she was distracted. After 2 weeks of thorough investigation, including neurological examination, there were no findings suggestive of any medically explained illnesses, and as she appeared severely mentally disturbed, she was transferred to the psychiatric ward. The referral diagnosis was suspected as early onset schizophrenia, and the differential diagnosis was conversion disorder. |
A 21-yr-old female was presented to the oral medicine and radiology department with a chief complaint of underdeveloped lower jaw, which was first noticed during childhood and gradually progressed. Due to unfavorable socioeconomic conditions, it was not possible to get the treatment done for the patient. At the anamnesis there was no history of any trauma or any systemic diseases. Patient's parents gave a history of consanguineous marriage. There was no family history of the present problem.\nGeneral Physical examination did not reveal any abnormalities. Her vital signs were within normal limits. Extraoral examination revealed facial asymmetry with severe retruded mandible giving a bird face appearance (Figures and ). Mouth opening was restricted (10 mm) with no deviation or deflection (). On palpation condyles were not detected on both sides. Intraorally there was crowding in upper and lower anterior teeth with increased overjet and overbite (). Molar relation was Angle's Class II bilaterally. She had deep palate and generalized enamel hypoplasia (fluorosis). No other important clinical extraoral or intraoral findings were observed. Based on clinical findings, a provisional diagnosis of bilateral ankylosis and differential diagnosis of bilateral condylar hypoplasia or aplasia were given.\nAfter clinical examination, radiographic examinations were performed. Panoramic radiograph showed complete absence of condyle on the right side and rudimentary condyle on the left side. Glenoid fossa was not developed on the right side and underdeveloped on the left side. Antegonial notch was prominent bilaterally (). PA view findings were inconclusive (). Lateral skull views showed severe retruded and micrognathic mandible (). Findings of panoramic radiograph were confirmed by lateral skull radiographs. CBCT was advised for additional information. CBCT also confirmed the findings of OPG and lateral skull radiographs (Figures , , , and ). After radiographic confirmation patient was advised complete systemic evaluation and referred to general medicine, cardiology, ophthalmology, ENT, and orthopedics to rule out any syndromes. Medical evaluation revealed no abnormalities. Based on the clinical and radiographic findings, a final diagnosis of nonsyndromic agenesis of the right condyle and hypoplasia of the left condyle was given. Patient was referred to oral surgeon and orthodontist for the best possible treatment. |
We present a case of a 54-year-old Caucasian lady, who three months following the diagnosis of PsA, presented with persistent cough and worsening dyspnoea at her outpatient clinic appointment. On examination, the patient had reduced chest expansion on the right side and a dull percussion note at the right lower zone. There was no clubbing and no lymphadenopathy. Urgent chest X-rays showed a suspicious 2 cm lesion in the right lower zone. During this time, the patient was being worked up for specific disease-modifying antirheumatic drug (DMARD) therapy, which was halted due to the unusual chest symptoms and imaging abnormalities. CT-guided transthoracic biopsy confirmed a 26 × 23 mm soft tissue lesion located at the bifurcation of the lower lobe bronchus. No mediastinal lymphadenopathy was noted. Histological analysis of the biopsy tissue revealed a benign typical carcinoid tumour ().\nConsequently, a right lower lobectomy was performed, with complete resection of the tumour. The patient was monitored without requiring any further treatment. Following the successful resection of the lesion, a 6-week trial of Methotrexate was commenced specifically for PsA but did not prove efficacious. She was then switched to Leflunomide with a better clinical response. Ten months after the lobectomy, the patient complained of new onset night sweats, weight loss, and flushing, albeit with a 2-year background of abdominal bloating. Two months later, she experienced a change in bowel habit. An urgent colonoscopy revealed a rectal polyp that was resected as per standard procedure. Benign typical carcinoid was confirmed histologically (). An octreotide scan and 5-hydroxyindoleacetic acid (5HIAA) urine levels were negative following polypectomy.\nShe was diagnosed to have multicentric carcinoid syndrome (CS), in the background of PsA. This was a histopathological diagnosis and not based on clinical evidence of more than one site involvement. Interestingly, a small number of patients with CS develop symptoms of arthritis compared to the general population, the causes for which remain elusive []. Albeit, paraneoplastic syndromes such as CS can rarely present in patients with Rheumatoid Arthritis [], where three cases have been reported in the literature. However, to our understanding none have been reported for patients with PsA []. This is likely to be incidental as there is no recognised relationship between PsA and CS. |
A 20-year-old man visited our hospital due to periumbilical pain lasting approximately 20 hours, followed by localized pain in the right lower quadrant for 6 hours with nausea and vomiting. The patient had a history of underlying pectus excavatum which had been corrected by Nuss procedure (minimally invasive repair of the pectus excavatum) and a right inguinal mass that reduced 4 years ago, although the patient never visited a hospital for this latter condition. At the outpatient department, the patient presented with a right groin mass that could not be reduced. Vital signs included an oral temperature of 37.7°C, a regular pulse of 113 beats per minute, blood pressure of 180/100 mmHg, and a respiratory rate of 18 breaths per minute. Physical examination revealed, marked tenderness and voluntary guarding at the right lower quadrant with a right inguinal mass, 7×10 cm, extending to the scrotal sac with no skin redness, and mild tenderness at the groin mass which was irreducible. Both testes were normal.\nBecause diagnosing these two diseases at the same time was uncommon, we requested an abdominal computerized tomography (CT) scan to confirm the diagnosis. The CT scan reported circumferential enhancing wall thickening and dilated fluid-filled appendix, 1.5 cm in maximal diameter () and a herniation sac containing omental fat through the right inguinal canal, compatible with indirect inguinal hernia with no evidence of bowel obstruction ().\nAfter the CT scan, the patient was prepared for surgery with hydration and intravenous antibiotics (2 g of ceftriaxone and 500 mg of metronidazole). Intraoperatively, the patient was in a supine position under general anesthesia.\nThe first procedure was an appendectomy, utilizing the Lanz skin incision; the external oblique aponeurosis was divided, followed by the muscle split technique, entering the peritoneum into the abdominal cavity. We found an acute gangrenous-type appendix, a divided and ligated mesoappendix, and the appendiceal artery was ligated with silk No. 5–0. Appendectomy was done by the double ligation technique, with silk No 0.\nAfter finishing the appendectomy, we tried to reduce the omentum, that is the content of the incarcerated hernia, from the hernia sac, but we were unsuccessful. Before starting the second procedure, all drapes were changed and a sterile scrub was repeated. An incision to repair the hernia was separated by a transverse skin incision at the right inguinal area (), two-finger breadth above the inguinal ligament, then the external oblique aponeurosis was divided and identified, and the iliohypogastric and ilioinguinal nerves were preserved. The spermatic cord was encircled to isolate it from the posterior wall of the inguinal canal with a penrose drain, after which we opened the hernia sac. The content of the hernia was omentum, without intestine; a partial omentectomy was performed, due to adhesions in the hernia sac. The remaining omentum was reduced into the abdominal cavity. A herniotomy was performed by the purse-string technique with atraumatic silk 2-0. The inguinal floor was repaired by “Bassini” technique and the skin was closed with nylon 3/0 vertical mattress.\nThe total operative time was 2.5 hours, with minimal blood loss (appendectomy 1 hour and incarcerated hernia operation 1.5 hours). The pathology report confirmed acute necrotizing appendicitis. The patient was discharged without any complications on postoperative day 7. The Research Ethics Committee of Chiang Mai University approved the study. Written, informed consent was obtained from the patient. |
34-year-old female Gravida 5, Para 4 in her 39th week of gestation with spontaneous labor was transferred to our hospital. Her pregnancy was uncomplicated. She denied smoking and had a BMI of 24 kg/m2. Her past medical history was unremarkable.\nThe first stage of labor was uneventful and lasted for 4 h. At the end of the second stage of labor which had lasted for 30 min, the patient complained of breathlessness and right-sided chest pain. Physical examination revealed the blood pressure was 140/90 mmHg, pulse was 102/min, and the oxygen saturation was 95 % on room air oxygen. The patient was found to be moderately tachypnoeic with breath rate of 22 cycles per minute. She received oxygen supplementation by facial mask and after pushing for 10 min, the labor ended with a vaginal delivery. A healthy, live female infant (4400 g) with Apgar score of 8/9 in the first and fifth minute was delivered.\nTen minutes later she felt worse. Palpation revealed mild subcutaneous crepitus in the neck and subclavian regions corresponds to subcutaneous emphysema. In a few minutes there was marked emphysema extending from the upper anterior chest wall up to the face. The deterioration of respiratory status was observed, still the patient's condition remained stable.\nChest X-ray was performed which showed pneumomediastinum and pneumothorax. Computed tomography () confirmed right-sided tension pneumothorax, pneumomediastinum and subcutaneous emphysema. A team of thoracic surgeons were required urgently. A pigtail catheter was placed in the 5th intercostal space. The pneumothorax was resolved during few hours. The chest radiograph was repeated 5 h later which showed resolution of pneumothorax. As the clinical condition had stabilized, the chest tube was removed. The patient was observed closely for 7 days in hospital, without a specific medication with proper analgesia during the first 48 h. The symptoms regressed and repeat chest X-ray confirmed disappearance of pneumomediastinum. She was discharged home with no other postpartum problems. No complications were seen afterwards. At 2 years follow-up she is healthy and asymptomatic. |
The patient is a 28-year-old male with prior history of a grade 3 astrocytoma, who presented to our emergency department (ED) after having a general tonic-clonic seizure (GTC). Initially, his disease was appreciated four years prior when he presented with a GTC and was found to have a grade 3 astrocytoma of the left occipital lobe (Figure ). He underwent maximal safe resection followed by adjuvant EBRT to 45 Gy in 25 fractions at an outside institution. Due to reasons outside the patient’s control, he received only two cycles of adjuvant temozolomide following EBRT. He developed disease recurrence, presenting as a GTC six months prior to this ED presentation, where imaging demonstrated tumor progression with increased extension into the parietal lobe (Figure ). He subsequently underwent a second maximal safe resection later that month. Final pathology returned as GB. Follow-up imaging four months afterward demonstrated disease progression and he was started on bevacizumab as well as a tumor treatment field (TTF) device. He unfortunately only tolerated TTFs for one month, ending one month prior to the ED presentation.\nUpon admission to our hospital from the ED, MRI demonstrated progression of disease in the left occipital-parietal lobes with extension into the splenium and anterior-inferior extension into the left thalamus and basal ganglia (Figure ). His physical exam was notable for mild right-hand weakness, but he was otherwise neurologically intact. Despite changes to his antiepileptic medication, he had seizure recurrence a few weeks following admission. Presuming that the area of tumor recurrence received a definitive dose in the past, the consensus decision was to proceed with repeat maximal safe resection with GammaTile placement. A dose of 60 Gy was prescribed to a 5 mm depth using a total of eight tiles, each containing four Cesium-131 3.5U seeds, to line the post-operative cavity volume of 17.6 cc. A significant portion of the occipital-parietal disease was debulked, with final pathology again demonstrating a grade 4 astrocytoma with molecular studies indicating an IDH-mutated, ATRX mutated, and MGMT promoter methylated phenotype with hypermutation. Postoperatively he was noted to have right upper and lower extremity weakness/spasticity with right foot drop and mild right face weakness. He required a cane to assist with ambulation and reported word-finding difficulties and decreased short-term memory. He was planning to start adjuvant temozolomide; however, he developed a severe GTC with increased muscle weakness and altered mental status two months later. MRI following this episode demonstrated mildly increased enhancement to the tissue surrounding the surgical bed with the progression of disease in the splenium and left thalamus/basal ganglia (Figure ).\nGiven this symptomatic disease progression, a treatment plan was made to take the progressive regions of disease outside the irradiated GammaTile volume to 35 Gy in 10 fractions. As shown in Figure , an initial planning target volume (PTV) was delineated consisting of the T1 post-contrast-enhancing disease with a 5 mm margin. The volume that received greater than 35 Gy from the GammaTile treatment was excluded from the PTV. The PTV was then separated into two portions by subdividing the remaining volume between tissue that received less than 17.5 Gy (PTV1), and that which received 17.5-35 Gy (PTV2). Using a volumetric modulated arc therapy plan with five arcs, one non-co-planar, a homogeneous dose of 35 Gy in 10 fractions was delivered to PTV1. This dose was calculated to have a biologically equivalent dose in 2 Gy fractions (EQD2) of 45 Gy. Dose painting gradually decreased the dose from 35 Gy to as low a dose as achievable approaching the resection bed border of PTV2 (Figure ). A composite of the GammaTile dose volume (Figure ) with a dose delivered from PTV1 and PTV2 resulted in a homogeneous dose of approximately 50 Gy EQD2 to the residual disease extending into the splenium and the left thalamus/basal ganglia (Figure ). Digital imaging and communications in medicine (DICOM)-RT data from the initial 45 Gy delivered from the outside institution were obtained, and cumulative dose to critical organs at risk (OARs), including the brainstem, ocular structures, and cochlea, were within established constraints.\nDue to the hypermutation phenotype of his tumor, the patient was started on CCNU (100 mg/m2) and completed his EBRT with no issue or progression of his current neurological symptoms. He was last seen in follow-up by our team three months after his most recent EBRT, at which point he reported significant improvement in the right leg tremors/spasms and improved headache. He demonstrated decreased right foot drop but continued to have word-finding difficulties and short-term memory deficits. He has not developed any new areas of muscle weakness or paresthesia, visual loss, or other forms of altered sensoria. The current plan is to complete six cycles of CCNU with alternative systemic therapy thereafter upon disease progression. While temozolomide could be used to this effect, the high mutational burden found in his most recently resected disease would permit entry into currently available trials of immunotherapy. |
A 56-year-old female with a prosthetic left wrist implanted 8 years before due to chronic polyarthritis presented herself to our emergency department with a massive painful swelling and signs of hand phlegmon (Figure 3a ). Her daily medication was methotrexate and prednisolone. Methrotrexate was paused, while prednisolone was reduced and the pain therapy adjusted by the pain therapist. We immediately performed a first debridement with relieving incisions. Daily wound lavage and antibiotic treatment were initiated. With no tangible improvement of the local wound signs and continuously increasing white blood cell count, we decided to remove the prosthetic wrist (Figure 3b ), stabilizing the hand by external fixation. After two more debridements and wound closure, the patient could be discharged 4 weeks later with external fixation and ongoing low dose prednisolone. After full recovery, the patient was hospitalized again for the removal of the external fixation and for autologous wrist arthrodesis by performing a free fibula flap to the left wrist (Figure 3c ). Once more, the wound healing was not adequate and both, staphylococcus and enteroccus could be verified within the wound. Antibiotic therapy was adjusted and additionally, we performed a gracilis flap to close the wound on the left wrist (Figure 3d ). However, the elevation area of the flap on the lower limb showed wound healing problems requiring a debridement and local skin grafting for wound closure.\nWith an overall hospitalization of more than 14 weeks, we could finally discharge the patient. 6 months after the last procedure was performed, again the patient was transferred to our hospital with the status of an acute sepsis and encephalitis with the left wrist being the focus of infection. As life-saving procedure the left hand needed to be amputated at the distal forearm. Finally, the patient recovered from both sepsis and encephalitis and could be discharged from our clinic to rehab. |
A 63-year-old gentleman was found by his primary care physician to have a new right inguinal canal impulse bulge upon presentation for an unrelated symptom. The patient was referred to a general surgeon, to whom he reported a one year history of an asymptomatic groin mass and possible urinary changes. On physical examination, the abdomen was soft, slightly obese, nontender, and nondistended. There was mild right testicular tenderness with a right inguinal canal impulse bulge. The left testicle was normal and there was no left inguinal canal impulse bulge.\nUltrasound ordered to evaluate hernia contents and rule out testicular pathology demonstrated a mild, unilateral right-sided varicocele measuring 3 mm (). Otherwise, the exam was unremarkable: there were no focal lesions of either the right or left testicle and there was no definite bowel-containing hernia visualized on examination of the right scrotum.\nA CT abdomen/pelvis with intravenous contrast was performed in order to rule out a mass in the right retroperitoneum that could have been compressing the right gonadal vein and causing venous congestion. This CT demonstrated a 10.3 × 7.4 × 18.1 cm predominantly fat density lesion with small internal focal areas of soft tissue density in the right retroperitoneum extending into the right lower quadrant along the right paracolic gutter and anterior to the iliopsoas muscle (Figures and ). The retroperitoneal location and presence of soft tissue components made liposarcoma much more likely than a benign lipoma [].\nThe white arrow on highlights the right-sided inguinal hernia contents, which have the same homogenous hypointensity as the large fatty lesion in the retroperitoneum. The liposarcoma had likely extended through the inguinal rings resulting in indirect inguinal hernia appreciated on physical exam. Indicated by the white arrowhead in , a section of the right gonadal vein courses through the deep inguinal ring where it was likely compressed by the liposarcoma, causing the patient's right-sided varicocele. There is also a nonlipomatous nodular focus of intermediate signal density seen starred in Figures and consistent with soft tissue elements. Additionally, the retroperitoneal tumor was exerting mass effect with leftward displacement of bladder and anterolateral displacement of bowel.\nThe patient's metastatic workup (chest CT with IV contrast) was negative and he underwent tumor resection. Surgical exploration demonstrated an obvious large, palpable, lobulated mass encapsulated within regular adipose tissue of the right retroperitoneum. The mass was removed with wide margins. Frozen section of the 17 × 17 × 8 cm specimen demonstrated adipose tissue with scattered chronic inflammation and rare histiocytes, though low-grade liposarcoma was not ruled out. The patient's postoperative course was unremarkable and he was discharged from the hospital on postoperative day 7.\nPathology confirmed the diagnosis of well-differentiated liposarcoma (). The tumor was histologic grade 1 with a mitotic rate of 1/20 high-power fields in most cellular areas. No necrosis or lymphovascular invasion was identified. The superomedial, lateral, and inferomedial margins were positive on microscopy. The pathologic stage was T2bNxM0 and clinical stage 1 b, which is based on a deep tumor of size greater than 5 cm. Immunohistochemical stains performed on formalin fixed and paraffin embedded tissue showed that the highly atypical cells in the area of well-differentiated liposarcoma, inflammatory type, were negative for lymphoid marker CD45. Cytogenetic studies were attempted; however, the cells from the tumor specimen failed to proliferate in culture.\nDue to positive microscopic margins, the patient proceeded to resection of residual disease including right orchiectomy, omental flap, and appendectomy at an outside, regional sarcoma center six months after the initial surgery. One microscopically positive margin persisted. The patient did not undergo any radiation or chemotherapy as part of his treatment.\nNow two and a half years after his initial diagnosis, this gentleman continues to be monitored for local and distant recurrence of disease with biannual abdominal/pelvic CTs and annual chest X-rays. |
A 73-year-old woman was referred to our hospital due to a left acetabular fracture. She had undergone L4-5 fusion for spinal stenosis 5 years ago, and after surgery she had persistent radiating pain. For pain control, a check lumbar myelography and spinal cord stimulator insertion was planned 4 months ago.\nBut she had a grand mal seizure when the myelography was being performed. The seizure developed at the moment of contrast media injection of about 7 mL of the total 10 mL (). The contrast media used was Iohexol (IO-Brix, Taejoon Pharm, Seoul, Korea). Emergency management was performed. During seizure attack, there was no fall or trauma. She had no history of a previous seizure.\nOn regaining consciousness, she began to complain of increasing pain in the left hip, and 2 days after injury, plain radiographs were taken of the hip. The radiographs revealed an acetabular fracture with medial displacement of the left femoral head (). After discussion with the patient and her family, it was decided to treat the fracture nonoperatively at another hospital. Three days after the seizure, a spinal cord stimulator was inserted. During hospitalization, femoral skeletal traction of 9 kg was applied for 12 weeks to reposition the fracture dislocation. She was then discharged. However, the pain did not subside, and finally she was referred to our clinic, where plain hip radiographs showed disrupted congruity of the left hip and joint-space narrowing (). The bone mineral-density scan done in our hospital revealed severe osteoporosis with T-score of −3.6 at L1–3 and −2.4 at proximal femur. Endocrine reference to diagnose secondary cause of osteoporosis was taken, but after complete evaluation, no cause was found and a diagnosis of postmenopausal osteoporosis was made.\nIn view of the pain, joint-space narrowing, and femoral head destruction, we decided to perform total hip arthroplasty. The bone defect, caused by a previous fracture at the acetabulum, was filled with an autologous bone graft from the femoral head. To reconstruct the acetabulum, we used an acetabular roof-reinforcement ring with a hook (Ganz cup; Zimmer, Warsaw, IN, formerly Centerpulse, Winterthur, Switzerland) and a cementless tapered stem to reconstruct the femoral side (M/L taper; Zimmer, Warsaw, IN). After surgery, the patient was maintained non-weight-bearing for 6 weeks and then allowed partial weight-bearing. Full weight-bearing ambulation was allowed 3 months postoperatively. Till date, she had been followed for 1 year and there was no pain or limping on her left hip (). Also, no other event of seizure attack was reported. |
A 30-year-old male patient known to have epilepsy was referred to the neurology clinic at our tertiary facility for evaluation and control of recurrent breakthrough seizures. He was born to nonconsanguineous parents, with an uneventful delivery and normal early development. He was of average intelligence and had completed 12 years of school education. Family history was negative for epilepsy, neurological or skin conditions. His epilepsy started at age 16 and has previously been controlled with sodium valproate 500 mg twice daily, with infrequent generalized tonic-clonic seizures over the last few years.\nNeurological examination was normal. Electroencephalography “EEG” was unrevealing. Brain CT revealed a hypoattenuating lesion at the left parasagittal parietal area, with sulcal effacement and mass effect. It also revealed gyriform calcifications in the left fronto-temporo-parietal region, with bilateral cerebral atrophy and dilatation of both lateral ventricles which was significantly more prominent on the left side (). Incidentally, bone cysts were noted in the left mandibular condyle extending down to the ramus. Brain MRI with contrast () revealed a dura-based mass in the left parasagittal posterior parietal region, with enhancement and extensive surrounding vasogenic edema. It also confirmed the left gyriform calcifications, parenchymal atrophy and ventricular dilatation noted on brain CT. No vascular anomalies were noted in any of the images. The patient was referred to neurosurgery and the intracranial mass was excised (). Cytopathology and immunostaining revealed the mass to be due to fibrous meningioma, WHO grade I.\nAlso, during evaluation, it was noted that the patient had several left-sided skin lesions over his face (), scalp, posterior neck, abdomen, back and the left inguinal area. He also had multiple subcutaneous lipomas of different sizes, which were also restricted to the left side of his body. One of these lipomas was in the left frontotemporal area (). The skin lesions and lumps have been present for “as long as he could remember”. The abundance and diversity of lateralized Central Nervous System (CNS) and skin manifestations prompted a thorough evaluation for a neurocutaneous syndrome, and based upon a review of literature, ECCL was suspected. No intraspinal lipomas were revealed on whole-spine MRI. Biopsies from the skin lesions revealed them to be due to nevus sebaceous of Jadassohn, excluding nevus psiloliparis. There was no nonscarring alopecia, skin tags or aplasia. Ophthalmological evaluation was normal except for left melanosis sclerae. No evidence of aortic coarctation was discerned on clinical examination, chest x-ray or Transthoracic Echo. A comprehensive bone survey looking for cysts other than the ones detected in the left mandible was not performed, but none were detected upon revision of the available previous radiographs that were taken for different reasons.\nDuring follow up, the patient suffered a left mandibular condylar fracture which was precipitated by a minor trauma and which was later complicated by osteomyelitis. Two years after meningioma excision, the patient’s epilepsy is again well controlled with medical treatment. This case demonstrated involvement of three systems (skin, central nervous system, and other), with major criteria in the latter two. Based on these findings, and the exclusion of alternative diagnoses, it meets Moog’s criteria for a definite case of ECCL. |
A 45-year-old man working in navy presented to our institute with the complaint of painless, gradually increasing swelling in the right temporal region for the last two years. Clinical examination revealed swelling to be cystic and fluctuant on palpation. It was a 6.2×3.5 cm subcutaneous swelling not adherent to the underlying bone []. The medical and medication history was noncontributory. No other family member had any such complaints.\nComputed tomography demonstrated an extracranial, oval complex cystic lesion in right temporal and infratemporal region. The lesion showed mild enhancement of its walls and was extending along the right temporalis muscle []. Based on these findings, possibility of dermoid cyst was considered. Fine needle aspiration biopsy (FNAB) was performed. Smears were paucicellular with proteinaceous background. A diagnosis of benign cystic lesion was rendered on FNAB. Other routine hematological investigations and plain chest radiograph were normal. Surgery was done under general anesthesia; circular incision was given around the margin of the cyst over the scalp. The mass was dissected from the surrounding structures and excised totally. Fortunately, the integrity of the capsule was maintained and there was no spillage of cyst fluid into the surrounding structures.\nThe mass was examined histopathologically. Grossly, there were multiple cysts measuring from 1.5 to 3.0 cm in diameter. On cutting, all the cysts were unilocular, filled with clear fluid and had tiny vesicular projections on their inner surface.\nOn microscopic examination, a lamellated ectocyst with germinative endocyst conforming to morphology of hydatid cyst was demonstrated. The patient was subjected to ultrasonography of the abdomen with CT scan of the thorax, to exclude the possibility of invasion of any of the visceral organs. But, surprisingly, the investigations did not reveal any other organ involvement by the disease process. Patient was given medical treatment with albendazole (800 mg/d) for six weeks. After 22 months of surgery, the patient has no complaints and is disease free. |
A 3-year-old boy presented with a whitish-clouded cornea and loss of vision on the left eye for one week and a physiologically appearing right eye. There was no trauma remembered or pain, family history showed no conspicuous eye disorders, no infections, or abnormalities in pregnancy or birth, and no genetic disorders were known. General pediatric physical examination was normal for all systems. The parents had Turkish origin, and history of parental consanguinity is known.\nThe clinical examination under general anesthesia showed a blue discoloration of sclera in both eyes. Intraocular pressure was within normal levels (8 mmHg in the left eye and 10 mmHg in the right eye). The posterior segment was examined by standardized echography and showed no retinal anomalies or retinal detachment. Axial length was 21.7 mm in the right eye and 22.1 mm in the left eye, suggesting myopic eyes. Retinoscopy in cycloplegia showed −4.0 diopter and astigmatism of −4.5 at 168 degrees on the right side. Retinoscopy in the left eye was not possible.\nThe cornea of the left eye revealed complete corneal edema with obvious stromal and bullous epithelial keratopathy. The epithelial layer was closed. Central corneal thickness was 745 μm in the left eye and the mean value of thickness peripherally was 550 μm as measured by ultrasound pachymetry (PalmScan AP 2000, Micro Medical Devices, USA).\nThe right eye showed corneal thinning with a central thickness of 212 μm and a peripheral thickness of 308 μm with corneal astigmatism in topography (−4.5D at 169 degrees). The inferior paracentral cornea showed a deep stromal scar with the remaining corneal surface clear. Funduscopy showed normal optic nerve and retina.\nReduced central corneal thickness with deep stromal scar on the right eye and the acute corneal edema suggested the diagnosis of keratoconus on both eyes, with acute keratoconus of the left eye. In addition, a connective tissue disorder causative for the blue discolored sclera was suspected.\nMolecular analysis of the genes ZNF469 and PRDM5 was performed by polymerase chain reaction amplification and direct DNA sequencing. No mutation was found in ZNF469, but the homozygous variant c.17T>G, p.V6G, was detected in PRDM5. To exclude a dosage effect, a quantitative real-time PCR analysis of PRDM5 showed no larger deletion or duplication. This variant c.17T>G, p.V6G, was not found in the 2504 control subjects in the “1000 genomes project” []. It does not affect any known functional domain but was predicted to be “disease causing” by MutationTaster. Based upon this prediction, the heterozygous state in the parents, and the absence in control populations the variant was thought to be likely disease causing.\nTo avoid amblyopia, there was an occlusion performed of the right eye for two hours per day and glasses were prescribed. Seven months after acute keratoconus, corneal cloudiness was nearly completely cleared under local antibiotic and hyperosmolar treatment with only little subepithelial scars remaining (Figures and ) and a visual acuity of 0.2 in the left eye. Optical coherence tomography (OCT) showed steepness of the cornea and a central pachymetry on the left side of now 259 μm (). A clear fundus view was given, so that at this moment there is no keratoplasty indicated.\nFive months after first consultation and while the left cloudiness was getting better, the 3-year-old boy presented with a large spontaneous corneal perforation on the right side. The spontaneous perforation extended from the pupil area to the peripheral 1 o'clock position with peripheral iris incarceration. The anterior chamber was flattened and fibrin was seen in front of the lens. After trying to reposition the iris incarceration, 4 corneal sutures were performed. Immediately there was cheese wiring () because of the extremely thin corneas of about 150–200 μm. No more sutures could be fixed. Because of the extreme corneal thinning, there was no opportunity to perform an amnion-transplantation or keratoplasty. Spontaneous wound healing was observed using a bandage contact lens, systemic carboanhydrase inhibitor therapy, and ocular compression bandage for 7 days (). The examination after 4 weeks showed a complete corneal epithelialization with iris incarceration but deep anterior chamber and negative Seidel test. Two months after spontaneous corneal rupture another examination under general anesthesia was performed. All 4 sutures were loose so that all of them had to be removed. The pupil seemed rarely round, the optical axis was clear (), and fundus evaluation showed normal results. Using OCT, the iris incarceration was seen to be only adherent on the rear surface of the cornea (). Six months after spontaneous rupture, iris incarceration was reduced so that optical axis was free and no more surgical intervention was necessary. Best corrected visual acuity (BCVA) of the right eye was 20/400. |
A 63-year-old African American male initially presented to the hospital with an elevated prostate-specific antigen (PSA) level of 9.41 ng/mL. The PSA from a year prior was normal at 0.80 ng/mL. The patient’s symptoms included occasional dysuria and infrequent nocturia. The patient denied any other lower urinary tract symptoms, hematuria, abdominal or pelvic pain, bowel abnormalities, hematochezia, weight loss, night sweats, or loss of energy. The digital rectal exam at the time revealed a large volume prostate with induration along the left side of the gland.\nThe patient was scheduled for a prostate biopsy. However, about two weeks after his initial visit, he presented to an outside hospital with complaints of urinary retention. He was found to have acute kidney injury from progressing bladder obstruction due to a combination of benign prostatic hyperplasia and his malignancy. A urinary Foley catheter was placed, but the patient quickly developed hematuria due to traumatic catheter insertion. When this did not resolve, he presented to this institution for further evaluation, during which time the patient denied any further subjective complaints. His kidney function eventually improved, and the patient subsequently underwent a transrectal ultrasound with a 12-core needle biopsy of the prostate. During the exam, the prostate appeared to have irregular prostatic tissue extension from the left base of the prostate. Pathology revealed small cell neuroendocrine carcinoma that was present bilaterally in the base, mid-prostate, and apex of the prostate gland (12 out of 12 cores positive) (Figures -). There was evidence of perineural invasion. Immunostains showed cytokeratin and synaptophysin positivity, with a weakly positive PSA.\nShortly after his biopsy, the patient again presented to the hospital with worsening renal function. Ultrasound at this time revealed bilateral hydronephrosis. A CT of the abdomen and pelvis showed a prominent nodule in the inferior aspect of the prostate measuring 4.2 cm by 5.0 cm by 4.2 cm, which extended posterolaterally to the right (Figures -). There was evidence of invasion of the left anterolateral aspect of the rectum and the bladder anteriorly. There were also two markedly enlarged right pelvic sidewall lymph nodes lateral to the external iliac vessels, measuring 2.2 cm by 3.3 cm and 1.8 cm by 2.7 cm, respectively. Besides, there was evidence of disseminated sclerotic osseous metastasis across the axial and appendicular skeleton representing prostate cancer metastasis. Lastly, there was confirmed moderate bilateral hydronephrosis with ureteral prominence. He next underwent bilateral percutaneous nephrostomy (PCN) pigtail catheter placement, and then received his first cycle of carboplatin [area under the curve (AUC) 5] with etoposide (100 mg/m2). Soon after this, the patient developed gross hematuria from his right PCN and associated anemia, all of which ended up resolving in a week. A follow-up CT of the abdomen and pelvis two weeks after his first cycle of chemotherapy showed worsening of the numerous osteoblastic lesions scattered throughout the osseous structures, which was consistent with osteoblastic metastatic disease. The right pelvic lymphadenopathy also progressed in the given interval. The patient was planned for the second cycle of chemotherapy; however, after extensive discussion with him and his family, the patient decided to pursue hospice care. The decision to opt for hospice care was made in just over three months from the time of diagnosis. |
A 67-year-old male chronic smoker with medical history significant for chronic obstructive pulmonary disease was admitted for imaging guided biopsy of a 1.2 cm left lower lobe lung nodule found recently on a computed tomography (CT) scan of the chest. The nodule was highly suspicious for primary lung malignancy. The patient was placed in a prone position and lung parenchyma in the posterior lateral left chest was visualized. Under CT guidance, a 19-gauge guide was advanced into the left lower lobe and two separate 20-gauge core biopsy specimens were obtained from the mass. There was no hemorrhage or immediate post procedure complication. However, towards the end of the procedure, the patient started complaining of sudden onset of chest pain and became unconscious. No palpable pulses were identified. A code blue was called and cardiopulmonary resuscitation was begun according to Advanced Cardiovascular Life Support guidelines. The patient subsequently demonstrated ventricular fibrillation which responded to defibrillation shock and epinephrine. The patient had three cycles of chest compressions, one dose of epinephrine, and a shock of 200 J. He was successfully resuscitated and intubated for mechanical ventilation. A CT scan of the chest was obtained immediately after the resuscitation and it demonstrated development of a small anechoic area in the left cardiac ventricle consistent with air embolus (Figure ).\nThe vital signs recorded at the time were as follows: blood pressure of 130/80 mmHg, pulse rate of 90 beats per minute, respiratory rate of 18 breaths per minute, and normal oxygen saturation of 92% on room air. The patient was admitted to the intensive care unit. Bedside transthoracic echocardiogram (TTE) did not reveal evidence of an air embolus. Additionally, no cardiac wall motion abnormalities were noted. The patient remained hemodynamically stable for 24 hours, and he was successfully weaned off from the mechanical ventilator next day and discharged home in a stable condition. |
A 55-year-old-male patient, who was a farmer by profession, presented to our Dermatology out-patient Department with complaint of a long-standing, non-healing, painless lesion over his genitalia for last 12 month. Initially there was an erythematous nodule that gave rise to multiple ulcers over the glans penis and around the urethral meatus.\nThe patient was married with two children. There was no history of any high risk behavior including history of exposure in either the husband or the wife. The wife had no symptoms and her clinical examination was within normal limits. There was no history of any similar lesion in the past, bleeding from the lesions or any urethral discharge. He had no history of any evening rise of temperature or loss of weight in the recent past. He had been vaccinated with BCG vaccine at birth. He had tried different treatment modalities without any response. He was in good health overall, but concerned about the genital lesion.\nOn examination of the penis, the prepuce was mobile and retractable. There were multiple shallow ulcers, some of them confluent, with undermined edges and yellow granulomatous indurated base over the glans penis. They were of variable sizes and measured from 3mm to 5mm and from 4mm to 6 mm in size []. There were no signs of tenderness and inguinal lymphadenopathy. Examination of rest of the genitalia was within normal limits.\nLaboratory investigations revealed a raised ESR (40 mm for 1st hour). Serologic tests for HIV and VDRL tests were negative. Dark field examination for Treponema pallidum, Gram stain for Haemophilus ducreyi, smear for Donovan bodies and Tzanck smear for giant cells were all negative. Microscopic examination of urine and culture revealed no abnormality. Acid fast bacilli were not demonstrable in either scraping from the ulcer base, urine or sputum. Ultrasonography of the kidney, ureters and bladder was within normal limits. Mantoux test was strongly positive (22 × 25 mm) []. Chest radiograph was within normal limits. A biopsy from the lesion revealed characteristic findings on histopathological examination. The epidermis was ulcerated with an epithelioid granuloma with central caseation necrosis. Numerous Langhans type of giant cells were seen with lymphoid cell infiltration [Figures and ].\nBased on the above-mentioned findings, the patient was diagnosed with tuberculosis of the glans penis. He was started on anti-tuberculosis therapy (ATT) under Category I as per the Revised National Tuberculosis Control Program (RNTCP). The patient came for regular follow-up and there was complete subsidence of the lesions after 6 months of therapy with ATT []. |
A 50-year-old man, slaughter house worker by occupation, presented in August 2019 with an episode of loss of consciousness with subsequent right-sided weakness and giddiness. He was a tobacco chewer since the past 20 years. He was not a known diabetic or hypertensive and was presently not on any medications. The past history revealed that he was a treated case of DLBCL 5 years back. He had then presented with enlarged lymph nodes in the left supraclavicular region and multiple abdominal lymph nodes. His disease was confirmed on biopsy and his bone marrow was uninvolved. For the same, he was treated with six cycles of Dose Adjusted Rituximab, Etoposide, Prednisolone, Vincristine, Cyclophosphamide, Doxorubicin (R-EPOCH) regimen. In view of low cell counts during chemotherapy and an episode of pneumonitis, he was administered granulocyte colony stimulating factor and pegfilgrastim. Positron emission tomography scan after four cycles did not show the presence of any metabolically active disease in the body and was suggestive of complete remission. He completed six cycles of chemotherapy in July 2014 and was not given any radiation. He was on regular follow-up since then, without any evidence of recurrence.\nOn examination for his present complaints, he had reduced power in the right upper limb along with increase in tone. Clinically, a relapse of lymphoma with involvement of the central nervous system (CNS) was suspected. Magnetic resonance imaging showed a 5.5 cm × 4.8 cm × 3.2 cm well-defined hypodense lesion in the left parietal lobe with rim enhancement on postcontrast study. Disproportionate perilesional edema was seen extending into splenium and causing mass effect in the form of effacement of lateral ventricle, subfalcine herniation, and midline shift of 8 mm towards the opposite side []. These features were suggestive of CNS involvement by lymphoma. Bone marrow biopsy and examination of the cerebrospinal fluid did not show involvement by lymphoma cells.\nA stereotactic biopsy was planned and left parieto-occipital craniotomy was performed. Intraoperatively, the tumor was soft suckable, moderately vascular with cystic fluid and showed perilesional edema.\nFor pathologic examination, multiple gray brown, soft to firm tissue bits were received, aggregating to 5 cm × 4 cm × 0.8 cm. The largest bit measured 2.5 cm × 1 cm × 0.8 cm and on cut surface showed gray white areas, along with few yellowish necrotic and focally congested areas. Microscopic examination revealed a highly cellular tumor with varied morphology. Tumor cells were arranged in sheets amidst a fibrillary background. Some of the tumor cells were polygonal with abundant eosinophilic cytoplasm and vesicular nucleus having irregular contours, while other cells were spindle shaped and arranged in fascicles with plump hyperchromatic nuclei. Multi-nucleate tumor giant cells and bizarre cells were also seen. There was marked nuclear pleomorphism and anisocytosis. Many atypical mitotic figures, areas of palisaded and nonpalisaded necrosis along with microvascular proliferation were seen [Figure -]. Adjacent cerebral cortex included in the biopsy showed evidence of tumor cell infiltration. No atypical lymphoid cells were seen. On immunohistochemistry (IHC), the tumor cells were positive for glial fibrillary acid protein (GFAP) [] and negative for Leucocyte Common Antigen (LCA). Hence, an impression of a high grade malignant glial neoplasm favoring Glioblastoma – WHO Grade IV was given. The patient received external beam radiotherapy to partial brain at a dose of 59.4 Gy, 33 fractions over 6.5 weeks and concurrent treatment with temozolomide. The patient remains free of disease, two months post-radiotherapy and has been advised regular follow-up. |
A 74-year-old man experienced multiple episodes of hematemesis of quantity approximately 1,500 ml, over a span of 24 h. Initially he was taken to a local hospital where fluid resuscitation was done and he was transferred to our centre on inotropic support for further management. Upper gastrointestinal (UGI) endoscopy was done in view of hematemesis, which was normal. Patient was evaluated further with computed tomography (CT) abdominal angiography, which revealed no evidence of any mucosal or submucosal pathology in relation to the UGI tract. Patient developed two more episodes of hematemesis one on third and the other on seventh day of hospital admission. Repeat UGI endoscopy revealed fresh blood trickling from nasopharynx and oropharynx and clotted blood in esophagus and stomach []. In view of fresh blood in nasopharynx, CT angiography of neck and paranasal air sinuses was performed. The sphenoid sinus and posterior ethmoidal air cells showed areas of hyperdensities indicating hemorrhage. In addition, CT angiography revealed a large saccular aneurysm within right sphenoid sinus arising from cavernous ICA []. The aneurysm was projecting medially into sphenoid sinus with breach in the lateral sinus wall. Digital subtraction angiography revealed a wide necked saccular aneurysm arising from medial wall of proximal cavernous ICA measuring 17 × 15 mm with its neck measuring 8 mm. It is projecting medially and anteriorly with teat in its medial aspect. Manual cross-compression test done during angiography showed good cross-flow across Anterior Communicating Artery (AcomA) into right middle cerebral artery (MCA) and anterior cerebral artery (ACA) branches with venous delay of less than 2 s in right cerebral circulation []. In view of wide neck ruptured right cavernous ICA aneurysm, patient was given definitive options for surgical external carotid artery-middle cerebral artery bypass, endovascular flow diverter placement, and parent artery occlusion. Patient has given consent for flow diverter placement with additional coiling. However, on the day of the procedure, the angiogram revealed partial thrombosis of the ICA aneurysm with extension of thrombus into ICA. Hence, a decision for parent artery occlusion was taken. An 8 Fr femoral sheath was inserted in the right femoral artery. A 6 Fr Neuron Max (Penumbra, Inc. Alameda, USA) guiding sheath was placed in right proximal cervical common carotid artery. A 5 Fr Navien guiding catheter (Covidien Rendezvous, Paris, France) was placed in right proximal cervical internal carotid artery. A ballon occlusion test was performed which demonstrated good cross-flow across AcomA into right MCA and ACA branches with venous delay of less than 2 s. An Echelon10 (ev3, Toledo Way, Irvine, USA) micro-catheter was advanced coaxially through the guiding catheter into the aneurysm and segmental trapping of the aneurysm and petrous ICA was done using Axium detachable coils (ev3, Toledo Way, Irvine, USA). Check angiogram showed complete non-opacification of the aneurysm with good cross-flow across AcomA []. The procedure was uneventful and the patient was discharged in a stable neurological condition. On follow-up, over a period of 6 months there had been no further episodes of hematemesis. |
A 14-year-old male with severe MPS VI was referred due to a progressive reduction in mouth opening. The trismus was first noticed at laryngoscopy 2 years previously, and subsequent intubations for ophthalmic surgery had become increasingly difficult. Although he presented with typical MPS morphology, he was less affected than his older sibling, a difference attributed to treatment with enzyme replacement therapy from 8 weeks of age [].\nOn examination, the patient’s maximal inter-incisal distance was only 8 mm (). A CT scan confirmed marked coronoid hyperplasia bilaterally, and the thickened anterior borders of the rami created concave surfaces on the superolateral aspect of the mandible (). The condyles appeared hypoplastic although otherwise fully functional and non-ankylosed, and the superior aspect of the coronoid processes deviated laterally, curving over the superior aspect of the zygomatic arch on both sides. (). In addition, exophytic bone deposits had formed on the medial aspect of the zygomatic arches suggestive of either benign exostosis or reactive hyperplasia ().\nThe indications for surgery included difficulties with daily activities, as well as the projected likelihood of regular intubations and further surgery in the near future. The patient subsequently underwent bilateral coronoidectomies and removal of the zygomatic arch exostoses under general anaesthesia. Due to the predicted difficulty with direct laryngoscopy, a fibre-optic technique was used. Due to the young age of the patient, anaesthesia was induced by a mask, and a fibre-optic bronchoscope was placed through the nose and positioned in the trachea. An endotracheal tube was then guided over the bronchoscope, and the subsequent general anaesthesia was uneventful.\nAs expected, the lateral concavity of the mandible limited intra-oral access to the sigmoid notches, and a bicoronal flap was raised. Osteotomy and lateral reflection of the right zygomatic arch were required for access to divide the temporalis and complete the coronoidectomy on that side. A partial improvement in mouth opening resulted, facilitating easier coronoidectomy and removal of the exophytic bone without arch division on the left side (). The mandible was vigorously mobilised, and an intraoperative mouth opening of 40 mm was achieved ().\nThe patient was transferred to ICU for recovery, with the endotracheal tube left in overnight. The bite block was maintained in situ for 48 hours to stretch local soft tissues and encourage cognitive awareness of the expected range of mandibular movement. The patient subsequently made uneventful progress. At Day 7, the scalp sutures and staples were removed under general anaesthesia, and the bite block was reinserted for a further 48 hours.\nPostoperative physiotherapy commenced immediately after removal of the bite block, utilising a TheraBite® at maximal opening for 3 minutes, before meals. Excellent mouth opening has been maintained over the follow-up period with unassisted inter-incisal measurement of 45 mm, and assisted opening of 50+ mm, at 18 months post operation ().\nHistopathology of the zygomatic exostoses showed essentially normal cortico-cancellous bone, with small amounts of additional fibro-cartilage, and the aetiology remains uncertain. |
A 47-year-old premenopausal woman presented to the outpatient breast cancer clinic of our hospital because of a palpable mass in the upper outer quadrant of her left breast. Ultrasonographic examination revealed heterogeneously dense breasts, with a solid mass measuring 3.6 cm × 2.6 cm in her left breast and one smaller solid mass. Axillary ultrasound showed multiple enlarged lymph nodes in the ipsilateral axilla, with a maximum diameter of 7.4 mm. Ultrasound-guided core biopsies of the masses in the left breast and fine-needle aspiration cytology (FNAC) of the lymph node in the left axilla were obtained. Pathological examination of the tissue from each of the solid masses revealed grade III invasive ductal carcinoma. The tumor expressed estrogen receptor and progesterone receptors but did not over-express Her2/Neu. The specimen from the axillary lymph node contained malignant cells. Subsequent whole body positron emission tomography with 18-fluorodeoxyglucose (18FDG-PET) scanning showed positive uptake in both left and right axilla and in the left breast. Consequently, FNAC of right axillary lymph node was obtained, which also contained malignant cells. Computed tomography (CT) of the chest and abdomen after the administration of contrast material and a bone scan showed no evidence of metastatic disease outside of the breast and axillae.\nThe patient underwent neoadjuvant chemotherapy consisting of 5-fluorouracil, epirubicin, and cyclophosphamide for six cycles, every three weeks. After extensive discussion, she elected to undergo modified radical mastectomy (MRM) of her left breast, bilateral axillary lymph node dissection, and prophylactic MRM of her right breast. Tissue from the left breast contained a grade III invasive ductal carcinoma 2.0 cm × 2.0 cm, with negative margins. There was no evidence of carcinoma in the tissue from the right breast. Of 5 dissected lymph nodes from the left axilla, 4 contained metastases. Eleven of 14 dissected lymph nodes from the right axilla were positive. Following surgery, she underwent locoregional radiotherapy for a dose of 50 Gy in daily fractions of 2 Gy to the chest wall via opposing tangential fields with 6 MV photons. Overlapping fields were avoided by matched midline technique using the CT simulator. The supraclavicular and internal mammary fossae were not included in the target volume. After discussing the risk and benefits of adjuvant hormonal therapy, she elected to start with adjuvant letrozole.\nUnfortunately, at followup 27 months after primary surgery, a mass was found at the presternum on physical examination. Ultrasound examination revealed a subcutaneous mass measuring 3.3 cm × 1.1 cm. Ultrasound-guided core biopsies of the lesion showed grade III invasive ductal carcinoma that was positive for ER and PR, but negative for Her2/Neu. Subsequent 18FDG-PET/CT showed no evidence of distant metastatic disease. |
A 54-year-old woman was referred to us by the Department of Plastic and Reconstructive Surgery in our medical center for further evaluation of prolonged right nipple erosion. She had previously received a diagnosis of right breast cancer and undergone total mastectomy and axillary dissection 15 years ago in another hospital. Histopathology identified an invasive ductal carcinoma with a tumor diameter of 0.9 cm and a nuclear grade of 2; one of 23 lymph nodes showed metastasis. Tissue profiling revealed the following: estrogen receptor (ER), positive; progesterone receptor (PgR), negative; and human epidermal growth factor receptor 2 (HER2), undetermined. As adjuvant therapy, she received six cycles of cyclophosphamide, methotrexate, and 5-fluorouracil (CMF), followed by tamoxifen for 3.5 years. Then, she defaulted her endocrine therapy. Seven years after the surgery, the doctors from our medical center performed ABR with a deep inferior epigastric perforator (DIEP) flap at another facility. The following year, her right nipple was reconstructed by V-shaped resection of the left nipple and an autologous grafting/nipple-sharing technique. The right areola was reconstructed with a penetrating skin graft from the proximal thigh and left areola. Concurrent mastopexy was performed for the left breast (Fig. a).\nAfter 7.5 years, right nipple erosion appeared, and she visited the Department of Plastic and Reconstructive Surgery in our medical center. At first, it appeared that she had an eczematous nipple lesion caused by an infection, and she was treated with antibiotics; however, the erosion progressed and enlarged over the course of a few months. She was eventually referred to our department. The skin on the right side of her chest around the NAC and the subcutaneous adipose tissue consisted of transferred tissue from her abdomen, as the DIEP flap and grafted nipple were constructed on the skin graft. In the right nipple, normal tissue was almost completely affected by erosion, and there was no abnormality, itching, or pain in the right areola (Fig. b). Scrape cytology revealed malignancy of the epithelial cells, and that the right nipple carcinoma originated from the tissue taken from the left nipple. On magnetic resonance imaging (MRI), the malignant lesion seemed limited to the area around the grafted right nipple (Fig. a), with no malignancy observed in the left breast on MRI and computed tomography (CT) (Fig. b). In addition, no distant metastases were observed on CT. Paget’s disease was clinically suspected, and we performed surgical treatment. Though the standard surgical operation for mammary Paget’s disease is mastectomy, we performed partial breast excision including the right nipple with sufficient lateral and deep margins (Fig. a–c) because there was no mammary tissue in the right reconstructed breast, except for the nipple and areola. The incision was closed with investing sutures.\nThe specimen submitted for surgical pathology was composed of epithelial and adipose tissue grafted from the abdomen, areolar tissue grafted from the base of the thigh and left areola, and a nipple graft from the contralateral side. Macroscopically, the lesion spread around the nipple and adipose tissue (Fig. a). Pathological examination identified invasive ductal carcinoma with a few comedo ductal components within the nipple, extensive infiltration of grafted epithelial and adipose tissue (Fig. b), and a tumor diameter of 25 mm. The nuclear grade score was 2 (nuclear atypia score was 2 and mitotic count score was 2), there was no lymphatic or vascular invasion, and the lateral and deep margins were negative. Immunohistochemical staining showed strong positive for ER, weak positive for PgR, positive for HER2 with a score of 3+, and 35% cells showing positive Ki-67 staining.\nAs the biological profile classified the tumor as a luminal HER2 type, weekly paclitaxel, trastuzumab, and endocrine therapy were administered as adjuvant therapies. No distant metastases or local recurrence were seen 1 year after the surgery. |
A 50-year-old female with no significant past medical history initially presented to an urgent care center for 1-day history of acute abdominal pain associated with nausea and emesis. Medical evaluation included an upright chest X-ray and abdominal X-ray which demonstrated free air under the diaphragm (Fig. ). The patient was immediately transferred to the emergency department.\nIn the emergency department, the patient's vital signs were notable for tachycardia at 110 bpm and hypotension at a blood pressure of 105 mm Hg systolic. Other vitals included 18 respirations per min, 98% oxygen saturation, and a temperature of 37°C. She demonstrated signs of dehydration with dry mucosa and cool, clammy, and slightly diaphoretic skin. She had clear lungs to auscultation. Abdominal examination showed mild distension but with absence of surgical scars or other lesions. On palpation, there was diffuse tenderness more pronounced in the right side of the abdomen, right upper quadrant, and epigastric region consistent with peritonitis concerning for perforated viscus. Fluid resuscitation and parenteral antibiotics were administered, and the patient was taken emergently to the operating room. A diagnostic laparoscopy was performed and demonstrated significant inflammation and contamination with enteric contents in the right side of the abdomen and pelvis. Due to the severity of inflammation and inadequacy of visualization, a laparotomy was performed. We found a perforated small intestine diverticulum located on the mesenteric side about 100 cm proximal to the cecum. Once the contamination was controlled, an oncologic segmental small intestine resection with adjacent mesentery with side-to-side functional end-to-end anastomosis was performed. Figure displays the perforated small intestine diverticulum at the time of surgery.\nFinal gross pathological examination displayed a 7.5-cm segment of small bowel with a 5.5 × 3.0 cm outpouching consistent with a diverticulum near its midpoint. The diverticulum shows a 1.0 × 0.7 cm perforation with surrounding hemorrhage, and at the tip of the diverticulum, a 2.7 × 2.5 cm firm mass-like area was noted and is 0.8 cm from the perforation and 1.5 cm from the mesenteric margin. Microscopic examination showed a moderately cellular, predominantly spindle cell neoplasm with moderate nuclear pleomorphism. Approximately 3 mitotic figures per 50 high-power fields were noted. No tumoral necrosis was appreciated. Immunohistochemical (IHC) staining for the CD117 antigen (c-kit proto-oncogene) and DOG-1 was performed and was positive, confirming the GIST diagnosis. Stains for S-100, actin, and desmin were negative. Hematoxylin and eosin (H&E) staining displayed characteristic spindle cells and epithelioid nature. Figure displays both the H&E and IHC staining utilized for GIST diagnosis confirmation.\nThe patient recovered well, and the postoperative course was uneventful. She was discharged home on postoperative day 5. At 4-month follow-up, the patient continued to do well. An upper GI endoscopy was performed and revealed no evidence of gastric or duodenal abnormalities suggestive of GIST. She continues to do well 2.5 years after surgery.\nBased on tumor size and mitotic index, tumor risk categories were evaluated according to the National Comprehensive Cancer Network (NCCN) Clinical Practice Guidelines []. The patient was found to be low risk, and therefore postoperative chemotherapy was not indicated. Both perforated GI diverticulum and GIST are rare pathologies and when combined warrant careful consideration for optimal patient care. |
A 37-year-old Turkish woman was referred to the department of oral surgery because of pigmented areas in her mouth. She had noticed the pigmented lesions in her oral cavity 3 years ago and till the beginning of the treatment no major change in the oral cavity was observed by the patient. Oral examination revealed multiple, painless brown-black pigmentation, on the buccal mucosa, lower lip and posterior of the palate, bilaterally (). No cutaneous or fingernail lesions were observed. She was systemically healthy and was not on any medication. She was neither a smoker nor a habitual drinker of alcohol. There was no family history of abnormal mucocutaneous pigmentation.\nLaboratory investigation results, including a full blood count, hematinic levels, serum chemistry and inflammatory markers, were all within normal range. The patient underwent an upper gastrointestinal endoscopy, as well as a colonoscopy, which revealed no evidence of polyps. To rule out Addisons disease, the serum cortisol and adrenocorticotrophic hormone (ACTH) levels were measured and the values were normal. A biopsy was taken from the buccal mucosa with the aim of excluding melanoma. Histopathological examination revealed lentiginous proliferation of melanocytes. Inflammatory changes or malignant features were not noted in any area. A diagnosis of LH syndrome was made based on the clinical presentation of lesions coupled with the absence of systemic findings.\nAn Er:YAG laser with a wavelength of 2940 nm (Fotona Fidelis Plus III, Slovenia) was used because of its capability of superficially ablating oral soft tissues to treat oral pigmentation, especially on the lips, soft palate, and buccal mucosa, bilaterally. The parameters used were 120 mJ output energy, 10 Hz frequency, 1000 μs pulse duration, and a 0.8 mm spot size with the noncontact hand-piece. The fluence was 25 J/cm2 and the lasing was continued until the pigmentation on the intervention area was visibly ablated as there is no accumulation of energy or heat by Er:YAG lasers. The treatment was performed under local anesthesia with no water and air spray. No sutures or other means of bleeding control were needed at the end of the procedure. Postoperative healing was uneventful, only mild “burning” sensation was reported by the patient for a few hours postoperatively. The reason of the need for anesthetics is that the water spray was turned off in order to obtain hemostasis. The procedure was straightforward, scanning all the pigmented areas of the oral cavity and the lips, ablating the superficial layers of the tissues including the buccal mucosa and the soft palate.\nAs the area of pigmentation was rather large the surgical treatment was performed in 4 sessions. In each session one quadrant was chosen and cleared in order to reduce scarring. After the 4th session, the healing process occurred without any complication and the entire pigmentation had been cleared (). Two months later, however, a small pigmented area was seen, and it was cleared again in one appointment. By the 12th month of followup, the pigmentation had recurred, being observed in an area close to half of the initial situation (). |
A 45-year-old man presented to our clinic requesting evaluation for surgical treatment of chronic low back pain of more than 20 years duration. He started Judo at the age of 12 and suffered severe low back pain at age 17 and could not practice Judo for 2 months due to the pain. He did not visit a hospital that time and did not receive a diagnosis for this pain. It disappeared within 2 months of stopping Judo practice and resting at home. After this episode, he started to suffer from chronic low back pain. He continued Judo until he graduated from technical college and became a sailor.\nDuring his first year at sea, he experienced very strong back pain again and visited a hospital for the first time. Triple-level spondylolysis at L3–5 was diagnosed from plain radiographs. The pain subsided with a NSAID (nonsteroidal anti-inflammatory drug) and sick leave. For more than 20 years after, he suffered from strong back pain, requiring sick leave for a couple of days, 3 to 4 times a year. He consulted many orthopedic surgeons to solve the problem, and all doctors recommended NSAIDs and rehabilitation. He visited us seeking possible surgical treatment.\nPlain radiographs from his first visit to our clinic are shown in . Bilateral spondylolysis at L3–5 is evident on oblique films. There is no instability apparent on dynamic films (). CT scan () demonstrated pseudarthrosis at all three sites of spondylolysis, which cannot be expected to achieve bony healing with conservative care []. On magnetic resonance imaging (MRI) (), no other degenerative spinal disorders such as herniated nucleus pulposus or spinal canal stenosis were found. Slight disc degeneration at L3-4, L4-5, and L5-S1 was seen. Effusion was also seen around the pars defects and surrounding facet joints (), indicating inflammation in the space [].\nOn initial presentation, he rated his low back pain at 1-2 out of 10 on a visual analog scale. The pain increased on lumbar extension but not flexion. Tenderness was noted on the spinous processes of L3–5. All neurological findings were normal, and there were no positive tension signs, including the femoral nerve stretch test and the straight leg raise test. It was difficult to decide the surgical indication for this case, since usually his pain was moderate at consultation. Usually, direct repair surgery would be indicated after confirming the pain to originate from the defects by steroid infiltration into them [, ]. In this case, we decided to conduct direct repair surgery for all three levels since there were no other obvious degenerative disorders causing low back pain on radiological investigation.\nA pedicle screw and hook-rod systems were utilized for L3–5. After debridement and decortication of the pseudarthroses, these systems were installed (). Autologous bone was harvested from the iliac crest and was grafted on the defects. One month later, he returned to his job as a captain of an international passenger ship. His chronic back pain disappeared after the surgery. Six months after the surgery, he complained of an abnormal metallic sound and discomfort in his back during lumbar twisting motion. Amongst the implants, the hook for L3 and the pedicle screw head for L4 were closely located (), and therefore we hypothesized that contact of these two components was causing the sound. After confirming bony union of the L3 pars defects, the L3 implants were removed. After removal, the noise and discomfort were resolved. At the 2-year followup, the patient had no complaints of low back pain and had not experienced any further pain attacks. Dynamic radiographs that demonstrated motion had been preserved (). |
A forty-four year old woman presented to us with a five days history of multiple painful papulo vesicular lesions distributed along the right D9 dermatome. She had no known co-morbidities prior to the onset of the skin lesions. She was clinically diagnosed a case of HZ. Tzanck smear performed from the lesions revealed multinucleated giant cells confirming the diagnosis. Other investigations were found within normal limits. Acyclovir therapy was deferred as she had presented on the fifth day after the onset of the lesions. She was managed symptomatically with analgesics and topical soothing agents. Lesions started healing with crusting and post inflammatory pigmentary changes.\nTwo days later, she reported again with multiple erythematous itchy papules all over the body. In view of dissemination of lesions, the possibility of HZ with dissemination was kept. However, on closer examination, it was observed that some of the lesions were targetoid papules and plaques with central hyper pigmentation and at places fluid filled vesicles ().\nTherefore, in view of morphology of lesions, provisional diagnosis of EM secondary to HZ was kept. She was in good health without any sign and symptom of immunosuppression. There was no mucosal involvement or lymphadenopathy. She was clinically suspected to have EM secondary to HZ. Tzanck smear prepared from new lesions did not reveal any multinucleated giant cell. Other Investigations revealed leukocytosis of 10,000/mm3 with 70% neutrophils, 1% eosinophils, and 29% lymphocytes. The C-reactive protein was of 5.9 mg/dl. Her liver function test and urine analysis were within normal limits. Her serology for Human immunodeficiency Virus, cytomegalovirus and mycoplasma were found negative. Antinuclear antibodies were also found negative. Histological examination of a skin biopsy showed a sub epidermal bulla with eosinophilic necrosis in the epidermis. A few inflammatory cells were present in the necrotic epidermis. There was a slight perivascular lymphocytic infiltrate in the papillary dermis with few histiocytes and plasma cells. There was no evidence of viral infection. These histopathological findings were consistent with our diagnosis of EM. VZV DNA studies could not be performed due to lack of availability. |
A 58-year-old woman was referred from a primary clinical to the Department of General Surgery at Asan Medical Center (Seoul, Korea) for an inguinal hernia. She had noticed reducible swellings on both inguinal areas for 10 years but did nothing until the swellings became problematic. These swellings had increased 7 months earlier, accompanied by pain. Ultrasound examination showed a well-defined cyst in the right inguinal area, and a mass with cystic and solid portions in the left inguinal area. Computed tomography showed a 7.4 cm round homogeneously enhanced mass in the left inguinal canal with an enlarged lymph node in the left para-aortic area ().\nThe mass excised from the left inguinal area was well-defined and firm, measuring 7×7×6 cm in size. The cut surface was homogenously creamy white and vaguely lobulated with focal hemorrhage (). The mass in the right inguinal canal was heterogeneously golden yellow to pinkish gray and encapsulated (). Microscopically, the mass of left inguinal area consisted of large uniform cells growing in broad sheet divided by thin septa which infiltrated by lymphocytes. The nuclei of the tumor cells were centrally located and round to polygonal with finely granular chromatin and prominent nucleoli. The cytoplasm was eosinophilic to clear and had distinct cell border (). These features were consistent with seminoma of classic type. Adjacent to the mass was soft tissue composed of atrophic hyalinized seminiferous tubules and interspersed Leydig cell nests in between, compatible with testicular tissue (). The mass from right inguinal canal also had testicular tissue with atrophic hyalinized seminiferous tubules, but the leydig cell nests were larger and more abundant, consistent with leydig cell hyperplasia (). This mass was removed surgically, as was the reducible mass in the right inguinal canal, followed by herniorrhaphy.\nPostoperatively, the surgeon in charge requested a detailed examination of this patient by a gynecologist for further evaluation and treatment. The patient was amenorrheic for her entire life, is unmarried, and a virgin. She was 170 cm in height and weighed 80 kg. Physical examination showed that her breasts were normally developed, but her pubic hair and axillary hair were sparse. She had slightly enlarged external genitalia with a blindly-ending vagina about 2 cm long. Chromosomal analysis showed a 46,XY karyotype, but none of her previous gynecological examinations had diagnosed any distinct features. Her serum testosterone concentration was 0.25 ng/mL (normal male range, 2.6-15.9 ng/mL) and her estradiol concentration was 12.1 pg/mL (normal male range, 0-44 pg/mL). Based on these findings, the patient was diagnosed with AIS. Because computed tomography suggested metastasis along the left para-aortic lymph node, adjuvant radiotherapy and chemotherapy were recommended. |
In October 2012, a 28-year-old female was referred to the local hospital due to two missed menstrual periods one month previously. Abdominal ultrasonography revealed a single normal fetus in the uterus and a right adnexal hypoechoic solid mass of 4.1×3.2 cm in size. The mass had been identified three years previously with a diameter of 2.5 cm during a routine gynecological check up and was followed-up by an ultrasound scan every six months for three years, which showed that the size of mass had remained stable. No further measurements were recorded due to the stability of the mass for a long period of time, without related symptoms. However, 16 days following the initial visit, the patient presented to the local hospital once again with a complaint of acute right lower abdominal pain following activity. An emergency ultrasound examination revealed that the adnexal mass had increased to 7×6.5 cm in size, without characteristics of torsion or rupture. The pain was significantly alleviated 1 h later without any therapy. Due to the increasing size of mass and the presence of pain, the patient was referred to Zhejiang Provincial People’s Hospital (Hangzhou, China) for surgical intervention.\nDuring the following days, mild intermittent abdominal pain was experienced, without fever, vomiting, vaginal bleeding or uterine contractions. Approximately one week later the patient was admitted to Zhejiang Provincial People’s Hospital (Hangzhou, China) with slight abdominal pain on the right side. Physical examination revealed a tender mass of ~11×10 cm in size, located in the right upper abdominal quadrant region. The mass was palpable and movable, with clear edges. During the pelvic examination, the cervix was normal and no amniotic fluid or bloody discharge was observed. The uterus was movable with no tenderness, and was of the size corresponding to the gestational age.\nUltrasound revealed a single live fetus and a partial cystic adnexal mass of 10.4×10×6.6 cm in size ().\nSurgery was scheduled to remove the mass one week later. The patient underwent laparoscopic ovarian tumorectomy under endotracheal intubation and general anesthesia. During the surgery, basic vital signs, including oxygen saturation and the end-tidal carbon dioxide level of the patient, were monitored continuously by the anesthetist. The laparoscope revealed an enlarged uterus of the size corresponding to that expected for a three-month pregnant uterus, normal bilateral fallopian tubes and a normal left ovary. A mass arising from the right ovary was ~18×16×10 cm in size and grossly appeared solid, with smooth/shiny surfaces and marked superficial vascularity. No adhesion or infiltration was observed in the pelvis. The ovary was preserved and the mass was excised. Following the completion of tumor enucleation, the ovarian defect was sutured with 2–0 absorbable VICRYL suture (Johnson & Johnson Medical Ltd., New Brunswick, NJ, USA) in a continuous manner, while bipolar electrocoagulation was used to secure hemostasis. The samples were removed using the bag-retrieval technique, where a 35×25 cm bag was used to remove the mass through the extended left trocar incision, and were subsequently sent to the Department of Pathology (Zhejiang Provincial People’s Hospital) for histological testing. The examination of frozen sections reported a benign spindle cell tumor with focal ischemic infarction. Pathological examination of the paraffin section following surgery demonstrated that the mass was comprised of typical smooth muscle cells, which formed strands and bundles arranged in a whorled interlacing pattern. Microscopically, ischemic infarction focus of the mass was observed, while significant nuclear atypia or pleomorphism was absent. Immunohistochemical staining with antibodies against vimentin, inhibin, α-smooth muscle actin and the α-helical rod domain of desmin was performed to confirm the diagnosis of this rare tumor. The tumor cells stained positively for vimentin, inhibin and α-smooth muscle actin, but not for the α-helical rod domain of desmin, which aided in confirming the diagnosis of ovarian leiomyoma ().\nFetal surveillance monitoring via ultrasound prior to and following surgery indicated that the surgery had been successful. Prophylactic antibiotics and progesterone at a dose of 20 mg twice daily for four days were prescribed following surgery to prevent miscarriage. The surgery time, defined as the period between the skin incision and the closure of the skin, was 118 minutes and the estimated blood loss was 800 ml. The patient experienced an uneventful postoperative recovery. Routine follow-up after discharge was conducted every six months for three years and no complications were observed. A 3.4-kg healthy baby was delivered spontaneously at full term. |
A previously healthy 57-year-old woman, with no significant past medical history, presented to the surgical department of our hospital for definite management of a primary pancreatic leiomyosarcoma, after being treated with adjuvant chemotherapy.\nOne year before her last admission, she was initially admitted to our emergency department due to abdominal pain, fatigue, and weight loss. She was totally healthy prior to these symptoms. She then underwent magnetic resonance imaging (MRI) that was indicative of a pancreatic head lesion along with possible metastatic liver lesions, superior mesenteric vein occlusion, and portal vein infiltration (Fig. a, b). The decision was to undergo an endoscopic ultrasound (EUS) biopsy in order to determine the exact nature of the lesion. EUS report was indicative of pancreatic leiomyosarcoma.\nMultidisciplinary team’s decision was to use gemcitabine- and docetaxel-based chemotherapy as up-front treatment to assess tumor response. Follow-up CT scan and magnetic resonance imaging (MRI) after the completion of chemotherapy regimen showed downsizing of the pancreatic mass, as well as downsizing of suspicious for malignancy segment III liver lesion (Fig. c, d).\nBased on the response to chemotherapy, tumor characteristics, and physical status of the patient, multidisciplinary team’s decision was to proceed to surgical exploration. Due to local expansion of the pancreatic tumor, its relation with the superior mesenteric and portal vein, and the underlying SMV thrombosis, excision of the pancreatic tumor was not feasible. Intraoperatively, a small piece of tumor was excised in order to be sent for histopathology. Surgeon’s decision was to ablate the tumor with irreversible electroporation (Fig. ). Metastatic liver lesions were identified with the use of intraoperative ultrasound. Segment III liver lesion was resected, while smaller lesions of the right lobe were ablated using microwave ablation.\nThe patient had an uneventful postoperative recovery and complete resolution of her symptoms. Histopathological examination of pancreatic lesion as well as segment III liver lesion revealed sarcomatous tissue of high cellularity with fascicular pattern, increased mitotic activity, and diffuse cytoplasmic immune reactivity for SMA, desmin and h-Caldesmon, and chromagen DAB (Figs. and ). Surprisingly enough, pathological report of a smaller liver lesion was indicative of angiomyolipoma staining positive for HMB45 and Melan-A. The lesion was a benign hamartomatous, circumscribed but unencapsulated hepatic mass composed mainly by mature lipocytes and limited mesenchymal component (smooth muscle cells), showing no marked atypia and thick-walled vasculature. Myoid component was positive for ΗΜΒ-45 and Melan-A. Based on the histopathological report, tumor board decided that the patient should be treated with adjuvant therapy for leiomyosarcoma after surgery. A regimen with anthracycline and olaratumab was used for 3 months. Follow-up imaging in 6 and 12 months showed no progression of the disease (Fig. a–d). |
A 45-year-old right-handed woman with a past medical history of hypertension and stroke presented to the emergency department (ED) with a 2-day history of severe left shoulder progressively worsening pain and limited movement due to the pain. Aside from the pain on her left shoulder, the patient reported no associated trauma, weakness, numbness, left upper-extremity tingling, shortness of breath, chest pain, or fever. Her ED vital signs were as follows: temperature 36.9°C, blood pressure 132/70 mmHg, heart rate 73 bpm, respiratory rate 18 bpm, and oxygen saturation 98% on room air. The patient's electrocardiogram showed a normal sinus rhythm with no ST or T-wave abnormalities. Physical examination results were normal except for the left shoulder exam, where patient expressed severe pain with passive and active abduction of the humerus and some mild trapezial muscle and subacromial and subdeltoid area tenderness to palpation near the greater tuberosity of the humerus. The patient was able to actively abduct her left shoulder to 60°, but pain limited her ability to abduct further. The left arm was neurovascularly intact with normal motor and sensory functions of radial, median, ulnar, and axillary nerves. The patient had 5/5 motor strength of her shoulder upon internal and external rotation and with forearm supination; she stated no tenderness along her anterior shoulder near the long head of the biceps tendon in the bicipital groove.\nOn initial assessment, the triage nurse suspecting possible shoulder calcific tendonitis, considering the severe pain that the patient was experiencing, notified the ED physician assistant and the ED physician to facilitate rapid pain medication administration and a rapid bedside ED ultrasound evaluation of the patient's left shoulder. A focused bedside ED ultrasound examination of the patient's supraspinatus tendon was performed by an ultrasonographer ED attending physician, with more than 10 years of experience, after administering opiate analgesia to the patient (see Additional files and available as supporting information in the online version of this paper). With the patient in modified Crass position (where the patient placed her palm on her ipsilateral iliac wing and moved her elbow as posterior as possible), long-axis evaluation of the patient's supraspinatus tendon revealed calcification near the attachment to the greater tuberosity of the humerus, and a dynamic bedside ultrasound long-axis evaluation of the patient's supraspinatus tendon showed elevation of the greater tuberosity cranially to the level of the acromion and impingement of the supraspinatus tendon underneath the acromion of the scapula when the patient actively abducted her left humerus (Figures and ; Additional files and ). With a dynamic humerus abduction maneuver, we were able to directly see the calcified and thickened supraspinatus tendon causing the impingement syndrome with limited abduction, and this would not be possible with X-ray calcification diagnosis alone.\nIn the radiology department, proximal humerus plain film radiography showed two small soft tissue calcifications in the region of the supraspinatus tendon of the left shoulder, likely thought to be calcific tendinitis (Figure ). The patient's pain was controlled in the ED, and she was treated and released with oral pain medications, a left arm sling, and urgent orthopedic follow-up. |
An 81-year-old man with a major complaint of painful mass at the lower calf was referred to our institution. Pain and swelling in his left lower leg started to develop 2 weeks prior to his presentation to our clinic and progressively worsened, eventually rendering him unable to walk. While he denied any trauma to that leg, he had been taking aspirin 100 mg, amlodipine 5 mg, candesartan cilexetil 8 mg per day for 7 years to control his hypertension, and had a history of several times of injections in the left knee for 1 year because of osteoarthritis.\nPhysical examination showed severe calf swelling with tenderness; however, no local heat or redness was observed (). The patient's body temperature was 36.8℃, his dorsalis pedis artery and posterior tibial artery pulses were strong at the level of ankle and foot, and the neurological findings were normal. Knee joint line tenderness and 10° of flexion contracture were observed in the left knee. The left knee score was 51 points.\nThe patient's blood parameters were normal: white blood cell (WBC) was 8,450, C-reactive protein (CRP) was 0.49, and coagulation parameters and autoimmune parameters were normal. But platelet aggregation function assay was delayed. A plain X-ray of the left leg revealed Kellgren-Lawrence grade 4 osteoarthritis of the knee joint and increased soft tissue density along the medial aspect of the proximal lower leg (). Computed tomography angiography (CTA) showed a large mass along the subfascial layer in the medial aspect of the gastrocnemius, and no evidence of deep venous thrombosis. MRI revealed a large mass extending distally along the subfascia in the superficial aspect of the medial gastrocnemius with muscle compression. The mass measuring 13×5×3 cm showed a low-intermediate signal intensity on T1-weighted images and an intermediate-high signal intensity on T2-weighted images. These MRI findings suggested the presence of a hematoma and the inhomogeneity in signal intensity was attributed to hemorrhage within the mass (). Before operation, we could not rule out the possibility that MRI did not reveal any opening or stalk for direct communication between the mass and joint cavity. Therefore, an open mass excision was performed.\nWith the patient in the supine position under spinal anesthesia, a longitudinal skin incision was made along the medial side of the left calf. The mass was located along the subfascia in the superficial aspect of medial gastrocnemius and dark brown blood clots suggesting a hematoma was noted within the mass. During detachment from the surrounding tissues, communication between the mass and the knee joint was detected. After removal of the mass, communication with the joint cavity was repaired. The gram stain and bacterial culture of the aspirate, acid-fast, fungal staining, and culture were negative.\nHistopathological examination showed that the hematoma was surrounded by synovial membrane and was composed of thick fibrous tissue; it also contained macrophages that had phagocytosed red blood cells and hemosiderin (). The pathologic diagnosis was hematoma in the Baker's cyst.\nThe patient was followed up until 2 weeks after surgery when his symptom was relieved. |
A 73-year-old man who had undergone total gastrectomy for pathological T4aN2M0 gastric cancer according to UICC-TNM classification 8th edition [] followed by adjuvant chemotherapy with S-1 and docetaxel for 10 months developed liver metastases 1 year after gastrectomy. The tumors were located in segment 6 (17 mm in diameter) and segment 7 (27 mm in diameter). He was then enrolled in a phase 2 clinical trial of lenvatinib plus pembrolizumab and received the study treatment (20 mg oral lenvatinib once daily and 200 mg intravenous pembrolizumab every 3 weeks) for 5 months []. He had grade 2 oral mucositis and proteinuria according to the National Cancer Institute Common Terminology Criteria for Adverse Events (CTCAE version 4.03) during the study treatment []. Thyroid function tests revealed transient hyperthyroidism, which promptly returned to normal. He continuously achieved a partial response with the study treatment, and liver metastases were decreased in size to 8 mm in segments 6 and 11 mm in segment 7 (Fig. ).\nSince the tumors were judged to be systemically under control due to decrease in size of liver metastases and no new lesions by positron emission tomography/computed tomography images, they came to be considered resectable. Four weeks after the last dose of pembrolizumab and 2 weeks after the last dose of lenvatinib, the patient underwent laparoscopic partial hepatectomy for segments 6 and 7. The operation time was 152 min, and blood loss was 109 mL (Fig. ).\nOn the 1st postoperative day, his body temperature was elevated to 40.4 °C. The patient complained of general fatigue, which seemed to be unproportional to the physical stress caused by laparoscopic resection of tiny lesions. Fasting blood glucose level remained 80–90 mg/dl, which was slightly lower than that before the surgery. His serum sodium and potassium levels were within the normal ranges. On the 4th postoperative day, laboratory examination for irAE was performed, and prednisolone (100 mg/day) was empirically started for possible adrenal insufficiency. His body temperature and fasting blood glucose levels promptly returned to within a normal range, and general fatigue disappeared. Laboratory data on the 4th postoperative day revealed hypothyroidism (thyroid-stimulating hormone (TSH) 11.56 μIU/ml [reference value: 0.50–5.00 μIU/ml], Free-T3 0.94 pg/ml [reference value: 2.30–4.30 pg/ml], Free-T4 0.94 ng/dl [reference value: 0.90–1.70 ng/dl]) and hypopituitarism (adrenocorticotropic hormone (ACTH) < 1.5 pg/ml [reference value: 7.2–63.3 pg/ml]), Cortisol 2.75 μg/dl [reference value: 6.24–18.00 μg/dl]). Considering the transient hyperthyroidism during lenvatinib plus pembrolizumab administration (Fig. ), we diagnosed hypothyroidism and hypopituitarism caused by lenvatinib plus pembrolizumab.\nAfter the diagnosis of combined hypothyroidism and hypopituitarism, he received hydrocortisone (40 mg/day) first on the 9th postoperative day. Then, his fever, general fatigue, or any abnormality of fasting blood glucose level dissolved, and he was discharged on the 12th postoperative day. Following recovery of adrenal insufficiency, he received levothyroxine on the 14th postoperative day. He was alive without recurrence of gastric cancer and his laboratory data for thyroid and pituitary function remained stable while receiving hydrocortisone and levothyroxine for 2 years and 5 months. |
A 46-year-old nulliparous woman visited our hospital due to progressive lower abdominal pain. Sonography revealed uterine myomas of size 4.4 cm × 3.5 cm and 5.5 cm × 5.3 cm at the anterior and posterior walls, respectively. She underwent laparoscopic supracervical hysterectomy in May 2013 []. Laparoscopic observation revealed two globular protruding uterine myomas with prominent vessels on the serosa and a bilateral adnexa that appeared normal []. After cervical transaction, the uterine body together with myomas was removed using a power morcellator through the 10-mm port. The pathology report revealed leiomyoma with focal hyaline degeneration.\nApproximately 2 years later, in 2015, she again presented to the emergency department due to progressive lower abdominal pain. Computed tomography (CT) revealed multiple heterogeneous enhancing pelvic tumors ranging from 2 to 10 cm []. Diagnostic laparoscopy revealed four solid tumors on the dependent part of the abdomen [], which were attached to the sigmoid colon, right side of the bladder [, PM1 and PM2], peritoneum of the right common iliac artery [, PM3], and cul-de-sac. Sigmoid colon injury had occurred during parasitic myomectomy, which was repaired by a general surgeon. The four solid tumors were removed using manual morcellation with the specimen contained in a surgical tissue bag [LapSac, Cook Medical, USA; ]. The whole peritoneal cavity was carefully explored and washed with 3000 mL of normal saline; no residual specimens or morcellation remnant of PM was noted. The pathology report indicated the solid masses to be leiomyomas with variable cellularity and focal degenerative change. No increased mitosis was noted. The specimen from the sigmoid colon was adhered to the adventitial adipose tissue of the colon, and no evidence of mural wall invasion was observed.\nTen months later, in February 2016, the patient developed progressive lower abdominal pain again. Transvaginal ultrasonography revealed a pelvic mass of approximately 4 cm in diameter, which was suspected to be a recurrent PM []. The patient then received a third surgery of robotic single-site parasitic myomectomy. On exploration, three PMs were found on the peritoneum of the left adnexal anterior near the residual cervix, the right pelvis near the external iliac artery, and the right external iliac artery []. In the lithotomy position, all PM were excised and contained in the same surgical tissue bag before manual morcellation. The whole peritoneal cavity was carefully explored, and no complication and no residual tumor or remnant of PM was noted. Histopathology confirmed the masses as leiomyomas composed of fascicles of the spindle to epithelioid smooth muscles. After the third surgery, the patient was prescribed a selective progesterone receptor (PR) modulator – ulipristal acetate (UA), 5 mg daily, for preventing recurrence. She exhibited good health status and remained asymptomatic until 3½ months, after which she presented with lower abdominal pain again []. A solid pelvic mass was detected on transvaginal ultrasonography []. The fourth surgery was performed in June 2016, and a 7.5 cm × 5.0 cm recurrent PM with prominent vessels on the tumor surface firmly attached at the junction of the mesentery and sigmoid colon was identified []. Due to multiple peritoneal dense adhesions, laparoscopic surgery was converted to laparotomy, and tumor excision with bilateral oophorectomy was performed. Sigmoid colon serosal injury occurred during parasitic myomectomy. We carefully explored the whole abdominal cavity through palpation and found no residual tumor. Peritoneal cavity irrigation was performed with 3000 mL of normal saline. The pathology revealed leiomyoma with interlacing myometrial bundles composed of bland smooth muscle cells. The patient was followed up for approximately 3 years after the last surgery, and she is currently in a stable condition with no evidence of recurrence. |
A 57-years-old man with a history of smoking interrupted 6 months prior, and no other significant past medical history, was admitted in the emergency department for acute epigastralgia and vomiting. On arrival, he was alert and oriented with a blood pressure of 130/90 mmHg, a heart rate of 85/minute, and a respiratory rate of 19 breaths/minute. Cardiovascular examination was unremarkable. His blood glucose was 260 mg/dl without acetonuria. His electrocardiogram revealed normal sinus rhythm with ST segment depression in lateral, inferior, and posterior leads. His cardiac enzymes were elevated (First Troponin I at 755 ng/L, the second at 1600 ng/l), serum creatinine and electrolyte levels were normal. Echocardiography demonstrated an akinesia of the lateral wall with a left ventricular ejection fraction of 50%. The patient was admitted to the intensive care unit and prepared for cardiac catheterization. He received 300 mg of aspirin, 600 mg of clopidogrel and full dose 6000 UI of enoxaparine subcutaneously, with a slight regression of the pain. An electrocardiogram performed 6 h after his admission showed an ST elevation in the lateral and posterior leads (). Urgent coronary angiography revealed a concomitant occlusion of the mid LAD and the proximal Left circumflex artery with a collateral flow from the RCA (). We proceeded with primary per cutaneous intervention of the circumflex occlusion that was crossed by a floppy guidewire and predilated with a 2 × 15 mm balloon, then we deployed a 2,75 × 38 mm drug eluting stent (DES). Afterward, we proceeded to the PCI of the LAD using the same procedure to deploy a 2,75/28 mm DES. The angiographic result showed Thrombolysis In Myocardial Infarction III flow in both arteries (). Unfortunately, IVUS was not available. Aside from an inaugural diabetes mellitus (an incidental finding during this case presentation), there was no clinical or biological feature suggestive of an underlining condition predisposing to multiple thrombosis. The clinical course was uneventful and the patient was discharged well on day 4. |
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