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A 73-year-old male smoker presented with a mass in the right upper lobe associated with mediastinal lymph node enlargement. The radiological profile was consistent with primary lung cancer; however, during endoscopic aspiration biopsy of the subcarinal lymph node, uncertainty arose as to whether it was a granulatomous lesion. Therefore, we opted for performing a transthoracic biopsy, as well as a mediastinal lymph node biopsy by mediastinoscopy, and, depending on the results of the intraoperative analysis, we would also insert an indwelling central catheter for chemotherapy. The patient was brought to the HOR, and the procedures were performed sequentially. Elective endotracheal intubation, large-bore peripheral venous access, and an indwelling urinary catheter were used. The patient was initially placed in the right lateral decubitus position for performance of a percutaneous Tru-Cut needle biopsy, with the gantry (Artis Zeego; Siemens, Munich, Germany) set to tomographic mode, by the interventional radiology team. Pathological examination of the biopsy specimen revealed that it was a carcinoma. The patient was then placed in the supine position to undergo mediastinoscopy with biopsy of the lesion, with the gantry set to fluoroscopic mode. Examination of a frozen section biopsy specimen confirmed an epidermoid carcinoma in the infracarinal lymph node. In view of the diagnosis of (stage N2) lung cancer, we proceeded to insert a catheter for chemotherapy, again with the gantry set to fluoroscopic mode.\nThe use of an HOR in the case described here allowed all procedures to be performed sequentially, in the same setting, without need for transportation. In addition, we would have the flexibility of adding pulmonary lobectomy if the pulmonary mass was positive for cancer and did not extend into the mediastinum. We were planning to use the gantry set to tomographic mode during mediastinoscopy in order to assist the intraoperative localization of the mediastinal lesion. However, the vertical size of the video mediastinoscope system precluded free rotation of the gantry, which is required to generate the tomographic image, and it was possible to use only fluoroscopy, which dispenses with complete rotation of the gantry. |
A 72-year-old Korean woman presented to our emergency department with right upper limb hemiparesis without speech impairment. Her medical history was significant for hypertension, hyperlipidemia, and diabetes. She reported no previous history of cigarette smoking and alcohol drinking. On admission to the hospital, her right upper limb hemiparesis resolved rapidly and completely. The results of laboratory examinations, including complete blood count, serum electrolyte levels, and coagulation studies, were within normal limits, except for slight hypertriglyceridemia. An electrocardiogram showed a normal sinus rhythm, and a brain computed tomography scan appeared normal. Urgent magnetic resonance imaging (MRI) of the brain revealed no definite evidence of acute infarction. Neither significant steno-occlusive lesions nor cerebral aneurysms in the intra/extracranial vessels were demonstrated by magnetic resonance angiography (MRA). Transthoracic echocardiography (TTE) showed a well-defined echogenic mass in the LA with a broad-based attachment to the interatrial septum (). The LA was not enlarged, and left ventricular (LV) systolic function was normal, with an ejection fraction (EF) of 60%. Transesophageal echocardiography (TEE) confirmed the presence of a heterogeneous mobile mass (33×25 mm) with internal echo-free spaces, and color Doppler flow mapping showed flow signal within the mass suggesting hypervascularity (). The mass did not involve the heart valves directly, and Doppler echocardiography revealed no impairment of flow across the mitral valve. The diagnosis of an embolic transient ischemic attack (TIA) caused by a LA myxoma was made, and the ABCD2 score was 6. She was urgently referred for surgical intervention to prevent further embolic strokes.\nA median sternotomy was performed and right atriotomy was created with a trans-septal approach. The LA mass was then excised en bloc for complete resection and the iatrogenic atrial septal defect was directly closed without using a patch. Histopathological examination confirmed a cardiac myxoma with a clear resection margin, and without an overlying thrombus. The postoperative course was uneventful and free of major complications. On postoperative day 4, TTE demonstrated no evidence of any remnant mass or thrombus in the LA (). The patient was discharged without antiplatelet therapy or anticoagulants.\nOne month after surgery, the patient was readmitted to our hospital because of a transient left-sided weakness in her arm and leg without speech impairment. Her neurological examination was normal, and brain MRI performed using the stroke protocol demonstrated no acute lesion with diffusion restriction. MRA of the cranial vessels showed neither steno-occlusive lesions nor aneurysmal dilatations, which was the same as before. TTE and TEE were performed to identify the source of the TIA. Scanning of the LA revealed a polypoid mass (13×7 mm) attached to the mid portion of the interatrial septum, with an irregular lobulated surface (). Lipomatous hypertrophy of the interatrial septum was also noted, and no thrombus was seen in the left atrial appendage. Although LV systolic function was slightly decreased (EF 47%) without regional wall motion abnormalities, the echocardiographic findings of the mass in question were more suggestive of a remnant myxoma. However, given the short period of time after the surgery, thrombus formation at the site of direct closure of the interatrial septum was a reasonable possibility. Therefore, after discussion, intravenous heparin administration was started as a therapeutic trial and it was continued to help differentiate a remnant mass from a thrombus.\nOne week after the initiation of anticoagulation, a follow-up TEE examination revealed a relevant reduction in the burden of the mass in question from 13×7 mm to 8×3 mm, suggesting that the mass was most likely to be a thrombus (). Therefore, long-term anticoagulation with warfarin was continued to maintain an international normalized ratio of 2-3. At the 4-month follow-up after warfarinization, the mass attached to the interatrial septum had resolved completely on TEE examination, confirming the diagnosis of a thrombus (). The patient is now being maintained on warfarin and kept under regular follow-up. |
A 76-year-old Caucasian female who was a non-smoker, with a history of heart ischemic disease, hypertension, anemia and diabetes, underwent a laparoscopic left radical nephrectomy for a renal neoplasm of 10×7 cm in the superior left renal pole in 2011. The postoperative course was uneventful and the patient was discharged after 6 days. The histological examination revealed the presence of a renal cell carcinoma, Fuhrman grade 2, with extensive necrosis and phlogosis areas (TNM 2009 RCC pT2a). Informed consent was obtained from the patient.\nAfter discharge, the patient was readmitted for a sciatica episode and underwent two abdomen ultrasonography examinations, with no notable pathological findings.\nAfter a few days, the patient noted an indolent swelling in the proximal third of the right thigh. The patient subsequently underwent ultrasonographic evaluation, revealing the presence of a solid, vascularized mass that was ∼40×22 mm in size.\nThe subsequent CT documented the presence of a pathological, solid, dishomogeneous bulk that was ∼4.5 cm diameter, in the front of the right iliac vessels, immediately cranial to the inguinal region. Another smaller (1.5 cm) lesion was observed in the ipsilateral inguinale region, while an additional solid expansive lesion was noted in the rectus femoris muscle ().\nThe fine needle biopsy of the muscle mass documented a metastasis of renal cell carcinoma (). The histochemical analysis revealed positive staining for vimentin and CAM, and negative staining for cytokeratin 7.\nMoreover, we performed a cerebral CT and a total body bone scan, in order to achieve a complete stadiation of the patient. Neither of the examinations revealed further meta-static localization.\nA joint evaluation of the patient was performed with the oncologist, the general surgeon and the radiotherapist. With regard to the pathological stage of the disease and the comorbidity of the patient, it was decided to refer the patient for targeted therapy with sunitinib.\nTherefore, the patient underwent 2 cycles of sunitinib therapy. Subsequently, due to the onset of edema in the right lower limb, the patient underwent an abdominal CT, revealing a nodal progression of the disease along the right femoral and iliac vessels. The patient underwent 6 further cycles of salvage therapy with sorafenib, which were well tolerated. The subsequent CT scan revealed a lymphonodal progression of disease. |
A 40-year-old man, with a past medical history of temporal lobe epilepsy, presented to the emergency department with altered mental status for three days. As per his records, he was taking topiramate 100 mg twice daily and lamotrigine 200 mg twice daily, but compliance had been an issue.\nOn examination, he was uncooperative. He was constantly making religious remarks, saying “God is with me and I do not need doctors or medications.” He would interpret every question asked to him as questioning his faith and at times attempted to convert doctors and staff to Islam. He believed everyone around him was preventing him from obtaining salvation. The remainder of the neurological exam, including cranial nerves, motor examination, sensations, reflexes, coordination, and gait, was grossly normal. Initially, he was thought to have an acute psychotic episode. Psychiatry, however, was concerned that his religious comments and paranoia regarding his treatment in the hospital may be a part of his epilepsy.\nHis family was eventually contacted after multiple attempts and they reported that he had a generalized tonic-clonic seizure two days prior to being hospitalized and after the generalized seizure he started having increasing religious thoughts. The wife called an ambulance because she was frightened by his excessive religious speech. She was not sure when he stopped taking his medications. She reported that he had a history of epilepsy of unknown etiology since the age of 20. The most common seizure semiology was generalized tonic-clonic seizures but occasionally, he would have episodes of hyperreligiosity with secondarily generalized events. There was no mention of aura preceding any of the generalized seizures. These similar episodes of hyperreligiosity in the past occurred when he did not take his antiepileptic medications (AEDs). He did not have a history of psychiatric disease. An EEG showed right-sided frontotemporal sharp waves. His MRI was unremarkable which included thin cuts over temporal lobes and hippocampus. His hyperreligiosity was deemed to be due to his epileptic activity. He refused intravenous AEDs, as he believed it was antireligious poison but was convinced to take his home medications. After three days of treatment, he became more alert. His wife noted that he returned to baseline and was discharged home with follow-up with his neurologist. |
In August 2010, a 57-year-old Chinese male presented with epistaxis and decreased hearing for 1 month. No additional symptoms, such as a neck mass, nasal obstruction, headache, diplopia or other cranial nerve palsies, were noted. The patient had no history of previous or synchronous tumours or any family history of cancer. Nasopharyngoscopy revealed a large exophytic tumour that was covered by smooth mucosa, which grew from the right posterolateral nasopharyngeal wall in the right posterior naris. Magnetic resonance imaging (MRI) scans of the nasopharynx and neck using gadolinium enhancement demonstrated a 2.0 × 1.5 × 2.0 cm well enhanced mass over the right posterior nasopharynx with right retropharyngeal node enlargement. The tumour extended across the right parapharyngeal space and infiltrated into the medial pterygoid muscle. In addition, skull base erosion was detected with right alar lamina involvement (Fig. ). Cervical lymph node metastasis was not observed. Hematologic, hepatic and renal function tests revealed no abnormalities. The patients underwent chest and abdomen computed tomography (CT) as well as a bone scintigram, and no distant metastasis was found. A biopsy of the nasopharynx was performed.\nIn the biopsy specimen, normal salivary tissue was not present. The tumours were ill demarcated without encapsulation. Tumour cells were arranged in nests and nodules. Two morphologic patterns of the tumour cells were observed. Some small round cells exhibited dark nuclei and scant cytoplasm. Other large cells contained round to oval pale nuclei and eosinophilic to amphophilic cytoplasm. In the central region of the tumour cell nests, large cells displayed a solid growth pattern. Small dark cells were clustered at the periphery of the tumour cell nests and appeared palisaded. Prominent nucleoli and mitosis can be observed, and an average of three mitotic figures were observed per 10 high-power fields (original magnification × 400).\nIn the immunohistochemical analysis, the tumour cells were immunoreactive with P63, vimentin, and cytokeratin (CK7 and CK14) antibodies and focally immunoreactive with a calponin antibody. This case of BCAC was not positive for smooth muscle actin or CD117. The proliferative index as demonstrated by Ki-67 was approximately 10%. Based on the immunohistochemistry results and the pathological findings, which included tumour islands with solid proliferation, basaloid-like cells containing large pale and small dark cells, an infiltrative margin, cellular and nuclear pleomorphism, and prominent mitosis, the patient was diagnosed with a solid-type minor salivary gland BCAC (Fig. ).\nBased on the 2002 American Joint Committee on Cancer (AJCC) Tumor, Node, Metastasis (TNM) staging system [], the tumour was classified as stage III (T3N0M0).\nIn our case, the patient received intensity-modulated radiation therapy (IMRT) with 6 MV X-rays. The delineation of the gross tumour volume (GTV) was based on the primary tumour volume determined from the physical and imaging examinations. The clinical target volume (CTV) was defined as the whole nasopharyngeal cavity, the clivus, the skull base, the pterygoid plates, the parapharyngeal space, the sphenoid sinus, the posterior one-third of the nasal cavity, the maxillary sinus, and the drainage of the upper neck (levels II, III, and Va. A total dose of 70.4 Gy/32 F/6.2 W was administered based on the planning target volume (PTVg) (GTV with 0.5 cm margin). The PTV60 was defined as 60 Gy/30 F (CTV with 0.5 cm margin) (Fig. ). After radiotherapy, MRI and nasopharyngoscopy revealed complete disappearance of the tumour (Fig. ). The patient was followed up every 3 months for the first 2 years, every 6 months for another 3 years, and then every 12 months. A follow-up at 72 months did not detect any evidence of disease recurrence. The patient developed moderate mucositis as an acute adverse event. However, he did not exhibit any grade 3/4 late adverse events, such as xerostomia, dysgeusia, or hearing impairment. |
A 56-year-old male presented to the emergency department (ED) with significant substernal chest pain starting 30 minutes prior to arrival. The patient was immediately brought back to an exam room after an electrocardiogram (ECG) was performed and was seen by a provider within 10 minutes of registration (Figure ).\nThe initial ECG revealed atrial fibrillation with a rapid ventricular response, rate of 147, with minimal ST depression within the lateral leads but was without apparent ST-segment elevation. On initial assessment, the patient had point-of-care labs immediately drawn (a basic metabolic panel and troponin), and a chest X-ray performed to evaluate for a possible aortic dissection which revealed no gross abnormalities (Figure ).\nWith a non-diagnostic chest X-ray alternative diagnoses were pursued. The ultrasound fellow in the department was consulted for an immediate cardiac ultrasound for evaluation of right heart strain secondary to pulmonary embolism. During the bedside cardiac ultrasound, the patient experienced ventricular fibrillation (Video ), and CPR was immediately started.\nThe ACLS algorithm was followed for pulseless ventricular fibrillation, and the patient received multiple rounds of epinephrine, 450 mg of amiodarone (300 mg and then 150 mg), and three conventional defibrillations with increasing joules at 150 J, 200 J, and 200 J (the departmental defibrillators are biphasic and have a maximum output of 200 J). The patient continued with ventricular fibrillation throughout the ACLS algorithm, and the decision was made to attempt DSD. The patient had a second set of pads applied in the anterior-posterior orientation in addition to the conventional right upper chest and left lateral chest with successful conversion of the ventricular fibrillation. The patient was additionally given Lidocaine, 100 mg, due to a wide-complex tachycardia and apparent non-responsiveness to the previously given amiodarone (Figure ). An improvement was noted after the use of Lidocaine, and a Lidocaine drip was started.\nOnce the patient was stabilized, he was taken for computed tomography (CT) imaging to further evaluate for the possibility of a pulmonary embolism. In the CT room, he developed bradycardia and subsequently lost his pulse. CPR was again started, the patient was given atropine, and return of spontaneous circulation (ROSC) was achieved shortly after. The CT scan did not reveal any evidence of aortic dissection or pulmonary embolism and a repeat ECG was performed which showed a persistent wide complex tachycardia with no obvious ST-segment changes. Due to the morphology of the QRS complexes and length of resuscitation time from initial arrest (nearing 90 minutes), tissue plasminogen activator (tPA) was used as a thrombolytic for what was presumed to be a large vessel occlusion myocardial infarction. Hundred milligram of tPA was administered (50 mg as a bolus and 50 mg as a drip given over 60 minutes) with an apparent reperfusion rhythm followed by a "normal" appearing sinus tachycardia. Another ECG was repeated which revealed sinus tachycardia, at a rate of 114, now with ST-segment elevations present in aVR, V1, V2, V3, and V4 with depressions in leads II, III, and aVF (Figure ).\nGiven the patient's persistent elevations despite thrombolytic therapy, interventional cardiology was consulted, and the patient was transferred to a tertiary care facility for cardiac catheterization revealing a thrombotic occlusion in the proximal left anterior descending coronary artery.\nAfter transfer to the tertiary care facility for cardiac catheterization, the patient developed cardiogenic shock. The patient was started on ionotropic medications with no improvement, and he was placed on venous-arterial extracorporeal membrane oxygenation (ECMO) therapy (~20 hours after his cardiac arrest). Before initiation of ECMO, the patient was awake, alert, and following simple commands (although still intubated). Unfortunately, despite ECMO support, his cardiac function did not improve, and the patient was not a candidate for cardiac transplantation. Seventeen days after the patient's initial presentation to the ED, the family decided to withdraw care. The patient was extubated, had ECMO discontinued, and time of death was documented shortly after. |
An 82-year-old male was admitted to the emergency department for worsening shortness of breath and hypoxia. He was admitted a week after he was diagnosed with a left ninth rib fracture secondary to a fall. He had long-standing history of chronic obstructive pulmonary disease, coronary artery disease, and peripheral vascular disease. Chest radiographs revealed a left pleural effusion and possible infiltrate. The patient was initially treated with a nebulizer, prednisone, and empiric antibiotic coverage with ceftriaxone and azithromycin. The patient failed to improve with the medical interventions and a therapeutic thoracentesis was performed. The thoracentesis was completed with ultrasound guidance, with the puncture made above the 11th rib at mid chest on the left. The pleural effusion was found to be frank blood. No immediate complications were noted, and the patient was taken to recovery. The next day the patient was found to be in respiratory distress. A chest x-ray revealed an opaque left hemithorax that was likely rapid accumulation of pleural fluid (Fig. ). A follow-up contrast-enhanced computed tomography (CT) of the chest performed during the arterial phase revealed a left intercostal pseudoaneurysm with hemothorax and adjacent compressive atelectasis (Fig. ). Ultrasound of the left chest wall was performed (Fig. ) directly over the thoracentesis site and doppler flow revealed bidirectional fluid flow, indicating the presence of a large pseudoaneurysm (Fig. ).\nFollowing identification of the left intercostal pseudoaneurysm, the patient underwent a thoracic aortogram and multiple-level left intercostal angiogram (Fig. ) under IV conscious sedation. Selective catheterization of the T5, T6, and T7 intercostal arteries was unsuccessful in identifying the pseudoaneurysm. Selective catheterization of T10 and T11 intercostal arteries was performed with a C2 Cobra catheter, following multiple catheter exchanges due to the patient’s atherosclerotic vessels. The pseudoaneurysm was ultimately found to have a left T10 origin and the C2 Cobra catheter was exchanged for a microcatheter. Once access was gained, coil embolization of the pseudoaneurysm was performed with a series of 15 Axium micro coils. Significant room was left on both sides of the pseudoaneurysm and a follow-up angiogram was performed via the microcatheter, then a 5-French Cobra catheter. The follow-up angiogram demonstrated no further filling of the pseudoaneurysm (Fig. ). The catheter was removed and a Perclose device was placed in the left groin for hemostasis. Following completion of the procedure, the patient was taken to recovery. The patient proceeded to return to his baseline following medical management during the remainder of his hospital stay and was discharged home after 5 days. |
A 55-year-old non-diabetic, non-hypertensive male with a history of recurrent colicky left lumbar pain presented with acute urinary retention. Catheterization was attempted; however, it was unsuccessful. Ultrasound revealed an overdistended urinary bladder with a normal-sized prostate and scarring and focal caliectasis in the left kidney (). The right kidney was normal and no calculi were seen on either side on ultrasound. Serum electrolyte, renal and liver functions were normal. The haemogram revealed neutrophilia. The prostate-specific antigen was within normal limits. A rigid urethroscopy was performed owing to suspicion of a left urethral calculus and a 11-mm size calculus was removed from the posterior urethra. The patient was discharged and had no difficulty with micturition for 2 weeks thereafter. The patient subsequently developed burning micturition with hesitancy and induration in the perineal region. The urine was turbid and microscopy revealed the presence of Escherichia coli. A perineal ultrasound revealed an abscess in the perineum, which extended to the proximal parts of the corpus spongiosum (). This abscess was drained under saddle block. The patient subsequently developed a discharging sinus at the operative site () leaking purulent fluid. A retrograde urography revealed periurethral extravasation of the injected contrast material with a lytic lesion in the left pubic ramus (). A repeat perineal ultrasound revealed a linear hypoechoic tract leading from the skin surface to the corpus spongiosum (). A retrograde CT urography was performed to look for the extent and ramifications of the abscess. On the non-contrast CT scan, a lytic lesion was seen involving the left inferior pubic ramus (). The pubic symphysis and bodies of both pubic bones were normal. A proximal femoral nail was noted in situ on the left side, which was inserted 10 years before for fracture of the proximal shaft of the femur owing to accidental trauma. On injecting iodinated contrast into the urethra, there was extravasation of the contrast in the periurethral region in the soft tissues surrounding the bulbar and the posterior penile urethra. An extension of the contrast through the external anal sphincter into the intersphincteric plane () with inflammatory stranding in the ischioanal fossae was seen. The contrast also extended into the lytic lesion present in the left inferior pubic ramus (). The contrast also extravasated through the cutaneous opening in the perineum and the natal cleft (). E. coli were isolated on pus culture. The patient was treated with intravenous antibiotics and suprapubic cystostomy was performed. The patient is presently being considered for elective urethroplasty. |
An eight-year old boy was referred to the pediatric department of Shiraz University of Medical Sciences (Shiraz, Iran) with the extraction order of his permanent maxillary central incisors. He had experienced a car accident 3 months before his referral, however nothing was done for him in private setting except for glass ionomer dressing of the exposed pulp horns, systemic antibiotic treatment, radiographic and photographic documentation at the time of injury. 3 months after the trauma, he was visited at that dental office with the chief complaint of ugly smile. New radiographs were taken. After radiographic examination, the dentist had interpreted that both permanent maxillary central incisors were no longer preservable. The patient was then referred to the pediatric department to have his teeth extracted.\nAlthough a remarkably long time had elapsed since his accident, the traumatic dental injury guidelines were followed. After assessing the medical history, the patient was examined for extra-oral signs. He had no facial swelling, changes in skin color, facial and mandibular asymmetry, limited mouth opening, areas of ecchymosis, crepitus or pain on palpation and nostril perforation.\nIntra-oral examination revealed dental traumas including complicated crown fracture with severe intrusive luxation of both permanent maxillary central incisors. The patient's history of pre-existing anterior open bite was reported by his mother. The patient had poor oral hygiene. The pulp vitality tests including electric pulp test (EPT), heated gutta-percha test and ice test were performed for control and traumatized teeth. Both maxillary central incisors showed no signs of vitality. Reaction to percussion was tested by tapping non-traumatized and traumatized teeth lightly with the handle of a mouth mirror in vertical and horizontal direction. The intruded incisors revealed normal sound upon percussion. The patient was asked to bring the photographs and radiographs he had taken at the time of injury [].\nPeriapical radiographs evaluating permanent maxillary central incisors showed areas of internal and external root resorption, wide apical foramens, with concomitant complicated crown fracture of both teeth. The neighboring teeth had normal socket.\nThe parents and patient were informed about treatment options and the expected prognosis of each procedure. Instead of getting the teeth extracted, they decided to give the teeth a second chance by following a distinct treatment and follow up regimen. Because of severe multiple intrusions (about 6 mm), without spontaneous re-eruption, we decided to choose surgical extraction as repositioning technique. After the administration of local anesthesia, the intruded teeth were initially luxated and grasped mesio-distally with a forceps. The teeth were then brought down into their normal position by applying careful and very gentle force incisally. Finally we splinted the teeth with round wire (0.028 inch) and light cure resin composite (Kerr, Romulus, MI, USA; microhybrid). Another radiograph was taken to confirm the correct repositioning. Systemic amoxicillin 500 mg three times per day, 0.2% chlorhexidine mouth rinse twice daily were prescribed for 7 days. Eating habits and vigorous oral hygiene maintenance were instructed to the parents and patient.\nOne week after the surgery, the endodontic intervention for both permanent maxillary incisors were initiated since both teeth had exhibited external and internal root resorption. The access to the root canals were gained and the necrotic pulp tissue was removed with step-back technique and copious irrigation with 0.5% sodium hypochlorite while care was taken not to weaken the fragile dentinal walls in the cervical and apical area which may impair healing. The canals were then filled with Vitapex® (Diadent® Group International Inc., Burnaby, B.C., Canada). 6 weeks after the surgery, the splint was removed and a follow-up radiograph was taken. The root canal dressing was checked every 3 months. After 6 months, the root resorption was controlled based on radiographic evaluation and calcium hydroxide was removed gently. The canals were flushed and dried while being careful not to stimulate bleeding. The mixed MTA (Dentsply, Tulsa Dental Products, Tulsa, OK, USA) with wet sand consistency (based on manufacturer's instruction) was positioned to the desired depth with plugger. After placing a 4-mm MTA plug, a moist cotton pellet was kept coronal to MTA and the access opening was then filled with glass ionomer restoration (GC America, Alsip, IL, USA). At the next day, both teeth were then filled with gutta-percha points with warm vertical compaction technique and restored with a light-cured resin composite []. Clinical and radiographic controls of the treatment condition were planned at 3, 6, 12 and 24 months.\nIn a 2-year follow-up, the teeth had remained functional and esthetically acceptable. The percussion sound and mobility were normal. In radiographic examinations, external and internal root resorption were stopped in both teeth, no signs of replacement resorption was detected and the adjacent anterior teeth were asymptomatic []. Despite our, the patient had not followed our oral hygiene instruction since his last visit [].\nThe parents and the patient were happy with the functional and esthetic results and were reluctant of orthodontic final corrections. Although acceptable results were achieved, further yearly observations are planned at least for 5 years. |
A 35-year-old female presented to the emergency surgery unit for diffuse abdominal pain and distension for two months and increased intensity for five days. She also complained of bleeding per rectum for two months and suprapubic discomfort while urinating for two weeks.\nShe was diagnosed with carcinoma rectum and bilateral adnexal mass one year back. However, she did not follow up for further planning and managing the disease due to her financial conditions and non-severity symptoms. One year later, she presented to the emergency surgery department with features suggestive of intestinal obstruction.\nPhysical examination revealed a large, firm, tender mass occupying the entire abdomen. Abdomen radiography revealed multiple air-fluid levels. Computed tomography (CT) abdomen and pelvis showed Irregular circumferential wall thickening involving the mid and lower rectum for a length of 8 cm with a maximum wall thickness of 22 mm. The distal margin of the lesion is 3 cm from the anal verge. Anteriorly lesion showed loss of fat planes with the cervix. Proximal to the lesion, large bowel loops appeared dilated with air with a maximum caliber of 6.7 cm. A large abdominopelvic multiloculated cystic lesion with multiple thin septations and few solid enhancing areas occupying the abdominopelvic region was noted, measuring 19x16x24 cm in its axial and longitudinal planes. Bilateral ovaries were not separately visualized; hence lesion origin was possibly from the ovaries. The lesion had indistinct fat planes with the anterior abdominal wall and was displacing the small bowel loops postero-superiorly and abutting them.\nLaboratory results revealed elevated serum carbohydrate antigen 125 (CA125) levels (276 U/mL). Based on clinical, laboratory, and image analysis, she was diagnosed with an acute intestinal obstruction and was taken for emergency laparotomy. Intraoperatively we found a gross dilatation of the large bowel up to the proximal part of the descending colon and collapsed distal part of the descending colon (Figure ).\nThere were two large ovarian cysts of size nearly 20x15 cm, each involving fallopian tubes compressing the descending colon's proximal part, with multiple serosal, peritoneal, omental, liver deposits. Hence palliative bilateral salpingo-oophorectomy was done with diversion loop sigmoid colostomy (Figure ).\nPathological gross examination showed bilateral enlarged ovaries with a smooth external surface. On microscopy, both ovaries showed multiple cystic spaces lined by columnar to cuboidal tumor cells along with signet ring cells in mucin-filled pools. Immunostains of cytokeratin 7 (CK 7) and cytokeratin 20 (CK 20) were performed, and tumor cells were positive for CK 20 and negative for CK 7. The histopathologic findings were of the Krukenberg tumor of the ovary, and the immune profile favored a metastatic rectal carcinoma (Figure ).\nShe was started on orals after three days following surgery which she tolerated well, and the stoma started moving. She was discussed in the tumor clinic board for palliative chemotherapy and was planned to start the same. |
A 32-year-old Caucasian pregnant woman (G2, P1) presented at the antenatal department due to reduced fetal movements the day before. She was 31 weeks and four days pregnant after spontaneous conception. This was her second pregnancy. During this pregnancy, she had a 12 weeks screening and a 20 weeks scan. Both of these screenings were reassuring. Her first pregnancy was three years ago with normal delivery. The first baby was a girl who weighed 3210 grams.\nInitially, she had a cardiotocography (CTG), which was reassuring. The baseline was around 130 bpm with accelerations while no contractions were recorded. Afterwards, she had a scan, which was done by a consultant specialised in fetal medicine. During this scan, significant small bowel dilation was observed and the diagnosis of interstitial obstruction was made (Figure ). The amniotic fluid index was normal (22 cm) and fetoplacental dopplers were reassuring. During the scan, the peristalsis of the fetal bowel was observed.\nThe patient was admitted to the antenatal unit for daily observation and assessment. On Day 2 of hospitalisation, the patient had an amniocentesis to detect any chromosome abnormalities, genetic disorders etc. The results of the amniocentesis came back as reassuring. During this time and after liaising with the neonatal unit, an obstetric scan was repeated twice weekly to monitor any bowel changes. During these scans, the bowel enlargement was stable, the bowel was not hyperechoic and peristalsis was present. Therefore, it was decided to wait until 37 weeks for labour induction unless there was a significant deterioration in either the scan findings or the CTG.\nThe patient delivered at 36 weeks and two days after spontaneous rupture of membranes. A male infant who weighed 2620 grams was delivered. The newborn was transferred immediately to the neonatal intensive care unit where a group of specialised doctors assessed him. After several examinations, such as X-rays and MRI scans, the diagnosis of interstitial obstruction was established and the newborn infant was transferred to another unit with specialised surgeons after having a nasogastric tube and being stable. On Day 2 post-delivery, the newborn underwent a laparotomy. The findings of the laparotomy were as follows: a large tumour that blocked the jejunum was identified (Figure ). A part of the bowel was necrotic. The tumour was excised and a part of the jejunum was removed. The jejunum was connected at the same surgery. The tissues were sent for histology.\nThe newborn boy was hospitalised for a total of 28 days. The results of the histology showed lymphoid hyperplasia, which was benign, and its possible origin was regional lymph nodes. After a multidisciplinary meeting, it was decided that no further treatment was necessary, and the male infant was to attend a follow-up appointment in three months. |
A 67-year-old male patient with severe lumbar back pain and weight loss was primarily treated in a community hospital. The preoperative CT scan showed a suspicious mass in the pancreatic head, without any evidence for extrapancreatic tumor manifestation (Fig. A). An explorative laparotomy was performed by the local surgeon and the tumor was classified as locally not resectable. A tumor biopsy was taken and a palliative gastroenterostomy was performed. The histopathological analysis revealed a poorly differentiated ductal adenocarcinoma of the pancreatic head. After the primary operation, the patient was referred to our center. As the tumor was judged borderline resectable, our interdisciplinary tumor board recommended ‘neoadjuvant’ chemotherapy with Gemcitabine/Oxaliplatin for 10 cycles. Restaging after the complete neoadjuvant therapy showed local tumor regression without evidence of distant metastasis. Therefore, a pancreatoduodenectomy (Whipple's operation) was subsequently performed. The histopathological examination showed an ypT3, N0 stage, Grade 3 tumor. The dorsal resection margin revealed an R1 resection, and the resected specimen revealed severe intrapancreatic NI by cancer cells (Fig. B). In the postoperative period, the strong pain sensations disappeared completely 2 weeks after tumor resection. With postoperative pancreatic enzyme supplementation, the patient experienced slight weight gain. At this time, an additive chemotherapy with Gemcitabine was initiated. Five months postoperatively, the patient had no pain, but suffered from mild polyneuropathy of hands and feet due to the side effects of the neoadjuvant treatment. Seven months after resection, the patient developed severe lumbar pain which again required the hospitalization of the patient. The performed CT scan showed local tumor recurrence around the inferior vena cava and liver metastasis (Fig. C and D). The chemotherapy regimen was changed from Gemcitabine to 5-Fluoruracil and a symptomatic adjunct analgesic therapy with Pregabalin was initiated in addition to Metamizol, which led to a short-term pain relief. However, 4 weeks later, the pain syndrome of the patient exacerbated again and was not responsive to current pain medication. Only a CT-guided blockade of the celiac plexus enabled considerable, but only temporary pain relief. Four weeks later, the patient's condition deteriorated again, with severe recurrence of uncontrollable lumbar back pain. Only a continuous morphine infusion provided a palliative pain control until patient's death.\nPrior to the Whipple's operation, the patient gave written informed consent for an extensive postmortem histology analysis. The respected ethical approval was obtained from the ethical committee of the Klinikum rechts der Isar, Technische Universität München. This enabled us in this unique case, to correlate the symptoms of the patient with the postmortem histological samples from the peripancreatic local recurrence area. All samples from the retropancreatic region demonstrated large extrapancreatic nerves embedded in dense fibrotic tissue areas. Remarkably, nearly all visible nerves were infiltrated by very large clusters of cancer cells, and the neural structures were entirely destroyed (Fig. E and F). Hence, these fibrotic areas with severe NI represent the direct histological correlate of the increased retropancreatic signal density (hyperdensity) seen on the re-staging-CT scan. |
The patient was a 30-year-old Japanese woman with a history of FA. The initial diagnosis of FA was made at 4 years old because of presence of anemia and malformation. She received HSCT from a full-matched unrelated donor at the age of 13 years. The conditioning regimen included cyclophosphamide (10 mg/kg × 4 days), anti-lymphocyte globulin (25 mg × 2 days, 50 mg × 2 days), fludarabine phosphate (25 mg/m2 × 6 days), and thoracoabdominal irradiation (3 Gy), and steroid therapy was continued about for 4 years after HSCT. Then, she and her family moved to our prefecture and she had been regularly followed up at our hospital. She developed type II diabetes requiring insulin therapy in 2007 (at the age of 18 years). In 2014, she noticed a tongue protrusion and was diagnosed with tongue cancer (left tongue margin, T2N0Mx, SCC, negative for human papillomavirus). Complete partial tongue resection without reconstruction was performed. Subsequently, she had been regularly followed up by magnetic resonance imaging (MRI) examination every 6 months. However, in 2017, swelling and mass lesion on the cervical esophagus were detected by magnetic resonance imaging and endoscopic examination revealed esophageal SCC (Fig. ). The stage was T3N1M0 and she refused surgery because of vocal code removal at the surgery. On physical examination, dwarfism with central obesity-like body shape was observed (height 132 cm, weight 34.5 kg). Radiation therapy (1.8 Gy × 23 fractions, total 41.4 Gy) was performed without any specific toxicities and complete response was achieved (Fig. ). In 2019 (at the age of 30 years), she presented with a cough that gradually worsened. She sometimes complained of stridor and was referred to our respiratory department. She had no history of smoking or drinking alcohol. Her parents had no hematological abnormalities and there were no specific relevant findings in her family history, including cancer. Body weight (36 kg) was also similar after the radiotherapy for esophageal cancer. Wheezing was detected on lung auscultation. Fasting blood sugar and HbA1c were 166 mg/dL and 9.3%, respectively, and other blood chemistry findings were almost within normal limits. Tumor markers, including carcinoembryonic antigen (14.5 ng/mL), SCC antigen (2.4 ng/mL), and cytokeratin 19 fragments (CYFRA 21-1; 7.4 ng/mL) were elevated. Chest computed tomography showed nodular shadows in the upper lobe of the right lung, and atelectasis and a mass shadow in the lower lobe of the left lung (Fig. ) suggesting lung cancer. Bronchoscopic findings revealed reddish and swollen mucosa causing stenosis of left upper and lower bronchi and a polypoid white mass in the left upper lobe (Fig. ). Histological examination indicated SCC (Fig. ). Immunohistochemical examination of the biopsy specimen indicated that the lesion was positive for p40 and p63 and negative for thyroid transcription factor-1. The tumor cells were negative on immunohistochemical staining with anti-PD-L1 antibody. Genetic analysis revealed that EGFR-sensitive mutations (exons 18, 19, and 21), BRAF, ALK, and ROS-1 fusions were negative. In the present case, the left lung cancer lesions were located very close to the field of previous irradiation for esophageal cancer (Fig. ). Chemotherapy using cytotoxic agents was not recommended based on her performance status of 2, respiratory condition, and background diseases, including diabetes and FA. Her respiratory condition deteriorated gradually and she died 3 months after the diagnosis of lung cancer. |
A 48-year-old female with a known history of hypertension, diabetes mellitus, and hypothyroidism was admitted to the emergency department after she experienced two syncope episodes that were accompanied with involuntary jerking movements. It was learnt that she had no prodromal manifestations of dizziness, palpitations, or diaphoresis. While awaiting discharge she had a third witnessed collapse followed by a cardiac arrest in the setting of a shockable rhythm. Her home medications included aspirin and levothyroxine. It was also revealed that these two episodes resolved spontaneously and she had no confusion afterward. Upon arrival to the emergency department she was hemodynamically stable. Of note patient had a son who died at age of 19 years. The initial electrocardiogram (ECG) obtained in the emergency department is shown ().\nQuestion: Does the Patient Have an Underlying Primary Electrical Disturbance? This ECG shows normal sinus rhythm with a widened QRS complex (140 ms) at 66 beats per minute with a left axis deviation. On careful inspection, ST-segments in V1 and V2 show a <1 mm elevation and flattening of T waves in V1, in this otherwise normal ECG.\nWhen the cardiac arrest occurred, the initial rhythm was found to be ventricular fibrillation. The patient received resuscitation per ACLS protocol, including being defibrillated five times in the emergency room with successful return of spontaneous circulation. The angiogram did not demonstrate any obstructive coronary disease. The ECG, obtained two days after these events, is shown (). A right bundle branch block (RBBB) with an ST-segment elevation of ≥2 mm followed by a negative T wave with no isoelectric separation and a QTc 440 ms are seen as well as nonspecific T wave changes. These findings suggested a spontaneous intermittent Brugada type 1 pattern. In the majority of the reported cases, there is a coved-type ECG in the right precordial leads before the development of ventricular arrhythmias (2). The patient was found to be febrile at this time. Cardiac MRI was performed and demonstrated neither structural heart disease nor abnormal myocardium. The patient was discharged home after a successful single-lead implantable cardioverter defibrillator (ICD) placement. She remained asymptomatic. |
A 26-year-old male prisoner injured himself by punching a wall with his dominant right hand at his prison facility. He experienced immediate pain in the dorsal-ulnar wrist region and presented to the emergency department the next day. The patient denied previous injuries, numbness, or tingling of fingers. Clinical examination revealed tenderness of the dorsal-ulnar aspect of the right wrist with minimal swelling. No distal neurovascular deficits were found.\nThe posteroanterior, oblique, and lateral radiographs demonstrated dorsal displacement of the base of the fourth and fifth metacarpals at the corresponding CMC joints consistent with dislocation without fracture (Figure ).\nA CT scan was performed that confirmed the above findings with no additional lesions (Figure ). Closed manipulation attempted in the emergency department under light sedation anesthesia failed to achieve successful reduction. He was offered operative treatment, explaining the associated risks as well as benefits, and informed consent was obtained.\nThe patient was brought to the operating room four days post-injury. He was positioned supine and the right hand was placed on a hand table. Under general anaesthesia, a closed reduction was attempted using longitudinal traction with direct thumb pressure, and the displaced bones were reduced. The fluoroscopic views confirmed the relocation of the fourth and fifth CMC joints but were found to translate dorsally with wrist flexion. In light of instability, a decision was made to perform pin fixation. Under fluoroscopic guidance, a 0.062-inch Kirschner wire (K-wire) was inserted through a stab incision on the dorsal aspect of the base of the fourth metacarpal and was driven across the reduced CMC joint into the body of the hamate.\nA second K-wire was inserted, in a similar fashion, stabilizing the fifth CMC joint to the body of the hamate (Figure ). The stability of reduction was confirmed in dynamic views and the trailing ends of the K-wires were protected with Jürgen pinballs. A well-padded short arm plaster cast was applied to keep the hand in the intrinsic-plus position and the patient was discharged back to the prison facility on the same day.\nThe plaster and K-wires were removed in the office at 3.5 weeks postop after repeating the radiographs (Figure ). Superficial infection was noted at the pin insertion sites and the patient was advised a one-week course of oral antibiotics along with local wound care. His hand was positioned in a removable splint and range of motion exercises of the wrist and fingers were begun. He was examined at the six-week mark with a resolved infection.\nThe posteroanterior, oblique, and lateral radiographs had confirmed the anatomical position of 4-5 CMC joints (Figure ). He also had regained full range of wrist motion and grip strength. Further follow-up was not possible due to the onset of the coronavirus disease 2019 (COVID-19) pandemic and the release of the patient from prison with no contact information. |
A 52-year-old male patient presented to the urology outpatient department with a history of decreased urine passage along with burning, hematuria and pain lower back and abdomen. History of loss of appetite, vomiting was also elicited, he also gave a history of smoking and alcohol consumption. Patient was advised an ultrasonogram (USG) along with biochemical and hematological investigation. Routine examination of urine was done which revealed few red blood cells. No atypical or malignant cells are seen in three consecutive specimen of urine for cytology His renal function tests were markedly deranged, with blood urea 346 mg/dl, serum creatinine 25 mg/dl and deranged electrolytes. His blood pressure was raised 230/100 mmHg and fundoscopy showed hypertensive retinopathy. USG showed features of cystitis with hypoechoic areas extending into the thickened bladder wall with features of pyelonephritis. An infective pathology was offered as diagnosis on imaging. A clinical diagnosis of bilateral obstructive uropathy due to tumor with chronic kidney disease was made, and the patient was put on hemodialysis and cystoscopy planned at a later date. Cystoscopy findings were of a growth with small fronds/papillae, prominent at the bladder neck with a very rigid thickened bladder wall. Trans urethral resection of the bladder tumor was done and sent for histopathological evaluation. The microscopic evaluation showed a high-grade tumor arranged in discohesive cords and sheet with plasmacytoid features and prominent retraction artifact [] penetrating through the entire bladder wall into the serosa. Histopathological features of the malignant epithelial cells were uncommon and lead to a differential diagnosis of undifferentiated transitional carcinoma, metastatic or primary lymphoma plasmacytoma, lymphoepithelioma-like bladder carcinoma and a plasmacytoid variant of bladder tumor. Immunohistochemistry was done for CD138, cytokeratin (CK) CK7, leukocyte common antigen (LCA), κ and λ light chain, with positivity for CK7, CD138 [Figures and ] and negative for other markers. Based on these findings, a final histopathological diagnosis of PUC was made. The patient was advised to undergo palliative cystectomy, however he is lost to follow-up. |
A seven and half year-old boy visited the outpatient clinic of Pediatric Dentistry Department, Faculty of Dentistry, Cairo University in June 2015 with a chief complaint of pain on the lower right molar area. The patient’s mother stated that the pain was at times throbbing in nature, and child is not able to chew on this side.\nClinical examination showed a badly decayed, lower second primary molar with related localized intraoral abscess, where the lower first primary molar was intact. The patient had poor oral hygiene; he had not received any professional dental care, and was very apprehensive.\nRadiographic examination revealed root resorption and bone rarefaction related to lower second primary molar. The interesting finding was a considerable amount of root resorption of the distal root of the adjacent lower first primary molar (\n).\nThe case was managed by performing pulpectomy\n to the lower second primary molar, with root canals filled with calcium hydroxide paste with iodoform (Metapex, Meta Biomed, Republic of Korea). The tooth was then restored with high viscosity glass ionomer (GC Fuji IX GP capsule, GC corporation, Tokyo, Japan) (\n). The lower first primary molar was not touched and instead monitored. No antibiotics or analgesics was prescribed.\nUnfortunately, the patient’s mother did not want follow-up appointments in person, however, she was contacted on the phone, after 2 weeks, 3 months and 6 months, and she said everything was fine and there was no swelling or pain.\nAt about 8 months from the treatment appointment, the patient’s mother visited the outpatient clinic with the patient for other reasons, and decided to pass by the Pediatric Dentistry Department for patient follow-up. Clinical examination showed no signs or symptoms, occlusal restoration was intact, and radiographic examination revealed arrested root resorption, on both molars, and an increase in the density of bone although this was not at a normal level yet (\n).\nshows the patient’s timeline of symptoms, treatment and follow-up. |
A 30-year-old male with no past medical history, except a diagnosis of schizophrenia, had a second psychiatric hospitalization for the treatment of worsening delusions and hallucinations. His first hospitalization occurred approximately 1 month prior, and discharge medication was olanzapine 20 mg at bedtime. Also, at that time his platelet count was 156 × 103/μL; reference range is 135 × 103/μL to 317 × 103/μL (). Three days prior to the current admission, the patient had self-discontinued olanzapine because of blurry vision. During the hospitalization the patient was trialed on multiple antipsychotics without benefit. Because of persistent psychotic symptoms, clozapine 25 mg at bedtime was initiated on hospital day (HD) 24. A complete blood count (CBC) with differential at that time revealed no derangements, including a platelet count of 156 × 103/μL.\nBy HD 29, clozapine was titrated to 175 mg at bedtime, at which time the patient started to complain of sialorrhea with nighttime predominance. One drop of ophthalmic atropine 1% sublingually administered at bedtime was initiated but was ineffective after 5 days of use, and the patient was not agreeable to an increase of the drops. The patient reported awakening at least 5 times throughout the night to “spit into a water bottle” and complained of having a wet pillow every morning. Both the bottle of saliva and wet pillow were observed by staff. Clinically, clozapine was increased to target psychotic symptoms, but it was divided as 50 mg in the morning and 150 mg at bedtime in an attempt to minimize sialorrhea. This was not successful to reduce the excessive salivation. Clozapine was further increased to a total daily dose of 250 mg by HD 36 with improvement in psychotic symptoms, although sialorrhea persisted. Clonidine 0.05 mg by mouth twice daily was added on HD 37 to target sialorrhea. On HD 39, the patient reported improvement of sialorrhea, and objectively his pillowcase was found to be dry during morning rounds.\nDuring the first week of clonidine treatment (HD 37-43), the patient's platelet count decreased from 146 × 103/μL to 132 × 103/μL. Clozapine had reached a total daily dose of 300 mg by HD 43. On HD 50 the platelet count remained the same, but on HD 52 the platelet count was found to be 117 × 103/μL. All other cell line values were within normal limits. There were no concerns for bleeding, evidence of bruising, or petechiae, but the decision was made to discontinue clonidine given the new thrombocytopenia. Sialorrhea was treated with continued sublingual ophthalmic atropine 1%, 1 drop at night, with the patient accepting additional as needed doses, using on average an extra 2 to 3 drops per day. Five days later, on HD 58, a CBC with differential revealed that the platelet count was within normal limits at 153 × 103/μL. At this time the patient was also noted to be psychiatrically ready for discharge. Delays related to placement issues resulted in the patient leaving the acute care psychiatric hospital on HD 84. Discharge medications included clozapine 150 mg in the morning and 150 mg at bedtime; atropine 1% drops, 1 drop sublingually at nighttime and as needed; melatonin 3 mg at bedtime; and senna/docusate 8.6/50 mg twice daily as needed for constipation. |
We present the case of a 67-year-old gentleman with no history of autoimmune disease, was diagnosed with clear cell renal cell carcinoma (RCC) stage T3b, Fuhrman grade 4/4, and underwent a right radical nephrectomy, right adrenalectomy, and vena cava tumor thrombectomy in November 2016. After a follow-up computed tomography in March 2017 demonstrated multiple lung nodules, the patient underwent a left upper lobe wedge resection in April 2017 which confirmed metastatic renal cell carcinoma to lung. Nivolumab 240 mg in sodium chloride 0.9% 100 mL infusion every 2 weeks was started in May of 2017 progressing to 480 mg nivolumab every 4 weeks on May 1, 2018. The patient reported no irAEs with nivolumab for the following 38 months.\nIn April 2020, computed tomography demonstrated oligoprogressive disease with increase in size of 2 lung nodules, but otherwise stable disease. The patient was referred to radiation oncology and was treated with stereotactic body radiation therapy 5000 cGy in 5 fractions to both progressive lesions. Maximum dose to skin was 1481 cGy with less than 5 cc of skin receiving 1000 cGy or more (200 cGy per fraction). The associated dose to skin was minimal with only 5 cc of skin receiving 1000 cGy or more. Radiation was well-tolerated and completed on May 22, 2020.\nAfter completion of short-beam radiation therapy, nivolumab 480 mg in sodium chloride 0.9% 100 mL infusion was continued on schedule with the next infusion provided on May 26, 2020. Following this, the patient developed diffuse, mildly pruritic skin lesions with blisters and presented to dermatology 1 week later with multiple erythematous bullae on the trunk, as well as the upper and lower extremities. Some bullae coalesced into crusted plaques and others were hyperpigmented patches. At this presentation, less than 10% of the patient's body showed involvement characteristic of bullous dermatosis and he was appropriately characterized to have mild to moderate cutaneous toxicity. A topic steroid (betamethasone 0.05% cream) was prescribed to manage the rash and nivolumab was continued accordingly.\nFollowing the next 480 mg nivolumab in sodium chloride 0.9% 100 mL infusion 1 month later, the patient's bullous dermatosis worsened, with progressive involvement of the upper and lower extremities. The patient presented with progressive foot involvement and linear erosions of the face. Additionally, the patient had developed edema of the lower extremities resulting in a follow-up with dermatology on July 6 and punch biopsy on July 14.\nSections of the biopsy demonstrated complete subepidermal detachment of the epidermis from the underlying dermis (Fig. ). Within the papillary dermis there was a moderate band like inflammatory infiltrate composed of lymphocytes, a prominent number of eosinophils and rare neutrophils (Fig. ). Direct immunofluorescence staining was performed on a separate skin biopsy and demonstrated linear staining for C3 and immunoglobulin-G along the basement membrane with no intercellular staining of the keratinocytes (Fig. ). The histologic features in conjunction with the immunofluorescence staining supported a diagnosis of bullous pemphigoid.\nGiven the progressive symptoms despite topical corticosteroids and the biopsy proven development of bullous pemphigoid (BP), Nivolumab was held and the patient began treatment with 100 mg doxycycline and 80 mg prednisone daily for a week, reduced to 60 mg during the second week. On follow-up, the patient showed significant improvement and over the next 10 weeks was tapered down to 20 mg of prednisone. Four months following the initiation of treatment of BP, 480 mg nivolumab in sodium chloride 0.9% 146 mL infusion was restarted and the patient continued low-dose tapering of prednisone until December. Since completing the prednisone course, the patient has shown no recurrence of bullous pemphigoid and has not developed any other irAEs to nivolumab upon rechallenge. Follow-up through October of 2021 demonstrates the patient's sites of disease, both in- and out-field, have remained responsive to treatment. |
A 79-year-old female who was previously healthy admitted to our hospital with a 1 year history of constant dull right upper quadrant pain and tenderness. All laboratory tests including tumor markers were in normal limits. There was not any story of hepatitis or chronic liver disease. Abdominal ultrasound revealed a hypoechoic solid lesion that located in the right and caudate lobe of liver with irregular border. MDCT examination reveals a liver mass with 52 mm × 43 mm diameters located in segment VIII and caudate lobe of the liver []. The lesion showed heterogeneous enhancement in arterial phase with hypointense central area also. The mass become hypodense to isodense to adjacent parenchyma on delayed venous phase. The region of nonenhancing hypointense area was likely to represent central necrosis, which was frequently seen in malign PEComas. The patient underwent MRI, after first assessment with MDCT for better evaluation of vascular invasion. In MRI, the tumor had intermediate isointense signal on T1-weighted images and heterogeneously hyperintense on T2-weighted images relative to the liver parenchyma []. There was not seen any fat component in the lesion. On gadolinium (Gd)-enhanced images, the mass showed significant and heterogeneous enhancement. Nonenhancing hypointense area in tumor center representing necrosis. Inferior vena cava (IVC) invasion with tumor thrombus into the lümen is also demonstrated, especially coronal Gd-enhanced images []. There was also marked diffusion restriction of lesion on diffusion-weighted images as totally hypointense signal in apparent diffusion coefficient maps [].\nCalcification and/or hemorrhage were not detected in the lesion. Metastasis in other systems was also not found. The patient consented to tumor biopsy after radiologic assessment. The patient underwent biopsy of the liver mass, and pathology revealed a hepatic perivascular epithelioid cell tumor or “PEComa.” Histologic findings were a mixture of large epithelioid cells and clear-to-eosinophilic granular cytoplasm []. There was severe nuclear atypia with large nucleoli and increased mitotic activity detected microscopically. However, immunochemistry study indicated that tumor cells were positive for human melanoma black (HMB)-45 marker. Following the surgery, the patient has not shown any evidence of recurrence for 6 months. |
A 54-year-old woman presented with 8-month history of progressive atypical left facial pain and numbness. She had no previous illness, and her elder brother died few years ago from parietal glioblastoma multiforme. General physical examination was unremarkable; there were no cutaneous stigmata of neurofibromatosis. The neurological examination demonstrated facial hypoesthesia in the distribution of the second and third divisions of the trigeminal nerve. The rest of cranial nerves and neurological examination were within normal.\nMagnetic resonance imaging (MRI) of the brain revealed a 40 × 25 × 40 mm isointense lesion that enhanced heterogeneously following intravenous Gadolinium administration enhancing lesion in the anterior part of left middle cranial fossa involving the anterior part of the temporal lobe and extends to the infratemporal fossa through foramen ovale (Figures , , and ). The tumor had intra-axial enhanced solid and cystic unenhanced components in the left anterior temporal lobe with surrounded vasogenic edema. The radiological findings were likely consistent with trigeminal schwannoma. Surgery was performed via temporal extradural and intradural approach, with neuronavigation guidance, through left orbitozygomatic-temporal craniotomy. The tumor was seen extradurally easily separable from the dura. It was noticed that it is smaller than the expected size from the MRI. The dura was then opened and a striking intra-axial soft tumor was noted in the superior and middle temporal gyri. Complete microsurgical resection, with ultrasonic aspirator, of the intradural portion of the tumor was performed through anterior temporal lobectomy and the dura was closed. The extradural portion was exposed with drilling of middle fossa floor into the infratemporal fossa and enlarging foramen ovale laterally. The tumor was encapsulated and removed completely, with ultrasonic aspirator, and few of the involved trigeminal nerve fascicles were sectioned.\nMicroscopically, the tumor showed two components. The first component of temporal lobe specimen revealed a mitotically active malignant glial neoplasm, with no endothelial proliferations or necrosis (). These morphological features were consistent with anaplastic astrocytoma (WHO grade III). Immunohistochemistry study revealed a strong glial fibrillary acidic protein (GFAP) and P53 positivity (). Ki-67 proliferative index showed a mild to moderate (5–10% per 10 HPF) proliferation (). The second component from the extradural specimen revealed a malignant spindle cell neoplasm arranged in a fascicular pattern and embedded in a loosely textured background, with apparent numerous mitosis and tumor necrosis (). Immunohistochemically, the spindle cells were strongly positive for vimentin and S-100 proteins, and apparent P53 expression (). Ki-67 proliferative index showed a mild to moderate (5–10% per 10 HPF) proliferation (). The final diagnosis was consistent with two coexisting intermingled anaplastic astrocytomas and malignant trigeminal nerve sheath tumor (MTNST).\nThe patient had uneventful postoperative period, with significant improvement of facial pain. Postoperative brain MRI scans demonstrated a complete resection of both tumors (Figures and ). After counseling, the patient decided not to receive any further adjuvant therapy. She was readmitted 8 months later for palliative care due to progressive cognitive function decline. Brain MRI scans revealed left temporal leptomeningeal enhancement at resection cavity at the temporal and infratemporal regions, as well as new heterogeneous enhancing lesion at anterior part of corpus callosum; the latter is likely from the anaplastic astrocytoma component (). She passed away 9 months from surgery from tumor progression causing raised intracranial pressure and brain herniation. |
A 30-year-old woman reported complaining of disfigurement of face due to swelling in gums since seven years, which was causing functional and masticatory difficulty. She presented with a generalized severe gingival overgrowth, involving the maxillary and mandibular arches and covering almost the whole dentition. The patient was also concerned about the progressively increasing space between her upper front teeth and their movement away from their original positions. Extra oral examination () revealed a convex profile with bimaxillary protrusion, incompetent lips, and malocclusion.\nPatient's medical and family history was noncontributory. The patient was not receiving any antiepileptic, antihypertensive, or immunosuppressive medications that could contribute to the gingival enlargement. Past dental history reveals that patient got surgical treatment (gingivectomy) done for the same twice, but the condition had reoccurred.\nIntraoral examination revealed enlargement of the gingiva on both buccal and lingual/palatal sides with pinkish red, fibrous inconsistency and absence of stippling. Gingival enlargement enclosed the major surface of the teeth present except the incisal/occlusal surfaces. Severe diffuse enlargement involving the marginal, interdental, and attached gingiva of both arches, covering almost all the surfaces of the teeth, was found. There was generalized spacing in the dentition with proclined maxillary anteriors ().\nClinical examination revealed mobility in all the teeth present and severe pathologic migration, especially of the upper anterior teeth. There were deep pockets present and there was an increase in the intermaxillary rest position. Physical examination of the whole body and blood investigations were advised to eliminate any medical abnormalities.\nThe radiographic findings corroborated those of the clinical examination and revealed severe generalized alveolar bone loss, which could be attributed to the local factors which must have exaggerated the hyperplastic condition. The peripheral blood results were normal and correlated with an absence of any history of systemic disease. Based on all these findings, a provisional diagnosis of idiopathic gingival enlargement was made.\nTreatment decided was full mouth undisplaced flap surgery.\nAfter routine phase one periodontal therapy a treatment plan was formulated which comprised of quadrant-wise undisplaced flap surgery (the only treatment of choice in this condition as we had to treat the patient's underlying periodontal disease). The treatment procedure was explained to the patient and written consent was obtained. The surgery was planned under local anesthesia containing 2% lignocaine with 1 : 200000 epinephrine. Undisplaced flap surgery was performed to excise desired quantity of soft tissue. The wound was irrigated with betadine and a Coe-Pak (noneugenol, hard, and fast set) was given for seven days. Patient was advised to take analgesics and rinse twice daily with 0.2% chlorhexidine mouthwash. The excised tissue was sent for histopathological evaluation.\nThe sections revealed moderately dense collagenous connective tissue with collagen bundles arranged in a haphazard manner. Connective tissue was relatively avascular along with scanty inflammatory cell infiltrate showing dense wavy bundles of collagen fibers containing numerous fibrocytes and fibroblasts. The overlying epithelium was hyperplastic with elongated rete ridges. The histopathologic features led to the final diagnosis of idiopathic gingival fibromatosis.\nThe case was followed up for 6 weeks postoperatively and then every 3 months for 2 years. The mobility of the teeth was reduced to physiologic at the end of 3 months. No recurrence was observed within 2 years. Patient is still following the follow-up regime. Mild recurrence in the right maxillary posterior palatal segment was seen after 1 year (). |
A 42-year-old male was referred to his ophthalmologist regarding the complaint of sudden blurriness of vision in his right eye. He was diagnosed five months ago with a case of ENKTCL, nasal type, stage IV-B with testicular and central nervous system involvement with 23% natural killer (NK) cells on cerebrospinal fluid (CSF) flow cytometry. He was on an active therapeutic plan consisting of cycles of chemotherapy (i.e., SMILE protocol: dexamethasone, methotrexate, ifosfamide, L-asparaginase, etoposide), to which there was a complete response in the form of negative flow cytometry and computed tomography (CT) of the chest, abdomen, and pelvis. Five months after the initial diagnosis, prior to the fourth cycle of treatment, a complaint of blurriness of right eye vision was made. Brain CT was done aiming to rule out ocular involvement, which turned out to be unremarkable. At the time of the complaint, visual acuity was 20/30 -2 in the right eye as opposed to 20/20 in the left eye. The right eye showed relative afferent pupillary defect. Extraocular muscle movements were competent in both eyes. On examination of the anterior chamber, +1 cells were visualized in the right eye only. On dilated fundus examination, there was vitritis in the right eye which obscured the vision. Left eye examination was insignificant. B scan ocular ultrasonography revealed retinal detachment in the right eye. An optic CT revealed vitritis in the right eye; an impression of disease infiltration of the eye was made. The therapeutic plan was a diagnostic vitrectomy followed by systemic therapy. A pars plana vitrectomy was made and the vitreous specimen was sampled revealing the presence of 10% viable lymphoid cells expressing CD2 and CD56 on immunohistochemistry stain. An orbital magnetic resonance imaging (MRI) was ordered revealing evidence of an enhancing retinal lesion centered on the optic disc with diffuse restriction consistent with lymphomatous infiltration; minimal proptosis of the right eye was noted (Figure ). Fundus photography of both eyes was performed postoperatively with the retina flat under the silicon in the right eye as seen in Figure and minimal disease involvement of the left eye which further confirmed the diagnosis (Figure ). In addition, CSF flow cytometry revealed immunophenotypic evidence of disease (75% mature T cells and 20% NK cells expressing CD2, CD16 and CD56). Follow-up examination revealed 6/9 vision in the left eye, as opposed to no light perception in the right eye. The patient received 35 Gray units of radiotherapy to the optic apparatus and posterior globe of both eyes and the entire cranium down to the third cervical spine region. Post-radiotherapy MRI revealed interval regression of the disease in the form of resolution of bilateral optic disc infiltrative nodule. Despite medical efforts, the patient’s condition deteriorated, and he passed away. |
A 73-year-old female, without a prior history of bleeding or hemophilia, presented with difficult to control bleeding, large ecchymoses over her body, petechiae, and hemarthrosis over the prior 2 months. She was in her typical state of health until she fell in her home and landed on her left hip in early November, 2012. A large ecchymosis extended both distally and proximally from the site of injury. Over the next month, she noticed spontaneous bleeding, after minor or no trauma, over her body to include bilateral arms, right thigh, and left ankle. Both her right knee and left ankle became swollen and were limited in their range of motion. Several days prior to admission to an outside hospital, the patient reported increasing dyspnea on exertion, with new right thigh and tongue swelling. She was subsequently discharged, readmitted, and transferred to Johns Hopkins Hospital.\nThe past medical history revealed hypothyroidism, vitiligo, Raynaud phenomena, hypertension, coronary artery disease, and a rectal polyp. Previous surgeries included an appendectomy in 1958, herniated disc surgery in 1983, bare-metal stenting in 2007, and a polyp removal in 2012. The 6 cm rectal polyp was removed in August, 2012, which was complicated by postoperative bleeding after being discharged the same day, but the patient did not require blood transfusion or hospitalization at that time. All previous surgeries were uncomplicated without incident of excess blood loss. The family history was absent of bleeding diatheses. She previously drank significant amounts of alcohol but had cut down in 2012 and had a smoking history of 50 pack-years.\nThe outside hospital initially evaluated her in December and at that time she had a normal PT, fibrinogen, and platelet count. The aPTT level was elevated to 50–60 seconds. Erythrocyte sedimentation rate was reported as 54 mm/hr and C-reactive protein of 1.1 mg/dL. Coombs' test was negative with a normal serum protein electrophoresis and haptoglobin level.\nWhen she arrived to JHH, she was found to have a low factor VIII level at 16% with active bleeding. Despite this finding we did not measure any inhibitory activity in her plasma using Nijmegen modification of the Bethesda's assay. It is of note that we found in the aPTT evidence of an inhibitor. Immediately, the 4 : 1 mixed aPTT was delayed at 34.3 seconds (reference range: 23.3–30.3 s). After 2 hours, the 4 : 1 mixed aPTT continued to be prolonged at 39.7 seconds. All of her other factor activity levels were normal: FII: 138%, FV: 192%, FVII (measured after recombinant FVIIa given): 1212%, FIX: 137%, FX: 190%, FXI: 90%, and FXII: 60%. Because acquired inhibitors may have variable kinetics in inhibiting coagulation, we varied the incubation times in the Bethesda assay from 30 minutes to as long as 180 minutes and detected no inhibition. Additionally, the liver function tests were within normal limits.\nChromogenic assay revealed a significantly low factor VIII antigen of 16%. Dilute Russell viper venom time was normal at 37 seconds. A heparinase test did not shorten the aPTT. Euglobulin lysis (>60 mins), von Willebrand antigen (>150%), and ristocetin cofactor (>149%) were all normal. Both an antinuclear antibody screen and hepatitis B and C studies were negative. Serum immunoglobulin assay showed a slightly low IgG level of 629 mg/dL with normal IgA and IgM levels of 147 mg/dL and 161 mg/dL, respectively. The serum protein electrophoresis showed no monoclonal gammopathy. The remainder of the work-up laboratory data can be found in .\nDespite the negative Bethesda assay, her clinical presentation was consistent with the presence of an inhibitor. Thus, a trial of recombinant FVIII was given and serial FVIII activities were done to determine recovery time. Fifty units/kg of factor VIII was given and at 60 minutes FVIII activity was 62%. Subsequent drop-offs of the factor VIII activity are shown in .\nTo control bleeding, recombinant activated factor VII (NovoSeven RT) was given every two hours for 24 hours. The patient's hemoglobin stabilized and she was started on prednisone at one mg/kg a day. Cyclophosphamide was initiated the same day at 100 mg/day. Over a period of 8 months, the factor VIII activity steadily increased to a maximum of 220% without any further bleeding complications. However, cyclophosphamide was stopped 38 days after initiation due to toxicity. Prednisone has been slowly tapered over the 8-month treatment course. The laboratory data, clinical course, and treatment can be followed in . |
A fifty-four year old male presented with an eight month history of right-sided level II neck lump. He had a life-long history of smoking 10 cigarettes a day and heavy alcohol use of 40 international units per week. Examination revealed a 2 cm right sided tonsillar mass extending to the tonsillar pillar and the soft palate with a 6 × 4 cm right sided neck mass spanning levels II and III. Tonsil biopsy confirmed squamous cell carcinoma. The patient was treated with two 3-weekly cycles of induction chemotherapy comprising cisplatin 80 mg/m2 on day 1 and continuous infusional 5-fluorouracil 1000 mg/m2 on days 1–4, followed by radical chemoradiation. IMRT consisted of 65Gy in 30 fractions to the oropharynx and right neck levels Ib, II and the upper part of level III and 54Gy in 30 fractions to the right neck lower level III, IV and V and left neck levels II to V. Concomitant cisplatin 100 mg/m2 was delivered on days 1 and 29 of radiotherapy. A 3 month post-treatment FDG-PET-CT scan showed an equivocal FDG uptake of 4.5 maximum Standardised Uptake Variable (SUV) noted at the original site but with no mass lesion correlated on CT imaging and only low-grade FDG uptake in minimally enlarged right sided level II neck nodes. A multidisciplinary review recommended surveillance with repeat FDG-PET/CT scanning after three months.\nIncisional biopsy of the right tonsil showed a biphenotypic carcinoma with features similar to those of Case 1. In addition, the differentiated component demonstrated a focal papillary architecture lacking keratinisation and centred on the tonsillar crypts (Fig. and ). Both components demonstrated strong nuclear positivity for p63 and strong nuclear and cytoplasmic staining for p16 in greater than 70 % of tumour cells (Fig. and ). By contrast to Case 1, the differentiated component showed greater percentage and intensity of staining for CK7 (Fig. ) and CK19. Furthermore, unlike Case 1, both components showed similar percentage and intensity for CK8/18 and similar percentage of cells in cycle (data not shown). Also by contrast to Case 1, high-risk HPV ISH showed diffuse nuclear staining in the differentiated compartment and a punctate pattern in the undifferentated areas (Fig. ). A slightly lower density of TILs was present in the former component (Fig. ). The relative expression of Vimentin in the two components was similar to that of Case 1 (Fig. ). The immunohistochemical profiles for cases 1 and 2 are summarised in Table . |
We present a 77-year-old male with a past medical history of NHL/SLL diagnosed almost 10 years ago, who presented to the hospital with abdominal swelling, altered mental status, and difficulty in urinating associated with hematuria. On physical examination, diffuse bulky lymphadenopathy was found in the cervical, axillary, and inguinal areas. Detailed oncologic history and treatment regimens that were taken by the patient have been well explained in Table .\nLaboratory workup showed hypokalemia, hypophosphatemia, and elevated lactate dehydrogenase levels. During the hospitalization, computed tomography (CT) scan head was done that showed negative findings for any acute events. Due to altered mental status with underlying worsening of NHL and metabolic disturbances, the toxic and metabolic encephalopathy were the differentials under consideration. Metabolic derangements were corrected during hospitalization and that improved his mental status as well. Idelalisib treatment was discontinued abruptly a week prior to patient’s presentation to the hospital due to pancytopenia and a mixed response on the CT scan imaging. Upon admission, a repeat CT of the abdomen and pelvis showed diffuse bulky lymphadenopathy in the abdomen; one of the nodes in the anterior para-aortic region was measured about 5 cm × 5 cm × 8 cm (Figure ). Bilateral iliac, inguinal, and retroperitoneal lymphadenopathy was also significantly increased in size compared with prior CT scan. There was diffuse lymphadenopathy along with axillary and cervical regions as well (Figures -).\nBone marrow biopsy was done that showed a cluster of differentiation 5 (CD5) and CD23 positive B-cell population (37% of the lymphoid gate), lambda-restricted. Almost all of the B-cells showed immunophenotypic expression of CLL/SLL with lambda light chain restriction that was found in previous cases of this patient. Interestingly, a kappa light chain restricted population of monoclonal plasma cells co-expressed with CD56 (1.1% of total events) is also identified. Urology was consulted for urinary complaints of difficult voiding and hematuria. However, the patient was further diagnosed with paraphimosis and scheduled to have an elective circumcision that alleviated his urinary complaints later. Idelalisib treatment for SLL/NHL that patient took for almost one year was stopped a week prior to current clinical symptoms. Based on progressive SLL/NHL, the patient was started on venetoclax (B-cell lymphoma 2; BCL-2 inhibitor) 20 mg/day for seven days and 50 mg/day afterward. The patient did not develop any tumor lysis syndrome after starting the therapy and is tolerating the treatment well based on both imaging and clinical finding. |
A 45-year-old woman presented with a painful swelling on the left side of her jaw of 5 months duration. The swelling had recently started to grow rapidly. There were no associated sensory symptoms. In addition, a few teeth adjacent to the swelling had become loose, carious and had fallen off. Local physical examination showed a 4.0 × 3.2 × 3.0 cm, tender, firm, irregular lump that appeared to arise from the left mandible. Intra orally, the tumor could be seen as a pink, bulging and fleshy mass. Two lymph nodes were palpable, one in left submaxillary region measuring approximately 1 × 1 cm and the other in the left cervical region and was barely palpable. Remainder of the physical examination was not contributory and no other swelling was noticed. Orthopantomogram (OPG) of the jaw showed a radiolucent lesion with irregular margins, centered in and destroying a portion of the left mandibular body (Figure ). The teeth adjacent to the mass were carious and some were missing. A computed tomography (CT) scan of the oral cavity and neck was advised but was not done due to financial reasons. A clinical diagnosis of ameloblastoma with suspicion of lymph node involvement was given.\nAn incisional biopsy of the lesion was submitted for pathologic evaluation. Microscopic examination of the tissue showed a neoplasm composed of epithelial cells arranged in irregular nests separated by fibrovascular septa. The cells adjacent to the fibrovascular septa were cuboidal to columnar with high nuclear-cytoplasmic ratio and eosinophilic cytoplasm, while those in the center of the nests were larger and polygonal, with abundant clear cytoplasm (Figure ). Each cell had a single nucleus with fine chromatin and prominent eosinophilic nucleolus. The cells at the periphery of the nests occasionally demonstrated nuclear palisading away from the basement membrane, i.e., reverse nuclear polarity (Figure ). These peripheral cells also showed occasional mitoses. The central areas of the nests and fibrous septae showed infiltration by neutrophils. The cells were immunoreactive for pan-cytokeratin, epithelial membrane antigen, and S-100 protein; they did not stain for vimentin, smooth muscle actin, and HMB-45. The abundant, clear cytoplasm of the cells was strongly positive for periodic acid-Schiff (PAS) (Figure ). This PAS positivity was diastase sensitive indicating intracytoplasmic glycogen (Figure ). A diagnosis of clear cell odontogenic carcinoma was entertained. Fine needle aspiration from the submaxillary node was done but was inconclusive.\nThe patient was referred to a specialty cancer hospital where she underwent left hemimandibulectomy with ipsilateral functional neck dissection (level1 to level 5). The reconstruction was done using ipsilateral 12th rib and the soft tissue coverage was given by pectoralis major flap. Postoperative histopathological examination showed tumor morphology similar to incisional biopsy and 1/11 lymph nodes (submaxillary) was positive for the tumor metastasis without any extranodal spread. The tumor was extending into the soft tissue and the left bony margin was positive for the tumor cells. Due to the positive margin, the patient was referred for adjuvant radiotherapy for which she refused to give consent. At the time of discharge the patient was accepting soft diet and the suture line on the neck and chest were healthy. Subsequent to the discharge, the patient was lost to follow-up only to reappear after 8 months and at that time had no signs of local or regional spread. Since then patient is again lost to follow-up. |
A 45-year-old woman was admitted to the hospital because of a 5 month history of paroxistic abdominal pain and vomiting. The patient's past medical history included ovarian endometriosis treated with bilateral oophorectomy in another hospital seven years earlier. She subsequently received treatment with medroxyprogesterone and transdermal estrogens.\nOn physical examination the abdomen was distended and bowel sounds were increased. Laboratory values were unremarkable, and abdominal x-rays confirmed the diagnosis of intestinal obstruction. Laparotomy revealed an ileal mass protruding into the lumen. A right hemicolectomy including 40 cm of ileum was performed. The female genital tract and pelvis had no macroscopic evidence of endometriotic lesions.\nHistopathology revealed the following: the resection specimen consisted of 16 cm of right colon and 39 cm of ileum. A tumor measuring 5.5 cm was found within the wall of the ileum 2 cm from the ileocaecal valve. Morphologic and immunohistochemical features were typical of an endometrioid adenocarcinoma (cytokeratin (CK) 7-positive, vimentin-positive, CK20-negative) (figure ). The high nuclear grade tumor involved the mucosa, the muscularis propria and the subserosal fat. Vascular invasion was noted as well as metastatic involvement of 5 out of 40 isolated lymph nodes. Several foci of endometriosis were identified adjacent to this mass and in the caecum. Both, the epithelial and the stromal cells tested positive for estrogen receptors and for CK7, while tests for CK20 proved negative. The tumor itself showed only weak positivity for estrogen receptors.\nAfter surgery the patient received subsequent chemotherapy with 5-fluorouracil and leucovorin. Twenty months later the patient noted rectal bleeding. A colonoscopy discovered a mass at 15 cm from the anal edge. The biopsy showed an adenocarcinoma. A second endometrioid adenocarcinoma was not suspected before surgery.\nDuring laparotomy a tumor in the anterior wall of the rectum was seen. It was fixed to the uterus, occupying the recto-uterine pouch which it seemed to invade. No macroscopic endometrioid foci were seen in the pelvis. En bloc resection including rectum, uterus and the fallopian tubes was performed, as an invasion of the uterus was suspected. Transit restoration was achieved by termino-terminal anastomosis using the EEA-stapler device.\nHistopathology revealed the following: The resection specimen consisted of 18 cm of rectum, the uterus and the fallopian tubes. Histological examination confirmed the presence of a tumor measuring 3.5 cm in diameter, located in the anterior wall of the rectum and embedded to the posterior wall of the uterus. The poorly differentiated adenocarcinoma involved all layers of the intestine up to the uterus which was not invaded microscopically. The uterus and the fallopian tubes had no major microscopic changes. Morphological and immunohistochemical features of this endometriotic adenocarcinoma were similar to the previously removed tumor (CK7- positive, vimentin-positive, and CK20 negative) (figure ). There was no lymph node involvement in any of the 17 removed lymph nodes. Multiple microscopic foci of endometriosis were seen, especially in the peritumoral and peritubaric areas. Once again, immunohistochemical features of these foci included positive estrogen receptors and a positive result on CK7 in the epithelial and stromal cells, and negative results for CK20. After the second operation, the patient started treatment with raltitrexed (Tomudex®) and 5-Fluorouracil. After five years, she remains asymptomatic and has no evidence of recurrence. |
This study is approved by our institutional review board and this patient has signed informed consent.\nA 33-year-old Chinese male initially presented with an epileptic seizure. Magnetic resonance imaging (MRI) detected two extra-axial, homogeneous, well-defined, dural-based lesions. The large one is in the left frontotemporal parietal region, and the small one is in the right frontal region. T1-weighted images show isointense and slightly hyperintense signal intensity, and T2-weighted images show isointense with intralesional hypointense foci, obviously uniform enhancement with an enhancing dural tail sign (Fig. ). This patient was initially diagnosed with multiple convex meningiomas and underwent a craniotomy for resection of the large lesion. After the left lesion was resected, this patient recovered very well and restored normal speech function. We recommended adjuvant hormone therapy or radiotherapy but the patient declined any further treatment until progression was seen on the MRI and the patients suffered from a symptomatic seizure 20 months after surgery. MRI showed the size of the right lesion increased obviously. Then the second craniotomy was taken to remove the right lesion, and the patient recovered again with no neurological deficit. There was no seizure nor tumor recurrence on follow-up so far (2 years follow-up).\nIn the sections from the first operation, microscopic examination showed a large number of lymphocytes and plasma cells infiltrated in fibrous tissue scattered large histiocytic cells with abundant cytoplasm. Immunohistochemical staining showed positive for S100, CD68, but negative for CD1a (Fig. ). Both morphological and immunohistochemical profiles of the left lesion supported the diagnosis of RDD.\nHistopathological examination for the sections from the second operation exhibited similar results as the first one (Fig. ). Histiocytes were the predominant cells and contained abundant pale to vacuolated cytoplasm and mildly pleomorphic vesicular nuclei with multiple prominent nucleoli and emperipolesis.\nBased on whole exome sequencing (WES) data, we calculated the tumor mutation burden (TMB), obtained driver genes, and performed clonality analysis. The larger (left) neoplastic lesion has a higher TMB (3.06/Mb) and more tumor-driven mutations than the smaller (right) one (1.03/Mb). Four driver gene mutations, including PIK3R2, MED12, SUFU, and SOX2 were found in the larger lesion, while only one tumor-driven mutation (NOTCH2) was found in the smaller lesion. There are seven common truncal mutations in the two lesions (Fig. ). None of them are driver gene mutations. These mutations rarely appear in other populations, indicating that these two lesions may originate from the same main ancestor clone. |
A 37-year-old female was admitted to the thoracic surgery department with dysphagia that had progressively worsened during the previous year. Endoscopy of the upper gastrointestinal system revealed a yellowish-colored, adipose-tissue-like appearance of a polypoid mass with a smooth surface covering between 25 and 34 cm of the esophageal wall (fig ). Computed tomography (CT) of the thorax revealed a posterior mediastinal mass (height × width × depth: 10 × 5 × 3 cm; fig ), which was compressed between the vertebral column, aorta and heart and pushed the lumen of the esophagus leftward (fig ). The mass had low-density tissue absorption, which was characteristic of adipose tissue when compared with surrounding tissues. A barium-swallowed contrast study disclosed an intraluminal space-occupying polypoid lesion protruding through the esophageal wall, which narrowed the lumen of the esophagus (fig ). Therefore, esophageal lipoma was considered the most likely diagnosis, and the decision for surgical excision was made.\nA right-sided mini-thoracotomy was performed, the mediastinal pleura was opened and tumor location was identified. Enucleation of the lipoma was the planned operative strategy. The overlying muscle layer of the lower third of the esophagus was incised to expose the tumor, which was then completely enucleated (fig ). Expansive dilatation of the esophageal mucosa due to the outward pushing effect of the giant lipoma was observed on the barium-swallowed contrast study. After excision of the tumor, the mucosa of the esophagus, which expanded into the lumen, turned outside and led to a diverticulum (9 × 4 cm) on the right side of the esophageal wall. For confirmation of the diverticulum, a nasogastric catheter was inserted and deployed in the proximal part of diverticulum. The distal part of the diverticulum was obstructed manually; high-pressure air was applied through the catheter, and the diverticulum became more visible. Subsequently, diverticulectomy was added to surgery, and the operation was completed without complications. Gross examination of the mass showed a yellowish, well-circumscribed, encapsulated mass measuring 10 × 5 × 3 cm (fig ). Histopathologic examination revealed lipoma with a collection of matured adipose tissue next to the muscularis propria layer of the esophagus (fig ). The postoperative course was uneventful. The patient was discharged on the 7th postoperative day and was instructed to take her oral medication. |
Patient 2, a 48 years old woman, underwent several vascular surgery interventions: at the age of 26 years vascular prosthesis replacement of the ascending aorta with Bentall technique (composite graft and coronary arteries reimplant) for aortic aneurysm; eight years later, surgery for haematoma consequent to suture dehiscence at the level of the left coronary ostium; at the age of 43 years surgical repair of acute dissection starting from the distal anastomosis of the aortic graft extended to the abdominal aorta; at the age of 44 years, endovascular treatment with stent-graft of the descending aorta for increase of descending thoracic false lumen (Fig ). The patient showed: height 165 cm, velvety and translucent skin, and bilateral venous varicosities (Fig. ). The patient had a total of two pregnancies, the first uncomplicated, the second terminated at about 4 months of gestation because of the presence of the above mentioned aneurysm of the ascending aorta at high dissection risk. She had recurrent wrist dislocations in childhood. Proband's father suddenly died at the age of 33 years for unknown causes. The 26 years old proband's daughter did not present valvular, aortic or other anomalies during several echocardiographic evaluations. Her clinical history included a pituitary adenoma and she suffered from reactive hypoglycaemia and allergic rhinitis. Recurrent left patella dislocations were reported, without other signs of joint hypermobility. She showed mild myopia.\nIn Patient 1, sequencing analysis of genomic DNA disclosed in exon 6 of the TGFBR1 gene the c.1052A>G transition, leading to the substitution of an aspartic acid with a glycine in position 351 of the aminoacidic sequence (p.Asp351Gly) (Fig. ). This novel missense mutation was not detected in the proband's parents, suggesting that it arose as a de novo event. In Patient 2 and in her daughter, TGFBR2 genotyping disclosed the c.1529T>G transversion in exon 7, resulting in the p.Ile510Ser missense mutation (Fig. ). The proband's father was likely affected with LDS, as he suddenly died at a young age. Both missense mutations here identified were not detected in 250 chromosomes of control Italian blood donors, and substituted residues in the kinase domain of the proteins, which is highly conserved in the homologous of the receptor (Fig. ). Furthermore, in silico prediction algorithms, polymorphism phenotyping (PolyPhen) and sorting intolerant from tolerant (SIFT) [], characterized both the p.Asp351Gly and p.Ile510Ser substitutions as damaging and affecting protein function, respectively. |
A 42-year-old, gravida 2, para 0 woman was referred to our hospital at the 11th week of gestation. Before she got pregnant, she had had sterility treatment, but it hadn’t been successful. She subsequently received a laparoscopic adenomyomectomy in another hospital 14 months before referral. The findings in the operation were as follows: The lesion of adenomyosis existed at the posterior wall of the uterus and the lesion and rectum adhered to each other firmly. A vertical incision was made in the middle of the posterior wall of the uterus, and the adenomyosis lesion was enucleated as much as possible (50 g in total). Finally, the uterine wall was repaired with a three-layer suture. One year after the operation, she conceived spontaneously.\nThe patient’s singleton pregnancy was uneventful and she was admitted at the 30th week of gestation for the purpose of bed rest. This was done because past reports suggest that uterine rupture after laparoscopic adenomyomectomy typically occurs around the 30th week of gestation. The patient’s hospital stay was incident-free, and a cesarean section was due to be performed at the 34th week of gestation to achieve optimal trade-off between fetal lung maturation and fetal prematurity. However, the patient’s cesarean section was moved to the 35th week of gestation due to her strong request. On the scheduled date, no notable events occurred until the patient went into the operating room. The patient had undergone combined spinal epidural anesthesia, and was experiencing frequent uterine contractions when a weak pain suddenly occurred. After 13 minutes of uterine contractions, vaginal bleeding was observed while sterilizing the vagina. Cesarean section was performed immediately and a female neonate, weighing 2,283 g, was delivered 31 minutes after uterine contractions had begun. The neonate’s Apgar scores were 7 and 8 at 1 and 5 minutes, respectively. Umbilical arterial gas analysis indicated no acidosis (pH 7.267, base excess −3.4).\nThe rectum and omentum adhered to the posterior uterine wall, where there was a scar from the previous adenomyomectomy. Bleeding was found in the adhered tissue and therefore we peeled off those adhesions. A 1.5 cm hole was found in the uterus (). One area did not have a muscle layer and was covered with only uterine serosa adjacent to the ruptured area. This was repaired with a three-layer suture and total sutured length was 7–8 cm. The patient’s postoperative process proceeded without complications and the patient was discharged at a postoperative date of 12 days. Informed consent was obtained from the patient orally, in order to retain the anonymity of the patient. No ethics approval was required for this study. |
A 56-year-old male with a medical history of polysubstance abuse and hypertension was transferred from an outside hospital to the inpatient burn unit after sustaining traumatic and burn injuries in a firework explosion accident one week prior. Upon admission, he was afebrile and hemodynamically stable (blood pressure 127/82 mm Hg, heart rate 99 bpm, respiratory rate 16/min, and blood oxygen saturation level 96% on room air). His complicated injury complex included first and second-degree burns to the face, right upper, and right lower extremities; right deltoid wound with exposed humerus suggest picture reference of the abdominal wall, leg and shoulder; right lower extremity open wounds status post debridement now with vacuum-assisted closures; foreign objects in the scrotum, left medial thigh, and medial forearm status post scrotal repair with staples; and, a right open tibial-fibular fracture status post open reduction internal fixation (ORIF) with overlying wound and exposed hardware (). He had also undergone an exploratory laparotomy at the outside hospital. As the only culture data from the outside hospital yielded no growth, he was started on broad-spectrum coverage with vancomycin, cefepime, and metronidazole. He had daily hydrotherapy for his partial-thickness burns. On day 2, surgical debridement of his right shoulder and right leg was performed, with right leg muscle necrosis and purulent drainage visualized. Aerobic wound cultures of the right leg grew pan-sensitive V mimicus and Aeromonas hydrophilia, and the antibiotic regimen was narrowed to cefepime only. Vancomycin and metronidazole were discontinued. After wound cultures from a secondary surgical debridement of the right leg again grew V mimicus and now Pseudomonas stutzeri, the patient elected to undergo a right above-knee amputation for source control of acute osteomyelitis and soft tissue necrosis. Subsequent wound cultures from above the right knee amputation site grew Candida albicans. The patient was started on fluconazole as there were operative plans for a right latissimus dorsi flap. He was eventually discharged to a skilled nursing facility on fluconazole and cefepime for six weeks total from when he first cleared his wound cultures. This patient was fitted with a prothesis and made a full recovery with good return of functional performance status post-amputation. |
Our patient is a 3 year-old female who was previously healthy. Four months prior to hospitalization she began experiencing fatigue, recurrent fevers, progressive muscle weakness, and behavioral changes. The week prior to admission, she had decreased urine output, increased abdominal girth and respiratory distress. She was admitted to a community hospital for possible pneumonia. After 3 days, she was transferred to a tertiary center for oncologic assessment. There, her physical examination was concerning for a tender right axillary lymph node and hepatosplenomegaly. A chest radiograph demonstrated diffuse airspace opacities. PCR studies identified both rhinovirus and mycoplasma in nasal pharyngeal swabs. Twenty-four hours after arrival she developed hypoxemic respiratory failure requiring tracheal intubation and mechanical ventilation. This was complicated by an aspiration event and subsequent cardiopulmonary arrest, requiring resuscitation. Multiple subspecialties convened to facilitate an underlying diagnosis. HLH was considered given her hepatosplenomegaly, cytopenias, elevated CRP and normal ESR in the context of documented infection. Table presents key HLH laboratory markers ordered at onset and their results. MAS, possibly associated with sJIA, was also considered despite the absence of arthritis, because of an intermittent, pink, net-like rash in the preceding weeks. Bone marrow evaluation was performed soon after her admission and demonstrated only rare hemophagocytes, similar to those seen in children with an underlying infection [].\nOver the next month our patient continued to be dependent upon mechanical ventilation and developed acute respiratory distress syndrome (ARDS). Other complications included respiratory syncitial virus (RSV) infection and central line-related deep vein thrombosis requiring a six-week course of heparin sulfate. A brain MRI showed diffuse patchy white matter lesions most prominent in the right frontal lobe, suggestive of ischemia. Concern arose for adrenal insufficiency and she received several courses of stress dose corticosteroids with intermittent tapers. Intermittent episodes of fever and worsening cytopenias continued over the next month and HLH, possibly MAS, remained a consideration. For this reason an 18-day trial of anakinra was undertaken. However, fevers continued and abnormalities in liver enzymes along with hematologic abnormalities continued.\nAt this point, genetic testing results became available and identified two mutations in MUNC13-4: a known pathogenic mutation 1389(+1) G > A, a splice donor site of intron 15, and a second previously unidentified mutation, 1847 A > G. This second mutation was located in the splice donor site of exon 20, and the A > G change at this position would likely cause splicing error. It was unclear if this represented a compound heterozygous mutation or if the two variants were on a single chromosome. Parental testing was initiated. Given the genetic results and her persistent symptoms, the laboratory and bone marrow evaluations for HLH were reconsidered.\nInvestigations relevant to HLH before, during and after anakinra use were evaluated (Tables and ). When compared to the initial assessment, there was subsequent marked reduction in natural killer (NK) cell function, CD107a upregulation and an elevation in soluble IL-2 (soluble CD25) receptor. Importantly, a drop in ANC (Figure a) and platelet count (Figure b) was identified during the end of the anakinra trial. Children with active HLH have been noted to have elevated white cell and platelet counts initially that decrease over time [], as illustrated in our case. As a confounding feature, however, cytopenia is an uncommon but known side effect of anakinra. It is thus possible that addition of anakinra in our patient, with underlying MUNC 13–4 mutations, magnified the drop in platelet count and ANC that would have been seen with HLH alone. In addition, when ferritin was measured on the 15th day of anakinra therapy, the level was 629 ng/mL (above the aforementioned acceptable limit of 500 ng/mL), whereas one day after anakinra treatment was terminated, the ferritin level increased to 7129 ng/mL (Figure c). IL-1 is known to increase the synthesis of ferritin subunits in vitro[]. We hypothesize, therefore, that anakinra administration inhibited the surge of ferritin that would have otherwise been identified in association with clinical progression of HLH.\nA repeat bone marrow biopsy and the first and second bone biopsies were compared (Figure and Figure ). The first and second bone marrow biopsies were also critically compared (Figure ). The latter showing a substantive increase in hematophagocytes compared to the former. In addition, a lumbar puncture showed an elevated protein level (109 mg/dL) with a lymphocytic pleocytosis (12 WBC with 100% lymphocytes). These findings suggested central nervous system HLH involvement.\nTable : Initial laboratory values\nTable : Evolution of laboratory values\nFigure : Illustration of key laboratory value evolution\nFigure : Bone marrow aspirate; Wright stain post-anakinra\nFigure : CD 163 immunohistochemical stain of bone marrow biopsy\nWith this collective evidence, the HLH-2004 clinical protocol was initiated []. Our patient was treated with dexamethasone and etoposide. Cyclosporine was not initially utilized because of concern of abnormal kidney and liver function, but was added later as she improved.\nParental genetic testing was performed in order to determine if her case was likely primary HLH with familial genetic etiology. Testing confirmed that each parent carried one of the MUNC 13-4 mutations found in the patient. One parent possessed the novel variant identified while the other carried the known splice site mutation thus defining the patient as a compound UNC13D heterozygote consistent with FHL3.\nOur patient was hospitalized for almost 4 months prior to transfer to a rehabilitation institution. She received a tracheostomy tube prior to discharge because of her inability to be weaned from respiratory support. She developed mild hypertension which was attributed to cyclosporine. Her liver enzymes continue to decrease although she has a persistently enlarged liver and spleen. She has not had any recent fevers and has an age-appropriate neurologic exam. However, cognitive limitations cannot be predicted. An unrelated HLA-matched donor has been identified and she will be proceeding to bone marrow transplant. |
A 64-year-old male with a 92-pack/year smoking history was admitted to the emergency department of the institution with sudden shortness of breath. Chest X-ray and computed tomography on admission revealed the presence of massive left pleural effusion and a large tumor (diameter: 13 cm) occupying the patient’s left thoracic cavity (). Pleural effusion cytology and a bronchofiberscopic study failed to show any evidence of disease. Under local anesthesia, percutaneous needle biopsy performed through the left thoracic wall revealed the presence of a sarcomatous lesion (Fig. ) and positive immunohistochemical staining for CD56 and desmin. Based on these findings, the diagnosis of MTT was considered. There was no evidence of distant metastases.\nThe patient underwent surgical removal of the tumor approximately 1 month after admission to the hospital. Conventional thoracotomy was performed at the left fifth left intercostal space, with the patient in the right decubitus position. The tumor occupying the left hemithorax was gelatinous soft and had arisen from the anterior thoracic wall around the second left intercostal space. Moreover, numerous pleural disseminated nodules were identified throughout the left thoracic cavity. A subtotal tumor resection with extrapleural pneumonectomy (without diaphragmatic pleura) was performed due to the invasion of the tumor into the left lung. Broad chest wall removal was deemed excessively aggressive. Thus, the tumor of the anterior chest wall was not removed, and postsurgical radiotherapy was planned. Following tumor removal, the left thoracic cavity was irrigated with distilled water containing cisplatin (100 mg in total). The total operation time was 280 minutes, and the total bleeding volume was approximately 1640 ml.\nMacroscopically, the resected tumor was 14 × 8.5 cm in size, solid and gelatinous soft with broad necrosis. Moreover, it had widely invaded the subpleural fat tissue and left upper lobe parenchyma (Fig. ). Microscopically, short spindle tumor cells embedded in the myxoid stroma showed patternless proliferation, with tumor-free margins displaying broad necrosis. Immunohistochemical examination of tumor cells was positive for vimentin, CD56, CD57, desmin and myoglobin. In contrast, it was negative for pancytokeratin, epithelioid membrane antigen, cytokeratin 5/6, cytokeratin 7, thyroid transcription factor 1, S-100, neuron-specific enolase, CD34, c-KIT, bcl-2, calretinin, alpha-smooth muscle actin, muscle-specific actin, h-caldesmon and alpha-fetoprotein (Fig. ). The diagnosis of MTT was confirmed based on the evidence of bidirectional differentiation into neurogenic and myogenic components.\nThe initial postoperative clinical course was unremarkable. However, hypoxemia deteriorated 12 days after operation, and chest computed tomography revealed the presence of fibrotic shadows in the right lung and multiple nodules in the left thoracic cavity. Lymphangitis and pleuritis carcinomatosa were suspected, suggesting early recurrence of disease. Administration of steroidal pulse therapy and intravenous cyclophosphamide was ineffective. Acute respiratory failure progressed, and the patient expired 20 days after surgery. |
A 25-year-old Caucasian woman was initially diagnosed with invasive moderately differentiated squamous cell carcinoma of the tongue at the age of 23. She was treated with hemi-glossectomy with clear margins and had no involvement of cervical lymph nodes. Sixteen months later, the tumour recurred in the oropharynx and the left upper lobe of the lung. She was treated with a regimen containing carboplatin, paclitaxel, and cetuximab. The tumour progressed to submandibular lymph nodes, soft tissues, and lumbar vertebral body. She was initiated on immune checkpoint inhibitor pembrolizumab and radiation therapy.\nA detailed social and family history was taken in an attempt to understand the aggressiveness of her cancer. She worked in a private company involved in the environmental cleaning of lakes and ponds and was exposed to a significant amount of chemicals, including herbicides and pesticides. She has a scant history of smoking: half a pack per day for roughly 1.5 years. She drank alcohol socially on the weekends. Her maternal grandmother died from brain cancer. There was no other family history of cancer. Genetic testing was not performed.\nWithin 1 month of starting radiation therapy, she presented with progressively worsening severe, sharp, constant pain of the right shoulder and chest associated with right arm numbness, cough productive of clear sputum, and dyspnoea on exertion. Pain was exacerbated by right arm movement. She denied any trauma, travel, or oedema of the arm.\nOn examination, she appeared frail with evidence of weight loss. She had a heart rate of 86 b.p.m. and blood pressure of 121/74. On auscultation, coarse rales and rhonchi were present in the left lower lung field. Cardiac exam revealed a regular rhythm with no murmurs, gallops, or rubs. She had tender right supraclavicular lymphadenopathy. Additionally, she had swelling in the right suprascapular region and erythema of the overlying skin. Admission ECG showed ST elevation in the inferior leads and T wave inversions in the anterolateral leads (). Blood work was significant for an elevated troponin I, which peaked at 0.29 ng/L (normal <0.05 ng/L). The rest of her labs were unremarkable except for Hb 9.2 g/dL (normal 12–16 g/dL), Hct 28.8% (normal 35–47%), total calcium 10.5 mg/dL (normal 8.9–10.4 mg/dL), and ionized calcium 1.51 mmol/L (normal 1.14–1.33 mmol/L). Subsequent ECGs continued to demonstrate ST-segment elevation without resolution. A chest radiograph revealed a retrocardiac opacity concerning for left lower lobe pneumonia, worsening of a known left upper lobe cavitary lesion, and a new right clavicular lesion. Differential diagnosis at this point included myocarditis due to pembrolizumab, acute coronary syndrome (ACS), and Takotsubo cardiomyopathy in a cancer patient.\nA transthoracic echocardiogram (TTE) was performed, and it revealed focal areas of myocardial thickening within the basal to mid inferior and inferoseptal myocardium with associated hypokinesis ( and , Video S1). In addition, there was a highly mobile pedunculated echodensity, measuring 0.6 cm × 0.6 cm, which was adherent to and protruding from a region of inferior wall myocardial thickening (, Video S1). There was thickening of the right ventricular free wall as well. These findings were concerning for metastatic tumour implants within the myocardium with a mobile component within the left ventricular cavity, concerning for thrombus. There was also pericardial thickening and a small pericardial effusion. The TTE findings suggested that the ECG changes were likely secondary to infiltrative cardiac metastases, and as such, ACS was not felt to be the diagnosis. The chest pain was managed with analgesia.\nTo further characterize the lesions, cardiac magnetic resonance imaging (MRI) was performed. Cardiac MRI revealed infiltrative masses within four separate regions of the left and right ventricular myocardium (). These masses demonstrated increased T2 signal and heterogeneous enhancement on perfusion and delayed enhancement sequences (, ). The areas of infiltrative masses within the myocardium had associated hypokinesis, particularly notable in the inferior wall of the left ventricle. There was a 0.9 × 0.8 cm focus of intraventricular thrombus adherent to the inferior wall.\nFurther imaging with computed tomography (CT) scan revealed multilobulated, heterogeneous enhancement within the right trapezius muscle, indicative of intramuscular metastatic disease, which likely explained her right shoulder pain. There were lytic lesions within the right clavicle. Metastatic lesions were present in bilateral lung apices. There was interval development of a centrally necrotic 3.7 cm left adrenal mass, indicative of adrenal metastatic disease, which was not present on prior CT scan dated 4 months prior. After treatment of pneumonia and hypercalcaemia, she was referred to home hospice given her poor prognosis. |
A 37-year-old HIV-infected woman presented to pulmonary clinic in early 2016 for evaluation of years of persistent cough and dyspnea. Her relevant medical history included a prolonged hospitalization in 2010. At that time, she was not on ART and her absolute CD4 lymphocyte count was 6/mm3. She was hospitalized for evaluation of dyspnea and chest pain; she was found to have bilateral pneumothoraces. She was intubated for respiratory failure and had chest tubes placed. She was found to have Pneumocystis jirovecii pneumonia. She required mechanical ventilation for a total of 3 months.\nDuring her hospitalization, ART was initiated and an undetectable viral load was achieved. Although she subsequently was found to have viremia owing first to resistance mutations and later to medication nonadherence, she has maintained an undetectable HIV viral load since 2013. Her most recent CD4 lymphocyte count is 588/mm3. Her current ART program includes etravirine, dolutegravir, and ritonavir-boosted darunavir.\nSix months after her prolonged hospitalization in 2010, she had a pulmonary function test that showed no obstruction and significant restriction (total lung capacity was 48.5% of predicted). She was using inhaled beclomethasone twice daily and an albuterol inhaler as needed at the time of testing. Upon her evaluation in the pulmonary clinic in 2016, she complained of a frequent nonproductive cough that had been persistent since her illness in 2010, and although she functionally recovered after her critical illness, she continued to have significant dyspnea on exertion. She described shortness of breath walking 1 city block or climbing 1 flight of stairs. She had occasional wheezing and reported triggers included strong odors, seasonal changes, and her current living space. After her critical illness in 2010, she moved into a rental property, which had to be renovated twice due to mold, and then in 2014 she moved to another property, in which water damage occurred from a leaking air conditioner. The carpet was taken up and dried but ultimately remained in the home. At that time, she began to feel her pulmonary symptoms worsen in regards to coughing frequency and endurance limitations; these symptoms progressed through the time of her initial pulmonary clinic appointment. She denied orthopnea or lower extremity edema. She was a life-long nonsmoker and did not use illicit drugs.\nOn examination, she was in no acute distress, and she was obese (body mass index of 35.4 kg/m2) with oxygen saturation 100% on pulse oximetry at room air with no desaturation on ambulation. On percussion, she had small lung volumes, inspiratory rhonchi over the left upper lung field, but otherwise was clear to auscultation without wheezing. There was bilateral axillary scarring from prior chest tube insertions, with keloid formation. Her cardiac and abdominal exams were unremarkable. She had no lower extremity edema or clubbing.\nComputed tomography of the chest without contrast (Figure ) revealed fibrotic changes predominantly in the upper lobes (left greater than right) and upper lobe ground-glass opacities. There was varicoid and cystic bronchiectasis associated with the fibrosis in the left upper lobe, right upper lobe, right middle lobe, and lingula. Calcified pleural plaques were present along bilateral hemidiaphragms. There were calcified left hilar lymph nodes and a normal cardiac examination.\nUpdated pulmonary function tests obtained in 2016 showed significantly improved restriction (total lung capacity of 64.6% predicted), although concomitant obstruction was now seen (forced expiratory volume at 1 second [FEV1] was 58.5% predicted). Complete blood count revealed mild eosinophilia of 320/mm3. We were able to review laboratory testing from 2010, which included an absolute eosinophil count of 640/mm3, before therapy with inhaled corticosteroid.\nOut of concern for a diagnosis of ABPA, total serum immunoglobulin (Ig)E and Aspergillus-specific IgE were sent. Both were found to be markedly elevated (Table ), supporting a diagnosis of ABPA. In addition, given her identifiable triggers that correlated with dyspneic episodes, we ran an allergy (Rast Northeast) panel, which reported elevated IgE levels to allergens that included A fumigatus, cat, dog, and environmental stimuli (eg, birch, oak). Given the patient's clinical history, positive mold exposure, radiographic findings, and laboratory data, a diagnosis of ABPA was suspected. The patient was started on prednisone at 0.2 mg/kg per day, which is lower than recommended for initiation for ABPA, but given the patient's HIV infection and potential drug-drug interactions, this dose was decided on in a multidisciplinary discussion. Itraconazole 200 mg daily was also started and was then adjusted based on levels.\nThe patient returned after 4 months on the aforementioned regimen with significantly improved pulmonary symptoms: no coughing or wheezing, improvement in her endurance, and less usage of her as-needed inhalers. She had a new set of pulmonary function testing that revealed improvement in her FEV1 (60.2%), total lung capacity (69.6%), and diffusion capacity (65%; increased from 56%). Furthermore, her IgE levels had decreased from 2020 to 903 kU/L and her A fumigatus-specific IgE levels went from 6.6 to 3.01 kU/L. |
We present a case of a 21-year-old male who presented to the emergency department complaining of neck pain. The pain had started the night before presentation, a few hours after lifting heavy objects. The pain was in the anterior neck and upper chest, stabbing in nature, continuous, increases when moving the head, lying flat, and with deep breathing. The patient had no history of trauma, asthma, odynophagia, or dysphagia. He does not have any comorbidities or previous surgeries. Patient mentioned history of occasional hookah smoking.\nOn examination the patient was vitally stable. He looked well, sitting comfortably on emergency room bed without shortness of breath, or apparent facial or neck swelling. Oxygen saturation was maintained at 99% on room air. Neck examination showed central trachea, tenderness in anterior neck, and crepitation under the skin. No palpable masses, lymph nodes, or thyroid tissue. Endoscopy was performed to the nasal cavity, nasopharynx, oropharynx, and larynx, which showed no airway narrowing or edema. Chest and cardiovascular examination was unremarkable except for tenderness in the anterior chest wall.\nBlood investigations, including complete blood count and renal and liver profiles, showed results within normal limits. Computed tomography (CT) scan of the neck and chest with contrast showed diffuse subcutaneous, soft tissue, and intermuscular head and neck emphysema surrounding the visceral, carotid, retropharyngeal, and posterior cervical spaces reaching down to the anterior mediastinum causing mild pneumomediastinum. There is no pneumothorax, tracheal, laryngeal, or esophageal injury, as shown in ().\nThe patient was admitted to the ward for observation, monitoring of vital signs, analgesia, and prophylactic antibiotics. The patient was kept under observation for 8 days. During that time, neck pain improved gradually with no episodes of oxygen desaturation or vital signs deterioration. The patient was later discharged with very mild persistent pain. Two weeks after discharge, the patient was seen in outpatient clinic and was free of symptoms. Lateral neck and posteroanterior chest X-rays at presentation and 2 weeks later are shown in Figures and . |
We report on a 4 years and 6 months old Ethiopian boy who presented to Tikur Anbessa Specialized Hospital, Addis Ababa, with a recurrence of cough and fast breathing of five days. His respiratory complaints had been waxing and waning over the past few months and was at presentation on his 5th month of anti-tuberculous treatment which was not relieving his illness. He also had low grade fever, grunting, poor appetite and failure to thrive. His past medical history showed repeated treatment for pneumonia and otitis media. He also had epistaxis and occasional passage of stool mixed with bright red blood since infancy. He was on follow-up for generalized itchy skin lesions since 7 months of age. He was treated at age 2 years for pulmonary tuberculosis and declared cured.\nHe was the third child for his family, a product of non-consanguineous parents. His immediate older brother had died at age 2 years of similar illness. He has an apparently healthy older sister. All maternal brothers had died in childhood due to recurrent severe infections. There was no history of delayed separation of his umbilical cord. He received all ageappropriate vaccines with no post-vaccine adverse reactions reported. Despite not being vaccinated against meningococcal infections, he had record of being treated for neither meningococcemia nor meningitis.\nHe appeared chronically sick with extensive skin lesions but no dysmorphia. His pulse rate was 108/minute, respiratory rate 52/minute, temperature 36.4oC and saturation of oxygen in room air of 82%. He was underweight (weight = 12 kg). He had profuse purulent discharge from his right ear with an excoriated right pinna. He had no oral thrush. He had submandibular lymphadenopathy and hepatomegaly of 12 cm. He had crusted lesions on the scalp and face () and hypopigmented lesions on trunk and extremities (). He also hadumblicated small nodules over face and extremities and clubbing.\nHis total white blood cell was 6800/mm3 (normal for age: 4000 – 12000/mm3). His absolute neutrophil count was 3700/mm3, absolute lymphocyte count was 2300/mm3, absolute eosinophil count was 14/mm3.\nHis hemoglobin was 10.2 g/dl, mean corpuscular volume 81.4 fl, platelets 9000/mm3 (normal: 150,000 – 400,000/mm3) and mean platelet volume 8.5 fl (normal 9.4 – 12.3 fl).\nThe peripheral smear showed thrombocytopenia with no inclusions in lymphocytes, neutrophils or platelets. His chest X-ray showed bilateral reticulonodular infiltrates and no thymic aplasia. A GeneXpert molecular test from his gastric aspirate was negative for Mycobacterium tuberculosis. His abdominal ultrasound showed hepatomegaly with no focal lesions. His HIV serologic test was negative. His short hospital stay could not enable work-up for autoimmune disorders.\nA clinical diagnosis of WAS was made based on his gender, early death of male family members, recurrent pulmonary and ear infections, bleeding tendencies, eczema, thrombocytopenia, small platelets, normal lymphocyte and neutrophil counts. Despite parenteral and otic antibiotics and platelet transfusions, the platelet count remained below 20,000/mm3 and he succumbed due to his illness. Prophylactic Trimethoprim-Sulfamethoxazole and Acyclovir had been initiated. Intravenous immunoglobulins were not available in the hospital formulary. Hematopoietic cell transplantation (definitive treatment) is not performed in Ethiopia. |
A 36-year-old previously healthy man presented with abrupt flaccid paralysis of the lower limbs immediately following a traffic accident. Examination showed profound weakness of both legs (grade 1) combined with severe lancinating paresthesia at the posterior surface of the right leg. His left leg weakness spontaneously improved to grade IV within a few hours after injury. Deep tendon reflexes were absent. Bladder dysfunction was noted and catheterization was required. Pathologic reflexes, such as Babinski sign and ankle clonus, were absent. To treat apparent spinal cord injury or cauda equina syndrome, the patient was administered a high-dose steroid. However, a few days later, the patient complained of mild weakness and tremor in both upper extremities without improvement of right leg weakness.\nWhole spine magnetic resonance imaging (MRI) demonstrated mild central intervertebral disc herniation at L5/S1 that was of little clinical significance (). Otherwise, there was no significant pathology, including compressive lesion, cord contusion, or hemorrhage. Brain MRI also showed no significant abnormality. EMG/NCS was conducted 7 days after the accident, which did not demonstrate any abnormal findings. However, 3 weeks after the accident, a follow-up EMG/NCS was conducted. In the motor NCS, terminal latency was increased and conduction velocity was decreased in both upper and lower extremities. Sensory NCS showed decreased conduction velocity in both upper and lower extremities. There was increased F-wave latency in all 4 extremities. The assessment showed signs of demyelinating sensorimotor polyneuropathy. Furthermore, CSF assessment showed a mild elevation of protein levels, consistent with inflammatory demyelinating polyneuropathy and leading to a final diagnosis of GBS. The patient was transferred to the department of neurology, where he was treated with administration of IVIG.\nThree months after the accident, symptoms showed gradual improvement and the patient could walk stably with a cane. Bladder dysfunction had also improved. At the final follow-up, 5 months after the accident, the patient’s condition was stable. Further EMG/NCS still showed demyelinating sensorimotor polyneuropathy without significant changes since previous assessments. |
An 83-year-old female was referred to our hospital with a mass in the middle of the epigastrium for 10 days. The patient also complained of epigastric discomfort for the past 6 months. Her symptoms did not resolve even after taking some medicine prescribed in the clinic. She did not have a history of gastropathy and affirmed that she had been wearing dentures for many years.\nPhysical examinations found that the skin of the upper abdomen was red and swollen, and there was an apparent local tenderness with an inflammatory mass. The size of the skin protrusion measured approximately 4 × 5 cm, and there was no evidence of a surrounding scar or ulcer. There was no rebound pain or muscle tension in the remaining area of the abdomen. The patient had an average body temperature of 37.5°C, with an increased white blood cell count (18.12 x× 109/L [reference value 3.5–9.5 × 109/L]) on laboratory examination.\nAn ultrasound (US) examination of the abdomen revealed a 4 cm strip-like hyperechoic object entangled in the muscles of the abdominal wall, which was 2.5 cm away from the surface of the skin. The hyperechoic object penetrated the muscle layer and was partially located in the abdominal cavity, while the surrounding soft tissues were also hypoechoic (Fig. ). In the US finding, the atypical stripe echo in the abdominal wall was considered a foreign body.\nA computed tomography (CT) scan revealed a flaky, soft tissue density shadow between the anterior abdominal wall and subcutaneous fat, and a thin strip of bone-like hyperdense shadow. The fatty space in the lesser curvature of the anterior abdominal wall was turbid, with continually increasing density and high-density shadows. The CT results confirmed the presence of a foreign body entangled between the abdominal cavity and the abdominal wall that was causing infection (Fig. ).\nThe patient underwent a gastric foreign body removal by partial gastrectomy under general anesthesia. Intraoperative findings showed a sharp fishbone protruding from the lesser curvature of the stomach into the abdominal cavity, while a part of the bone remained in the gastric cavity (Fig. ). The foreign body measuring approximately 4.2 cm was removed. The sinus tract in the gastric wall was cleared and closed. The postoperative pathology of the tissue from the gastric wall revealed chronic suppurative inflammation with abscess and sinus canal formation. The foreign body was surrounded by proliferating granular and fibrous tissues with local bleeding (Fig. ). The patient received anti-inflammatory treatment following the surgery, and the symptoms of discomfort in the upper abdomen improved rapidly. The patient was discharged from the hospital 9 days after the surgery.\nAt the 1-month postoperative follow-up, the patient showed no discomfort in the upper abdomen, and the inflammatory mass of the abdominal wall was no longer present. |
A 65-year-old woman presented with a self-palpated small lump in the left breast. She already underwent three breast lumpectomies in another hospital for benign lesions; two of the left breast and one of the right breast. She did not take any medications and had no family history.\nClinical examination confirmed the presence of a small nodule in the left breast, situated medially at 9 o’clock. Mammography and echography revealed a spherical, homogenous, non-cystic, well-defined mass of 19 × 14 × 19 mm. The lesion was situated close to the pectoralis major muscle and was suspicious for muscle invasion. Doppler ultrasonography revealed an important perilesional and peripheral vascularization. Several enlarged ipsilateral axillary lymph nodes were noticed.\nCore needle biopsies (CNBs) were taken from one enlarged axillary lymph node and from the breast lump. The latter showed a spindle cell lesion with a sharply defined round border that separated the lesion from the surrounding fat tissue. At higher magnification, a variable cellularity was observed but no infiltrative permeation in the surrounding tissue was noticed. Both poorly and highly cellular areas consisted of bland-looking spindle cells. While the poorly cellular areas were characterized by a dense compact collagenous stroma, the more crowded areas showed haphazardly arranged cells embedded in loose connective tissue with myxoid appearance (Fig. a). Mitotic figures were absent. The CNB taken from the lymph node showed no malignancy.\nBecause of the discrepancy between the radiological findings (suggestive for an infiltrative malignancy) and the pathological findings (consistent with a bland-looking, sharply delineated spindle cell lesion), a complete local resection was recommended for definitive diagnosis.\nAt gross examination, the excised lesion presented as a sharply delineated nodule with homogenous white color, hard consistency and regular borders. The nodule had a diameter of 19 mm and was completely resected with a free margin of 8 mm. The lesion was surrounded by adipose tissue; no muscular tissue was resected during the surgical procedure.\nIn addition to the morphological features observed in the CNB, we noticed the presence of small clusters of epithelioid cells admixed with the spindle cells in the highly cellular fields of the resection specimen. The epithelioid cells contained oval nuclei with vesicular aspect, slightly irregular borders and often a prominent nucleolus. Occasional mitotic figures were present, as opposed to the CNB (Fig. b). Focally, despite a mainly sharply delineated margin, we also noticed small areas of invasion in the surrounding adipose tissue at the periphery of the lesion (Fig. c, d). In the areas of peripheral invasion, several entrapped ducts could be found, often surrounded by scattered lymphocytes. A distinctive central scar-like sclerotic zone admixed with bland-looking ducts was noticed as well (Fig. e). We considered the central scar-like sclerotic zone as a pre-existing sclerotic lesion. The spindle cell lesion was completely resected, albeit with a minimal margin of less than 1 mm.\nBased on these morphological features, we considered a broad differential diagnosis including DF, adenomyoepithelioma (AME), inflammatory myofibroblastic tumor (IMT), myofibroblastoma, pseudoangiomatous stromal hyperplasia (PASH) and LG-FLMC. A broad panel of immunohistochemical stains was performed to narrow this differential diagnosis.\nThe spindle cells showed a diffuse immunoreactivity for alpha smooth muscle actin (α-SMA) (Fig. a), p63 (Fig. b), and cytokeratin AE1/AE3 (Fig. c). Focal immunoreactivity for S100, desmin and caldesmon was also noticed. There was no immunoreactivity for estrogen receptor (ER), progesterone receptor (PR), human epidermal growth factor receptor 2 (HER2), CD34, B-cell lymphoma 2 (Bcl-2), CD10 and anaplastic lymphoma kinase (ALK). β-catenin immunostaining showed focal cytoplasmatic staining, but no nuclear expression (Fig. d). The Ki67 immunostaining revealed a labeling index of about 2%, on average.\nBecause of the lack of expression for ALK, CD34 and ER and because of the lack of nuclear β-catenin expression, we excluded the diagnosis of IMT, myofibroblastoma, PASH and DF. Given the spindle cell morphology, the presence of rare epithelioid cells, the mitotic activity, the clear co-immunoreactivity for CK AE1/AE3 and p63 and the triple-negativity for ER, PR and HER2, the differential diagnosis was limited to a borderline malignant ER-negative AME with myoepithelial overgrowth and LG-FLMC. Notably, a sharply delineated border and association with a centrally sclerotic region may be observed both in AME and LG-FLMC [, ].\nThe further characterization of this lesion included mutational analysis of 97 cancer related genes (Table ) with capture-based targeted next generation sequencing method in search of recurrent mutations in the HRAS and/or PIK3CA gene that could have helped in the differential diagnosis [, –]. The following pathogenic variants were detected: GNAS c.601C > T p.(Arg201Cys) with variant allele frequency (VAF) 22%, TERT-promotor c.-124C > T p.? with VAF 20%, PIK3R1 c.1365_1367del p.(Gln455_Phe456delinsHis) with VAF 21% and PIK3R1 c.2088dup p.(His697Thrfs*44) with VAF 22%. No mutations were found in HRAS and PIK3CA.\nEventually we favored the diagnosis of LG-FLMC based on the morphological and immunohistochemical findings.\nBecause of the negative resection margins (albeit with a margin less than 1 mm), the favorable prognosis of this entity and the lack of proof of usefulness, no adjuvant therapy was given. After 2 years of clinicoradiological follow-up, the patient was still free of disease. |
A 57-year-old male patient was admitted to our hand surgery department complaining of a progressive swelling located on the dorsoulnar side of the right wrist joint. The patient reported that the mass was started to grow for the past three months. On physical examination, there was no tenderness or erythema around the mass. The range of motion of the wrist joint was within the normal range. Neurovascular examination findings were normal. The patient was not diagnosed with any chronic disease previously, including gout or not under any medication. He had a history of an occupational injury 33 years ago in which he lost extensor muscles and tendons on the right forearm. Thus, the patient underwent a tendon transfer surgery in which the FCU tendon was transferred to EDC tendon (Figure 1). The patient’s family history was unremarkable for gout.\nMagnetic resonance imaging (MRI) revealed an iso/hypointense mass at proton density-weighted and fat-suppressed T1 images (Figure 2). We decided to perform excisional biopsy. A written informed consent was obtained from the patient. During surgery, it was confirmed that the FCU tendon was transferred to EDC tendon. The tumor was located within the junction where the FCU tendon was transferred to EDC tendon. Non-absorbable suture materials were observed within the tendon transferred region (Figure 3). A caseous material was reached following the opening of the paratenon suggestive of tendon necrosis, tuberculosis, or a foreign body reaction. An excisional biopsy was performed, and the specimens were sent for histopathological and microbiological evaluation. The weakened tendon was repaired with eight-strand and epitendinous sutures.\nNo organism was seen in the initial Gram staining, and there was no bacterial growth in the microbiology cultures. His preoperative blood laboratory test results were within normal ranges for complete blood count, complete metabolic panel, and uric acid levels. Microscopic examination revealed typical morphology of a gouty tophus. A granulomatous inflammation consisted of a fluffy cotton candy-like material and surrounding foreign body giant cells. At the periphery, fibrotic synovium was visible (Figure 4). Since urate crystals are dissolved in formalin fixation, microscopic examination under polarized microscope failed to demonstrate urate crystals. The patient was consulted with rheumatology department and antigout therapy was initiated. The patient's wound was healed without any complication. There was no recurrence at 18 months of follow-up. |
A 38-year-old lady who has no past medical history of chronic illness apart from a congenital heart defect that was discovered incidentally during her childhood, she was told it is a small defect and does not require any further treatment or follow up (no physical documents or medical reports were available).\nThe patient presented to the hospital with 3-month history of severe low back pain. MRI revealed L4/5 and L5/S1 left lateral disc protrusions with mild impingement on the left L5 and S1 nerve roots (). Patient underwent CT guided lumbar epidural injection with 80 mg methylprednisolone and 2 mL of 0.5 % marcaine. No immediate complications were encountered during or after the procedure.\nThree weeks post procedure, patient presented to ED with 1-day history of chest pain and shortness of breath. She described the pain as progressive non-radiating left sided chest pain, dull in nature, constant and 5/10 in severity. There were no upper respiratory tract symptoms. Patient reported that she still has residual low back pain but less severe than it was before the procedure. On physical exam, patient was found to have fever of 38.3 c but otherwise she was hemodynamically stable with respiratory rate of 21 breath per minute and O2 saturation of 97 % on ambient air. The residual of her physical exam including cardiac exam was unremarkable. EKG showed normal sinus rhythm with no ST segment or T wave abnormalities. Lab tests were remarkable for high d-dimer (5.19 mg/L), other routine lab tests including WBC and Trop T were normal. Pulmonary embolism was suspected, so CT pulmonary angiogram (CTPA) was done and showed no evidence of pulmonary embolism, however, it showed small patchy consolidations involving multiple segments of the lungs on both sides. The patient was admitted with an impression of pneumonia and to rule out COVID-19 infection given her CT Findings, she received antibiotic treatment with cefuroxime and clarithromycin, COVID-19 PCR was negative, no blood cultures were sent, she was discharged home within few days after her symptoms improved.\nTwo weeks later, patient presented again to ED with 3-day history of fever, dry cough, fatigue and generalized body ache. On physical exam; blood pressure was 91/58 mmHg, pulse rate was 97 beat per minute, temperature was 38.1c, respiratory rate was 20 breath per minutes, O2 saturation 96 % on ambient air. Chest was clear on examination and cardiac exam did not reveal any murmur. Lab tests showed WBC 10.3 × 103/uL, neutrophils: 76 %, C-reactive protein of 150 mg/L (normal < 5), her kidney function, liver function and serum electrolytes were normal. Subsequent lab tests for Brucella, COVID-19 PCR, HIV, Hepatitis B and C came back as negative. Chest X-ray showed small patchy opacity in the left upper zone. The patient was admitted again with an impression of community acquired pneumonia and she was started empirically on ceftriaxone and azithromycin. blood cultures were sent. Initial gram stain for blood cultures showed Gram positive cocci in cluster, subsequently vancomycin was added to her regimen. Later on, species identification reported coagulase negative staphylococcus. Final blood culture report showed Staphylococcus Lugdunesis growth, which was sensitive to Teicoplanin, Vancomycin, linezolid, Rifampicin, Trimethoprim/sulfamethoxazole and Clindamycin and resistant to cefazolin, cloxacillin and gentamycin.\nTransthoracic echocardiography was done to rule out IE given that she has bacteremia with virulent organism, it showed elongated chaotic highly mobile echo density attached to native tricuspid valve with mild tricuspid valve regurgitation. (). Transesophageal echocardiography showed vegetation mass attached to entire length of anterior tricuspid leaflet which is highly mobile with size of 1.5 *1.8 cm (). No defects were noticed in both echocardiograms. She was diagnosed as IE based on modified Dukes criteria, meeting one major criteria; positive echocardiogram, and three minor criteria: fever, positive blood cultures that do not meet major criteria and possible septic pulmonary emboli as CT chest showed bilateral small patchy consolidations.\nGiven the recent lumbar epidural injection with the current bacteremia and non-resolving back pian, a local infectious complication was suspected, lumbosacral spine MRI was done and showed L5-S1 spondylodiscitis with inflammatory right posterior paraspinal myositis ().\nPatient continued to spike fever and repeated blood cultures showed persistent bacteremia despite being on optimum antimicrobial coverage with teicoplanin and rifampicin for about one week. Eventually, she was referred for urgent surgical management. Cardiothoracic surgery was carried out through conventional median sternotomy incision, right atrium was opened longitudinally then the area was inspected, A large shaggy vegetation was found attached to the anterior leaflet of tricuspid valve, tricuspid leaflets were intact. A well-defined defect was identified between the right atrium and left ventricle at the level of anterior-septal commissure of the tricuspid valve presenting congenital Gerbode defect, its location was confirmed with intraoperative TEE. Vegetation was removed from the anterior leaflet and the Gerbode defect was closed by prolene suture placed underneath adjacent parts of anterior and septal leaflets. Tissue culture of the vegetation showed S. Lugdunesis growth.\nPost Operation, patient had recovered gradually, and subsequent blood culture showed clearance of bacteremia. she was kept on IV teicoplanin and rifampicin (since the primary source most likely is related to bone and soft tissue infection) for 4 weeks. Her back pain resolved, and she was discharged home on oral Co-trimoxazole and rifampicin for another 2 weeks to complete total 6 weeks of antibiotics. |
A 78-year-old woman was referred to our hospital after being diagnosed with a pancreatic tumor in a routine abdominal ultrasound exam. The patient was asymptomatic and did not report losing weight nor any other complaints. Her previous medical history was unremarkable.\nDuring the physical exam, the patient seemed well nourished, vital signs were within the normal range, and she was without masses in the abdominal exam. Routine blood laboratory results were within normal ranges. The referral ultrasound result reported a nodular hypoechogenic lesion with 4 × 3 × 3.3 cm in the pancreas tail, suspicious of an expansive process. An abdominal computed tomography (CT) revealed a globular shaped pancreatic body and a loss of cleavage plane between the splenic vein and the posterior face of the pancreas. For a better characterization, a pancreatic magnetic resonance imaging (MRI) was performed, showing a nodular lesion of the pancreatic body with 2.8 × 2.5 cm causing pancreatic tail atrophy and distal dilation of Wirsung's duct (Fig. ). These findings were mostly compatible with a neoplastic lesion. A thoracic CT was performed and was negative for metastatic pulmonary lesions.\nThe patient's clinical case was discussed in a multidisciplinary meeting, and she was proposed for a surgical procedure with the diagnosis of pancreatic neoplasia clinically staged as T2 N0 M0 (Stage IB – AJCC 8th edition) [].\nShe was submitted to a left subcostal laparotomy. During the procedure, a hard, bulky mass located in the transition of the pancreatic head/body was found. Intraoperative ultrasound showed close contact between the lesion and the portal vein (Fig. ), confirming local invasion after dissection. The performance of a tru-cut biopsy of the lesion and peri-regional lymphadenectomy (celiac trunk, interaortocaval, retropancreatic, and left renal artery lymph nodes) were decided on. There were no complications during or after the procedure, and the patient was discharged home on the ninth postoperative day.\nImmunological and histological analysis found a dense and diffuse inflammatory infiltrate with mononuclear cells (Fig. ) as well as some plasmocytes and fibrosis (Fig. ). There was positive marking for cytokeratins AE1/AE3 on a regular glandular pattern and highlighting of the lobulocentric pattern (Fig. ). Another cut showed dense plasmocyte (CD138 positive) infiltrates (Fig. ). Immunoglobulin (Ig)G4/IgG ratio was less than 40% of the plasmocytes. Positivity for CD34 was found only on vessels (Fig. ), excluding a solitary fibrous tumor. The expression for DOG-1 and CD34 was negative; also, anaplastic lymphoma kinase gene (ALK) marking was absent, excluding a myofibroblastic tumor (Fig. ).\nThe histological result was compatible with an inflammatory pseudotumor, without any signs of malign epithelial neoplasm, nor criteria for IgG4-related disease. The lymph nodes were also negative.\nTwo weeks after surgery, the patient was admitted to the emergency department complaining of vomiting and abdominal pain, exacerbated after meals. She underwent upper gastrointestinal endoscopy which showed a substantial gastric distention, with the stomach filled with stasis residue. Also, a marked decrement in peristalsis was noted, particularly on the gastric antrum. A barium-based contrast tract radiography was performed and confirmed the diagnosis of gastroparesis. A gastrojejunal anastomosis was performed and the patient fully recovered of the symptoms.\nShe was evaluated on a gastroenterology consultation, and postoperative PET/CT and MRI were scheduled. MRI showed an absence of the pancreatic mass, with a total resolution of pancreatic atrophy, and PET/CT also showed no sign of abdominal disease (Fig. ). Her blood IgG4 levels were 405.7 (normal range values of 39.2–864.0). To date, the patient has been well and without any symptoms of the disease. |
A 77-year-old female with hypertension, untreated hyperlipidemia, hypothyroidism, but without prior history of CAD or angina symptoms was referred to a cardiologist's office for a treadmill exercise test secondary to new onset palpitations. She denied any chest pain or pressure, shortness of breath, exertional dyspnea, or leg swelling. She quit smoking 36 years ago and has no family history of early cardiovascular diseases. She has a very distant cardiac work-up years ago, including a stress test and an echocardiogram, which the patient reported were unremarkable. Vital signs prior to the test were a blood pressure of 140/78, heart rate of 80, and a respiratory rate of 14. Physical exam was unremarkable except for a systolic ejection murmur that was graded II/VI at the base. EKG was at baseline with a normal sinus rhythm, normal axis, and occasional premature ventricular complexes (PVCs).\nThe patient underwent an exercise stress test using the Bruce protocol and was able to complete stage 1 with exercise for three minutes at a speed of 1.7 mph and a 10% incline. The test was terminated due to dyspnea and fatigue without chest pain. She reached a heart rate of 141 beats per minute which was 98% of predicted for her age. She accomplished 4.5 metabolic equivalents of exertion. With exercise, she had occasional atrial premature complexes and PVCs with a ventricular couplet in recovery. She started to notice tightness in her chest. Her peak blood pressure at the time was 218/90.\nThe patient was transferred onto a stretcher, and an IV line was started. She was given sublingual nitroglycerin, 325 mg of aspirin to chew, and one 5 mg IV push of metoprolol tartrate. She then received nitroglycerin paste and metoprolol tartrate IV every 5 min for two more doses. At that time, her EKG on the stretcher showed ST elevations in leads I, aVL, V5, and V6 with ST depressions in leads III, aVF, and V1-V3 consistent with a lateral wall evolving myocardial infarction (). She was transferred urgently to our institution for cardiac catheterization.\nThe patient underwent an emergent cardiac catheterization with left ventriculography and intravascular ultrasound (IVUS) within 2 hours after onset of symptoms. Troponin-I levels prior to the catheterization increased to 11.17 (normal less than 0.05 ng/ml). The rest of the laboratories were within normal limits including a thyroid-stimulating hormone (TSH) level. Coronary angiography showed nonobstructive coronary artery disease (pLAD 40%) and highly tortuous coronary arteries. IVUS of the proximal LAD revealed a minimal lumen area of 5.2mm2, and no ruptured plaques. Left ventriculogram revealed a left ventricular ejection fraction (LVEF) of 20% and severe mid-cavitary hypokinesis with basal and apical hyperkinesis (Figures and ). To our knowledge, this is the first case of treadmill exercise testing-triggered mid-left ventricular ballooning variant of takotsubo cardiomyopathy, whereby obstructive epicardial CAD and ruptured plaques were excluded with angiography and IVUS, respectively.\nThe patient was started on medical management with standard therapy for heart failure. A follow-up echocardiogram was done two days after the event which redemonstrated mid-left ventricular ballooning, with an improved LVEF of 35%. The patient remained asymptomatic during the course of her hospitalization and troponin levels trended down from a postcardiac catheterization peak of 16.06 ng/ml. An echocardiogram was repeated during an outpatient follow-up two weeks later which showed resolution of wall motion abnormalities and an LVEF of 45-50%. |
A 41-year-old Iranian female was referred to Yazd Faculty of Dentistry with six-month complaint from a painless mass in the right posterior portion of the mandible. According to the patient, the soft tissue mass enlarged gradually during the six-month time period. Her medical history was normal and she was in good general health condition.\nIntraoral examination revealed a firm pedunculated gingival mass which was 3×3.5 cm in extent, located in the posterior region of the right third molar tooth, extending anteriorly and buccally and covering crown of the tooth. The lesion was lobulated and covered mainly with a normal colored mucosa (). Slight expansion of buccal and lingual cortex of the third molar region could be seen. These plates were firm and nontender in palpation. The molar teeth did not have any restoration, caries or significant loosening. No asymmetry was obvious in extra oral examinations.\nDifferential diagnosis of gingival exophytic lesions in retromolar pad area along with fibrous nodules, pyogenic granuloma, peripheral giant cell granuloma, peripheral ossifying fibroma and other peripheral hyperplastic masses were considered., In panoramic view, a soap bubble multilocular radiolucency with well-defined margins was apparent. It measured approximately 3×3.5 cm in mesial, distal and periapical of third molar (). Incisional biopsy of gingival mass, extraction of third molar and curettage of intraosseous lesion was made under local anesthesia. The specimen was then sent for microscopic examination. Microscopically, hematoxylin and eosin stained section of soft tissue component revealed a stratified squamous epithelium. The lamina properia showed multiple islands with various sizes and shapes in a background of fibrous stroma with few blood vessels. These islands were lined peripherally by one layer of ameloblastic columnar cells. The islands contained stellate reticulum-like cells within them. Fusion of tumor islands with basal layer of epithelium was evident in some parts. Microscopic evaluation of curetted specimen ( and ) also revealed multiple island of follicular ameloblastoma in a mature fibrous stroma. Due to the diagnosis of follicular ameloblastoma, marginal resection was performed and the lesion resected completely (). Finally, on the basis of microscopic and clinicoradiographic findings, the present case was diagnosed as solid ameloblastoma (follicular type) with a peripheral ameloblastoma- like component. |
Mr. M, a 74-year-old gentleman, was diagnosed with advanced stage lung cancer with malignant pleural effusion. He also had a past history of NHL 13 years back which was completely cured after chemotherapy. Mr. M underwent pleurodhesis for malignant pleural effusion. He and his family were explained about the nature of the disease and given the option of palliative chemotherapy. But Mr. M chose not to undergo chemotherapy and opted for palliative care. He was doing well for 2 months followed pleurodhesis though his general condition and daily activity started declining. He started having mild right-sided chest pain which was responding to paracetamol and NSAID.\nWhen Mr. M came to palliative care, he suffered with grade IV dyspnea, severe right-sided chest pain, dry cough, reduced appetite, and nausea. He was started on oral morphine 10 mg every 4 hourly and SOS (as needed) for right-sided chest pain, and later on switched over to the fentanyl patch as he developed dysphagia secondary to intractable dyspnea. Cough was under control with cough suppressants and Ondansetron 4 mg three times a day took control of nausea.\nDyspnea was treated with broncholdilators (Salbutamol nebulization) and oxygen through nasal prongs 6 l/min as he developed cyanosis. He had anxiety attacks during severe dyspneic attacks for which he was started on T. Lorazepam 1 mg at bedtime.\nMr. M was aware of his diagnosis and the incurable nature of his disease. His only wish was to have a peaceful death at home. He had a very strong family support and family members were willing to take care of him at home as he wished. He did not express any psychological problem except his grief of permanent separation from his loving family.\nThe palliative care team planned for organized home care at regular intervals. Out-of-hours care was coordinated over phone and if required one of the team members would pay a visit to home during emergency.\nFew weeks later, Mr. M developed delirium. The various causes of delirium including metabolic causes were ruled out. He was administered Haloperidol 5 mg subcutanoues (SC) every 8 h through a SC line. The family member was taught to administer SC medication under supervision during subsequent home visits.\nMr. M's dyspnea worsened despite administering all possible measures and he became increasingly delirious. As he was suffering with refractory dyspnea and delirium, that was not responding to the conventional treatment, the team decided that PS could be the option to relieve these intractable symptoms.\nFamily members were explained about the refractory nature of dyspnea and delirium and the fact that all the options of treatment failed to relieve them. Therefore, the need to administer PS at this moment to relieve Mr. M's intractable dyspnea and delirium was discussed with his family. The terminality of patient's condition was explained and DNR consent was obtained. The family agreed and gave informed consent to administer PS. Also, they were willing to administer PS medications through SC line during out of hours. The out-of-hours care was coordinated by the author.\nMr. M was started on PS with Haloperidol 2 mg SC every 2nd hourly and Midazolam 2 mg SC every 2nd hourly. Mr. M's dyspnea and delirium was well controlled with sedation. Although Mr. M slept most of the time, he woke up in between to take oral fluids until the last day. The sedatives were maintained at the same dose as this dose was found to be adequate to achieve conscious sedation. Fentanyl patch 25mcg per hour was continued for pain and Metaclopramide 10 mg SC three times a day for nausea was administered. Mr. M developed death rattle on the day of starting sedation and hyoscine butyl bromide 20 mg SC three times a day was given to dry up the secretion. Mr. M passed away peacefully 1.5 days after starting palliative sedation at home in the presence of his loving family. |
A 52-year-old woman presented to her head and neck surgeon (ENT) in 2008 with a well-circumscribed, pigmented lesion of her left lingual gingiva/anterior hard palate. The clinical impression was focal melanosis due to local irritation and observation was recommended. Four years later, the pigmented lesion evolved into multifocal lesion involving both the right (tooth #2 region) and left (teeth #9, #11 and #12 region) anterior hard palate. Biopsy revealed melanoma in situ. Subsequently, the patient underwent resection of the anterior hard palate with neck dissection in 2012, which revealed malignant melanoma with positive (melanoma in situ) margins. The cervical lymph nodes were negative for melanoma. Mutation analysis revealed wild-type BRAF, KIT and NRAS. A positron emission tomography/CT showed no evidence of distant metastasis. Due to positive surgical margins, radiotherapy was considered. However, to avoid the morbidity associated with irradiation, a decision was made to administer adjuvant chemotherapy (temozolomide) for 3 months.\nOne year later, in 2014, a biopsy of a new pigmented lesion on the anterior hard palate extending both to the right and left of midline revealed melanoma in situ. The patient underwent another surgical resection of the hard palate. Histology revealed melanoma in situ, again with positive anterior margins. A second resection was performed with clear margins.\nIn 2016, two pigmented lesions were found in the left anterior hard palate and labial gingiva of tooth #10. An incisional biopsy of one of the lesions revealed atypical intraepithelial lentiginous melanocytic proliferation. Subsequently, an excisional biopsy was performed showing melanoma in situ extending to the lateral margins. An excisional biopsy of the second lesion was negative for melanoma. Given the non-invasive lesions and morbidity associated with multiple surgical resections, observation was recommended.\nShortly thereafter, the patient presented with a new pigmented mass of the anterior hard palate () as well as pigmentation of the labial gingiva of tooth #10 (). An excisional biopsy of the anterior hard palatal lesion revealed malignant melanoma () with melanoma in situ extending to the lateral margins. Given her extensive surgical history and high expected morbidity, further resections were deferred.\nThe locally recurrent multifocal lesion was highly suggestive of field cancerization. The decision was made to initiate topical imiquimod 5% cream, an immunomodulatory agent that has been Food and Drug Administration-approved for treatment of cutaneous cancerized fields, precancerous lesions, and carcinomas. Topical imiquimod was applied every other day to the pigmented areas of the hard palate and labial gingiva (October 2016). A custom application tray was fabricated to ensure adequate exposure of the tumor to imiquimod (). Biweekly evaluation was performed. The patient initially tolerated the imiquimod well with no adverse events. After 6 weeks of single-agent imiquimod therapy, there was evidence of inflammation in the hard palate lesion () and significant clinical response in the pigmented labial gingival lesion (). Due to the uncertain stage of her recurrent disease (residual melanoma in situ that may have progressed into malignant melanoma), we discussed the potential benefit for checkpoint therapy including its potential to improve outcomes in patients with metastatic disease. The patient subsequently was started on pembrolizumab 2 mg/kg every 3 weeks. She tolerated both imiquimod and pembrolizumab well, experiencing only constipation, fatigue, and arthralgias.\nAfter 2 months of imiquimod therapy, the patient reported irritation and bleeding of the hard palate. Treatment was reduced to once every 3 days. After 6 months of imiquimod and pembrolizumab therapy, repeat biopsy of the left hard palate/gingiva showed no melanoma (). Given the complete histologic response, topical imiquimod and pembrolizumab were discontinued. Surveillance examinations every 3 months and CT of the neck/chest/abdomen/pelvis every 6 months was done; as of April 2020, scans remained negative.\nIn August 2020, three new pigmented lesions involving the left anterior hard palate and another lesion involving the palatal gingiva of tooth #15 were detected (). An excisional biopsy revealed melanoma in situ of the palate with negative margins and melanosis of the gingiva. Despite negative margins, imiquimod treatment was re-initiated every other day to the affected areas as a preventive measure. After 4 weeks of treatment, the patient experienced significant discomfort near the palatal gingival lesion, dysgeusia, and flu-like symptoms including fatigue and chills. These symptoms abated following a reduction in treatment to every third day. After 12 weeks of treatment, the patient resumed every other day application with improved tolerance. At week 15, treatment was held due to a recurrence of pain and bleeding in the gingival treatment area which self-resolved. After 20 weeks of treatment, the patient developed an infected molar requiring extraction. Imiquimod was stopped at this time; the residual pigmentation at the gingival margin of tooth #15 was no longer visible (). There is no clinical evidence of recurrent melanoma as of April 2021.\nImmune cell profiles in the patient’s recurrent melanoma in 2016 and following treatment with topical imiquimod and pembrolizumab were assessed retrospectively using quantitative multiplex immunofluorescence assays (). The formalin-fixed paraffin-embedded tissue slides were stained with anti-human CD8 (a cytotoxic T-cell marker), FoxP3 (a regulatory T-cell marker), Tbet (a Th1-type helper T-cell marker), CD4 (a helper T-cell marker), and DAPI (a nuclear marker). Stained slides were scanned on the Leica Versa with positive and negative controls and the images were adjusted using Aperio ImageScope (V.12.4.2.5010). Data were analyzed using a macro derived from the Leica Quantitative Algorithm V.1. Inflamed oral mucosa following imiquimod treatment revealed almost a 10-fold increase in activated helper T-cell population (CD4+/CD8–/FoxP3–/Tbet–) and a 13% increase in cytotoxic T-cell population (CD8+/CD4–/FoxP3–/Tbet–) compared with the recurrent melanoma. |
This 16-year-old male who previously underwent surgical resection of a suprasellar tumor at the age of 4 years via a left-sided pterional approach was referred to our hospital. In spite of two cycles of adjuvant chemotherapy for treatment of the diagnosed hypothalamic pilocytic astrocytoma, the tumor massively recurred over the following years. At admission, the patient was alert but suffered from panhypopituitarism and severe left-sided visual impairment equaling almost blindness in this eye. Preoperative MRI demonstrated a giant tumor occupying the suprasellar space that extended into the left parahippocampal gyrus and lateral ventricles (Fig. ). Tumor volume amounted to 68.1 mm3, its depth to 44.4 mm and the laterality angle to 21.9 °. Surgical removal was considered the only reasonable treatment modality at this stage.\nA bicoronal skin incision, bifrontal craniotomy and basal interhemispheric approach were undertaken in a routine fashion. First, the tumor involving the optic chiasm was removed, and the pituitary stalk was identified below the chiasm. The ACA, ACoA and lamina terminalis were gradually exposed. Opening the lamina terminalis permitted wide access to the anterior third ventricle and allowed for significantly debulking the retrochiasmatic tumor portion that had filled this area up to the basilar bifurcation and P1 segment of the posterior cerebral artery. While the tumor was meticulously resected despite its firm adherence to the hypothalamus and posterior circulation vessels, the ACoA was unexpectedly lacerated by tensile stress. Since repair of the lacerated ACoA was impossible, it was decided to divide the ACoA and thus maintain the blood supply in both A2 segments of the ACA. Following temporary occlusion of both A1 segments, the lacerated point of the ACoA was trapped with mini clips and subsequently divided (Fig. ). Vascular patency was repeatedly controlled with a micro Doppler device. Removal of the residual tumor could then be readily accomplished. Near total tumor resection and normal perfusion in both A2 vascular territories were confirmed and documented on intraoperative MRI (Fig. ). The optic chiasm and tracts, pituitary stalk and hypothalamus remained intact.\nThe postoperative course was uneventful, and the patient continued the medical treatment of panhypopituitarism. At follow-up no additional neurological or cognitive deficits were noted. |
Patient is a 73 year-old right hand dominant female who initially presented to the office complaining of atraumatic right shoulder pain with activity and limited range of motion of longstanding duration. On physical exam, she was found to have significantly limited active range of motion of the right shoulder and clinical signs of impingement. Radiographs at that time demonstrated superior escape of the humeral head with impingement of the greater tuberosity on the acromion and early acetabularization of the acromion (Fig. ). MRI findings were consistent with her x-ray and also demonstrated a lack of contiguous supraspinatus or infraspinatus tendon. At this juncture, the patient was diagnosed with rotator cuff arthropathy and elected to proceed with reverse total shoulder arthroplasty.\nThe patient was brought to the operating room and placed in the beach chair position. An incision was made from just lateral to the coracoid to the medial border of the proximal humeral shaft in line with the axillary fold. Subcutaneous tissue was dissected and the cephalic vein was identified. As the cephalic vein was mobilized and the clavipectoral fascia was incised, a discrete, branching, fascicular nerve was identified lateral and deep to the cephalic vein within the deltopectoral groove (Fig. ). The nerve was further dissected and traced both proximally and distally. Distally, the nerve and all branches were found to be diving into the anterior deltoid muscle. Proximally, it was found to run deep to the conjoined tendon, towards the brachial plexus. The nerve was freed from the deltoid muscle belly, allowing enough excursion to access the glenohumeral joint via a small deltoid window. The remainder of the operation concluded without complication and the wound was closed primarily (Fig. ). The patient was neurovascularly intact post-operatively with intact sensation in the axillary nerve distribution and able to fire her deltoid muscle. She healed without complications. At 4-month follow-up, she was doing well and able to actively abduct and forward flex her right shoulder to approximately 120 degrees (Fig. ). |
A 23-year-old man (weight 65 kg, height 175 cm, and BSA 1.8 m2) with a diagnosis of primitive right atrial enlargement from foetal age was referred to our Centre for cardiological evaluation. Cardiac examination showed increased heart size on percussion and a grade II/VI Levine systolic murmur. No significant pathological findings were found on pulmonary examination. Electrocardiography showed a regular sinus rhythm with a rate of approximately 60 beats/min associated with an abnormal morphology and duration of P wave (enlargement of P wave with duration of 130 msec), together with a low amplitude of QRS complexes in the limb leads. All routine laboratory studies were within normal limits. Chest radiography showed an abnormal cardiac silhouette with increased convexity in the lower half of the right cardiac border and cardiomegaly ().\nTransthoracic two-dimensional echocardiography demonstrated a huge right atrium of about 6.2 cm and a volume of 230 ml/m2, with a thick smoke pattern and mild tricuspid regurgitation. The pulmonary arterial pressure was normal (). The tricuspid valve was normal without significant annular dilation. No stenosis or abnormal displacement of the tricuspid valve leaflets was detected. No significant regurgitation of the tricuspid valve was found despite a partial distortion of the anterior leaflet and compression of the right ventricle inflow. The right ventricle appeared small and compressed anteriorly by the right atrium (area of RV: 11 cm2).\nCardiac magnetic resonance imaging showed a marked right atriomegaly (right atrium area: 66.50 cm2, volume: 220 ml/m2) and normal size of the left atrium (left atrium area: 7.02 cm2). The right ventricle was regular in size and global contractility but was partially compressed and dislocated posteriorly, due to the massive enlargement of the right atrium. The left ventricle was regular in dimension, thickness of the wall, and global/segmental contractility (FE VS = 61%). No evident transvalvular jets or areas of late gadolinium enhancement were found. The pericardium was visualized without focal abnormalities or pericardial effusion ().\nDue to the high risk of arrhythmias and thrombus formation in the right atrium, which is a potential risk for pulmonary embolism, the patient underwent cardiac surgery. Through a median sternotomy, cardiopulmonary bypass was established with standard aorta and bicaval cannulation. After the pericardium was opened, the entire anterior surface of the heart was found to be covered with a thin wall in continuity with the right atrium. No atrial appendage as such was apparent. The right atrium was fully opened. The inferior border of the atriotomy was sewn around the anterior part of the tricuspid annulus, and the superior border was brought over the lateral wall of the right atrium as a flap and sewn near the interatrial groove. This provided adequate reduction of the atrial size and reinforcement of the atrial wall ().\nThe histology of the resected atrial wall showed focal hyperplasic areas of smooth muscle cells with polymorphic nuclei surrounded by a few scattered areas of hypertrophic fibrous tissue.\nPostoperative transesophageal echocardiogram showed a significant reduction of the right atrium area (23 cm2, volume: 93 ml).\nThe patient was extubated 11 hours after surgery. Complications arose postoperatively with the early appearance of pericardial effusion with leukocytosis and elevated inflammatory markers. This was resistant to conventional medical therapy, which in the end required surgical drainage. Medical therapy of the postpericardiotomy syndrome (ibuprofen 600 mg/TID and colchicine 1 mg/OD) was continued over the subsequent 6 follow-up months without further recurrence of pericardial effusion. |
A 35-year-old G7P2 female at 12 weeks gestational age presented to the emergency department with a six-day history of worsening rash and swelling of her right hand and ankle (Figure ). This started at the right fifth finger and progressed to involve the dorsum of the same hand, along with the second to fifth metacarpophalangeal (MCP) joints. There was associated pain throughout and decreased range of motion of the right wrist and hand joints. The patient noted recent chills and body aches but no fever. She had originally seen her family physician two days prior and was prescribed cephalexin and ceftriaxone for a presumptive diagnosis of cellulitis. On a subsequent emergency room visit the next day for no change in the disease course she was prescribed clindamycin, once again with no improvement.\nOn presentation to the inpatient unit, due to infectious disease, her antibiotics changed to daptomycin and meropenem for broad coverage. Further history included no recent travel, infection, or exposure to pets. She denied insect bites and close contacts with similar symptoms. Her medical history was significant for hypertension, treated with lifestyle changes, and gestational diabetes. Social history was negative for smoking and drug use, and she had no family history of autoimmune disease. There was no history of sexually transmitted infections. Physical examination revealed an edematous, light pink plaque on the right dorsal hand and wrist that spread proximally up her arm with satellite firm, pink papules on the forearm. Numerous similar appearing papules were present on the left forearm. A small lesion was present near her right fifth MCP that had a gray, dusky appearing hue. On her right foot, there was a dark red/tan edematous plaque. Lab results on admission included an unremarkable complete blood count (CBC) and comprehensive metabolic panel (CMP). Differentials considered were broad, and included cellulitis, parvovirus, Lyme disease, disseminated gonococcal infection, and autoimmune etiologies.\nOver the course of her hospital stay, her condition continued to worsen. This included the onset of nausea and loss of appetite. Rheumatology and dermatology were consulted. An x-ray of the right hand showed no acute findings and was followed by an MRI that showed marked subcutaneous edema, possible fourth finger flexor tendon tenosynovitis, and no evidence of infection or abscess (Figure ). Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were elevated; however, autoimmune antibody testing, blood cultures, Lyme serology, gonococcal testing, and parvovirus IgM were all negative. Trans-thoracic echocardiogram showed no evidence of valve vegetations. Punch biopsies of the right wrist and left forearm were obtained on the second day post-admission.\nAs the patient's rash was progressing despite empiric antibiotic therapy and all cultures were negative, the antibiotics were discontinued on the third day post-admission and empiric IV steroids were begun. IV methylprednisolone 40 mg once daily was started which led to rapid improvement in her rash, swelling, nausea, and loss of appetite within 24 hours.\nEventual punch biopsy results obtained after discharge showed an inflammatory cell infiltrate consisting of neutrophils in the subcutaneous fat lobules (Figure ). There was no vasculitis, and special stains and cultures were negative. Following these results, she was diagnosed with subcutaneous Sweet syndrome. |
A 55-year-old woman with no notable medical and family history was referred to our department by her general practitioner following the appearance of multiple lesions of the oral mucosa. The intraoral clinical examination revealed poor oral hygiene, hypersialorrhea, and extensive mucosal damage affecting the entire oral cavity and causing discomfort, pain, and dysphagia. The lesions were located in the internal surfaces of the lips and cheeks, the dorsal and ventral surface of the tongue, and the soft palate mucosa (). There were epithelial erosions and postbullous ulcerations with a positive Nikolsky sign indicating acantholysis with dislocation of the epithelial layers (). Bulloerosive umbilical involvement was associated with the oral impairment without noting any other cutaneous lesions for four months ().\nIn front of these signs, we conducted an oral biopsy and prescribed topical prednisolone (20 mg; 3 times per day) and chlorhexidine-based (0.12%; 3 times per day) mouthwashes in order to reduce the oral pain awaiting the biopsy result. In a while, her condition worsened (). We referred her to the department of dermatology where she was hospitalized and an umbilical biopsy was performed.\nOn histopathological examinations, the oral biopsy concerned a squamous mucosa lined with a partially visualized coating, with disappearance of the superficial layers and the presence of cells of the basal layer which remain attached to the underlying chorion, drawing a “tapestry nail” appearance, surmounted by a few acantholytic cells. In the chorion, we noted the presence of a polymorphic inflammatory infiltrate rich in eosinophilic polynuclear cells. Direct immunofluorescent examination was negative (). As for the umbilical biopsy, the sample received was carried to the deep dermis. It was bordered by the basal layer of the epidermis, drawing the same “tapestry nail” appearance with extensive suprabasal acantholysis and separation of the overlying epidermal layers. The dermis was the site of an inflammatory infiltrate rich in neutrophils and eosinophils of perivascular arrangement with extravasation of red blood cells and without alteration of their wall. Intercellular immunoglobulin G and C3 deposits were observed on direct immunofluorescent examination with a characteristic mesh-like appearance (). Based on clinical, histopathological, and immunofluorescence findings, a diagnosis of pemphigus vulgaris (PV) was established.\nOn laboratory investigations, we found an inflammatory syndrome with increased erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Hepatitis (VHB and VHC) and VIH serologies were negative. Mycological direct examination and cultures were negative. On bacteriological culture performed from the umbilicus, Staphylococcus aureus growth was observed.\nAs for the therapeutic management, due to oral pain, a nasoenteral probe was used to feed her. Daily intravenous administration of prednisolone (120 mg/day) and amoxicillin-clavulanic acid (1000 mg thrice a day) was started. The nursing team was instructed to clean the oral cavity and the umbilicus using chlorhexidine (0.12%) mouthwash and ointment. Her condition improved gradually during hospitalization. There was a complete remission of the lesions in the mouth and umbilicus. The patient was advised to continue oral prednisone intake at a dose of 20 mg/day. She underwent monthly follow-ups in dentistry and dermatology departments after discharge from the hospital.\nFour months later, the disease recurred. We noticed the appearance of severe cutaneous lesions added to the oral and umbilical ones located in the elbows, forearms, and the periungual area. She was newly hospitalized. |
A 75-year-old gentleman presented with a one-week history of bright red rectal bleeding and fever. His past medical history included: history of human immunodeficiency virus, hepatitis C, decompensated cirrhosis with ascites, portal hypertension, transient ischaemic attack, excess alcohol consumption and smoking. He also had severe aortic stenosis, for which he underwent a transcatheter aortic valve implantation, squamous cell carcinoma of the tongue, basal cell carcinoma and depression. His drug history included anti-retrovirals, aspirin, thiamine, vitamin B, folic acid, omeprazole and fluoxetine. On examination, the patient’s abdomen was distended with minimal tenderness. Digital rectal examination revealed active bleeding with no obvious masses or haemorrhoids. Blood investigations showed a haemoglobin level of 74 g/L, platelet count of 54 × 109/L and deranged clotting and liver function tests. A computed tomography (CT) angiogram demonstrated splenomegaly and ascites but did not identify any specific cause for rectal bleeding. He was optimised medically prior to endoscopy with intravenous antibiotics, tranexamic acid, vitamin K, human albumin solution and a transfusion of one unit of packed red blood cells.\nOesophago-gastro-duodenoscopy demonstrated patchy candidiasis and a single varix in the lower third of the oesophagus. There was mild portal hypertensive gastritis in the cardia and fundus of the stomach and the lower body of the stomach with no gastric varices. No fresh or altered blood was seen within the upper gastrointestinal tract. On flexible sigmoidoscopy, there was a visible point of continuous fresh bleeding on the anterior wall of rectum around 4 cm from the anal verge. With the exception of a possible rectal varix there was no overlying mucosal abnormality at this site. Bleeding persisted despite the application of eight resolution clips (Boston Scientific), adrenaline injections and rectal packing. The patient became haemodynamically unstable with a blood pressure of 70/46 mmHg. A massive haemorrhage protocol was activated with immediate transfusion of packed red blood cells, fresh frozen plasma, platelets and terlipressin.\nA decision was made to insert a Sengstaken-Blakemore tube per rectum and to inflate the gastric balloon using 100 mL of air to tamponade the lower rectum. The oesophageal balloon was then inflated (external to the anus) to provide gentle traction to ensure that the balloon was firmly situated in the rectum and exerting optimal pressure onto the bleeding point on the rectal wall. The luminal port was connected to the catheter bag for free drainage and the bleeding stopped immediately. The blood pressure responded and increased to 95/54 mmHg. A subsequent CT angiogram demonstrated no evidence of haemorrhage with the effects of balloon tamponade (). There was no free air noted in the abdomen and no obvious varices in the portal venous phase.\nThe patient was closely monitored in the intensive care unit following endoscopy where he remained haemodynamically stable. He was further managed with intravenous antibiotics and terlipressin. After 36 h, the haemoglobin level was 92 g/L with a platelet count of 70 × 109/L and the balloon was removed at this point to reduce the risk of pressure necrosis and ulceration of the compressed rectal wall. There were no further bleeding episodes with two months of follow-up. |
A 66-year-old male patient was admitted to our surgical department for elective total thyroidectomy with a symptomatic non-toxic multinodular goiter. He was recently diagnosed with atrial fibrillation and was taking rivaroxaban, which was suspended 48 hours before surgery and bridged to heparin subcutaneous therapy. He had known hypertension, coronary artery disease and type 2 diabetes mellitus. Physical examination revealed palpable neck swelling, which was movable with swallowing. The neck ultrasound showed an enlarged thyroid gland with multiple nodules on both sides; the thyroid volume was 26 mL. The preoperative thyroid function test, parathyroid hormone and serum calcitonin were within normal ranges.\nThe patient underwent total thyroidectomy. The parathyroid glands and recurrent nerves were recognized and preserved. After meticulous haemostasis and intraoperative Valsalva manoeuvre, the wound was closed. No drain was placed. The patient was sent to the recovery room and then to the normal ward.\nThe neck circumference was regularly measured at different intervals as follows: the preoperative measurement was 42 cm; immediately postoperative, 42.5 cm; 30 min, 43 cm; 60 min, 43 cm; 90 min, 44 cm; 120 min, 44 cm; 4 h, 45 cm; and 6 h, 47 cm. At this point, the patient complained of a pressure sensation in his neck but no respiratory distress or hypoxia.\nThe patient was transferred immediately to the operating room (OR). Our anaesthesiologist experienced no difficulties in intubation. After surgical draping, the wound was opened, and cervical exploration was carried out, which revealed a haematoma deep to the strap muscles that appeared as a diffuse oozing haemorrhage. Following the removal of several clots, irrigation of the surgical bed, meticulous haemostasis and application of thrombin-coated collagen (TachoSil) covering the entire bleeding surface were performed. Two drains were left in the thyroid bed. The patient was extubated and sent to the recovery room and later to the surgical ward. The postoperative course was uneventful. Anticoagulant was restarted 12 h after surgery (heparin twice daily). Oral intake was gradually increased as tolerated. The drains were removed on postoperative Day 2. Histopathology returned benign. The patient was discharged on postoperative day 4 in good general condition. |
A 10-year-old, 35 kg, American Society of Anesthesiologists physical status I girl was scheduled for outpatient foot surgery. Her past medical and surgical history was unremarkable.\nAfter premedication with midazolam 10 mg per oral, an uneventful induction of anesthesia was performed with sevoflurane, followed by placement of an intravenous (IV) line. Propofol 30 mg and alfentanil 0.125 mg were given to facilitate the placement of a laryngeal mask airway (LMA). General anesthesia was maintained with sevoflurane. IV medications administered during the 60 minute procedure included: fentanyl 0.01 mg, morphine 4 mg, ketorolac 15 mg and dexamethasone 4 mg. At the completion of surgery, the surgeon infiltrated the operative site with 4mL of 0.5% ropivicaine. Emergence from anesthesia was uneventful. The patient was transported to the post anesthesia care unit (PACU), where she recovered quickly. After about 45 minutes in the PACU, the patient was returned to the same day surgery unit where she received acetaminophen with codeine for pain control. Approximately two hours after emergence from anesthesia, the patient complained of nausea and ondansetron 3 mg was administered IV. Approximately 30 minutes later she started having jerking movements of her arms and torso every time she would drift off to sleep. Initially the movements lasted for several seconds but during the course of the day these movements developed into shaking, seizure-like movements of her upper body and head lasting several minutes. She was easily arousable and while awake she was asymptomatic. She was oriented to person, place, and time.\nDiphenhydramine 25 mg IV was given as an attempt to treat a dystonic reaction but did not result in any improvement. The patient was discharged to home at 0930h on postoperative day one. The symptoms persisted at home (Video 1 - provided by the patient's parents and oral and written consent to publish pictorial content of the procedure was obtained). Over the next four days the symptoms improved slowly and the patient returned to her normal preoperative self. |
A 25-year-old woman presented in 1994 to the ophthalmology department with sustained episodes of flashes affecting the vision in both eyes. She was morbidly obese weighing 140 kg and a smoker. On examination, her optic discs and fundi were normal, but she was noted to have a transient drop in her visual acuity that normalized over a few days. For the on-going troublesome migrainous phenomenon, she was referred to neurology where she reported episodes of blurred vision, paraesthesia in the left lower jaw, left arm and leg. She was experiencing monthly right-sided headaches, associated with photophobia, which resolved within 24 hours. Examination was normal with no evidence of focal neurological deficit. Multiple sclerosis was considered as a diagnosis but later deemed unlikely. She was thought to have complicated migraine, and she was discharged. In 1998, she was re-referred to the neurology department with intermittent tingling on both sides of her body. This was accompanied by a sensation of imbalance. She had some short lasting visual obscuration. Neurological examination was normal. A magnetic resonance imaging (MRI) of the brain was organized but could not be performed due to morbid obesity. In 2002, she presented to the emergency eye department with bilateral granulomatous uveitis. Retinal fundoscopy revealed multifocal retinal periphlebitis. The optic discs appeared healthy, and there was no retinal cystoid macular oedema. Blood tests showed a normal ANA, antiphospholipid screen, full blood count, an ESR of 28 mm/hour and the presence of an atypical ANCA. The ANCA prompted a referral to the rheumatology department. An MRI of the brain showed lesions that involved the deep white matter (Fig. ). Over the next few years, she was monitored in a combined rheumatology–neurology clinic. Neurological examination remained normal. A Lumbar Puncture (LP) was not performed due to the body habitus. Visual evoked potentials were normal. Between 2002 and 2004, she had episodes of vitritis, hypopyon and macular oedema needing large doses of oral prednisolone. There was debate about this being a demyelinating process versus a vasculitis process, and it was decided that she should be treated with six cycles of intravenous cyclophosphamide followed by mycophenolate mofetil. However, in spite of the treatment, in 2005 she had a further episode of retinal haemorrhage and a posterior circulation transient ischaemic attack. A repeat MRI of the brain revealed progression of the number and distribution of white matter lesions with multifocal deep grey matter and brain stem involvement (Fig. ). Considering this to represent small vessel disease, possibly due to vasculitis, she was commenced on aspirin, b-blocker and statin. From 2004–2010, she continued to have attacks of uveitis and retinal bleeds, which were treated with a high dose of prednisolone. At this stage, an MRI was repeated to monitor the central nervous system changes. The MRI demonstrated progressive disease, which is, though non-specific, more suggestive of multifocal small vessel disease. The unusual distribution of the lesions and clinical progression sparked the search for alternative diagnoses. A review of the entire history and a multi-disciplinary discussion between the ophthalmology, rheumatology and neurology departments considered potential differential diagnoses—CADASIL, Susac syndrome and neurological Behcet’s disease. A negative genetic screen ruled out CADASIL. The lack of auditory involvement made Susac syndrome unlikely. Further history of intermittent oral and vaginal ulcers in her teens was obtained. This history had been lost because the patient had not considered this to be important and this had not been a problem for a number of years because of on-going oral prednisolone (doses of > 10 mg daily). A clinical diagnosis of Behcet’s disease was made. She fulfilled the classification criteria as set by the International Study Group for Behcet’s disease []. She was commenced on infliximab and azathioprine in 2013, which was later switched to methotrexate due to azathioprine intolerance. Since then she had no further episodes of uveitis, and her subsequent MRIs of the brain revealed stable appearances. At last follow-up she remained stable and is back in full-time employment. |
Case 2 was a 57-year-old male from Guangxi Province who presented with cough and fever for a week. He had a 40-year history of smoking, and his father had nasopharyngeal cancer. He was admitted to the hospital with a temperature of 38.5°C but with no abnormalities in the other physical examination parameters on January 22, 2018. His white blood cell count was 15.68×109/L, hemoglobin was 91.90 g/L, and neutrophil percentage was 74.0%. His serum albumin was 27.5 g/L. His hypersensitive C-reactive protein was 163.71 mg/L. HIV antibody testing was negative. No abnormalities were found in renal function, blood glucose, autoantibody spectrum or T lymphocyte subsets. A chest CT showed multiple nodules in the upper lobe of the left lung, a mass in the left hilar lung and a mass in the middle lobe of the right lung with mediastinal and left hilar lymph node enlargement ( and ). Fiber bronchoscopy revealed that the trachea, main bronchus and bronchi segmentales were unobstructed, and no necrotic material or tumor was observed. TBNA of the right paratracheal lymph node and subcarinal lymph node was conducted. The pathology of those lymph nodes revealed purulent exudate and hemorrhage. He was given cefoperazone tazobartan at 2.25 g every 8 hours and vancomycin at 500 mg every 12 hours a week, but the patient had a recurrent high fever. A chest CT on January 31 showed that the pulmonary lesions had increased in size ( and ). T. marneffei was isolated from TBNA tissues on February 4 ( and ). Thus, voriconazole at 200 mg orally every 12 hours and amphotericin B at 30 mg/day intravenously were prescribed. However, amphotericin B was discontinued due to the development of renal impairment noted on February 13. The lung lesions were smaller 2 weeks later on February 22, 2018. He was discharged with voriconazole orally 200 mg every 12 hours. However, he repeatedly developed a cough and increased sputum in August 2018, and AIGAs were identified in his serum. There were no intracavitary neoplasms seen during tracheoscopy. TBLB was performed in the posterior segment of the upper lobe of the left lung, and TBNA of the subcarinal lymph node was repeated. TBLB revealed acute and chronic inflammation of the mucous membranes accompanied by local hemorrhage, but no cancer cells were observed. No tumor cells were found in the TBNA. We considered that the patient might have T. marneffei combined with a nontuberculosis mycobacteria (NTM) infection. Therefore, rifampin at 450 mg/day, ethambutol at 750 mg/day, moxifloxacin at 400 mg/day, and clarithromycin at 500 mg/day were started. Meanwhile, cefoperazone tazobartan 2.25 g every 8 hours + linezolamide 0.6 g every 12 hours and voriconazole 200 mg every 12 hours were used for antibacterial treatment and antifungal therapy, respectively. After one week, cefoperazone tazobartan and linezolamide were discontinued, and chest CT on August 27, 2018 showed that the lesion in the upper lobe of the left lung was larger, but the mass of the left hilum was smaller ( and ). The patient was discharged after his temperature gradually recovered. After discharge, he continued anti-NTM and antifungal therapy. However, he presented with left-sided chest pain without fever in October 2018. A chest CT showed that the lesion in the upper lobe of the left lung had enlarged, and on November 6, 2018, the mass of the left hilar was the same as before ( and ). An ultrasound-guided lung puncture biopsy was performed on November 12. The pathology was confirmed as infiltrating adenocarcinoma, and no epidermal growth factor receptor mutation was observed (–). The anti-TNM drugs were discontinued. A positron emission tomography/computed tomography showed lung cancer in the upper lobe of the left lung with metastasis in the lower lobe of the left lung, the left hilum and mediastinal lymph node, the left 3rd and 4th ribs, and the cervical, thoracic and lumbar spine. The final diagnosis was confirmed as primary lung adenocarcinoma (cT4N2M1 stage IV) with metastasis to the lower lobe of the left lung, ribs, thoracic and lumbar vertebrae and a T. marneffei infection. He took itraconazole capsules orally at 100 mg twice a day for antifungal therapy but refused chemotherapy and chose icotinib for antitumor treatment. We did not recommend this treatment because his EGFR status was negative, and he died in April 2019. |
A 54-year-old male presented to our institute with severe pain, redness, swelling, and reduced vision in the right eye of 2 weeks duration. Eight years before, he had undergone RD surgery with a scleral buckle. The following year, he underwent cataract surgery with intraocular lens implantation, but there was no significant improvement in vision. Both these surgeries were done at a different center and the patient did not have any documents related to these. A year prior to presentation to our institute, he suffered from a blunt injury following which there were chronic watering and foreign body sensation in the right eye. Since then, the patient was using ciprofloxacin eye drops on and off. He admitted being a chronic alcoholic. At presentation, the best-corrected visual acuity in the right eye was 20/400 and left eye was 20/20. There was mechanical ptosis with eyelid edema in the right eye. Slit-lamp examination of the right eye revealed an anteriorly migrated exposed scleral buckle with mucopurulent discharge in the superotemporal quadrant []. The cornea had a small epithelial defect and 360° peripheral vascularization. Fundus examination of the right eye showed an attached retina. Left eye examination was clinically unremarkable. A diagnosis of right eye buckle infection with pseudophakia was made. The patient was operated under local anesthesia to remove the infected buckle on the same day. A superior peritomy was done. A segmental buckle 277 was seen along the superotemporal and superonasal quadrant of 6 o’clock hours’ length along with an encirclage 240 band. The band, buckle, scleral sutures, and overlying exudates were removed and sent for microbiology evaluation. The underlying scleral bed was severely inflamed and there was marked thinning. The scleral bed was irrigated with 0.3% povidone-iodine solution. No microorganisms were seen on 10% potassium hydroxide wet mount preparation or Gram's stain of the necrotic tissue material. The patient was started on empirical treatment with oral moxifloxacin 400 mg once a day and topical moxifloxacin 0.5% 1 hourly. On the second day, cultures revealed colonies of Moraxella spp. that were sensitive to moxifloxacin []. There was a gradual improvement of the clinical signs over the next 2 weeks, with a decrease in pain, clearing of conjunctival congestion, and resolution of anterior chamber inflammation.\nHowever, after 2 weeks, he again presented with severe pain, swelling, and watering in the right eye of 2 days duration. On examination, his visual acuity was the perception of light. There was a large scleral abscess involving whole superior quadrant []. Anterior chamber showed cells 4+. Fundus examination showed an attached retina with 360-degree peripheral choroidal detachment. He was treated with intravenous ceftriaxone 1 g 8 hourly in addition to oral moxifloxacin. Topical 5% ceftriaxone was also added. Scleral abscess showed signs of improvement on the third day [] and oral prednisolone 60 mg was added. Systemic antibiotics were continued for 3 weeks until the clinical signs resolved completely. A year later, the visual acuity was 20/200. The patient was asymptomatic. There was a large ciliary staphyloma in the right eye []. |
A 25-year-old female presented to the emergency department (ED) for evaluation of persistent productive cough of yellowish sputum over the last four week and mild exertional dyspnea over the last two years. Her past medical history was unremarkable and she took no regular medications. There was no personal or family history of multiple endocrine neoplasia type 1 (MEN1) syndrome. She was in no distress on presentation to the ED with a resting hemoglobin oxygen saturation of 97% while breathing room air. Her physical examination was remarkable for absent breath sounds and decreased tactile fremitus on the left middle and lower lung fields. No wheezing or stridor were heard. Laboratory data were within normal limits.\nA chest x-ray (CXR) in the ED demonstrated opacification of the left middle and lower lung fields, hyperinflation of the right lung and deviation of the trachea to the left (Fig. ). A computerized tomography (CT) scan of the chest showed complete left lung atelectasis due to a mass obstructing the left main bronchus and excessive mediastinal deviation to the left with substantial herniation of the hyperdistended right lung into the left hemithorax (Fig. ). There was no evidence of tracheobronchial narrowing in the right lung or esophageal compression. The mass was well demarcated and of soft-tissue quality, demonstrating homogeneous contrast enhancement, starting 2.8 cm distal to the main carina, measuring 4.4 × 2 × 2.8 cm (Fig. ). Abdominal and head CT scans showed no abnormal findings. The patient subsequently underwent a diagnostic flexible bronchoscopy which revealed a pale hypervascular polypoid mass completely obliterating the left main bronchus which was biopsied using forceps (Fig. ). Histopathological examination of endobronchial biopsies disclosed a carcinoid tumor with a Ki-67 index of approximately 10%.\nFollowing thoracic surgery consultation, an open left pneumonectomy with concurrent complete lymph node assessment and dissection was performed. During surgery, the left lung was found completely atelectatic with adhesions between the pericardium and the left pleura which were dissected. No attempt of repositioning the mediastinum or placement of tissue expanders was performed, due to the absence of airway compression in the right bronchial tree during previous bronchoscopy and CT scan. The patient recovered well after surgery and no complications were noted. Post-operative histopathology disclosed an atypical carcinoid with a Ki-67 labelling index of 10% but no areas of necrosis (Fig. ). There was a radical resection of all tumor with clear operative margins, the periphery of the left main bronchus was infiltrated by tumor, but there was no invasion of the visceral pleura, and no infiltration of resected lymph nodes from lymph node stations 5, 7, 9 and 10 by carcinoid cells.\nPre-operative spirometry was as follows: FEV1: 1.51 lit (44% predicted), FVC: 1.54 lit (39% predicted), FEV1/FVC: 98%. Spirometry and static lung volumes 12 months after surgery were as follows: FEV1: 1.93 lit (58% predicted), FVC: 2.34 lit (61% predicted), FEV1/FVC: 82%, TLC: 3.28 lit (63% predicted), RV/TLC: 118% predicted. Although spirometry appears to be significantly improved after surgery, spirometry before surgery triggered fits of coughing and therefore preoperative values might not be representative.\nPostsurgical follow-up has included the following: Initial chest CT scan was carried out 2 months after surgery. Parathyroid hormone (PTH) and prolactin levels were within normal limits 1 year after surgery. The following investigations were carried out at 6 months and then every 6 months for the first 5 years: Chest CT scan, abdominal ultrasound, chromogranin A measurement and standard laboratory testing including complete blood count, renal function, liver function, calcium and glucose. Abdominal CT scan and fiberoptic bronchoscopy were carried out 1 year after surgery and then will be carried out annually for the first 5 years. Bronchoscopy would be performed earlier for any symptoms or imaging findings suggestive of local progression. Repeat chest CT scans after surgery showed no changes in mediastinal rotation compared to those prior to surgery, and no signs of tracheobronchial or esophageal compression. Repeat bronchoscopy showed a normal-appearing surgical stump of left main bronchus and no airway compression of the right bronchial tree. The remaining studies listed above have been normal. The chronic mild exertional dyspnea reported by the patient before surgery completely resolved on hospital discharge, 7 days after pneumonectomy. The patient has been asymptomatic for the last 16 months after surgery with excellent performance status. |
A 45-year-old male patient admitted on 17th October 2011 with history of blast injury at work place. Chief complaints were severe photophobia, blepharospasm, ocular pain, and loss of vision in the right eye. Examination revealed multiple charred open wounds on the face and the eyelids. Particles of sand and mud were seen embedded in and around the eyelids. Both the eyes were congested with swollen eyelids. Slit lamp examination of the right eye showed a full thickness stellate-shaped corneal laceration with iris and probably lens matter incarceration measuring 4–6 mm in the central cornea. The lacerated corneal edges were edematous and fragile. The anterior chamber was shallow and flocculent lens substance observed in partially reformed anterior chamber. Pupillary examination was insufficient due to clouding of the anterior segment structures with ruptured lens. Left eye examination revealed diffuse multiple foreign bodies mostly sand particles embedded in the superficial layers of the cornea with few abrasions. Traumatic mydriasis noted with normal anterior chamber and lens. The presence of light perception was doubtful in both eyes. Intraocular pressure could not be measured due to severe photophobia and blepharospasm. Computed ocular tomography revealed a medium sized intraocular foreign body (IOFB) located probably in the vitreous cavity of the right eye. No intraocular foreign bodies were seen in the left eye.\nAs a primary procedure corneal laceration was repaired and the ruptured traumatic cataract was extracted under general anesthesia. The eyelids and the conjunctival sac of the right eye irrigated with 5% povidone iodine as a prophylactic measure to prevent postoperative endophthalmitis. Multiple small to medium sized sand particles that embedded in the eyelid margins were removed. The surgical field was adequately exposed by eyelids skin stitches. Central corneal wound was repaired by continuous sutures using 10-0 monofilament Ethicon []. Through temporal scleral tunnel approach, hydrated lens matter was aspirated and giant posterior capsular rent was observed. Therefore, primary intraocular lens implantation was deferred to later dates. A fairly large sand particle was seen immediately floating in the anterior vitreous that was extracted by McPherson forceps. A mixture of air bubble and Ringer’s lactate was used to reform the anterior chamber and the scleral tunnel was closed with 10-0 Ethicon suture and a soft bandage contact lens was inserted. Multiple sand particles from the left cornea was simultaneously removed []. Postoperative B-scan ultrasound of the right eye revealed few hyper reflective echoes in the vitreous cavity. Intraocular pressure was 10 and 12 mmHg by rebound tonometer measured in the morning hours.\nPostoperative period was uneventful with an aphakic vision of hand movements in the right eye and 6/18 in the left eye. Prior to penetrating keratoplasty (PK) the right eye examination showed an irregular and dense central corneal opacification approximately measuring 4–5 mm obstructing the visual axis. Anterior chamber was optically empty with normally reacting pupil. Fundus examination was unremarkable. The best corrected visual acuity was 6/60 with normal intraocular pressure. Improved vision after the primary repair and decreased vision due to central leukomatous corneal opacity with normal posterior segment on ultrasound B-scan was the basis of the second surgery. In the month of February 2012, PK and scleral fixated intraocular lens implantation was performed in the right eye [ and ]. Postoperative vision improved to 6/60 in the right eye. During the 2 year follow-up no complications were noticed and the vision improved to 6/24 with surgically induced astigmatism of −4 diopters. The best corrected vision in the left eye was improved to 6/9. |
A 30-year-old male presented to emergency department after being involved in run over injury by truck. Examination revealed complete avulsion of skin and subcutaneous tissue from bilateral inguinal region up to bilateral nipples. Upon reflection of avulsed skin, a full thickness defect of size 25 × 20 cm with complete loss of sheath, muscle, and peritoneum was noted in anterior abdominal wall. Through the defect, intestines were protruding out. Tyre marks were present over whole of the avulsed skin. Other positive finding on the physical examination included wound in lateral aspect of left thigh approximately 10 × 5 cm in size. The bowel was easily reduced and saline-soaked gauze was used for temporary coverage over the wound.\nAfter resuscitation with intravenous fluids and blood components, patient was taken to the operating room for exploratory laparotomy. Exploration of abdominal cavity was done through the defect. There was complete transection of jejunum about 2.5 feet distal to duodenojejunal flexure and mesentery at that level was incompletely transected with bleeding []. There was also complete transection of sigmoid colon and retroperitoneal hematoma present at right paracolic gutter.\nAfter abdominal wash out, patient underwent primary end to end jejuno-jejunal and colo-colic anastomosis in single layer. After abdominal wound debridement, defect was covered temporarily with urobag (urine collecting bag) by taking continuous sutures with margin of defect and urobag []. This was done to prevent abdominal compartment syndrome. After that avulsed skin and subcutaneous tissue was repositioned over the defect. In postoperative period, patient developed necrosis of avulsed skin [].\nOn day 12th, second stage operation was planned which involved excision of whole necrosed skin and removal of the urobag. We found that whole of the defect was covered with healthy granulation tissue. Two vicryl meshes of 15 × 15 cm size were used to give the strength to the granulation tissue [].\nLater on vicryl meshes got infected and were removed. Infected wound was managed with saline dressing and antibiotics. Patient’s wound was well covered with healthy granulation tissue with no cough impulse. Therefore, we decided for skin grafting of wound. On day 37th, split skin grafting was done over healthy granulation tissue that was taken up very well [].\nThe patient was discharged from the hospital 7 weeks after the initial injury and did well in follow-up at 2 months, both from cosmetic and functional perspective. Further, there was no evidence of hernia at the site of the traumatic defect. |
A 74-year-old man was admitted due to acute abdominal pain, which had started 3 hours earlier, with a stool problem since the previous month. He had a history of hypertension, cerebral infarction and benign prostatic hyperplasia under medical treatment. Physical examination revealed direct and rebound tenderness of the lower quadrants of the abdomen. Computed tomography of the abdomen showed multiple air bubbles and wall thickening of the sigmoid colon with fecal materials in the pericolic space. Laboratory results were within the normal ranges except for an elevated carbohydrate antigen 19-9 (88.39 U/mL). Under the impression of panperitonitis caused by perforation of sigmoid colon cancer an emergency operation was performed. During the operation, sigmoid colon showed a perforating tumor, which was severely adhered to the jejunum. Hartmann's operation and segmental resection of the small intestine were carried out.\nGrossly, the specimen consisted of two separate segments of sigmoid colon and jejunum. Serosal surface of sigmoid colon showed a fibrotic torn area, which was considered to be a result of surgical detachment from jejunum. Inside was an irregular cauliflower-like mucosal lesion projected into the lumen. On section, a 3.0 cm-sized tumor with irregular infiltrating border was noted. The separately submitted jejunal segment demonstrated irregular serosal surface and intact mucosa. Sectioning of the jejunum revealed a 3 cm-sized gray-tan and rubbery tumor mainly located in the muscular layer, extending to the perijejunal soft tissue. Dilated duct-like spaces were seen in the involved muscular layer.\nHistologically, the jejunal wall displayed irregularly dilated cystic areas, invasive carcinoma and focal heterotopic pancreatic tissue (). The foci of invasive carcinoma were composed of well-formed glands as well as individual cells and clusters, featuring well to poorly differentiated adenocarcinoma. The tumor was distributed through the muscular layer in a haphazard fashion, and infiltrated into the submucosal layer of jejunum and into the perijejunal soft tissue. The mucosa of jejunum was intact. The heterotopic pancreatic tissue was composed of ducts and acini without islet, intermingling with the tumor in the muscular layer of the jejunum (). Some ducts showed features of pancreatic intraepithelial neoplasia (PanIN) (). And more ectatic ducts with intraluminal papillary growth were present. The papillae were composed of mucin-secreting columnar epithelial cells with extensive pyloric metaplasia and variable degrees of cytologic atypia, being consistent with IPMN (). This lesion measured 7 mm in diameter. Transitional areas from high-grade PanIN and IPMN to adenocarcinoma were noted (). In the sigmoid colon, adenocarcinoma showing similar patterns to the jejunal tumor was present. Neoplastic glands were dispersed through the colonic wall and mesocolon (). Heterotopic pancreas, PanIN, and IPMN were not found in the sigmoid colon. Immunohistochemically, both jejunal and colonic tumors including IPMN showed positive reactions for cytokeratin 7, MUC5AC and MUC6 and negativity for cytokeratin 20, p53, and MUC2. By operative, pathologic and immunohistochemical findings, this adenocarcinoma was assumed to have arisen from a jejunal heterotopic pancreas via precancerous conditions (such as IPMN and PanIN), and invaded directly into the sigmoid colon. |
Case 1 was a 60-year-old woman. She was raised in a coastal area in a small town, in what is described as a well-functioning family, and has been trained as a healthcare assistant. Prior to moving into the nursing home, she had been married and had two adult children, with whom she had no contact. She had gradually increased her consumption of alcohol over the years, and in spite of several contacts with outpatient services for alcohol dependence, her drinking had steadily increased. After losing her job, she became increasingly socially isolated. Prior to moving into the nursing home, her home nurses visited several times per day, and they often found her in severe withdrawal, occasionally convulsing. She was underweight and incontinent. Her apartment was untidy and rarely cleaned, smelled of urine and feces and evinced her lack of personal hygiene. She was depressed and talked about suicide.\nAfter moving into the home, she gradually became stable, and was able to manage her personal hygiene with minimal assistance. She ate at meals and began to look better. She was still drinking, but at a level that did not cause problems with other residents. Occasionally, she drank heavily for 1–2 weeks. Her contact with other residents and staff stabilized, and she participated in simple practical activities. She seemed less anxious, and did not go through serious withdrawal.\nDuring the 18-month period before moving into the nursing home, she had been hospitalized nine times for periods ranging from 1 to 19 days; in total, she spent 43 days in hospital, had one outpatient visit and several ER visits. The total cost of her hospital-based care was estimated to be 154,649 DKK (20,798.74 Euros).\nAfter moving into the nursing home, she was admitted to inpatient treatment on two occasions for a total of 4 days. The total cost was 25,226 DKK (3392.64 Euros). She also had two visits at an emergency room (ER) and four outpatient visits. |
This study was approved by the Ethics Committee of Orthopedic Surgery Department, Imam Khomeini Hospital, Tehran, Iran and a written consent was signed by the parents.\nA 7-yr-old boy, the only child of otherwise healthy parents was referred the Pediatric Orthopedic Clinic, Imam Khomeini Hospital, Tehran, Iran on July 2018 with the diagnosis of CP. The reason for referral was the parents’ concern about the increasing severity of disease despite regular occupational therapy.\nOn physical examination, the patient was developmentally delayed, unable to walk or stand, with obvious cognitional and gross and fine motor retardation. Flexion contractures were noted in elbows, wrists, knees, and hips. There was bilateral equinovarus deformity of feet and increased popliteal angle. Plantar reflexes showed extension response and DTRs were exaggerated. Spastic response of muscles was recorded after continuous stretching. Sitting balance was extremely unstable ().\nThe patient was the result of a consanguine marriage and normal pregnancy. Birth weight was 2950 gr and head circumference and height were 35 and 47, respectively. The few first months of his life showed normal weight gaining and development. He was able to hold his head in 5 months and roll over at 7 months age. The first time the parents had been told about the possibility of an abnormality was in a routine screening at 5 months age. The pediatrician noticed a decreased head circumference growth. Further investigation showed the head circumference reached a plateau (40 cm) in its growth around 12 months age (). His general and developmental condition seemed to experience a sudden pause with progressive delay in growth and development since then. He lost his ability to rolling over and never gained any gross motor milestones. His face became expressionless and his eyes started to sink into the orbits (). Other findings were: apparent cachectic dwarfism, microcephaly, loss of facial adipose tissue, pigmented retinopathy, thoracolumbar kyphosis, multiple joint contractures, senile appearance, photosensitivity, and thin and dry hair.\nAlthough physical examination had a lot of similarity to a patient with CP, the history was inconsistent with the diagnosis of CP in its almost all aspects. This made us reevaluate the diagnosis. After a thorough history taking, some clues were added to our knowledge which was critical to the correct diagnosis. These include rapid regression of all motor functions, regression of language and fine motor functions and facial changes which are not compatible with CP.\nAt 7-yr-old age, he was in a cachectic dwarfism condition. The progeroid appearance narrowed our differential diagnosis.\nOur first diagnosis based on clinical findings and progression of the disease was Cockayne syndrome. The diagnosis was later confirmed by molecular analysis for Cockayne syndrome. The patient was homozygous for ECCR6 gene (genotype: c.2551 T>A /p.W851R- c.2551 T>A /p.W851R). The parents were also heterozygous for the same gene. This was also true for the patient’s only sister. |
CC, a 25-year-old, had been referred to this unit after she developed erratic behavior while traveling abroad. As detailed in the summary that was brought to our attention, she was sectioned under the mental health act and upon returning to her premorbid state, she was allowed to fly back to Oman. In the discharged summary, she was given a tentative diagnosis of an acute psychotic/manic episode. She responded when prescribed with Olanzapine (10 mg BD).\nUpon arrival in Oman, she sought consultation with the present unit. The accompanying family member informed the clinical team of her distress while traveling abroad, of which CC has minimal recollection. The family informed us that she often experienced uneasiness with others and exhibited strongly inappropriate emotion and culturally devalued conduct in the last 5 years. They noted that the distress occurred periodically (approximately every 29 days) with abrupt onset during a full moon. In traditional Omani society, certain lunar cycles are thought to trigger bad omens and malevolent spirits. Her symptoms were deemed manageable by the family since they appeared to dissipate with lunar changes. Further exploration of her changed self and conduct appeared to occur during the second half of her menstrual cycle and end at the onset of menstrual bleeding.\nPremorbid, her life during childhood was uneventful. She excelled in her education, graduated with a university degree and was on the lookout for a job. CC denied having consumed any mind-altering substances including tobacco or its rejuvenated forms and alcohol. A routine urine drug screening did not reveal the presence of any illicit drugs in her system. Physical examination was unremarkable and her medical workup—including hormonal study, brain CT scan, and EEG—was inconclusive.\nCC and her family were offered the option of continuing with the same medication she was prescribed abroad. CC and her family refused the option under the pretext that the medicine (Olanzapine) left her feeling drowsy, constipated and with an insatiable appetite. The attending team labeled her of having something akin to a manic episode with psychotic symptoms. Using the ICD-10, she was registered in her medical records as having Manic episode, unspecified (F309)/brief psychotic disorder (F24) (Table ). She was also subjected to intellectual and neuropsychological evaluation (see Fig. ). CC scored 15 on the a score suggesting the presence of mild depressive symptoms. |
AA is a 23-year old single Omani woman. She was brought by her parent to the psychiatric clinic with a 3-year history of the cyclical presentation of a short episode of clouded sensorium and abrupt onset of psychosis. Her thoughts and emotions had been impaired to the extent that her family had begun to believe that she had been ‘possessed’ by a malevolent spirit. Her mother noted that her distress occurred during the second half of her menstrual cycle and ended at the onset of menstrual bleeding. During the second half of her menstrual cycle, AA was reported as showing symptoms of dysphoric mania, isolating herself and crying unremittingly without substantive reason. The episodes of negativistic behavior tended to be superseded by a state of overactivity and euphoria characterized by grandiose beliefs, inappropriate irritability and social behavior and increased talking speed or volume. AA’s symptoms were reported to recede upon the onset of menstrual bleeding. While sedulous dating was not feasible, she was described to have had approximately 6–8 episodes of ‘possession’ in a year, with a regular occurrence every month.\nShe had no relevant medical history and denied having a family background of persistent mental disorders. She also denied having consumed any mind-altering substances including tobacco or its rejuvenated forms and alcohol. A routine urine drug screening did not reveal the presence of any illicit drugs in her system.\nPremorbid, AA met her developmental milestones without difficulty. AA had her first menstrual cycle at the age of 13 with regular cycles. She acquired 12 years of formal education and graduated with a secondary school leaving certificate with average performance. But due to her recurring distress, she did not seek further education or employment.\nDuring her consultation in our unit (taking place after the onset of menstrual bleeding), the clinical team noted that she was asymptomatic, interactive and socially active. She informed the clinical team that she tended to have ‘strange feelings’ and ‘weird experiences’ starting during the second half of her menstrual cycle. Overall, she expressed foggy awareness of her recurring distress.\nPhysical examination was unremarkable and her medical workup—hormonal study, brain computerized tomography (CT) scan, and electroencephalogram (EEG)—was inconclusive. She was also subjected to neuropsychological testing and evaluation of mood (see Fig. ). AA scored 23 on the []. Such a score implied the presence of moderate depressive symptoms.\nOlanzapine (5 mg)—an antipsychotic—was instituted once every day to which she was deemed to be compliant. The clinical team noted that the severity of her distress significantly receded but still manifested in episodes of screaming and lability and, as often the case, dissipated upon the start of menstrual bleeding. As the symptoms appeared to be atypical to those featured in CIDI, the clinical team suggested the tentative diagnosis of a manic episode, unspecified (F309)/brief psychotic disorder (F24). During the subsequent follow-up, her antipsychotic, olanzapine, was tapered up to 10 mg once daily and it was noted that the patterns of her symptoms appeared to generally be aligned with her menstrual cycle.\nDuring subsequent follow-up (6 months later), it appeared that her distress was exacerbated after approximately 28 days. Overall, her episodes of manic and psychotic-like symptoms remained the same but significantly reduced in terms of duration and veracity of distress. |
A 74-year-old male presented in February 2016 with increasing dysphagia. There was no history of prior abdominal infection or surgery. On esophagogastroscopy, a necrotic and circumferential friable tumor was seen at 33 to 40 cm from the incisors, with an endoscopic appearance of involvement of gastroesophageal (GE) junction and the proximal 2 cm of the stomach. Biopsies of the distal esophageal tumor confirmed poorly differentiated adenocarcinoma. The patient was anemic with a hemoglobin of 89 g/L. Staging endoscopic ultrasound suggested a breach of muscularis propria and four enlarged paraesophageal nodes. Neoadjuvant chemoradiotherapy followed by esophagectomy was initially considered; however, a staging positron emission tomography (PET) scan demonstrated 18-fluorodeoxyglocose (FDG) uptake not only in the primary tumor, but also in the paraesophageal region near the GE junction and upper abdominal lymph nodes extending as far inferiorly as the right renal vessels, in a retrocaval location (Figure ).\nA radiation oncology consultation was sought regarding treatment options of such extensive lymphadenopathy. Palliative radiation therapy (RT) was recommended. The patient was also evaluated by a medical oncologist who advised that chemotherapy may be considered after assessing the response to palliative radiotherapy.\nFrom March 21, 2016 to April 5, 2016, the patient received palliative RT to the symptomatic primary tumor and closest adjacent nodes using a pair of anterior and posterior fields. A total dose of 30 Gray (Gy) was prescribed over 10 daily fractions. As the lymphadenopathy in the lower abdomen was not symptomatic, and would have contributed to increased toxicity, this region was deliberately excluded from the high dose RT volume (Figure ). Other than very mild odynophagia, the patient had no other RT-related side effects. On the first follow-up visit, one month following treatment completion, he had improved swallowing function and a weight gain of six pounds.\nFollow-up computed tomography (CT) scan was obtained on May 24, 2016 to evaluate for the suitability of chemotherapy and to serve as a baseline during systemic therapy. This demonstrated persistent thickening of the lower esophagus, with lymphadenopathy reported to have decreased in size and no significant retroperitoneal adenopathy. When given the option of receiving palliative chemotherapy, the patient declined and chose to continue on observation only. Further CT scans in August and October 2016 showed a complete response in the irradiated primary tumor and nodes, with a stable 10 mm lymph node at the right renal vein.\nIn January 2017, due to symptoms of increasing dysphagia, the patient was assessed by a thoracic surgeon for consideration of esophageal stent placement. Endoscopy on January 12, 2017 noted that there was a possible small amount of residual tumor at the GE junction, but there was no significant narrowing or stricture, and no biopsies were taken. A further CT scan on April 10, 2017 showed minor circumferential thickening of the distal esophagus, but unchanged from previous. Paraesophageal lymphadenopathy was reported to be unchanged. The PET-positive lymph node at the renal vein decreased from 10 mm to 5 mm.\nThe patient’s symptom of dysphagia resolved spontaneously, and an evaluation was made with a further PET scan on May 19, 2017 (Figure ). This demonstrated mild residual FDG activity within the distal esophagus, more likely inflammatory change rather than malignancy. The FDG activity within all the lymph nodes, both treated and untreated, had unexpectedly resolved. |
The patient is a 47-year-old male refugee from Rwanda who was referred for urologic evaluation by his primary care physician for a mildly painful, slowly enlarging nodule on his left hemiscrotum. The lesion had been present for two years, having become painful over the past 6 months. The patient reported no associated discharge or changes in color or texture. He denied any personal or family history of genitourinary malignancies or dermatologic issues.\nA genitourinary examination demonstrated a mobile, pea-sized, scrotal lesion near the left penoscrotal junction. No other masses, lesions, or inguinal adenopathy were noted. At the time, the lesion appeared to be most consistent with a sebaceous cyst. A complete dermatologic examination was also performed which did not demonstrate any other lesions.\nGiven the unknown etiology of this scrotal lesion, a scrotal ultrasound was performed to evaluate any deeper involvement. This demonstrated a 7 mm superficial, hypoechoic soft-tissue nodule (). An incidental right-sided varicocele and hydrocele and small bilateral epididymal cysts were also found. Of note, unilateral right-sided varicoceles are rare and may signify underlying pathology causing inferior vena caval obstruction, classically from tumor thrombus involving the IVC []. To further evaluate this right-sided varicocele, a renal ultrasound was performed which demonstrated an indeterminate vascular mass measuring 1.5 cm in the right kidney and a 1 cm mass consistent with a Bosniak type II cyst in the left kidney.\nDue to the uncertain nature of the scrotal nodule and the patient's report of bothersome pain, the patient was offered continued observation or surgical excision and elected for the latter. After infiltration of the area with 1% lidocaine, an elliptical skin excision was made encompassing the lesion measuring approximately 1.0 cm × 2.0 cm. This was then dissected free from the subcutaneous tissue, showing no obvious extension of the nodule into deeper tissue. The patient tolerated the procedure well and was able to return home in stable condition.\nGross evaluation of the specimen revealed a well-circumscribed, subcutaneous nodule measuring 0.8 cm. The nodule revealed homogenous, tan, whorled cut surfaces. Histologic evaluation revealed a well-circumscribed, nodular lesion arising from the tunica dartos, comprised of interlacing fascicles of bland, spindled cells with eosinophilic cytoplasm and blunt-ended, elongated, cigar-shaped nuclei with perinuclear halos and mild to moderate cytologic atypia (Figures and ). Rare mitotic figures without atypia (0-1 per 10 high-power fields) were present (), without myxoid change or hyalinization. Lymphoid aggregates were present within the lesion, often in association with blood vessels. Immunohistochemical staining demonstrated immunoreactivity of the spindled cells using antibodies directed against smooth muscle actin and desmin, but not CD34, S-100 protein, or AE1/AE3, supporting a diagnosis of a smooth muscle neoplasm (). The proliferative index of the lesion, as demonstrated by a Ki-67 immunohistochemical stain, was 1-2% ().\nAfter discussion of the pathology results with the patient including the close margin status of the original excision (less than 1 mm margin), the patient was offered observation or wide reexcision to prevent tumor recurrence. The patient wished to proceed with reexcision, which was performed without complication. Pathologic examination of the reexcision demonstrated chronic inflammation and fibrous tissue, consistent with a previous surgical site, but no residual leiomyoma. His follow-up two-week later examination demonstrated an appropriately healing surgical site.\nThe presence of a dartoic leiomyoma in this patient with a renal mass of undetermined etiology raised the possibility of hereditary leiomyomatosis and renal cell carcinoma syndrome (HLRCC). The underlying defect of this disorder is an inactivating germline mutation in fumarate hydratase (FH). Although not employed currently as a routine clinical screening method for HLRCC, we acquired an antibody directed against FH (J-13 fumarate hydratase, Santa Cruz Biotechnology, Santa Cruz, CA) and performed staining on the lesion using two benign uterine leiomyomas as positive controls. By this method, we found intact FH expression in the patient's lesion () suggesting that the tumor most likely arose in a sporadic fashion and offering no overt evidence of FH deficiency or the HLRCC syndrome in the patient, although it should be remembered that FH immunohistochemistry is only 83% sensitive and 75% specific for HLRCC, and intact FH expression does not entirely exclude the possibility of HLRCC []. We also performed a triphasic contrasted CT scan to further characterize the possible right renal mass. This study, however, revealed no vascular, cystic, or solid abnormalities in the kidneys. MRI for follow-up may be optimal given the improved soft-tissue resolution and lack of ionizing radiation. Although no specific evidence of HLRCC syndrome was identified either clinically or pathologically and our results were reassuring, this remains a poorly understood syndrome and we believe that long-term surveillance for development of new or recurrent leiomyomas and renal tumors is still prudent. |
A 10-year old boy with OI presented at the Stomatology service of the Federal University of Ceará (Sobral). The OI type was not identified, the genetic test was inconclusive, and the patient had no family history of OI. Physical findings included short stature, blue sclerae, and locomotion difficulties due to deformed and arched limbs (). The medical history included reports of 16 bone fractures and intravenous administration of pamidronate disodium infused over a period of 3 days and repeated every 4 months. Anterior and posterior cross-bites, Angle class III malocclusion, and mild mandibular prognathism were observed on oroscopy. The anterior teeth were brownish, especially the lower incisors. Hypomineralization was observed on the vestibular surface of the upper left lateral incisor and a gray band was seen on the posterior teeth, especially the molars, suggesting DI (). The remaining deciduous teeth (the upper right maxillary canine and the right mandibular molars) were more brownish than the permanent teeth and were undergoing exfoliation, requiring extraction. Periapical radiography revealed enlarged pulp chambers, with insidious early obliteration of the coronary pulp chambers of the lower incisors and a thin layer of dentin deposited along all the teeth producing a taurodontic appearance. The teeth had ample crowns and cervical constrictions, with molars taking on a bell-like shape resembling a crown (). Panoramic radiography confirmed large pulp chambers in all teeth, but no agenesis, impaction, or supernumerary roots (). The primary mandibular molar teeth (first and second left mandibular teeth) presented physiologic mobility for some time, but were retained in spite of the underlying erupting premolars, which generated dental plaque accumulation, pain, and gingival bleeding during mastication and tooth brushing. Thus, these teeth were extracted to prevent further discomfort and to allow proper dental eruption. The child's mother requested the extraction of the primary maxillary right canine, in fear of having her son present the previously described findings. Surgery was performed without suspending bisphosphonate therapy and without antibacterial prophylaxis since no risk of infection was perceived in the transsurgical period. Currently, after 18 months of follow-up, the patient shows no signs of osteonecrosis. |
A 50-year-old woman with an unremarkable medical history observed gradual growth of an asymptomatic mass in the left cervical region. Clinical examination revealed a firm, adherent painless mass, medial to the left sternocleidomastoid muscle with minimal lateral and vertical mobility, located close to the carotid artery pulse. She denied a family history of PGLs or other neuroendocrine tumors. Neurological examination was unremarkable, and we initially suspected a branchial cleft cyst. The patient underwent neck ultrasonography (U/S), which revealed a firm, welldefined, solid, hypoechoic oval mass measuring 3×1 cm, located in the left carotid triangle. Power Doppler imaging revealed that the mass was highly vascular, resembling a carotid body PGL (CBPGL). Magnetic resonance angiography (MRA) (), revealed a mass displacing the cervical vessels. Subsequently, digital subtraction angiography (DSA) was performed and revealed a tumor with a vascular “blush” compressing the internal and external carotid arteries just over the carotid bifurcation, displacing the latter towards the midline (). We proceeded with an operation for the tentative diagnosis of a VPGL based on the absence of the classical “lyre” sign associated with a CBPGL on imaging studies.\nThe patient was electively admitted to our Vascular Unit and underwent surgical excision of the tumor under general intratracheal anesthesia. We used the typical left lateral cervical incision that extended to the skull base. The mass and the carotid bifurcation were covered by a peculiar arachnoid web-like structure with strong adhesions and a venous web (). The tumor received its arterial supply from the external carotid artery that crossed the carotid bifurcation in the frontal plane (). This artery supplying the tumor was ligated. The tumor nearly extending to the skull base was resected en bloc with the vagus nerve. All attempts to separate the tumor from the vagus nerve failed; thus, we decided to remove it with the part of the vagus nerve to which it was strongly adherent. Notably, owing to the cranially directed growth of the tumor, the superior laryngeal nerve (SLN) was not affected, both ends of the vagus nerve were ligated (–). The patient experienced temporary hoarseness immediately postoperatively; however, the hoarseness subsided after approximately a month. Further investigations using neck U/S and brain magnetic resonance imaging (MRI) revealed normal findings. Histopathological examination of the resected specimen showed a VPGL measuring 3 cm in size. She was discharged on the 3rd postoperative day following an uneventful recovery. Regular follow-up over 7 years after surgery did not reveal any functional abnormality nor abnormal imaging studies, and she showed no evidence of recurrence. |
A 32-year-old female patient with Kniest syndrome presented at our department with a painful pseudarthrosis after femoral valgisation osteotomy at the left side 8 years ago. The patient was informed about total hip replacement with osteosynthesis removal, and a conventional X-ray was obtained as well as computed tomography of the pelvis and an orthoradiogram. The trochanter major was not apparent due to a non-union after femoral valgisation (). A Wagner cone stem (Zimmer, Warsaw, IN, US) was implanted since radiological imaging revealed a dysplastic femur with a small medullar space and short femur. The Wagner cone stem is normally anchored in the proximal femur. However, we applied the Wagner cone stem with distal fixation which is not designated for this kind of stem but for revision stem shaft types. We achieved an adequate hip stability in this specific case using an acetabular reinforcement ring (Zimmer, Warsaw, IN, US) with a cemented Ecofit cup 2M, and acetabular bipolar cup system (Implant cast GmbH, Germany) ( and ). The cemented acetabular bipolar cup prevents a dislocation to a certain extent per se and contributes to total hip stability. The gluteus muscles were detached and retracted with a bone flake using a lateral approach, whereas a subvastus approach was performed for surgical exposure of the proximal femur. The acetabulum was first addressed, and an acetabular reinforcement ring with cemented Ecofit cup was implanted with correct alignment. Due to the poor bone quality, we reinforced the acetabular component with screws ( and ). The proximal femur showed intramedullary ossification which required preparation with a chisel and a drill to prepare the medullary cavity for further stem implantation. In this special case, we could reduce the risk of dislocation using an Ecofit cup in combination with an appropriate torsion of the stem. Thus, the Wagner stem was implanted with more anteversion to reduce posterior luxation. In addition, the abductor muscle group was sutured to an applied attachment tube (Implant cast GmbH, Germany) based on the concept of tumorprosthesis providing adequate re-attachment of the bone flake with the gluteal muscles. The surgery was performed by the senior author (AMN). The patient was mobilized with weight bearing of 15kg and a flexion limit of 60° for 6 weeks after surgery. The wound healing period proceeded without any complications although type II collagen is important in early wound healing and known to be compromised by a mutation in the COL2A1 gene. This finding corresponds with another study about head and neck surgery in patients with Kniest dysplasia with no impaired wound healing []. Follow-up showed flexion of the hip up to 90° at 7 weeks after surgery. Weight-bearingwas increased to 30 kg and flexion was limited to 70° at sitting position. Weight-bearing and hip flexion were stepwise increased during follow-up time, and the patient was able to walk without crutches and no sign of Trendelenburg 2 years after total hip arthroplasty (). Her mobility and quality of life after surgery increased so that she was able to return to work. The patient is still satisfied with the range of motion 4 years after hip endoprosthesis and reported a significant increase in quality of life. X-rays revealed no signs of loosening or migration during the entire follow-up time (). |
A 55-years-old male without any comorbid conditions presented to the orthopedic outpatient department with a mass over right forearm for the past 30 years. The swelling was progressively increasing in size over the years and was not associated with any motor or sensory impairment. He was unable to eat comfortably, as it was causing a mechanical block to the elbow movement dueto its increasing size and atypical location. On examination, it was found that swelling was located over proximal half of forearm extending mainly on the anterolateral aspect. Skin overlying it was normal and non-adherent. The mass was soft in consistency and on supination and flexion of forearm muscles, it partially reduced in size indicating that it was in the submuscular plane, specifically deep to the brachioradialis. His elbow range of motion had a terminal restriction which probably was causing his disability (). On admission, all his blood parameters were within normal limits. Conventional radiographs of the forearm revealed spiculated periosteal bone formation at the junction of the proximal and middle one-third junction of the radius. There was an increase in the soft tissue shadow surrounding the lesion along with characteristic radiodensity of fatty tissue. Magnetic resonance imaging (MRI) showed multilobulated mass with the hypointense signal on T1-weighted images. The dimension of the mass was estimated to be 8 cm × 8cm × 4.5 cm (). The mass was explored using elliptical incision and it was found underneath brachioradialis muscle. Enbloc resection of the well-capsulated mass with the stalk was carried out. The tumor weighed approximately 220 g (). Histological examination confirmed the diagnosis of encapsulated lipoma except for its attachment at the bone. Postoperatively, he had a full range of motion at the elbow (flexion:0–145°, supination:0–85°, and pronation:0–90°) and the wrist. The patient was followed-up for 2 years and was found to have a complete range of motion with no radiological or clinical evidence of recurrence of the tumor (). |
The patient was a 16-year-old boy who was referred to the outpatient clinic with complaints of behavioral problems like difficulty in anger control, running away from school and home, smoking and telling lies. In his medical history, he was diagnosed with the attention deficit and hyperactivity disorder (ADHD) 5 years ago by a child and adolescent psychiatrist and received methylphenidate 54 mg/day alone, with significant benefit for 5 years. But, within the last year, he started to fight, tell lies, smoke and also to be a school truant. Finally, it was learned that he took his father’s car without permission and had an accident with it. His family told that he did not have attention deficit problems during methylphenidate treatment and his academic achievement was satisfactory. In addition, he did not want to use methylphenidate anymore. In the psychiatric evaluation, he did not have eye contact and he told that he did not want to talk. He seemed very nervous. And after ten minutes in silence, he shut the door hard and went out.\nAccording to psychiatric assessment, he was diagnosed with ADHD and conduct disorder (CD) according to Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM 5) and was prescribed aripiprazole 2.5 mg/day adding to extended-release methylphenidate at a dose of 54 mg/day. Next day, he had an episode of hiccups in the morning starting 12 hours after the administration of first dose of 2.5 mg/day aripiprazole at night and hiccups lasted for 4 hours, which led him to come to clinic in great concern. He was extremely disturbed from this side effect. Therefore, we were unable to convince the patient to use another drug for behavioral problems. Owing to this side effect, it was thought that he may be a poor or intermediate metabolizer and pharmacogenetic testing was requested from medical genetics laboratory for CYP2D6.\nWhile waiting for test results, behavioral problems of the case were aggravated and his mother gave aripiprazole again at the dose of 2.5 mg/day for 2 weekend days saying that it was a vitamin for his lack of appetite, due to methylphenidate because he had drug holidays for methylphenidate every weekend. She told that she observed no hiccup with only aripiprazole but when she gave it again in weekdays in combination with methylphenidate, the hiccup started 12 hours later again and lasted for an hour at this time. She thought that he was better with aripiprazole when administered at alone, so at present patient is on only aripiprazole 5 mg/day as the schools are closed, and no hiccup is observed. Moreover, his behavioral problems decreased markedly. Meanwhile, our case was genotyped as extensive/normal metabolizer for CYP2D6. Evaluation with the Naranjo algorithm revealed a score of 7 (probably adverse drug reaction). |
Our patient is a 68-year-old Caucasian woman with a past medical history significant for a tonsillectomy 50 years ago. She developed a constant, dull and severe right-sided pain in her throat, neck, teeth and jaw along with dysphagia beginning approximately 25 years ago.\nAt the outset she attributed her symptoms to dental origins so she underwent tooth extractions on the right side without relief of pain. She was initiated on multiple pain medications, but her pain was improved though poorly controlled with carbamazepine, baclofen, and high doses of opiates. Ten years following the initial onset of her pain she was evaluated by an oromaxillofacial surgeon who noted that her symptoms were consistent with Eagle syndrome. He performed a manual fracture of her styloid process, which provided nearly instantaneous pain relief for 10 years, at which time she experienced a recurrence of her symptoms.\nAt that time she underwent a computed tomography scan of the maxillofacial structures which revealed bilateral elongated styloid processes, particularly on the right, and she was officially diagnosed with Eagle syndrome. The extremely elongated right styloid process (approximately 6cm) and moderately elongated left styloid process (3.2cm) can be seen in Figure\n. A styloidectomy using an external approach significantly relieved her pain, and she was able to discontinue her pain medication regimen.\nTwo years after her styloidectomy the patient had an outbreak of shingles in her right ophthalmic division; the shingles healed without incident and left no residual pain or alteration of sensation. Five years post-styloidectomy she developed right-sided facial pain extending from her upper lip to the middle of her forehead. She described her pain as electric shock-like and transient. It was triggered by light touch and activities such as brushing teeth, talking, and blowing her nose.\nShe restarted 800 to 1600mg carbamazepine and up to 10mg baclofen daily, however, she took these medications irregularly due to side effects that interfere with work and activities of daily living. Due to the medications she typically slept for 12 hours each day after work, and the full-dose baclofen affected her balance and gait. Although the side effects were significant, the medications reduced the frequency and severity of her episodes.\nFollowing the development of her right-sided facial pain, she underwent magnetic resonance imaging of the brain and brainstem, which revealed scattered T2 hyperintensities in the deep white matter, a non-specific finding. No cranial nerve lesions were apparent. A loop of superior cerebellar artery was in contact with the right trigeminal nerve root, which can be seen in Figure\n.\nBecause of inadequate pain relief and medication side effects, the patient was referred for possible surgical management. The various surgical treatment options were considered and discussed with the patient, including microvascular decompression, percutaneous rhizolysis, and Gamma Knife® radiosurgery. She elected to pursue Gamma Knife® radiosurgery with the intent to taper oral pain medication as her pain subsides over time. No deficits were detected in her cranial nerves and pain was not elicited with touch or percussion. Otherwise, her physical examination was negative. |
A 55-year old man visited the emergency room due to acute ch-est pain. His vital signs were stable but his electrocardiogram (ECG) showed ST-elevation in leads II, III and aVF as well as Q waves in leads V 1 and 2 (). The patient had a history of repeated AMIs, for which he had received multiple stents in two different hospitals. Despite the higher risk of recurrent cardiovascular events, his compliance seemed poor and he was still a heavy smoker. His current medication included triple antiplatelet therapy, a lipid-lowering agent, a beta-blocker, and an angiotensin-converting enzyme inhibitor. The pain-to-door time was about 30 minutes and the PCI team was called after diagnosis of an acute inferior wall ST-elevation myocardial infarction (STEMI).\nDuring the stay in the emergency room, the patient suddenly collapsed from VF. CPR was initiated and repeated shocks at maximum energy were administered. Throughout about 30 minutes of conventional CPR, repeated shocks only stopped the VF for several seconds (). At this point, we decided to implement ECLS (CAPIOX EBS®, Terumo, Tokyo, Japan). The right femoral vessels were accessed guided by anatomical landmarks and ECLS was finally implemented, about 2 hours from the initiation of CPR. With full support, we achieved a flow rate of 4.8 L/min and mean arterial pressure of 70 mm Hg, but VF persisted.\nNow the patient could be transferred to the catheterization room to undergo primary PCI, without the need for chest compressions for his fibrillating heart. In the catheterization room, the left femoral artery accessed also guided by anatomical landmarks and a coronary angiogram (CAG) was performed. The left CAG, including stented segments, showed no significant stenosis. The right CAG revealed one stent in the proximal portion that was totally occluded by a thrombus (). Despite some difficulty in wiring, the coronary flow was successfully restored after ballooning, about three hours from the onset of chest pain. We implanted a long drug-eluting stent on the previous stent because the residual stenosis after ballooning was more than 80% (). After the PCI, a single 360 J shock proved sufficient for successful defibrillation. We finished the procedure by installing an intra-aortic balloon pulsation (IABP) catheter to assist recovery.\nThe patient regained full consciousness without any neurological deficit later that day. The IABP and ECLS could be weaned on the third day and the patient was transferred to general ward on the fourth day. He was discharged with chest wall pain, caused by the CPR, and a large hematoma around the ECLS cannula insertion site, which had required a transfusion. |
A 62-year-old male with past medical history of hypertension, diabetes mellitus, and hepatitis C was brought to the emergency department (ED) by Emergency Medical Service (EMS) after initial resuscitation of cardiac arrest at his home. He was found in asystole by EMS and return of spontaneous circulation (ROSC) was achieved after one dose of epinephrine. No further history was available. In ED patient was found intubated and unresponsive to painful stimuli. Patient was hypothermic (93°F) and physical examination revealed a puffy face and dry skin; the rest of the examination was unremarkable. EKG revealed sinus bradycardia and low voltage QRS with left axis deviation (). He was admitted to the intensive care unit (ICU) in view of status post cardiac arrest of unknown etiology. Work up was done to rule out most common etiologies of sudden cardiac death. Chest x-ray was negative for any acute pulmonary pathology. Echocardiogram revealed normal left ventricular function with no regional wall motion abnormality and no pericardial effusion. Head CT was unremarkable. Perfusion scan was not suggestive of pulmonary embolism. Electrolytes were normal on presentation, with no evidence of hyponatremia or hypoglycemia. Later, son revealed that patient had history of forgetfulness a week prior to presentation but he ascribed those symptoms to dementia. Thyroid profile revealed TSH level of 43.730 uIU/ml and free T4 level of <0.11 ng/dl and total T4 < 0.5 mcg/dl; the patient was diagnosed with myxedema coma with a score of more than 60. The patient was then started on intravenous levothyroxine and hydrocortisone. Adrenal insufficiency was ruled out as the serum cortisol am was 19.8 ug/dl. The patient was managed in the ICU and he continued to have episodes of bradycardia. The hospital course was complicated by another episode of cardiac arrest but he was successfully resuscitated; it was preceded by prolonged QT and bradycardia, leading to third degree heart block (). Because of persistent bradycardia the patient’s levothyroxine dose was increased. He was also started on T3 a week after treatment with levothyroxine. The patient showed gradual improvement in hemodynamic status and was able to be taken off pressor support and was successfully extubated. Clinical improvement was suggested by improvement in mental status, and TSH and T4 levels trending towards normal range and improvement in heart rate and QRS voltage. Eventually, the patient improved and was discharged home. |
A 23-year-old male was feeding his horse, and while stroking the horse's hair, the animal chewed the fourth finger of his left hand causing violent pain and total functional impotence of the finger. Both the patient and the horse were up to date on their required vaccinations at the time of the incident. The patient was transferred to an emergency department and was admitted six hours after the incident. He was conscious, in good general condition, and apyretic. An examination revealed a crush injury of the fourth finger with tendons and bone exposed ().\nCopious irrigation with normal saline (2 liters) at the injury site was performed along with injection of 0.5 ml tetanus toxoid and 500 IU of human tetanus immunoglobulin. Postexposure rabies prophylaxis (rabies immune globulin human 20 IU/kg) with the first-dose rabies vaccine was injected into the depth of the wound as well as around the wound. The remaining rabies immune globulin was injected into the deltoid muscle. The patient was also treated with prophylactic antibiotic therapy with intravenous amoxicillin-clavulanate, gentamicin, and metronidazole.\nAfter this initial treatment, radiography revealed a fracture dislocation of the proximal interphalangeal joint of the fourth finger with a third fragment (), prompting the patient to undergo surgery. Surgical exploration under locoregional anesthesia found that the ulnar digital pedicle was sectioned and thrombosed, the radial digital pedicle was intact, the flexor and extensor tendons were sectioned and shredded, and the skin was irreparably shredded ().\nSurgical procedures included removal of foreign bodies and excisional debridement of devitalized tissue, collection of bacteriological samples, copious irrigation with saline serum (3 liters), tendon striping, and finger amputation with coverage of the bone by the radial digital flap using separate stitches (). The surgery was followed by careful clinical and biological monitoring.\nA clinical assessment of the patient 1 day postoperatively showed that he was apyretic with no necrosis of the flap and no purulent discharge. A neurovascular examination was normal. Biological findings at this time showed a C-reactive protein level of 30 mg/dL and a white blood cell count of 11000/μL.\nThree days postoperatively, bacteriological samples found an evidence of Pasteurella species and Staphylococcus sensitive to amoxicillin + clavulanic acid with C-reactive protein levels of less than 10 mg/dL and a white blood cell count of 7000/μL. At this time, the patient received the second dose of rabies vaccine.\nOne week postoperatively, the patient was discharged with a prescription for a course of 10 days amoxicillin-clavulanic acid treatment to be reviewed weekly. At a three-month follow-up (Figures and ), the patient showed no sign of infection; he returned to his usual activities and was discharged from care. |
We report the case study of an 18-year-old Saudi male patient who was known to suffer from SCD. This patient was presented with a generalized body ache and headache, both of which had persisted for 2 days. His headache was most severe in the frontal region and was not relieved by his home-administered analgesics. There was no history of neck stiffness, weakness, or a decreased level of consciousness. There was no history of fever, chills, rigors, or contact with sick people. The patient was admitted to the medical ward as a case of SCD with vaso-occlusive crises (VOCs) and was started on King Fahd Hospital of the University sickle cell protocol; initial workup included complete blood count, retics, renal function test, liver function test, peripheral blood picture, urinalysis, chest X-ray, and electrocardiography. The patient was given half normal saline at a rate of 150 cc/h, and for the pain, he received 5 mg morphine sulfate every 4 h and 8 mg Xefo (lornoxicam) every 12 h. He was also kept on daily pantoprazole 20 mg and folic acid 5 mg. An intradermal skin test was made for ceftriaxone and was negative. One gram of ceftriaxone was administered every 12 h plus vancomycin 1 g every 12 h. The patient was evaluated by neurology to rule out meningitis.\nThis patient was referred to the on-call ophthalmologist as a case of bilateral periorbital swelling; the left eye was affected first, followed by the right eye. According to the patient, he developed a similar complaint 2 years ago and was believed to be an acute allergic reaction to an antibiotic.\nOn examination, the patient was conscious, oriented, and vitally stable, with a low-grade fever. The uncorrected visual acuity was 0.8 in both eyes. The external examination showed swelling of the bilateral upper lid with left inferior dystopia []. In addition, there was no erythema or hyperemia. Extraocular motility was full in both eyes, and color vision assessment by the Ishihara test was normal. The pupils were reactive with no relative afferent pupillary defect.\nFurthermore, the slit-lamp examination was unremarkable, and no disc edema or any other abnormality was noted on examining the fundus. The initial impression was orbital infarction. Hence, the ophthalmology plan was imaging to confirm the diagnosis, metronidazole 500 mg intravenous every 8 h to cover anaerobes and Vitamin C supplements.\nA magnetic resonance imaging (MRI) with contrast was undertaken and showed intermediate signal intensity on T1-weighted (T1-W) image and high signal intensity on T2-W image. These results occurred with a peripheral diffusion restriction and faint peripheral enhancement, representing multiple bony infarctions and subperiosteal hematomas within the frontoparietal region bilaterally. The MRI also showed a well-defined extraconal lesion, likely to be a hematoma, arising from a superior lateral orbital wall of the left eye, causing mass effect on the ipsilateral lacrimal gland as well as the left lateral rectus muscle [].\nIn addition to the periorbital swelling, the patient was found to have a low-grade fever, leukocytosis, and elevated inflammatory markers. Due to ongoing clinical concern, further surgical exploration was conducted via a subbrow incision on the left side, but neither pus nor hematoma was discovered. Interestingly, the bone appeared gray, which may correlate to the bony infarction seen on MRI [].\nDuring the follow-up visit, the patient's pain decreased, and the dystopia improved []. The patient was advised to maintain proper hydration and to take Vitamin C supplements. |
A 31 year old female with a history of two cesarean sections, the last one of them three
years prior, presented with a painful mass in the left lateral side of the Pfannenstiel
incision which had been steadily growing. The same patient went a month ago to our
institution's urology clinic with pain in the inguinal region. After a detailed history
and physical examination an ultrasonography was performed that revealed a 20X12 mm
hypo-echoic nodular mass, neighboring the rectus abdominis muscle, with minimal
vascularization. The differential diagnosis included endometriosis and a possible
desmoid tumor. The patient was referred to our clinic for further evaluation. The pain
started on the left lower quadrant and radiated towards the inguinal region, and was
associated with menstruation. In physical examination a 2 cm wide mass was palpated in
the previously described localization. Magnetic resonance imaging was performed which
revealed a 20x11 mm mass which is slightly hyper-intense in the T1 sequence, and
contrast enhanced after IV gadolinium injection in the T2 sequence, with increased
signal intensity and nodular appearance in diffusion weighted sequences. These were
found to be consistent with an endometrial implant ( and ). Examination of the
uterine cavity showed effusion which was at most 15 mm in width when measured. A little
free fluid, indicating peritoneal irritation was present in between the intestinal
loops. No pathological lymph nodes were present in the lower abdomen.
The patient was admitted for surgical removal of the mass 2 cm wide fibrotic appearing
mass was excised () and was sent for
pathological examination. The patient's complaints resolved after the surgery. She was
discharged with no complications two days after the operation. Four months after the
surgery, the patient came in for a follow-up visit, and had no complaints or
complications. Histopathological examination was consistent with glandular structures of
the endometrium with accompanying endometrial stroma |
A 69-year-old man had the past history of laparoscopic sigmoidectomy for pathological stage IV colon cancer with synchronous liver metastases (Segment 7 and 8) 4 years ago. He underwent sigmoidectomy due to intestinal obstruction, and liver lesions were planned for a two-stage surgery. Histological examination of the primary tumor revealed a well-differentiated adenocarcinoma with subserosal invasion and vascular infiltration, but no lymphatic or lymph node metastasis. Thereafter, he had undergone partial hepatectomy of Segment 7 lesion and radiofrequency ablation (RFA) of Segment 8 lesion for colorectal liver metastases 2 months after sigmoidectomy. After 2 years from the initial operation, he had undergone partial hepatectomy for the local recurrence of post-RFA lesion. He was followed without any adjuvant chemotherapy since the resection of primary colon cancer. After that, he was referred to our hospital because of the stenosis in a hilar bile duct. Enhanced CT revealed an enhanced wall thickness in the hilar bile duct (Fig. a). Magnetic resonance cholangiography, endoscopic retrograde cholangiography and intraductal ultrasound showed that the tumor had grown into the bile duct lumen (Fig. b, c). A brush cytology appearance indicated a class IV. According to the radiological findings, we diagnosed a hilar cholangiocarcinoma (Bismuth type II). Then, he underwent a right portal vein embolization and followed by the extended right hemihepatectomy with extrahepatic bile duct resection. The macroscopic finding displayed 3 cm tumor located in the hilar bile duct wall. Histological examination revealed moderately differentiated adenocarcinoma existing in the common hepatic duct. There is no tumor invasion into the right hepatic artery. Perineural infiltration was observed, but portal or vascular infiltration or lymph node metastases were not identified. There is no invasion of intraepithelial neoplasia at the margin of the tumor (Fig. ). The immunohistological stainings of the bile duct tumor showed the cytokeratin (CK)7-negative, CK20-positive, caudal-type homeobox 2 (CDX2)-positive and special AT-rich sequence-binding protein (SATB2)-positive, uncovering that the bile duct tumor originated from the sigmoid colon carcinoma (Fig. ). |
A 3-year-old Caucasian male patient presented to us with a swelling of 2 weeks duration in the left eye. The onset was gradual and was preceded by blunt eye trauma in a traffic accident. The patient was initially admitted to another hospital, where the initial examination revealed a left periorbital ecchymosis with subconjunctival hemorrhage and proptosis without any vision loss. After 2 weeks of surveillance, the patient presented a persistent proptosis for which he was addressed to our structure. The admission examination found a slight proptosis of the left eye with a tender swelling palpable at the inferior orbital region that was pushing the globe upwards (Fig. ). Movements of the globe as well as pupillary function were normal. No abnormalities were found in the examination of the anterior and posterior segments. His intraocular pressure in both eyes was in the average range. Neurological examination found no signs of intracranial hypertension, of sensory or motor deficiency. The rest of the physical examination found no signs of distant lesions such as osteoarticular pain, hepatomegaly, splenomegaly, enlarged lymph nodes, or dullness of the flanks. No notable history of hematopathy or weight loss were presented, and the patient’s parents are not related. A craniofacial computed tomography (CT) was performed showing a spontaneously hyperdense, oval-shaped, extraconal mass measuring 29 × 19 mm without significant enhancement after contrast injection. This mass repressed the inferior and external rectus muscles and was not associated with any bone abnormalities (Fig. ). In front of these clinical and radiological findings, the first diagnosis proposed was an inferolateral subperiostal hematoma. An inferior mediopalpebral incision was performed to drain the hematoma when a pale yellowish tumor was found against the orbital floor (Fig. ). An excision of the mass was done. Microscopy showed the features of a round-cell tumor infiltrating the fibrocollagenous tissue. On immunohistochemistry, cells expressed positivity for anti-Myeloperoxydase (anti-MPO) and CD-45, and were negative for S-100 and myogenin. Complete blood count revealed: hemoglobin (Hb) 11.9 g/dl, total leucocyte count (TLC) 19540/mm3 with 20% blasts. Liver and renal functions were within normal limits [alanine aminotransferase (ALT) 23 UI/L, aspartate aminotransferase (AST) 17 UI/L, total bilirubin 11.7 mg/L, urea 0.27 g/L, creatinine 6.8 mg/L]. Human immunodeficiency virus (HIV), hepatitis, and syphilis serology was negative. Blood culture and cytobacteriological examination of urine revealed no pathogens. White blood cells in the cerebrospinal fluid were < 5 elements/mm3. Bone marrow aspirate smears revealed the presence of 24% blasts, and the diagnosis of orbital myeloid sarcoma with acute myeloid leukemia was made. The patient was t (8;21) (q22;q22) positive, and the subtype was AML M2. Thus, the patient was started on induction therapy, with intravenous cytarabine (Ara-C: 15 mg/12 hours the first, second, and third day of chemotherapy) and daunorubicin (10 mg/day, the third and fourth day), followed by consolidation with both molecules plus intrathecal administration of methotrexate, Ara-C for two doses. On the 18th month follow-up, the patient was disease free. |
A 75-year-old female patient reported to the emergency room of Lata Mangeshkar Hospital, Nagpur, with chief complaints of pain and swelling in the lower lip and discomfort in the left cheek region for 5 days. The patient gave an alleged history of fall at home 5 days back. Since then, she had an open wound involving the lower lip and left commissural region. The patient reported to a government hospital 2 days back where approximately thirty maggots were removed. The patient was carrying relevant documents stating the same.\nOn extraoral examination, a diffuse swelling of approximately 4 cm × 3 cm in size involving the lower lip was noted. The examination also revealed incompetence involving the left corner of the mouth with overlying skin tense and shiny with multiple fenestrations []. The swelling was soft and edematous in consistency and tender to palpation with no suppuration. Intraoral examination revealed complete upper and lower edentulous arches.\nSeveral cavitations with deep burrows were noted. Multiple larvae were noted crawling within the lower lip and left cheek region. Ultrasonography (USG) of the lower lip and left cheek region was advised. On USG, multiple linear tubular echogenic moving structures were noted in a subcutaneous plane in the left cheek and lower lip. In view of history of the removal of maggots, the above features are likely suggestive of multiple maggots in the present scan [].\nCotton gauze impregnated with oil of turpentine was placed in the deeply burrowed cavitation in the lower lip and left cheek region for approximately 10–12 min. Around 15 Maggots were removed manually with the help of curved artery forceps and were collected in a sample bottle as shown in . The same procedure was carried out for the next 3–4 consecutive days with exploration, curettage and irrigation with warm saline, hydrogen peroxide, povidone-iodine and metronidazole till no further larvae could be found. A total of 73 maggots were removed in a span of 3–4 days. The patient was administered injection ceftriaxone, injection pantoprazole, and injection metronidazole for 5 days. No attempt of administering the antiparasitic drug ivermectin was made.\nOn the 7th postoperative day, there was no evidence of maggots on repeat USG. A magnetic resonance imaging of the oral cavity (plain + contrast) was suggestive of minimally enhancing mucosal thickening along the gingivobuccal and gingivolabial sulcus on the left side likely of inflammatory etiology postoperative changes. However, the clinical examination confirmed a serious loss of the lip tissue with necrosed margins. Competence involving left commissure was lost and constant drooling of saliva was visible [].\nThe defect was reconstructed with Bernard's modification of Gillies flap. The flap consists of an incision along the perioral area around nasolabial fold [Figure and ]. The flap viability was maintained by preserving the vascular bundle. After accomplishing sufficient mobilization, the flaps were medially oriented to allow closure in three planes, namely oral mucosa, orbicularis muscle and skin maintaining the integrity of oral sphincter. However, there is some microstomia with minimal impact on function [].\nDiagnosis of myiasis causing larvae was done based on the form of spiracles, cephalon-pharyngeal skeleton, shape and size of developed larvae as shown in . The posterior spiracles, a distinct button, and the number of lobes on the anterior spiracles are six, which would give a definitive identification of Chrysomya bezziana. The robust spine bands are other indicative criteria of C. bezziana.\nBased on the mentioned diagnostic keys and comparing the whole shape of the larvae, posterior spiracles and cephalopharyngeal skeleton, the larvae were diagnosed as C. bezziana (Diptera: Calliphoridae). |
Patient presentation
A 21-year-old female college student with a history of asthma presented to the neurosurgery office for consultation complaining of mass on the left side of her skull associated with increasing size over the past two days and intermittent headaches for the past two to three weeks. The left-sided headache included her upper jaw. She also reported a history of cellulitis and urinary tract infections, in addition to surgical removal of an impacted wisdom tooth in 2016. Family history was positive for diabetes mellitus (DM) type II in both her father and her grandfather and colon cancer and coronary artery disease in her other grandfather. She admitted to drinking alcohol one to two times per week but denied use of tobacco and drugs. At the time, she was taking Viorele birth control to regulate her menses. Review of systems was otherwise negative.
Clinical findings
Physical examination revealed a well-developed, well-nourished female in no acute distress. She was awake, alert and oriented to person, place and time with a Glasgow Coma Score (GCS) of 15. A soft left frontal lesion associated with tenderness to palpation, without erythema or drainage, was palpated slightly off midline. Her cranial nerves II-XII were intact. Strength in both upper and lower extremities was five out of five bilaterally. No pronator drift was noted. Sensation to light touch was intact bilaterally in V1-3, upper extremity, and lower extremity distributions. Her reflexes were symmetric. Her gait was within the normal limits.
Imaging
CT of the head without contrast (Figure ) revealed an expansive soft tissue mass with beveled edges and dimensions measuring approximately 3.5 x 2.1 x 2.3 cm in the left frontal calvarium. Bony destructive changes of the inner and outer table of the left frontal calvarium were apparent. Extension of the mass into the dura was noted. The mass did not extend into the |
A 54-year-old male, suspected of a left kidney tumor, was urgently referred to our urology clinic. The ultrasound examination incidentally revealed the lesion and reported it as a 3 cm mass in the upper pole of the left kidney. The patient was asymptomatic: he did not report any weight loss, and the lesion was undetectable by palpation. He presented with several associated diseases, including intervertebral disc degeneration, but had no history of surgical treatment. Multiphase contrast-enhanced computer tomography () revealed a 31 × 28 × 31 mm mass at the upper pole of the left kidney, showing strong and relatively uniform contrast enhancement. The lesion was located between the left adrenal and anterior kidney surface. The length of the tumor-kidney contact area was 22 mm. No other abnormalities were detected. Both kidneys were normal and they excreted urine into nondilated pelvicalyceal systems. The chest radiographs were also normal.\nThe patient was admitted to our Urology Department for urgent surgical treatment of a left kidney tumor. Laboratory tests showed no abnormalities; creatinine level was 1.0 mg/dl (GFR: 82.90 ml/min/1.73 m2). At the preanesthetic assessment, the patient was classified as ASA1. Surgery was started using lumbar access. A 3.5 cm tumor was seen at the upper pole of the left kidney without apparent involvement of the kidney or other organs. Gross total resection was performed. The postoperative course was uneventful. The patient was discharged home in good condition three days after the surgery.\nThe histologic examination revealed an extrapleural solitary fibrous tumor (35 × 30 × 30 mm) with a Ki67 proliferation index of 5%, a mitotic activity of 1 MF/10 HPF, and positive immunostaining for CD34. There was no histologic evidence of necrosis ().\nA follow-up examination performed three months after the surgery consisted of laboratory tests, CT of the abdomen, and chest X-ray. No abnormalities were detected. The patient continues to come in for scheduled appointments at our urology clinic. |
A 49-year-old Japanese man whose gloved hand was caught between a gas cylinder and a concrete floor was taken to our hospital by ambulance. His right middle finger had been amputated in the distal interphalangeal (DIP) joint region, and his right ring finger was connected only by nerve and blood vessel bundles, showing a compound fracture of the distal phalanx (Figures and ). Our institute’s approach in cases of trauma is to inject tetanus toxoid and human anti-tetanus immunoglobulin (TIG) if the wound is dirty. In this case, no tetanus toxoid was injected since there was no soil contamination. The bone was comminuted, and the crushing was found to be severe, but replantation was considered appropriate, and replantation of the middle finger and osteosynthesis of the distal phalanx of the ring finger were performed on the day that our patient was injured.\nSurgery was performed under axillary block anesthesia. After irrigation with 500mL of warm saline, the DIP joint of the middle finger was fixed, and the ulnar-side finger artery and dorsal cutaneous vein were anastomosed. In the ring finger, the distal phalanx was reduced and fixed. Minimal debridement was applied to only a part of the skin.\nOur patient was admitted for observation. There were no infectious signs in his hand. His middle finger was successfully replanted, but his fingertip was partially necrotized because of crushing (Figure ), and so additional reconstruction with a reverse digital arterial flap was performed 15 days after injury. The wound of his ring finger healed completely 18 days after the injury. No particular symptoms developed and there was no problem with the condition of the flap, but aggravation of lower back pain, trismus, and convulsion suddenly occurred 21 days after the injury (Figure ). The wound of his middle finger was immediately inspected, and discharge of a whitish turbid exudate from the region around the flap was noted. The wound was opened and irrigated with a large volume of saline. No bacterium was detected on exudate culture, and isolation or identification was not possible. On the basis of the clinical symptoms, tetanus was diagnosed and treatment was initiated. TIG (6000 units) was intravenously administered on the day of onset, and 6000 units of TIG and 0.5mL of intramuscular tetanus toxoid were administered the following day. We administered treatment with penicillin antibiotics. However, the convulsions did not remit and, in fact, slowly became aggravated. Thus, tracheal intubation was performed, and our patient underwent artificial respiratory management. Anticonvulsant and sedative were concomitantly administered, but convulsion was readily induced by light stimulation, such as irrigation of the wound. The frequency and intensity of convulsive seizures started to decrease slightly at about 10 days after onset, and a tapering of the intravenous anticonvulsant injection was initiated. Our patient was weaned from the ventilator 12 days after onset. The distal phalanx fracture of his ring finger healed 6 weeks after the injury. Conservative treatment of the open wound of his middle finger was continued, and the wound healed 8 weeks after the injury. The fingertip morphology of his middle finger was relatively favorable, but owing to rest for tetanus treatment, rehabilitation could not be performed, and joint contracture remained in his right middle and ring fingers. No systemic problem occurred afterward, and our patient was discharged 12 weeks after the injury. |
A 59-year-old woman was admitted for recurrent urinary tract infection (UTI). Past history revealed right hip prosthesis and hysterectomy for cervical cancer at age 56, followed by radiotherapy. An extensive stricture of the left ureter had been treated with ileal loop substitution in a General Surgery environment at age 58. The procedure was performed in a general surgeon setting instead of a urologist because the patient lives in a peripheral area with first level hospitals. Three months later, due to worsening of left hydronephrosis, the patient underwent pyeloplasty with placement of a 'stent' of unknown characteristics using a flank approach. Ten months after the second procedure, the patient was evaluated at our institution for recurrent UTI and fever, with irritative bladder symptoms (frequency and urge incontinence). Serum creatinine was 1 mg/dL; urine culture revealed >1 million E. coli CFU (Colony Forming Units) per mL.\nAs a second step a plain X-ray of the abdomen was performed (Figure ), that showed presence of a stent-like radiopaque device on the left side, with its upper part projecting on the left kidney area and three short interruptions at its proximal third. Furthermore, a renal scintigraphy was performed, that revealed severely reduced (10%) left kidney function.\nIn order to get more detailed information about the morphology of the left urinary tract, CT urography was subsequently carried out using a 64-row CT scanner. Axial images, Maximum Intensity Projection and Volume Rendering (VR, Figure ) reconstructions showed instead a large tube with multiple bends and a radiopaque marker inside it, crossing through the entire collecting system of the left kidney; this latter was smaller than the contralateral one, due to chronic parenchymal failure.\nThe patient underwent left nephrectomy and removal of the ileal loop, containing a 32F partially encrusted Silastic drain with a radiopaque marker (Figure ). The general surgery setting can, even partially, explain the use of a Silastic tube instead of a standard urological stent.\nIn our case, CT proved to be superior to plain X-ray due to its higher contrast resolution and its ability to display a body volume as a stack of cross-sectional images, rather than as a projective representation of the attenuation of all tissues crossed by a relatively wide X-ray beam [,]. Moreover, CT allowed to accurately distinguish the Silastic drain tube from its centrala radiopaque marker, while 2D and 3D reconstructions (and especially, VR) yielded an accurate depiction of the tube structure and course. In particular, MIP views were useful to show the metallic marker in its entirety (by extracting voxels with the highest intensity inside the slab), while VR were more suitable to display the relatively hypodense tube coating, owing to the VR capability to use the information from all voxels for generating a 3D view of the anatomy under investigation [-]. |
A 39-year-old male patient with the chief complaint of left-side motor weakness arrived at the emergency department. He was alert with no unusual medical history or ongoing medications. Blood pressure was normal, and results of laboratory investigation did not indicate coagulation abnormality or other specific findings. The patient began to show symptoms nine hours before arrival at hospital.\nBrain computed tomography (CT) and brain CT angiography taken at our hospital showed a subarachnoid hemorrhage (SAH) in the right sylvian fissure and a right proximal middle cerebral artery occlusion (). A series of brain magnetic resonance images (MRIs) and digital subtraction angiography (DSA) showed an acute cerebral infarction in the right striatocapsular area due to proximal right middle cerebral artery (MCA) occlusion (). The patient had both ischemic stroke and SAH. No salient lesions that might be considered a cause of SAH were observed. Therefore, considering it a large-artery ischemic stroke, we established a treatment plan. Because more than nine hours had passed since the patient's symptoms developed, we decided that medical treatment through dual antiplatelet medication (aspirin 100 mg daily, clopidogrel 75 mg) would be better for the patient than any other treatments.\nOn the fifth day after admission, he had an episode of tonic-clonic seizure and brain CT after seizure showed increased SAH at the basal cistern and both sylvian fissures (). DSA showed a lesion, apparently a dissecting aneurysm (DA), in the right supraclinoid internal carotid artery (ICA), that had not been visible earlier (). Due to the patient's stuporous consciousness level, it was impossible to perform a balloon test occlusion. However, trapping of the lesion in the ICA was considered viable based on the “Allcock test” and “Matas test” results. Thus, internal coil trapping was performed on the right supraclinoid ICA lesion. Subsequently, lumbar drainage was performed, and his consciousness level improved to drowsy.\nOn the 11th day, the follow-up brain MRI showed that the size of the cerebral infarction had increased, although the patient's condition did not worsen until the following day, reverting to the stuporous consciousness level. CT scans showed an increase in the size of the cerebral infarction. Therefore, we performed a decompressive craniectomy.\nOn the day of the decompression, his pupil size was normal. The next day, his pupil size had increased with no light reflex. The brain CT showed low density in both hemispheres due to vasospasm (). The patient was diagnosed as brain dead, followed by organ donation and death. |
A 62-year-old male known to have hypertension and dyslipidemia, presented to our hospital with shortness of breath, fatigue and dry cough. Chest x-ray for the patient showed patchy infiltrates in the right lung. The patient was admitted under Internal Medicine service as a case of suspected COVID-19 pneumonia, which was later confirmed by polymerase chain reaction (PCR) test and was put on Enoxaparin 40 mg daily, IV fluids and acetaminophen upon admission according to hospital protocol. On day three of admission, patient started to have increased respiratory effort and was requiring oxygen support via high flow nasal cannula. He was then admitted to the intensive care unit (ICU), and his enoxaparin was increased to a therapeutic dose and was started on steroids, broad-spectrum antibiotics and intravenous fluid. A 16 Fr Foley’s catheter was inserted for monitoring urine output and was a routine measure in all ICU patients. After 3 days in the ICU the patient became stable and was transferred to general ward. He noticed that he has a constant painless erection that he thought it is caused by the Foley’s catheter and did not report it to hospital staff. Because of the high volume of COVID-19 patients and as a safety precaution, physicians did most of their rounds via Telemedicine video calls. Therefore, genital exams were only done when there is a complaint made by the patient. After spending 21 days in the hospital the patient was ready for discharge, and while removing the patient’s Foley’s catheter in preparation for discharge, the primary physician noticed the erection and the Urology team were consulted for suspected priapism. The patient reported that he has been having a constant erection for at least 10 days, which he noticed after Foley’s catheter insertion. He denied any history of, pain, trauma, use of erectile dysfunction (ED) medications or treatment. He denied any voiding difficulty after removing the Foleys or prior to his admission. Patient vitals were stable. On examination, patient had a hard erection, no skin changes were noticed, no discoloration or tenderness. Patient labs revealed leukocytosis of 18.6 mostly neutrophils and platelet count of 470. Aspiration with 18 French gauge needle was difficult due to increased blood viscosity however, we managed to withdraw about 10 ml, which had no effect on the erection rigidity (). Blood gases sent from the penis came back with PH = 6.86, pCO2 = 33.3 and, pO2 = 26.9 confirming and diagnosis of ischemic priapism. Phenylephrine 200 μg was then injected into the corpora cavernosa twice 3 minutes apart, and aspiration was continued until detumescence was achieved (). The patient was given prophylactic antibiotics and was discharged 2 days later. On follow-up 2 weeks later via phone call, patient was still under home isolation and reported having nighttime erections with desire, no sexual encounters or ejaculation and, having mild itching at the aspirationsite. |
This is a case of a 54-year-old male who was treated at 13 years of age with a combination of chemotherapy, bone marrow transplant, and whole brain and spinal axis radiation (cranial 24 Gray) spinal (12 Gray-Gy) for acute lymphocytic leukemia (ALL). At the of age 46 years, while the patient was being evaluated with MRI scans for intermittent headaches, it was discovered that the patient had developed two intracranial tumors radiologically consistent with meningiomas. The tumors were a right cavernous sinus tumor of 13 x 10 millimeters and a larger multilobulated left tentorial tumor of 13 x 11 x 12 mm with direct compression on the left pons. The repeat MRI scans over a period of five years demonstrated the continued growth of the tumors, especially in the left tentorial notch. At the age 51 years, although the patient had no neurologic symptoms, because of continued tumor growth, he was referred for radiosurgical treatment. At this time, the right cavernous sinus tumor was 19 x 14 mm with a tumor volume of 792 mm3 and contiguous to the right anterior brainstem, while the left tentorial tumor was more lobulated and 24 x 14 mm with a tumor volume of 2002 mm3. There was marked direct indentation and pressure on the left lateral pons, including the root entry zone of the left trigeminal nerve (Figure ).\nBoth the tumors were treated in three sessions of radiosurgery using the CyberknifeR (AccurayR, Sunnyvale, California, USA) with the right cavernous sinus/posterior-clinoid tumor receiving 12 Gy to the 77% isodose line and the left tentorial tumor receiving 18 Gy to the 85% isodose line in three fractions of 600 cGy each (Figure ).\nNine months after radiosurgery to the tumors, the patient started developing lancinating left facial pain without numbness. He initially responded to medical management with carbamazepine 100 mg and gabapentin 300 mg daily. However, gradually over the next 15 months, his pain recurred and it was unresponsive to the medical treatment, despite increasing doses of carbamazepine to 600 mg daily and gabapentin 1800 mg daily. With high doses of carbamazepine and gabapentin needed to control his facial pain, he developed side effects which left him unsteady and somnolent. The repeat MRI scans showed the left tentorial tumor had reduced to 11.86 mm. The MRI scan, 24 months after original tumor treatment revealed 3.13 mm residual tumor adherent to the left fifth cranial nerve root entry zone with no tumor in the trigeminal cistern. He did not want to consider balloon compression and underwent another radiosurgery treatment of 70 Gy to the left Gasserian ganglion. He responded with loss of pain and was on baseline medication of gabapentin 300 mg two times daily and carbamazepine 100 mg daily (Figure 3). |
An 81-year-old man with past medical history including atrial fibrillation, coronary artery disease s/p myocardial infarction and ventricular tachycardia/ventricular fibrillation s/p automatic implantable cardioverter-defibrillator placement was diagnosed with metastatic BRAF wild-type melanoma. Ipilimumab was initiated however two weeks after the third dose, the patient developed diarrhea deemed immune-related (confirmed by colonoscopy and muscosal biopsy). An 8 week course of steroids and two doses of infliximab eventually lead to resolution of the colitis. In addition, the patient experienced immune-related hypophysitis with secondary adrenal insufficiency and hypothyroidism. Management of colitis was complicated by bacterial pneumonia requiring two hospitalizations but eventually resolved.\nEleven weeks following the third and last dose of ipilimumab the patient developed progressive subacute shortness of breath. An extensive work-up including a computed tomography (CT) of the chest, bronchoscopy, transbronchial biopsy, and bacterial/fungal cultures ruled out a recurrent infectious etiology, other respiratory etiologies, and metastatic disease. Echocardiography showed a left ventricular EF moderately-to-severely reduced at 35 % from a baseline of 47 % as measured by myocardial perfusion single-photon emission computed tomography 14 months prior. The left ventricle was mildly dilated with normal left ventricular wall thickness. On physical examination the patient had an irregularly irregular heart rhythm, mild pitting edema, and bilateral basilar crackles in the lungs. He did not exhibit anginal symptoms. Cardiac enzymes and troponins were negative. He was treated with diuretics resulting in complete resolution of his respiratory symptoms. Though the patient clinically improved over time with diuretics, cardiac function did not return to normal with subsequent echocardiography documenting persistently reduced ejection fraction. Treatment with corticosteroids was not given due to the patient’s clinical improvement with diuretics and previous toxicity from a prolonged steroid course for his immune-related colitis.\nRestaging revealed initial tumor shrinkage however subsequent slow progression over an extended period of time. The patient went without treatment for over a year before initiating anti-PD1 antibody treatment with pembrolizumab. No further cardiac events were observed while receiving pembrolizumab. |
The cadaver was that of an 86-year-old Japanese female who died of a subcutaneous abscess. No macroscopic evidence of abdominal surgery was observed either on the body surface or in the abdominal or pelvic cavity. The abnormal branches from CT, SMA, and IMA were specifically observed during a student course on gross anatomical dissection at Aichi Medical University. The branching of the CT, SMA, and IMA was observed and recorded while paying close attention to the positional relationships of the CyA with these arteries. No intestinal malrotation was observed in the cadaver (Fig. a).\nThe CT had four branches: one each to the left gastric, right hepatic, splenic, and left hepatic artery (Fig. a, c). The left hepatic artery, which supplied the left hepatic lobe, originated 21 mm distal to the CT bifurcation and branched to the gastroduodenal and right gastric arteries and did not pass through Calot’s triangle (Fig. a, b). The right hepatic artery originated 15 mm distal to the CT bifurcation and ran along the dorsal side of the portal vein and common hepatic duct through Calot’s triangle and supplied the right hepatic lobe (Fig. a, b).\nThe CyA was branched from the SMA, which also branched the inferior pancreaticoduodenal, jejunal, ileal, ileocolic, and right colic arteries. The middle colic artery was not found in the branches of the SMA. The CyA originated 15 mm distal to the SMA bifurcation and branched 10 mm distal to the inferior pancreaticoduodenal artery. The CyA ran along the dorsal side of the portal vein through Calot’s triangle, and then distributed to the dorsal side of the gallbladder (Fig. b). The total length of the CyA was 130 mm. There was no obvious abnormality in vascular endothelial swelling, stenosis and thickness compared to normal cyst arteries.\nThe IMA branched to the left colic artery, sigmoid arteries and superior rectal artery. The left colic artery originated 35 mm distal to the IMA bifurcation and passed through the left colic flexure until the vicinity of the right colic artery supplied from the descending colon to the whole transverse colon (Fig. a). |
A 24-year-old female with diagnosis of Cushing's disease (ectopic ACTH syndrome) had undergone adrenalectomy in 2000. She was kept on low-dose steroids postoperatively. The patient started having pain in the left hip 2 years postadrenalectomy, for which she was given analgesics and calcium supplementation. Her BMD (T score: –3.3; lumbar spine and –2.6; proximal femur) and radiological examination suggested severe osteoporosis. She subsequently suffered pathological fracture neck of femur for which she was operated and valgus osteotomy was done. Postoperatively, she was advised alendronate 70 mg once a week with calcium supplementation along with low-dose steroid for post adrenalectomy supplementation. After 6 years, the patient again started having pain in the right hip and thigh. Her BMD was found to be improving (T score –1.1) and there was no radiological evidence of osteoporosis as well. She was advised analgesics, exercises, along with the previous treatment, but her pain did not subside. After 5 months, she had aggravation of pain and on radiological evaluation was found to be having pathological subtrochanteric fracture for which she was operated and internal fixation with long proximal femoral nail was done []. The fracture united at 10 months and pain in thigh and hip subsided []. The patient continued to take alendronate along with low-dose steroid for post adrenalectomy supplementation. Two years later the patient started having diffuse dull ache, insidious in onset, activity related pain in the right foot which did not subside completely with analgesics and physical therapy for 3 months. The radiographic evaluation showed incomplete, transverse, diaphyseal fracture of fifth metatarsal shaft with thickening of lateral cortex []. Patient's BMD showed normal mineral density (T score –0.7). She was advised plaster. Alendronate therapy was discontinued and teriparatide therapy 20 mcg subcutaneously was started and continued for 6 months. She improved and the fracture healed, and after 5 months, she was able to walk without walking aid and was doing all her household activities []. |
A 72-year-old man presented to the emergency department with pain in his right lower abdominal quadrant of 4 hours. His past medical history was significant for smoking (50 packs/year) and poorly controlled hypertension. He had no history of previous abdominal surgery. On admission, the patient had hypotension (84/46 mm Hg) and tachycardia (106 beats/min). Palpation of his abdomen revealed a large, tender pulsatile mass around the umbilicus.\nEmergency contrast-enhanced computed tomography revealed a giant infrarenal AAA extending to both common iliac arteries (\n) with evident rupture at the medial wall of the right iliac aneurysm (\n). This AIA measured 17 cm × 10 cm at the aortic bifurcation, 12 cm × 10 cm at the ruptured common right iliac artery aneurysm, and 9 cm × 8 cm at the left common iliac aneurysm. The aortic and both iliac aneurysms presented with extensive thrombus within the aneurysmal sacs. Both internal iliac arteries were patent.\nThe patient was immediately transferred to the operating theater.\nAn open transperitoneal approach was chosen. Through a midline incision, aortic cross-clamping was performed within a few minutes. Both external iliac arteries were dissected and controlled. Patent internal iliac arteries were controlled from within with inflatable balloons after opening the AIA. A\nY\n-shaped Dacron graft 18 mm × 9 mm was used. Proximal anastomosis at the infrarenal aorta was performed just below the renal arteries. Distal anastomoses were performed in an end-to-end fashion with the external iliac arteries. The right internal iliac artery was oversewn from within at the ostium, while the left was revascularized via a jump graft from the iliac limb of the bifurcated graft. The jump graft was anastomosed in an end-to-end fashion to the ostium of the left internal iliac artery.\nAt the end of the procedure, the sigmoid colon had no signs of ischemia. During surgery, the patient was stable and received 8 units of packed red blood cells.\nHis postoperative course was uneventful and he was discharged from hospital on postoperative day 8. The patient continues to do well 3 years later. The last follow-up ultrasound imaging at 3 years from surgery revealed normal findings (\nand\n). |
A 50-year-old woman was admitted to our hospital in June 2013 owing to back pain radiating to the lower left abdomen for 2 months with progressive exacerbation. Physical examination revealed a tender point on the back, paraparesis with motor strength of 4/5 in both lower limbs, and decreased left inferior abdominal wall reflex. Magnetic resonance imaging (MRI) revealed destruction of the T11 vertebra and a soft tissue mass. To prevent rapid neurological deterioration owing to tumour growth, local curettage was planned using the posterior approach. However, pathologic examination of the neoplastic specimen using instant frozen section showed that the tumour was more likely to be malignant; thus, the involved vertebral body and upper and lower intervertebral discs were completely resected. Thereafter, spinal reconstruction was performed with a screw system and titanium mesh.\nFollow-up computed tomography (CT) showed tumour recurrence at 14 months after surgery (Fig. a-c). The patient was advised to undergo surgical treatment again, which she refused owing to lack of obvious discomfort.\nTwenty months after surgery, the patient was readmitted owing to back pain. On admission, radiography, CT, and MRI showed lytic bone destruction at the left edge of the T12 vertebra with a huge soft tissue mass shadow in the left thoracic cavity (Fig. a-f). The tumour volume was too large to be safely removed; thus, conservative treatment of denosumab was performed. After 1 year of denosumab therapy, the tumour growth was controlled, and its edges from the T10 to the T12 vertebral body were markedly calcified, and its boundary line became clear (Fig. a-i).\nTo prevent tumour recurrence, three-level TES was performed after 16 months of denosumab therapy. A 50-cm incision was made from T7 to L3 at the posterior median line. Twelve pedicle screws of appropriate length were implanted on both sides of the T7–9 and L1–3 vertebrae. The ribs of the right T10-T12 and the left T7-T12 were excised, and tumour tissues were separated. The T9-T10 and T12-L1 intervertebral discs were excised to divide the tumour, and the tumour and spinal tissues at the T10-T12 level were extracted from the left side of the spine (Fig. a-c). The tumour was approximately 20 × 15 cm in size, pale yellow, and hard. After resection of the tumour tissue, the left lung and diaphragmatic muscle tissues were explored and found to be severely compressed by the tumour. Some lung and diaphragmatic tissues were damaged. During the surgery, the thoracic surgeons performed emergency repair of the lung and diaphragmatic tissues. Next, two titanium rods were placed on both sides of the T7-L3 vertebrae. The cylindrical titanium mesh with appropriate length implanted into the autologous ilium was placed between T9 and L1. The pathological diagnosis was GCT of the bone (Fig. a-c).\nSix-month postoperative radiographic examination revealed that the implant was in a good position and showed no loosening. The patient was instructed to wear a thoracolumbar brace for 3 months postoperatively.\nAt the last follow-up (32 months after the second surgery), the patient had recovered well and could work normally. Moreover, no local tumour recurrence was observed on MRI and radiography, and three-dimensional CT showed successful biological reconstruction of the spine (Fig. a-d). |
A 79-year-old female with a past medical history of coronary artery disease (CAD) and coronary artery bypass grafts (CABG), bioprosthetic mitral valve replacement (MVR), hypertension, hyperlipidemia, obstructive sleep apnea, and atrial fibrillation presented to the clinic for the evaluation of worsening dyspnea. She underwent transthoracic echocardiogram (TTE) that revealed mild left ventricular hypertrophy, interventricular septum about 1.4-cm thick and normal left ventricular ejection fraction. In addition, she was found to have severe AS at outside hospital, thought to be causing her symptoms and was referred to us for TAVR evaluation.\nAfter she underwent a comprehensive evaluation and reviewing her treatment options by the heart team, TAVR was recommended as preferred treatment. She had preprocedural computed tomography of chest/abdomen/pelvis for evaluation of access sites and for procedural planning. Her preprocedural transesophageal echocardiogram (TEE) revealed no thrombus in left atrial appendage and normally functioning mitral valve prosthesis and severe AS with peak/mean gradients of 67/44 mm Hg respectively, across her aortic valve (Figure ).\nShe subsequently was scheduled for and underwent transfemoral TAVR using a 26-mm Edwards Sapien S3 TAVR valve. Bilateral femoral arterial access was obtained and TAVR valve prepared, aortic valve crossed with a wire in usual fashion and TAVR valve positioned across the native aortic valve without any difficulty. Rapid ventricular pacing was initiated and after confirming no loss of capture and no premature ventricular contractions (PVCs) with adequate reduction in systolic blood pressure to 45-50 mm Hg range, the valve inflation was done. During the slow inflation of balloon expandable TAVR valve, the valve appeared to move a little (Video ).\nShortly after the withdrawal of valve delivery system and balloon into the descending thoracic aorta, the implanted TAVR valve embolized into ascending aorta. All the available options were thought of and it was carefully withdrawn into the aortic arch and positioned just after the great vessels with a inflated Edwards 25-mm balloon aortic valvuloplasty (BAV) catheter (Video ).\nThen, using the same Edwards 25-mm BAV catheter, valvuloplasty was performed x 2 to plan for second TAVR valve implantation but the BAV balloon water-melon seeded repeatedly during inflation (Video ).\nAlternate reasons for such occurrence like loss of pacing capture, PVCs or inadequate drop in blood pressure to <50 mm Hg were excluded. The intraprocedural TEE revealed normally functioning bioprosthetic mitral valve and thick valve struts encroaching on the non-calcified LVOT as shown (Video ).\nWe concluded that the rigid struts of bioprosthetic mitral valve encroaching on the left ventricular outflow tract (LVOT) likely resulted in TAVR valve embolization and further attempts at TAVR valve implantation were aborted. She was discharged from hospital uneventfully and clinically followed and underwent SAVR two months after the index TAVR procedure with a 23-mm Carpentier-Edwards bovine pericardial tissue valve. At the time of SAVR, her TAVR valve in aortic arch was visualized and it had a freely mobile echodensity attached to the leaflet consistent with thrombus. She recovered well from surgery and discharged on warfarin anticoagulation. At clinical follow-up four months after the surgery, she was doing well with no other complications. |
A 73-year-old woman presented to her family physician with an unexplained drop in weight from 90 to 65 kg in the last 5 months, upper abdominal pain and nausea, acholic stools, and jaundice. She had a history of depression, osteoarthritis, dyslipidemia, a previous laparoscopic cholecystectomy 15 years earlier, and a mesenteric venous thrombosis. She declared a smoking history of 23 pack-years, consumed less than 1 alcoholic drink per week, and denied any use of illicit drugs. The patient's family history was significant: her mother had gall bladder cancer and two maternal aunts had liver and lung cancers, respectively.\nTwo endoscopic retrograde cholangiopancreatographies (ERCPs) were performed which revealed a common bile duct stricture – a “fuzzy” possibly mass-like area in the adjacent pancreas – and a common bile duct stent was placed to relieve the obstruction. The stomach appeared unremarkable on standard and retroflex views. Further imaging determined that the “fuzzy” appearance of the pancreas was likely due to a perforation from the first ERCP and not a mass.\nThree months later, following an episode of cholangitis, she underwent a choledochojejunostomy that revealed an unresectable pancreatic mass extensively infiltrating the root of the small bowel mesentery. A biopsy of the mesentery extension of the mass confirmed a well-differentiated adenocarcinoma. Imaging confirmed her disease as T4N1M0 adenocarcinoma of the uncinate process of the pancreas. Worsening symptoms suggestive of GOO led to another ERCP; it revealed no significant evidence of duodenal obstruction up to the fourth section of the duodenum so a duodenal stent was not placed.\nAs a result of her advanced disease the patient was referred for consideration of palliative radiotherapy to relieve her gastrointestinal symptoms. At that time she had lost an additional 6 kg in the past month, and was admitted to hospital with an Eastern Cooperative Oncology Group (ECOG) performance status of 3. She took regular venlafaxine, risperidone, ramipril, rosuvastatin, furosemide, potassium chloride, and dalteparin, and had recently been prescribed senna glycoside, lactulose, docusate, hydromorphone, dexamethasone, and metoclopramide. She complained of aching pain across her epigastrium and upper abdominal quadrants, anorexia, and fatigue. While following a predominantly liquid-only diet, she was suffering from new bouts of projectile vomiting, nausea, and distention. In a 3-week period, her symptoms had worsened to the point where she could barely tolerate oral solids or liquids. On examination she was obese and tender over the epigastrium but devoid of distinct palpable masses. Her abdomen was somewhat tympanic but body habitus obscured more detailed findings.\nShe underwent a CT scan for radiation planning which surprisingly revealed profound dilatation of the stomach and proximal duodenum (Fig. , ) with extrinsic compression and likely invasion of the duodenum by the uncinate mass. She was diagnosed with malignant GOO, and several liters of fluid were urgently removed via nasogastric tube suction. In retrospect, it was thought that the obstruction had progressed from transient to complete since the time of her last endoscopy 13 days previously that had ruled out obstruction. Radiation therapy was cancelled and a gastrojejunostomy was performed to bypass the obstruction and relieve her symptoms.\nA week later, her symptoms had resolved and she was able to consume solid foods. She was discharged home and began palliative gemcitabine. Unfortunately, the patient later became too weak to complete her third cycle, and 4 months following the diagnosis of GOO she passed away in a hospice. |
An 82-year-old man complained of serious backache accompanied by radiating pain to the right lower extremity.\nThe patient presented to a local hospital for acute exacerbation of chronic lower back pain 3 mo ago. Magnetic resonance imaging (MRI) revealed a high signal area in T2-weighted images of the vertebral bodies of L3 and L4, with the L2/3 presenting corresponding segment canal stenosis (Figure ). However, the patient did not exhibit any obvious lower extremity neurological symptoms. Therefore, PVP was performed based on the diagnosis of OVCF (L3, L4). Unfortunately, bone cement leakage occurred during the surgery, which led to severe neurological symptoms. Computed tomography (CT) revealed that the right intervertebral foramen of L3/4 had a high density shadow (Figure ). Leaked bone cement compressed the root of the L3 nerve, which caused a stabbing pain radiating to the skin of the anterior-lateral thigh. A local surgeon administered an epidural steroid injection that can temporarily alleviate neurological symptoms. However, after 2 d, the patient’s pain recurred and was unbearable. The patient was transferred to our hospital and complained of lower back pain accompanied by radiating pain in the right thigh. The patient was maintained in bedridden status because even light activity resulted in unbearable pain. We therefore performed a partial facetectomy to retrieve the leaked bone cement using a posterior approach. Following this procedure, the neurological symptoms completely resolved, although slight backache persisted. The patient was subsequently discharged from the hospital.\nAfter 3 mo, the patient experienced serious backache accompanied by radiating pain to the right lower extremity and revisited our hospital. The radiating pain was mainly located from the back of the thigh to the inner side of the shin. The visual analog scale score was 8.\nThe patient had a previous diagnosis of high blood pressure and coronary heart disease.\nThe patient had been drinking for about 20 years, intaking about 200 mg per day. He had no history of smoking and no significant family history.\nNumbness and hypoesthesia at the medial lateral of the right shin skin, and the muscle strength of the dorsiflexion ankle decreased to level 4. A positive Lasegue’s sign on the right lower extremity was noted. Bilateral patellar and Achilles tendon reflex could not be evoked. Perianal sensation and anal sphincter muscle strength were normal. Babinski sign was negative.\nTroponin-T and N-terminal pro-brain natriuretic peptide were normal. Arterial blood gas analysis revealed partial pressure of blood oxygen was 81.7 mmHg.\nMRI revealed a right lumbar disc protrusion located at L3/4 and spinal canal stenosis at L2/3 (Figure ). |
An 86-year-old female with a BMI of 33 kg/m2 presented to the operating room to undergo a thyroidectomy for removal of a large suprasternal goiter. The thyroid mass limited her neck ROM and obstructed her airway by causing a tracheal deviation as seen on the computed tomography exam. This indicated that she would have a “difficult airway” even though the rest of her airway indices were normal. Specifically, her airway exam showed she had a Mallampati class II airway with an oral aperture of 3 fingerbreadths and a thyromental distance of 3 fingerbreadths. Since airway management was expected to be difficult, she was given 0.2 mg of IV glycopyrrolate to dry secretions, 50 mcg of fentanyl for patient comfort, and a 4% lidocaine nebulizer to topicalize her posterior pharynx prior to an awake intubation using a 5.2 mm inner diameter flexible fiberoptic bronchoscope (FFB) in order to optimize maneuverability. Within two attempts, her vocal cords were visualized and the 6.0 mm ETT was advanced through them. The finesse and maneuverability of the FFB helped to prevent hemodynamic instability by decreasing the amount of force and, presumably, number of direct laryngoscopic attempts that might have been needed if a conventional laryngoscope blade was used. The patient underwent surgical thyroidectomy without complications and was extubated awake, following complete neuromuscular blockade reversal, when she could follow commands – open her eyes and squeeze the clinician’s hand.\nBoth case examples demonstrate a common occurrence faced by anesthesia clinicians in the general operating room arena – patients appearing to have straightforward airways during their preoperative examinations often receive conventional airway management rather than the incorporation of specialized airway devices (SADs). Although in young and middle-aged patients this is generally deemed to be an appropriate approach, among elderly patients it could lead to undesirable events. A common complication related to difficulties with BMV is inadvertent insufflation of the abdomen with a concomitant heightened risk for aspiration. Moreover, during multiple laryngoscopic attempts, hemodynamic instability might ensue placing the elderly patient at an increased risk for myocardial ischemia. On the other hand, patients with expected airway difficulties receive SAD that limit the risk for adverse events during the induction phase of anesthesia. In Case 2, an awake FFB, using minimal sedation, was used to intubate, which not only limited the risk of aspiration by avoiding BMV but also enhanced maneuverability of the ETT, limiting the risk of a sympathetic response (eg, tachycardia and hypertension) to laryngoscopy, which occurred in Case 1. Accordingly, an increased awareness among anesthesia care providers and the formation of more appropriate “standard” or specialized techniques should be considered to decrease the risk of complications during airway management in the elderly surgical patient.\nElderly patients are prone to structural and functional changes surrounding the airway, including, but not limited to, an edentulous mouth, oropharyngeal tumors, atrophy of the glottic muscles, and decreased neck ROM ( for comparison with a younger patient).– These attributes may make it more difficult to BMV and/or intubate during airway management. In addition, age-related comorbidities such as COPD, gastroesophageal reflux disease (GERD), and diabetes increase the risk of aspiration pneumonia., Diminished cognitive function from Alzheimer’s disease may impair the patient’s ability to cooperate during an awake FFB if the airway is deemed to be difficult. Problems during airway management may be isolated to a specific aspect of care, or may include challenges related to a combination of tracheal intubation, BMV, oxygenation/desaturation, and aspiration.\nIn this review, we provide the reader with a comprehensive overview of the anatomic and physiopathologic changes that may impact airway management of the elderly surgical patient. Finally, we discuss various airway devices () and corresponding management strategies () that may be considered in the armamentarium of the anesthesia care provider who oversees the perioperative course of elderly surgical patients in order to help minimize the risk of complications related to airway management in this vulnerable and growing surgical population. |
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