text
stringlengths 746
31k
|
|---|
A 60-year-old man with alcoholic hepatic cirrhosis and ascites developed perianal epidermoid cancer, for which he underwent combined chemotherapy and radiation therapy with good initial response. Nine months later, he was found to have an enlarging lesion in his perianal region, which upon biopsy was shown to represent a recurrence. Abdominoperineal resection was performed, with malignancy-negative margins. At the time of this surgery, 4 L of ascites were evacuated. Because of prior radiation, the patient's perineal tissues were extremely frail. Although primary closure of the perineal wound was possible at the time of the abdominoperineal resection, the patient began to leak ascitic fluid through the wound soon on postoperative day 2.\nOn postoperative day 3, a reinforcing line of cutaneous sutures was placed in an attempt to control the process, but this ultimately proved inadequate. A concurrent medical therapy aimed at decreasing ascites was undertaken, and consisted of a regimen of diuretics and paracentesis sessions.\nBy postoperative day 5, the leak was so severe that frequent bedside dressing changes (every 2–4 hours) were inadequate, and VAC dressing was applied to an open anterior area of the perineal wound (Fig ). It was hoped that the VAC would contribute to closure by preferentially channeling the flow of ascitic fluid and protecting the remainder of the wound, and that the subatmospheric pressure would promote “collapse” of the perineal tissues and subsequent gradual wound closure.\nInitially, it was hoped that the patient would be able to tolerate limited ambulation while on VAC therapy. However, every time he stood or sat up, more than 1 L of ascitic fluid drained with gravity and overwhelmed the drainage capacity of the VAC device and canister. With each such occurrence, complete VAC dressing change was required. Therefore, it was decided to place the patient on strict bedrest. This maneuver decreased the fluid drainage from 400 mL on day 2 to less than 30 mL on day 4 of VAC therapy. At this point, the patient was allowed to ambulate, and no further drainage was noted. The wound appeared to have sealed and was granulating well.\nThe patient was discharged to home on postoperative day 12, and continued to do well with continued VAC therapy as an outpatient. He had no further adverse events or any ascitic fluid leaks at the 3-month follow-up. |
A 46-year-old Caucasian female patient with a dermatological history of actinic keratoses and rosacea and a positive family history of melanoma in her paternal uncle presented to a dermatologist for evaluation of a skin lesion located on the dorsal aspect of the left third finger overlying the proximal interphalangeal joint that had been present for the last 11 years (Fig. ). The patient had treated this lesion intermittently with over-the-counter wart medication, without any significant improvement. Over several months leading up to presentation, the lesion had become increasingly irritated, erythematous, and pruritic. A shave biopsy was performed at the time of presentation, with dermatopathology revealing hyperkeratosis, papillomatosis, and acanthosis with the top of a dermal cystic lesion lined by the dysplastic epithelium forming papillary fronds without stroma plus focally cystic comedo necrosis, consistent with an aggressive DPA (Fig. ). The lesion extended to the deep tissue edge of the biopsy specimen. Re-examination of the patient following diagnosis failed to identify lymphadenopathy or hepatosplenomegaly.\nThe patient was referred to hematology/oncology services for further evaluation of the malignancy. Physical examination revealed no evidence of metastatic disease, but the length of time that the lesion had been present provided some cause for concern. WLE versus digital amputation was discussed at length, with the decision to treat with WLE plus skin grafting for closure and left axillary lymph node dissection by a combination surgical oncology and orthopedic surgery services (Fig. ). Sentinel lymph node biopsy (SLNB) was negative at that time. Assessment for potential metastatic disease revealed two-subcentimeter lung lesions on computed tomography scan of the chest, which were monitored with serial computed tomography imaging every 3 months for 1 year without significant changes. The patient was also seen by her dermatologist every 3 months for 1 year following initial treatment and has been returning for annual skin checks since.\nSix months following the lymph node evaluation, the patient presented to her surgeon complaining of some difficulty using her left hand. Physical examination revealed mild to moderate lymphedema in the left upper extremity. She was referred to physical therapy for active and passive range of motion and supportive care, including compressive bandages and elevation. On re-evaluation 4 months later, the patient's functional status had significantly improved; she was using her hand without difficulty and minimizing edema with compression. To date of this report, the patient has not suffered any other complications from treatment nor has metastatic disease become evident in follow-up examinations. |
In August 2013, a 63-year-old woman presented with a gradual onset of decreased exercise tolerance, dyspnea, and a heavy feeling in the chest. Otherwise she was in good health with no relevant concomitant diseases. She denied fever, hemoptysis, weight loss, or other constitutional symptoms. There was no drug, alcohol, or nicotine abuse. She took no medication and there was no recent surgery or trauma. On physical examination we found a decreased breath sound over the right hemithorax and dullness to percussion at the right side of the thorax. There were no signs of peripheral oedema or ascites. She did not have any peripheral lymphadenopathy or enlarged spleen and liver. Laboratory values were normal for a white blood cell count of 5.9 × 109/L, a hematocrit of 44%, and a platelet count of 192 × 109/L. Her creatinine was within normal range and albumin was 42 g/L, with a total protein of 76 g/dL. Furthermore lactate dehydrogenase and tests for glutamic-oxaloacetic transaminase, serum glutamic-pyruvic transaminase, alkaline phosphatase, and bilirubin were all normal. The posteroanterior chest radiograph on admission confirmed a large right pleural effusion (). On admission, the effusion was drained showing a turbid and milky white appearance. Biochemical tests on the fluid showed a high triglyceride content (79 mmol/L) in the form of chylomicrons with cholesterol levels as low as 4.6 mmol/L. Further cytological analysis showed the presence of lymphocytosis but no malignant cells. Flow cytometry confirmed the absence of B-cell or T-cell lymphoma. Further imaging with PET-CT was initiated searching for the underlying disease. This revealed a PET positive bulky intraperitoneal tumour around the abdominal aorta and pancreas extending to the pelvic inlet (). There was a heterogeneous contrast enhancement and clear encasement of nearby arteries and veins. No enlarged mediastinal lymph nodes or suspect gynaecological malignancy was seen. In our case, the disruption occurred apparently below the fifth thoracic vertebra, and with the thoracic duct being on the right side of the vertebral column a right-sided chylothorax developed. The differential diagnosis of lymphoma or pancreatic carcinoma was made. Biopsy from the large epigastric mass revealed a mixed lymphoid cell population with a significant amount of CD 20 positive B-cells next to a small population of CD 3 and CD 5 positive T-cells. CD 23 staining was negative and only very few neutrophilic granulocytes showed CD 15 positivity. In the CD 30 staining there was no increase in blast cells. Fluorescent in situ hybridization detected IGH/bcl2 translocation and the final diagnosis follicular lymphoma grade 1-2 was made. Consequently a bone marrow biopsy was planned showing no arguments for lymphoma localization or stage IV disease. In conclusion she was diagnosed with a stage IIE retroperitoneal follicular lymphoma. Despite the high tumor burden, only her age (>60 years) was an adverse prognostic factor according to the FLIPI index. The patient is considered low risk with an overall survival at 10 years that is estimated to be 70%. Although the FLIPI is a validated prognostic index for follicular lymphoma, there is no consensus on any of the treatment approaches. In an initial attempt to reduce chyle production the patient received an oral diet with medium-chain triglycerides (MCT) [, ]. Unfortunately, the pleural effusion and its associated respiratory symptoms reoccurred. After discussion at the multidisciplinary board we decided to treat her with low dose radiotherapy. We argued that her low FLIPI indicates that the optimal treatment has to avoid toxicity and preserve quality of life.\nAfter CT-based simulation she was treated to a total dose of 4 Gy given in 2 fractions of 2 Gy per fraction over 2 consecutive days. Radiation was delivered with a linear accelerator with 6 MV photons and anterior-posterior/posterior-anterior (AP/PA) fields (). The low irradiation dose was chosen because of the large treatment volume and no clear known dose-response relationship to obtain a good long-term control of symptoms [, ]. We decided to limit the treatment volume to the abdomen. We mention one case report of a patient with lymphoma-associated chylothorax where chemotherapy resulted in good response for mediastinal lymphadenopathy but progressive disease in the abdomen. Noteworthy, also the pleural effusion remained present []. As expected, treatment was tolerated excellently with a clear clinical response four weeks after radiotherapy. Until present, she has no respiratory problems and even rides her bicycle for 40 kilometers without any problems. Two months after treatment restaging CT showed a partial remission of abdominal lymph nodes and no presence of chyle fluid in the thorax. Since she remained asymptomatic we decided to defer further systemic treatment. Of note, no recurrence of pleural effusion occurred during the complete 6 months of follow-up after radiotherapy. |
A 50-year-old woman was referred to the colorectal surgery clinic for abdominal discomfort and chronic constipation. She had a past history of pelvic endometriosis. At consultation, she complained of chronic constipation, abdominal and perineal discomfort, and incomplete evacuation. She had to spend a long time on the toilet and strain intensely before opening her bowel. Frequently, she had to push on the posterior wall of her vagina or use her finger to empty her rectum. She had to use daily laxatives and occasional enemas; nonetheless, she could open her bowel less than once a week. Our initial impression was ODS. In fact, her Longo's score was 22, and her symptoms severity score was 22 as well.\nAt physical examination she had perineal descent with no external rectal prolapse and no external hemorrhoids, but she had rectoanal intussusception, a redundant anterior mucosa and a 5-cm anterior rectocele. Her sphincter tone was fine, and her squeezing pressure was slightly reduced. Magnetic resonance imaging confirmed perineal descent and a rectocele. Endoanal ultrasound found a small anterior sphincteric tear, probably due to obstetric trauma. Rectal manometry was normal. With the diagnosis of ODS, she was referred for pelvic physiotherapy. After 6 months of rehabilitation, she had some slight, subjective improvement, but her Longo's score was still 22. To rule out a recurrence of her endometriosis or any other pelvic disease, she had diagnostic laparoscopy, which was completely negative.\nA STARR procedure was offered and accepted. This was straightforward, with no intraoperative complications. At the end of the surgery, the rectal suture was 5 cm above the dentate line and was complete. No bleeding points were noted. A 5-cm prolapse was removed (). An anal absorbable tampon with a Foley catheter was left into her rectum, as usual. During the first night after the surgery, she had minimal rectal bleeding, which continued over the first postoperative day. She had some perineal and pelvic discomfort and was quite pale and tachycardic. Blood tests on postoperative day 2 showed a significant drop in her hemoglobin levels from 147 to 88 g/L. Fluid resuscitation was started, and an urgent abdominal and pelvic CT scan showed a huge extraperitoneal and retroperitoneal pelvic hematoma. No active arterial bleeding was demonstrated at the arterial phase. The anastomotic ring was complete, but a small amount of free gas in the peritoneal cavity was noted just above the bladder (). A diagnostic laparoscopy revealed no free gas, free fluid, blood or enteric contamination in the abdomen and no sign of peritoneal inflammation; only a vast extraperitoneal hematoma of the pelvis tracking the mesorectum and mesosigmoid up to the mid sigmoid and the lateral pelvic walls was found (). A flexible sigmoidoscopy showed a very small breakdown of the rectal suture (), which was closed with endoclips.\nThe patient responded well to conservative treatment with transfusions and laxatives, although she complained of perineal pain for more than 2 weeks. She was always clinically stable and non-septic. She was discharged in good conditions 28 days after the first operation. At the 30-day postdischarge follow-up, the patient was completely pain free, and her Longo's score was back to 14. |
A 54 year old Caucasian woman presents to the Emergency room with progressive fatigue and proximal muscle weakness for two months. She had history of hypertension, type 2 diabetes mellitus and hyperlipidemia. She was on atorvastatin for the last 4 years. Her initial blood work was flagged with markedly elevated CK levels of 16,000 Units/Liter, which triggered admission for statin induced rhabdomyolysis. Her CK levels did not improve and her symptoms continued to worsen despite aggressive hydration. Eventually an MRI of the lower extremities was done which showed multifocal edema and enhancement of bilateral thigh muscle. Suspecting inflammatory myositis, a biopsy of the thigh muscle was done which showed mild to moderate necrotizing myopathy with myofiber degeneration and necrosis and myophagocytosis highlighted on the acid phosphatase, MHC-1 and CD68, CD8, CD3 stains. Serology showed positive ANA, HMC-CoA ab and anti-Ro antibodies. Diagnosed as anti-HMG-CoA type of Immune mediated necrotizing myopathy, the patient was trialed on methotrexate (MTX) 15mg and prednisone 40mg orally without improvement and severe gastrointestinal upset after the MTX. The patient was then started on high dose pulse methylprednisolone 1g for 3 days followed by intravenous immunoglobulins (IVIG) for 5 days. Her CK dropped from 16,000–3000. However, her condition was complicated by diabetic ketoacidosis (DKA) and mild oropharyngeal dysphagia noted on fluoroscopic study. A pulmonary function test (PFT) showed mild restrictive disease. A transthoracic echocardiogram (TTE) showed no cardiomyopathy. With mild improvement of her symptoms, the patient was discharged on azathioprine 50 mg and prednisone 60 mg orally to taper. She was discharged to a sub-acute rehabilitation center with significant objective improvement in terms of muscle strength and muscle endurance. The patient was then scheduled for a biweekly IVIG infusion with a goal to taper off prednisone, and eventually space out IVIG infusion and continue on azathioprine for long term management. After 6 months of treatment including rehab and home physical therapy, her CK levels dropped to 155 Units/Liter and she went from a Hoyer lifter to walking with a cane. |
In 1998, a 17-year-old male reported to an ophthalmologist with a complaint of visual disturbances, and ended up with the diagnosis of Marfan syndrome (MFS). Eleven members of his family were diagnosed with MFS by 2008 and as the children were born, this number was raised to 17 members by 2018 [].\nMFS diagnosis was established according to Ghent Nosology criteria for 17 of 21 members of this family. We believe such a high genetic penetrance is a rare entity []. Further molecular and genetic tests confirmed the diagnosis of MFS with a high level of confidence. For 20 years they went under periodic examinations. They were followed up by a single cardiologist and a single ophthalmologist.\nCardiovascular involvements were seen in 16 members of this family, representing for more than 90% of affected individuals. The most common pathology was mitral valve disease (including mitral valve prolapse and regurgitation) followed by aortic root dilation. Three patients were operated electively for aortic root replacement and one patient underwent urgent surgery for aortic dissection via the Bentall procedure [details are shown in ].\nIn this study, ocular manifestations included lens ectopia (100%), flat cornea (23%), on gated eyeball (29%), hypoplastic iris or ciliary muscle hypoplasia (41%), glaucoma, cataract, and retinal detachment. Four patients needed ocular surgery [details are shown in ].\nUnfortunately, during the 20 years, three members were deceased including two patients and one healthy individual which indicates a mortality rate of 11% among our MFS patients (2/17).\nAlthough it is well known that cardiovascular pathologies are the leading cause of death in patients with MFS; nevertheless, no individual in this family died following cardiovascular events.\nThe patients died in two separate car accidents while driving, which given to their history of ocular pathologies makes us think acute visual disturbances as a possible cause of trauma and eventually death. |
A 30-year-old Japanese man visited his doctor with the complaint of epigastric pain. Abdominal enhanced computed tomography (CT) revealed a hypovascular tumor in his pancreatic body. He was referred to our hospital for further examination and treatment. He had no significant past medical history and no surgical history, but had a family history of pancreatic cancer (his uncle had had this disease). Although he had no history of smoking, he regularly consumed alcohol.\nOn examination, his abdomen was soft and flat, without any evident mass or tenderness. His laboratory data were unremarkable, except for the carbohydrate antigen 19-9 (CA-19-9) level, which was elevated to 139 U/mL. Abdominal enhanced CT revealed an 18-mm hypovascular tumor in the pancreatic body and a dilated main pancreatic duct in the tail side of this tumor (Figure ). The tumor was compressing the splenic vein. Endoscopic ultrasound showed a 15-mm low echoic tumor that had ill-defined borders and was located next to the splenic vein. Endoscopic retrograde pancreatography demonstrated disruption of the main pancreatic duct in the pancreatic body (Figure ). We diagnosed PDAC with invasion of the splenic vein and performed distal pancreatectomy, lymph node dissection, and splenectomy.\nMacroscopic findings of the resected specimen showed a 35 mm × 18 mm tumor in the pancreatic body and no other lesion (Figure ). Pathological findings revealed a moderately differentiated tubular adenocarcinoma with invasion of neutrophil in the main lesion (Figure ). The tumor had infiltrated the tunica externa of the splenic vein. At a 20-mm distance to the tail side from the main lesion, there was a 0.6-mm cancer nest, which was a moderately differentiated adenocarcinoma with invasion of neutrophil (in resemblance with the main lesion) (Figure ). There was no connection to the main lesion, and we diagnosed this small lesion as intrapancreatic micrometastasis of PDAC. The patient was administered 5-fluorouracil and heparin-based infusion chemotherapy combined with cisplatin and mitomycin C (PI4W) as perioperative chemotherapy[], and was discharged without any complications. He was administered S-1 (tegafur, gimeracil, and oteracil potassium combination) for 6 mo as adjuvant chemotherapy. However, he developed a recurrence in the liver 6 mo after surgery and underwent FOLFIRINOX therapy following GEM and nab-PTX therapy. Nonetheless, he died 25 mo after surgery. |
A 77-year-old female with a medical history significant for hypertension presented with left upper quadrant abdominal discomfort for 1 day accompanied by generalized weakness. She denied any fever, chills, night sweats, weight changes, chest pain, shortness of breath, palpitations, hematemesis, melena, hematochezia, diarrhea, or constipation. Her physical examination was unremarkable except for a large hard, nontender mass palpable in the abdomen's left upper quadrant. She underwent CT scan of the abdomen with contrast material which showed a 13.7 × 11.5 × 12.9 cm mass in the left mid and upper abdomen with an irregularly enhancing rim (Fig. ). The mass was predominantly fluid in density. The origin of the mass was not clear on a CT scan of the abdomen. The initial impression was that the mass could be a large mesenteric cyst versus pancreatic pseudocyst. Subsequently, an MRI of the abdomen with contrast material re-demonstrated the large left upper quadrant mass lesion with predominant nonenhancing central fluid component and a peripheral rim of soft tissue (Fig. , ). The origin of the mass was again not clear on an MRI of the abdomen. Upper gastrointestinal endoscopy was performed, which showed a submucosal lesion on the greater curvature of the gastric body (Fig. ). The esophagus, duodenal bulb, first portion of the duodenum, and second portion of the duodenum were normal. Upper gastrointestinal endoscopic ultrasonography (EUS) showed an anechoic lesion suggestive of a cyst in the perigastric region. The outer wall of the lesion was thick. There was internal debris within the fluid-filled cavity (Fig. ). A fine-needle biopsy of the thick wall was performed. His biopsy results were consistent with spindle cell type GIST. The patient was started on imatinib. A follow-up CT scan of the abdomen done 3 months later did not show any significant change in the tumor size. Because of inadequate response to imatinib, the patient was referred to surgery. She underwent exploratory laparotomy showing a 15 × 15 cm exophytic gastric wall tumor arising from the posterior wall of the body of the stomach. Gastric wedge resection was performed. The patient tolerated the procedure well. The pathology report of surgery specimens showed that all the surgical margins were free of tumor. The patient follows with oncology and gastroenterology services regularly and is slowly returning to her normal state of health. |
A 6-month-old previously healthy male patient was admitted to the emergency center with cough and respiratory distress. Posteroanterior chest X-ray demonstrated increased aeration and infiltration in the reticular pattern that established the diagnosis of bronchopneumonia, and treatment was initiated. In the follow-up, the postero-anterior lung graft suggested progression to respiratory distress syndrome, so acyclovir and oseltamivir were added to the treatment. On the 7th day of the follow-up, the patient’s general condition deteriorated, and he was taken into the intensive care unit because of the necessity of intubation.\nOn the respiratory tract pathogenetic agent panel, upon cytomegalovirus (CMV), and CMV PCR positivity, we switched from acyclovir to ganciclovir treatment 4 weeks after the initiation of ganciclovir therapy. The CMV PCR negativity was detected, so the treatment was discontinued. The patient experienced seizures, and the head control was difficult during extubating and weaning from mechanical ventilation while he was followed up in the intensive care unit. Therefore, cranial magnetic resonance imaging (MRI) was obtained which revealed a 4.5x3 cm lesion with restricted diffusion consistent with the abscess formation. The patient was operated with the indication of cranial abscess.\nBacterial growth was not detected in the cultures of the abscess material, and gram staining did not reveal the presence of any infectious agent. The CMV-PCR test result of the abscess material was reported as negative.\nThe patient was referred to the Pediatric Immunology Clinic for further investigation of possible underlying immunodeficiency. The first physical examination of the patient did not reveal any pathology regarding skin, cardiovascular and respiratory systems, and pulmonary vasculature. Lymphadenopathy, hepatosplenomalgy, and microcephaly were not observed, and examinations of other systems were unremarkable.\nTo differentiate between congenital and acquired CMV infection of the patient, eye examination, hearing test, cranial magnetic resonance (MR), and CMV avidity tests were performed. Calcification was not observed on cranial MR in the patient, whose ocular examination and hearing test results were within the normal limits. The CMV avidity test result was negative. The patient was evaluated in terms of genetics and neurometabolism. In the evaluation of pediatric neurology, no pathology was detected, and the convulsion experienced was interpreted as a manifestation of the secondary to intracranial abscess. Metabolic tests were normal.\nA broad-spectrum antibiotherapy (meropenem, vancomycin) was initiated based on the blood culture results.\nThe hemogram of the patient was reported as follows: WBC, 4500/mm3; neutrophils, 470/mm3; lymphocytes, 3360/mm3; Hgb, 9.5 gr/dl; and Plt, 445,000/mm3. On peripheral smear, atypical cells were not detected, and only few neutrophils and toxic granulation were observed. Immunoglobulin results were the following: IgG, 609 mg/dl; IgM, 17.3 mg/dl; IgA, 5.81 mg/dl. Lymphocyte subgroups were CD3, 69.7%; CD4, 39.6%; CD8, 25.2%; CD19, 22.8%; and NK, 5.0% (). The dihydrorodamine test was normal.\nRepeated control MRI obtained in our clinic was evaluated in favor of bleeding (). The patient was consulted to neurosurgery for operation. During the operation, pus was drained from the area, and it was interpreted as hemorrhage. The operation was completed by placing the catheter in the loge. Microbiological examination of the material collected during the operation, and abscess material retrieved from the catheter were unremarkable. Gram staining and antibiogram could not reveal any bacterial growth in both specimens. Neutropenia was not detected in the evaluation of whole blood counts calculated during healthy periods of the patient, and at the starting time of his first complaints (WBC, 9600/mm3; lymphocytes, 4700/mm3; neutrophils: 4550/mm3).\nThe bone marrow aspiration was performed to exclude the primary etiologies, drug-related secondary neutropenia was considered, and maturation arrest was not observed. The samples were sent for genetic analysis to exclude possible congenital causes of neutropenia, and the result was reported as normal. The cyclic pattern was not observed in the weekly hemogram follow-ups performed to detect cyclic neutropenia.\nAfter the exclusion of primary etiologies, since the number of neutrophils calculated during the clinical follow-up was within the normal limits, infection associated with drug-related secondary neutropenia was considered in the patient who developed neutropenia after the CMV infection and ganciclovir treatment. It is thought that the cranial abscess developed secondary to these risk factors because of the presence of risk factors such as intubation in the intensive care conditions during this period of neutropenia.\nGanciclovir treatment was continued with the CMV PCR follow-ups, and when the CMV PCR results became negative, neutropenia resolved spontaneously. The patient whose lung infection and cranial abscess treatment completed was discharged with cure. Neutropenia was not detected during the clinical follow-up. |
A term female infant was delivered by a 39 year old woman in a peripheral hospital after an uncomplicated 3rd pregnancy with 41 week of gestational age. Delivery was assisted by vacuum extraction. Apgar score was 8/9/10 after 1/5/10 minutes respectively, and the infant had a birth weight of 2920 g (8th percentile), a length of 50 cm (20th percentile), and a head circumference of 35 cm (50th percentile). The postnatal physical examination showed grouped pustular lesions and annular erythema, partly with central lightening, yellowish crusted plaques or slight scaling on the skin, exclusively in the face and on the forehead (Figure ). The rest of the integument and the mucosa were inconspicuous. The child was transferred to a neonatal tertiary care center under the tentative diagnosis of a herpes simplex virus (HSV) infection, and antiviral therapy with aciclovir was promptly initiated. About six hours postnatal, the baby developed focal clonic seizures of the right arm. An electroencephalogram showed focal temporo-parieto-occipital changes over the left hemisphere with low amplitude, slight increase of slow waves and a reduced basic activity. Further, intermittent short-term focal rhythmic parietal theta activity on the left as well as rhythmic occipital delta activity on the left could be detected while no typical epileptic discharges occured. A lumbar puncture showed no sign of infection, with a normal protein and white blood count. In particular, HSV-PCR was negative. Anticonvulsive therapy with phenobarbital was initiated but seizures could only be controlled after adding phenytoin. A diffusion- weighted magnetic resonance imaging was performed on the 3rd day of life, revealing multiple ischemic brain areas in the distribution of the middle and posterior cerebral artery on the left side (Figure A-C).\nNo evidence of sinus venous thrombosis was found. Thrombocytopenia (minimal 91000/μl) was present, and coagulation blood test showed evidence of an increased fibrinolysis and normal plasmatic inhibitors of coagulation, normal antithrombin III (56%) and protein S (58%) but low protein C (22%). Therapy with low molecular weight heparin was started. Echocardiogram revealed no structural abnormalities, and an inconspicuous electrocardiogram with a normal PQ interval and QRS complex was analyzed. Central and peripheral bleeding spots were found in the ocular fundus, most likely caused by trauma during birth. Renal function test, albumin, transaminases, bilirubin and calcium were normal. A smear of the grouped pustular lesions in the face showed no infection with staphylococcus. But a biopsy of the affected skin showed evidence of cytoid bodies compatible with an interface dermatitis. Serological studies of the infant detected maternal HSV and varicella zoster virus (VZV) specific IgG antibodies, IgM and a HSV-PCR of a swab of a skin lesion were negative. However, antinuclear antibodies (ANA), anti-U1-snRNP antibodies and anti-Sm antibodies were present, while antiphospholipid, anti-Scl 70, SSA/Ro, SSB/La, anti-Tm, anti-Jo-1 and anti-ds DNA antibodies could not be detected. Test results of the asymptomatic mother revealed the same autoantibody pattern.\nFour weeks postpartum, the mother developed bilateral erythema on the face and both forearms, muscle weakness of both arms, a Raynaud’s phenomenon, arthralgia in hands and swollen fingers. She was subsequently diagnosed with mixed connective tissue disease (MCTD), since she exhibited three typical clinical symptoms (swollen fingers, muscle weakness, Raynaud’s phenomenon), and one of the possible antibody constellations (ANA, anti-U1-snRNP and anti-Sm antibodies), both characteristic signs and symptoms of the disease [,]. There had been no occurrence of symptoms of rheumatic diseases in the infant’s siblings, a seventeen years old sister and a seven years old brother. Further questioning of the mother revealed that about six weeks after the second delivery, transient arthralgia occurred, which responded to oral steroids. No diagnosis was made at that time.\nThe newborn infant was discharged on his 16th postnatal day, the lesions were healing (in parts showing scars as residual lesions), and until reassessment at the age of 10 weeks with phenobarbital and low molecular weight heparin as medical therapy displayed no symptoms; he was consequently carefully weaned off medication. A transcranial duplex ultrasound after 4 weeks of birth showed a normal cerebral artery blood flow in all areas of the brain. A follow-up MRI of the brain (Figure D-F) at the age of 32 months showed postischemic defects with involvement of the gyrus supramarginalis and the peripheral visual cortex. The rest of the brain showed age-appropriate myelinization and no further over lesions. At the age of 6 years there were no signs of cerebral palsy, seizures or amblyopia but language development was delayed and complicated by a speech disorder. Other cognitive development as tested by the Snijders-Oomen non-verbal intelligence test was in the lower norm and a somatic retardation with dystrophy and microcephaly was diagnosed.\nIn summary, we describe a newborn infant who had cutaneous manifestations and the antibody pattern of neonatal lupus erythematosus (NLE), complicated by neurological symptoms. |
The patient was a 53-year-old man who was suffering from a moderate pain in his right shoulder because of lifting a heavy object. Two days after the incident, due to falling on an outstretched hand while climbing, he felt a very severe pain in the same area. Three days after the second incident, he visited Rasool-E-Akram Hospital's Sports Medicine Clinic. He reported a severe pain in his right shoulder in the first visit (Visual Analog Scale (VAS) = 9-10). In evaluation with goniometer, right shoulder had 150 degree forward flexion and 130 degree painful abduction. Internal rotation could be done very painfully, not suitable for goniometer measurement. The other movements of the shoulder were relatively normal and the left shoulder was perfectly normal. Tests on the right shoulder showed positive result for Neer impingement sign, Empty Can test, Hawkins Kenedy test and Speed test. In evaluating the neck, range of motion was normal and there was no pain. In patient's posture evaluation, there was thoracic kyphotic posture, forward head posture, right scapular winging; and tightness in right upper trapezius and levator scapulae muscles.\nDuring the mentioned 5 days, no treatment had been used other than manual massage on the suffered shoulder. There was no disease in the patient's medical history and he didn't use any specific medication.\nHis sport history showed that in the last 10 years he had regularly gone swimming twice a week for 2 hours each session, he had gone mountain climbing approximately 10 hours per week, and ran 20 kilometers at least 2 days a week.\nIn ultrasonography of the right shoulder there was a complete tear in supraspinatus tendon and the other cuff tendons were suspicous for rupture and fluid was reported around the biceps tendon which was in favor of biceps tendonitis.\nIn MRI without contrast on the patient's shoulder, a 17-mm gap was reported in insertion of supraspinatus tendon. Fluid in joint space of sub-acromial sub-deltoid was also reported. Rupture in the other cuff tendons and biceps tendon was not reported in MRI. Bone marrow edema was evident in greater tuberosity.\nAfter the first visit, 10 sessions of physical therapy modalities (TENS, US, Superficial heat) were done once every two days, each session for 45 minutes. Beside modality, 15 mg Meloxicam tablet was prescribed for the patient twice per day, which was taken for only the three first days by the patient. An exercise program began from the fifth session of physical therapy modalities (2 weeks after injury).\nExercise program was under the physician's supervision twice per week in the first month and trained exercises were performed daily at home. Patient's cooperation during 6 months of treatment was excellent and he performed taught exercises with high precision. In the initial days of exercise program, the patient consulted two shoulder surgeons, both of whom suggested surgery on cuff tendons. However, because of his fears he decided to perform exercise program for 6 months. If the exercise program had failed, he would have undergone surgery.\nIn the first phase of the exercise program, passive ROM exercises were done in the form of forward flexion, internal rotation, external rotation, and abduction. Pendulum exercise, isometric exercises on the shoulder, chin tuck, back extension, shrug exercise were done in this phase too. All of these exercises were done 30 times daily. Stretching exercises for posterior capsule, anterior capsule, the inferior capsule, and trapezius muscle were done twice per day. Each time 5 repetitions were done for 15 seconds. During the first month of treatment, training exercises were supervised twice a week for one hour under the supervision of the physician. If there was a problem in conducting exercises by the patient, necessary recommendations were given. In other days the exercises were done at home.\nAfter obtaining full passive ROM in the involved shoulder and reducing pain, strength training for rotator cuff was initiated from the second month as the second phase of the treatment. The phase included scaption exercise (0 -30 degree abduction), internal, external rotator strengthening exercise (0 degree of abduction, scaption (0-60 degree), internal and external rotators (45 degree of abduction), and biceps (0-90 degree) exercise. Three weeks later exercises of the third phase started and strength training and stretching exercise on neck were conducted.\nAt the beginning of the third month of therapy, strength training for periscapular muscles was added to the previous exercises. Wall push up, wall push up with medicine ball, push up, push up plus were conducted as 3 sets 10 repetitions daily beside other exercises. Cuff strength training severity was increased gradually during the next months as 3 sets 15 repetitions and abduction was done in higher angles. Exercises on medicine ball were done as push up, quadruped exercise, diagonal exercise. At the beginning of each treatment sport session, 10-15 min systematic warm-up as brisk walking was done. At the end of each treatment session, ice was applied on the right shoulder for 20 min. After three months of exercise therapy, the patient was allowed to swim twice per week. Given a slight pain in full abduction, ha was advised not to have full stroke.\nAt the end of 6-month exercise program, patient's pain reached to 0 according to VAS. In the evaluation after the 6-month exercise program, there was a negative result for Neer impingement sign, Empty Can test, Hawkins Kenedy test, and Speed test; however, winging of scapula persisted. These results continued to the 9th month (to this report's time). In goniometer examination, there were 146 degree abduction and 170 degree forward flexion in the right shoulder.\nAt the end of the 6 months of treatment, ultrasonography of the shoulder showed no difference compared to the time before the treatment. In MRI without contrast on the patient shoulder, a 14-mm gap was reported in insertion of supraspinatus tendon. The fluid in joint space of sub-acromial sub-deltoid was reported. Bone marrow edema was evident in greater tuberosity. After termination of the program, the patient initiated mountain climbing twice per week, and he swam twice per week each time for 2 hours. |
A 6 month-old boy fractured his right tibia, when he fell from a couch (50 cm height). One month later, he accidently kicked his leg against a desk. He was diagnosed with a fracture of the right femur. The following month, he again fell from a couch, sustaining a new fracture of the right femur. In addition to the fracture, his x-ray showed thin, osteopenic bones ().\nAt the age of 11 months, the boy was assessed in our institution, in order to reach a diagnosis which would explain his three low-energy fractures. An eye check revealed blue sclera, heart sounds were normal, as were his teeth. His full metabolic bone profile (), showed low levels of procollagen type 1 C-terminal propeptide (PICP).\nAt 17 months, further laboratory tests were performed. The boy had microcephaly as well as generalised floppiness and delay in gross motor milestones (not sitting unsupported at 11 months, not walking independently at 17 months). His weight and head circumference were both below the 3rd centile for age and sex in all visits. Due to faltering height (initially at the 50th centile, then at the 10th centile in the last visit), a failure to thrive work up was undertaken, which revealed no comorbidities (e.g. celiac disease, hypothyroidism).\nBoth parents were present during the assessment. The mother, who had been pregnant for the 3rd time, had had a miscarriage at 9 weeks. The father bore a striking resemblance to the child. He had a history of 5 fractures during adolescence and also reported multiple dental procedures as a child. His sclerae were also blue. His sister had sustained over 5 fractures during childhood and has been on treatment with IV bisphosphonates since the age of 25 years.\nThe clinical geneticist of our institution suggested a fibroblast biopsy of the patient, with a working diagnosis of OI. Also, DNA testing was performed on both the child and the parents. |
A 72-year-old man was referred to our clinic with severe bilateral ectropion. In 2015, an abdominal mass was detected in the left lobe of the patient’s liver during his follow-up. The mass was biopsied and pathologically diagnosed as cholangiocellular carcinoma for which he was maintained on treatment with capecitabine, 500mg daily, after the completion of gemcitabine and cisplatin combination regimen. At the end of the 3rd month of capecitabine treatment, the patient noticed increased tenderness and progressive bilateral ectropion in addition to upper lid shortening, hyperpigmentation, and cutaneous sclerosis around the head and neck region. He was consulted to the dermatology department for further assessment, and an incisional skin biopsy was planned. Biopsy results showed nonspecific findings with areas of interstitial granulomatous dermatitis accompanied by elastophagocytosis. These findings without the evidence of any previous periorbital surgery were considered as an adverse effect of the capecitabine treatment. The patient was followed up by the dermatology and ophthalmology departments, and as he had no corneal abrasion and visual problems, he was managed medically with topical Vaseline and clobetasol propionate cream (0.05%), moxifloxacin antibiotic eye drops, and artificial tears for ocular symptoms. Capecitabine was stopped in the 5th month of treatment because of severe bilateral corneal erosion in addition to the progression of lower lid retraction during follow-up. His upper lid shortness did not change, whereas the corneal erosion and lower lid ectropion did not resolve and continued to progress; therefore, he was referred to the plastic and reconstructive surgery department 1 month after the capecitabine treatment was stopped. Physical examination revealed bilateral lower lid ectropion, bilateral vertical shortening of the upper eyelids, and lagophthalmos []. During the 1-month drug-free period leading up to the surgical procedure, the patient’s findings seemed to worsen with the exception of upper lid shortening, which necessitated surgical intervention. He was treated with surgical release of the lower eyelid skin followed by bilateral full-thickness skin grafting, with grafts obtained from the supraclavicular area. Healing of the grafts and the early postoperative period were uneventful [], yet recurrent ectropion was noted on the 6-month follow-up examination on the right lower eyelid []. A second surgery was planned and ectropion repair was undertaken with surgical release of the lower eyelid skin accompanied by a lateral tarsal strip procedure followed by full-thickness skin grafting. No complications were noted in the postoperative period and later, the patient was followed up without any significant ocular complaints []. |
A 47-year-old woman presented to the emergency department with generalized abdominal pain for a duration of four days. The pain was intermittent and colicky in nature with a mild response to analgesia. It was associated with vomiting and abdominal distension, with absolute constipation. The patient denied experiencing a similar previous attack. Her medical and surgical histories were unremarkable, and she reported only having an irregular menstrual cycle. She also reported no history of trauma.\nUpon physical examination, the patient was found to have stable vital signs: temperature, 36.7°C; heart rate, 88 beats per minute; and oxygen saturation, 100% room air. An abdominal examination revealed mild abdominal distention with generalized tenderness but no guarding or rigidity; the abdomen was tympanic to percussion, and a digital rectal examination was unremarkable. A provisional diagnosis was made for intestinal obstruction.\nLaboratory investigations, including a complete blood count, urea and analysis, and coagulation profile, were all within normal limits. An abdominal X-ray showed a dilated large bowel with few air fluid levels, no gas in the rectum and no air under the diaphragm (Fig. ). An abdominal CT scan with oral and intravenous contrast showed that the cecum was flipped superiorly and positioned anteriorly to the transverse colon, and it herniated into the foramen of Winslow (opening between the inferior vena cava and portal vein). The cecum occupied the lesser sac, and it was distended with gas and caused a mass effect on the stomach and hepatic root, with associated intrahepatic bile duct dilatation. This was associated with proximal small bowel dilatation and collapsed large bowel loops distally (Fig. ). These findings were consistent with an obstructive internal hernia into the lesser sac through the foramen of Winslow.\nA surgical consultation was requested, and laparoscopic assisted internal hernia reduction with ileocecal resection and side-to-side anastomosis were offered. The cecum and terminal ileum, along with the appendix, had herniated through the foramen of Winslow as evidenced during laparoscopy (Video 1). Both the cecum and terminal ileum were markedly distended and probably less vascularized (Fig. ). Consequently, the cecum and terminal ileum were resected (Fig. ).\nThe patient’s postoperative was uneventful. She was started on a clear liquid diet and then advanced to solid foods by Day 2. The patient was discharged, and she did not show any signs or symptoms suggestive of complications or recurrence during the last follow-up. |
This is the case of a 98-year-old female with a long history of severe end-stage osteoarthritis, for which a total knee arthroplasty had been recommended for many years but the patient refused any kind of surgery due to her advanced age. She was treated with nonsteroidal anti-inflammatory drugs (NSAIDs), bracing, and physical therapy with bi-annual cortisone and hyaluronic acid injections to control discomfort. She was also treated at the practice intermittently for a large popliteal cyst with recurrent effusions via aspiration. The patient ambulated mostly without assistive devices, occasionally using a cane, and was high functioning in her activities of daily living. She lived at home with her daughter. Her past medical history was significant for multiple joint osteoarthritis, hypercholesterolemia, allergic rhinitis, and a remote history of large intestine adenocarcinoma in remission for over 10 years, successfully treated with resection and maintained colostomy.\nThe patient presented to the office complaining of several days of increasing knee pain. Her chief complaint was pain and swelling in the posterior knee although she was able to ambulate and bend her knee. On exam, she had a large popliteal cyst. She had mild swelling of the knee, no discernable effusion, no redness, no warmth, and intact skin. Her range of motion was 5-130 degrees, which was typical for her. She denied trauma, fevers, chills, or other constitutional symptoms. She was treated with aspiration of the cyst and cortisone injection in the joint and was discharged with a plan to inject hyaluronic acid in two weeks.\nThe next morning, the patient presented to the emergency room. She stated that she had experienced marked improvement in her symptoms following treatment in the office, however, awoke the next morning with a hot, erythematous, swollen knee with limited range of motion or ability to ambulate. On exam, the patient was afebrile (temperature: 98.3°F) and stable (blood pressure: 147/76 mmHg, HR 60 beats per minute, oxygen saturation on room air >96%). Her knee was aspirated in the emergency room and found to have elevated WBCs (87,000 cells/mm³) with 98% neutrophils and a positive Gram stain. Her serologies showed leukocytosis to 24.7 cells/mm³, elevated erythrocyte sedimentation rate (ESR) of 37 mm/hr, and C-reactive protein (CRP) of 2.1 mg/L. Plain radiographs of her left knee showed end-stage osteoarthritis consistent with prior imaging without evidence of new trauma or osteomyelitis (Figure ). The patient was then taken to the operating room for arthroscopic irrigation, debridement, and synovectomy. Purulent thick synovial fluid was encountered and sent for analysis. There was severe end-stage tri compartmental arthritis, most severely patellofemoral and lateral, with little remaining joint cartilage. This pre-existing degenerative disease limited the ability to assess infectious cartilage damage. There was also extensive edematous, hyperemic, and boggy tri compartmental synovitis. Care was taken to remove as much of the synovium as possible, and a drain was left in the joint to allow for effective postoperative drainage. The patient was started on broad-spectrum antibiotics (vancomycin and ceftazidime) and probiotics at this time per infectious disease consultant recommendations.\nThe cultures of the initial aspiration and washout grew C. perfringens. The patient denied recent trauma to the knee and denied recent possible outdoor exposures due to Covid-19 pandemic restrictions. She did, however, have a history of colon cancer treated greater than 10 years prior. Colon adenocarcinoma has previously been found as a nidus for the hematogenous spread of atypical bacteria such a C. perfringens to joints []. The patient was status post resection with a permanent colostomy and adjuvant chemotherapy with resultant eradication of the disease. She did not have active disease at this time to her or her family’s knowledge. The diagnosis of C. perfringens septic arthritis prompted a full work-up with a CT scan of her chest/abdomen/pelvis and a PET scan. No recurrence of the adenocarcinoma was found.\nThe cultures demonstrated penicillin sensitivity, prompting a switch from broad-spectrum antibiotics to IV penicillin. The patient’s pain improved significantly (9/10 to 3/10) after the arthroscopic irrigation and debridement, and she required up to two doses of opioids daily for analgesia. Her swelling, redness, and pain also improved drastically, with an associated increase in range of motion from minimal to 5-60 degrees. The patient was however slow to bear weight on the knee, with physical therapy started on the second postoperative day. Aspiration postoperative day four returned minimal fluid. The patient received appropriate deep vein thrombosis prophylaxis throughout her hospital course.\nThe patient was discharged to rehabilitation, where she spent one month recovering. In week six of treatment, she developed an allergic reaction to penicillin (rash) and was readmitted to the hospital with a medication change to clindamycin. An aspiration of her knee at that time did not return fluid. Interventional radiology then performed a guided aspiration with a minimal fluid return. Cultures of this fluid were negative. As the patient’s leg was still swollen and her weight-bearing difficulty remained, an MRI was ordered to evaluate for osteomyelitis or the presence of abscess or soft tissue involvement. The MRI showed only a mild effusion and severe arthritis of the joint (Figure ). Doppler ultrasound was also negative for DVT. Antibiotics were discontinued at this time as there was no sign of persistent infection with negative serologies and culture. Over the following week, the patient’s leg swelling and ability to ambulate improved with physical therapy, and her residual swelling was attributed to post-infectious sequelae in the setting of severe osteoarthritis. The patient returned to rehabilitation and continued to improve until discharge to home. Throughout her course, the patient did not develop fevers, chills, or any other constitutional symptoms. |
A 42-year-old male patient (height 170 cm, weight 63.7 kg) visited our pain clinic with complaint of severe right groin pain. Two years earlier, the patient had been diagnosed with bilateral cam type FAI and underwent arthroscopic surgery in the right hip after hip magnetic resonance imaging which showed fibrocystic change of the right femur and superior labral tear and minimal joint effusion on both hips. The recurrent pain began about 1 year prior to his visit and walking was impossible. The degree of pain was 10/10 on the visual analogue score system (VAS, ranging from 0 = no pain to 10 = absolutely intolerable pain) and the Oxford hip score (OHS, function of hip joint, excellent = below 19, good function = 19-26, fair = 27-33, poor = 33 or more) was 47/60. During the physical examination, the straight leg raising test (SLR) was right 45° and left 80°. The FABER test and anterior impingement test (flexion and internal rotation of knee) were all positive at the right hip. The frog lateral view of the X-ray showed left superior labral calcification and an osteophyte at the right femur neck. In addition, the head-neck offset of the left femur was decreased (). The alpha angle in the translateral view was 78.2° (). The ultrasonographic finding showed mild effusion and capsular thickness. The patient had no previous past medical history and took tramadol 200 mg, NSAIDS 200 mg and gabapentin 600 mg per day. We decided to perform IA steroid injection under C-arm guidance. Written informed consent was received after sufficient explanation about the procedure and related complications. With the patient in the supine position, C-arm fluoroscopy was focused on the hip joint in the anterolateral view. After insertion of a 22 gauge spinal needle, 0.75% ropivacaine 5 ml and triamcinolone 40 mg injection was achieved in the right hip joint (). The patient visited our clinic again checking his VAS, OHS and satisfaction scores (5-point Likert scale; 5 = very satisfied, 4 = somewhat satisfied, 3 = neither satisfied nor dissatisfied, 2 = somewhat dissatisfied, 1 = very dissatisfied) at 2, 4, 8 and 12 weeks after injection (). The patient took NSAIDS 200 mg intermittently during the 12 weeks. Although moderate right hip pain remained, the patient could walk and return to work.\nA 59-year-old female patient (height 161 cm, weight 59 kg) visited our pain clinic with left hip joint pain. The patient had been taking medications for rheumatoid arthritis. The patient had been diagnosed with pincer type FAI about two years prior to her visit. The hip AP (anteroposterior) view of the X-ray showed suspicious FAI, with labral calcification and excessive coverage by the superior margin of both acetabula (). The pain site was in the lateral and subgluteal area of only the left hip joint. The degree of pain was 7/10 on the VAS and the OHS was 26/60. During the physical examination, the SLR was right 90° and left 90°. FABER test was positive on the left side. However, the anterior impingement test was negative for both hips. No abnormality was found in the ultrasonographic image. We decided to perform ultrasound guided IA steroid injection. Written informed consent was received after sufficient explanation of the procedure and related complications. With the patient in the supine position, the hip was internally rotated about 15-20°. An ultrasound convex probe (2-5 MHz, MicroMAXXTM, Sonosite, USA) was aligned with the long axis of the femoral neck, including the acetabulum and the femoral head. A 22 gauge spinal needle was then advanced under direct ultrasonographic guidance into the anterior synovial recess at the junction of the femoral head and neck (). Injection of 0.75% ropivacaine 5 ml and triamcinolone 40 mg in the left hip joint was performed. The patient visited our clinic again checking her VAS, OHS and satisfaction scores at 2, 4, 8 and 12 weeks after injection (). The patient took her previous medications for rheumatoid arthritis during the 12 weeks and additional analgesics were not prescribed. Three months after the injection, the pain in the trochanteric area was partially reduced.\nA 50-year-old male patient (height 175 cm, weight 73 kg) visited our pain clinic with complaint of pain in both hip joints. The pain had been ongoing for one year prior to his visit. The patient had been prescribed NSAIDS and muscle relaxants at a local pain clinic whenever he felt pain. The pain in the right anterior groin area was more severe than that of the left side. Because of the hip pain, the patient could not sit crossed-legged on the floor. The degree of pain was 3/10 on the VAS scale and the OHS was 21/60. During the physical examination, the SLR was right 90° and left 90°. The FABER test and anterior impingement test were positive on both sides. The frog lateral view of the X-ray showed the possibility of mixed type FAI of the right hip (). The head-neck offset of the right femur was decreased and there was excessive coverage by the superior margin of the right acetabulum. The alpha angle in the translateral view was 75.3°. No abnormality was found in the ultrasound image. We performed C-arm fluoroscopy guided injection of 0.75% ropivacaine 5 ml and triamcinolone 40 mg in the right hip joint just like case 1. His symptom was much improved after the injection. The patient visited our clinic again checking his VAS, OHS and satisfaction scores at 2, 4, 8 and 12 weeks after injection (). The patient took NSAIDS 200 mg during the first month after injection and thereafter, was discontinued. The patient could sit crossed-legged on the floor 2 weeks after the injection although mild right hip pain remained. |
We report a case of a 12-year-old boy with no significant past medical history who presented at Phoenix Children's Hospital Emergency Room following a motor vehicle accident (MVA). The patient was sharing a seatbelt with one other sibling in the back seat of a 4-door sedan. The vehicle was involved in a “T-bone” collision, which resulted in a temporal head injury against the side door. At the time of presentation, the patient complained of neck tenderness, pain at the right skull base, and nausea. The patient did have poor recollection of the accident; however, there was no indication of loss of consciousness. The patient denied any neck or head pain prior to the accident, and denied any fever, chills, emesis, or seizures. Prior to his presentation, the patient was otherwise healthy, performed well in school, has no family history of coagulopathies, was not taking any medications, and had a negative review of systems except what was discussed earlier. On physical examination, the patient was alert and oriented and appeared well nourished and well hydrated. His pupils were equal and reactive to light, extraocular movements were intact, his smile was symmetric and his hearing present in both ears. He had no otorrhea or rhinorrhea, but some ecchymosis was noted around the right ear. There was some neck tenderness at the level of C3, a neck brace was applied as a precautionary measure pending imaging results. He had full power and normal tone in his upper and lower extremities. His pulses were equal bilaterally to palpation. Overall, the patient was clinically stable but remained somewhat confused with a Glasgow coma scale (GCS) of 14. The patient was admitted to the pediatric intensive care unit pending imaging studies.\nA computed tomography (CT) of the head [] on presentation revealed a small petrous bone fracture on the right side of the skull. Several small pneumocephalic collections were evident surrounding the fractured mastoid air cells. Bony fragments were visible within the vicinity of the sigmoid sinus. A magnetic resonance venography (MRV) [] was ordered to evaluate the cranial vascular integrity, which demonstrated a flow void of the right sigmoid sinus and the right external jugular vein.\nThe patient's coagulation profile was within normal limits, he was admitted and started on IV heparin and monitored with serial exam for changes in neurologic function. The patient remained stable and on the third day of admission, a repeat MRV [] demonstrated persistent flow void of the sigmoid sinus, but improved flow through the external jugular sinus. At this point, the patient's nausea and headache had resolved and he was switched to low molecular weight heparin (enoxaparin) and transferred to the pediatric floor. The remainder of the hospitalization was uneventful, the patient was discharged on day 7 with 6 weeks of low-molecular weight heparin (LMWH) therapy with weekly clinical follow up. |
A 68-year-old man presented to us with complaints of a recurring cough and occasional blood-streaked sputum. The problem started after a dental procedure during which he had a choking episode and aspirated a dental drill, which was being used for a root canal treatment. He denied any fever, chest pain, or shortness of breath. He had no other medical problems of note beside the above. Clinical examination was essentially normal. The patient had brought with him an X-ray of the chest, which was normal, and CECT of the thorax with reconstructions in which the slender metallic FB was seen lodged in one of the basal sub-segments of the left lower lobe [Figures –]. A decision for bronchoscopic extraction was taken. After obtaining informed consent the patient was taken up for fiberoptic bronchoscopy under conscious sedation.\nA bronchoscopy for evaluation was done with a videobronchoscope, outer diameter 5.8 mm, under conscious sedation with intravenous midazolam. The trachea-bronchial tree was largely normal except for the left lower lobe lateral basal segment, where the tip of the FB, a dental drill, was seen pointing straight up. The plastic blunt end of the drill was lying distally embedded in the subsegmental bronchus. An attempt was made to hold this metallic sharp end with a rat tooth forceps but failed. Then, a snare was coiled around the tip, but it slipped through on account of it being too thin. A magnetic tip probe for extraction of metallic FBs also failed to pull out the FB. Finally, a thin fogarty catheter was pushed from the side of the FB with the intention of inserting it distal to the FB, inflating the balloon and then pulling it up to dislodge the FB. However, this attempt resulted in the FB getting pushed in with the catheter and then slipping out of sight into the distal subsegment.\nThe video bronchoscope was pulled out and in its place a thin pediatric fiberoptic bronchoscope, outer diameter 3.2 mm, was inserted. The FB was again located further down in the bronchial tree in a fourth order basal subsegment []. A thin dormia basket was inserted through the working channel of this bronchoscope to snare the FB. This attempt also failed because the distal end of the FB was impacted in the bronchus and, therefore, did not allow the dormia basket to open alongside. Taking into account the failure of the various instruments to extract the FB it was decided to attempt extraction with the rigid bronchoscope. The patient and the family were informed and counseled about the failure of the procedure so far, the need for rigid bronchoscopy under general anesthesia, and the possibility of needing surgery in case the rigid bronchoscopy also failed.\nThe next day the procedure was undertaken in the operation theater under general anesthesia. Initially, a 7.5 mm outer diameter, 43 cm barrel, rigid bronchoscope was inserted up to the left main bronchus. A zero degree Hopkins telescope was passed through it for visualizing the FB. However to our dismay, we found that the tip of the Hopkins telescope was too thick to enter the subsegment, and the FB could not be seen. Furthermore, the outer barrel of the rigid bronchoscope after entering the left main bronchus was at such an angle that maneuvering the Hopkins telescope towards the segment of interest was very difficult. The situation demanded that a short bronchoscope barrel be inserted only up to the proximal part of the left main bronchus so as not to restrict the maneuverability of the extraction instruments. Then a long thin telescope had to be passed through it to reach the FB and extract it with an optical forceps under vision.\nFor this purpose, a 5.5 mm outer diameter, 30 cm barrel, pediatric rigid bronchoscope was inserted just inside the left main bronchus. Since the adult Hopkins telescope was too thick and the pediatric telescope too short to reach the left lower lobe, a ureteroscope 6F, 3 mm outer diameter, length 45 cm, was selected to extract the FB. To our delight, this telescope indeed could be passed and maneuvered into the left lower lobe subsegment, and the FB glimpsed. However, another challenge confronted us. The flimsy bronchial walls of these narrow sub-segments, collapsed around the telescope making insertion and visualization difficult. Fortunately, this was averted with the jet venturi system connected to the rigid bronchoscope by which frequent, high velocity bursts of oxygen enriched air helped to keep the distal airway open and keep the tip of the FB in view. Finally, the FB could be extracted under vision with a tri-pronged 3F forceps passed through the ureteroscope. This prong is normally used for extracting stones from urinary passage, but here it served admirably to extract this sharp metallic FB from an almost inaccessible location [].\nA postprocedure check fiberoptic bronchoscopy revealed a normal bronchial tree. The patient remained well on follow-up with complete recovery of the symptoms. |
A 29-year-old woman was admitted our hospital on August 1999, had been diagnosed as mucinous tumor of borderline malignancy a year ago. She was followed up at a local clinic. On the follow-up study, the patient's CEA level was raised to 43.32 U/L and CT scan showed splenomegaly with cystic lesion. Her past medical history was not significant. She already underwent two surgeries. The first surgery, right salpingo-oophorectomy, was performed at the age of 22 after being diagnosed as dermoid cyst. She was healthy thereafter. Seven years later, left ovarian mass was found on the routine check. At the second surgery, left ovarian mass excision, mucinous tumor of borderline malignancy was diagnosed.\nDuring follow-up after the second surgery, she was referred to our hospital on the suspicion of carcinomatosis peritonei. On the preoperative evaluation (Figure ), splenic lesion, which had been existed for 2 years, was merely noticed as simple cystic lesion unrelated to the ovarian mass.\nTo exclude peritoneal carcinomatosis, open laparotomy was perfomed. On opening the abdomen, no abnormal gross findings were found except the splenic lesion, which reported as probable metastatic adenocarcinoma on frozen sections. After splenectomy was carried out, peritoneal washings and multiple biopsies on the omentum, peritoneum, mesentery, and left ovary were performed to rule out possible microscopic peritoneal dissemination. Suspecting the transabdominal metastasis, 100 mg of cisplatin was infused into the peritoneal cavity at the end of the operation; however no intraperitoneal recurrence was confirmed after tissue diagnosis.\nFinal pathologic examination (Figure ) showed metastatic mucinous cystadenocarcinoma. Peritoneal washings and multiple biopsies were all negative. The patient was recovered from the surgery without the evidence of sepsis of severe thrombocytosis. The patient received 5 courses of Taxol and Carboplatin as postoperative chemotherapy.\nTwo years after the surgery, 3 × 3-sized mass on the left ovary, which assumed to be recurrence, was detected. She has been followed up at outpatient department receiving symptomatic treatment and chemotherapy. |
Our patient is a 20-year-old female who initially presented to an outside hospital in July 2017 with a 1-month history of progressive low back pain. Initial magnetic resonance (MR) showed a destructive, osteolytic, expansile lesion measuring 4.8 × 3.8 cm centered in the left aspect of the sacrum at the level of S2–S3 with areas of anterior cortical destruction extending into the left sacral neural foramina from S2 to S4. There was also a heterogeneously enhancing soft tissue component that was measured at 6.4 × 3.7 cm that was along the left piriformis muscle. A CT-guided core needle biopsy performed in August 2017 yielded an initial diagnosis of a high-grade osteosarcoma. She was subsequently treated as an osteosarcoma patient with MAP chemotherapy. She completed a total of 12 weeks of chemotherapy, with cumulative doses of cisplatin 360 mg/m2, doxorubicin 225 mg/m2, and methotrexate 24 g/m2. Surgical intervention was determined to result in substantial morbidity, and therefore, the patient underwent proton beam radiation in three fractions for a total dose of 54 Gy. However, due to the lack of increased bone mineralization in response to chemotherapy, the question of osteoblastoma rather than osteosarcoma was raised.\nAfter reviewing the initial pathology, a second pathologist described the tissue specimen as a cellular tumor composed of proliferating osteoblasts with atypia and frequent mitotic figures associated with trabecule of woven bone. Neoplastic cells demonstrated mild cytologic atypia and scattered mitoses of normal configuration. In different areas, the tumors had undergone secondary hemorrhagic and cystic changes. Although difficult to definitively differentiate between the two, an overall impression of osteoblastoma was favored. As a result, a second biopsy was later obtained in December 2017. It in fact demonstrated no neoplastic cells, but instead a cystic wall with bland-appearing spindle cell proliferation, hemosiderin deposition, lamellar and woven bone with osteoclast-type giant cells along with a foci of fibrous tissue with plasmacytoid and epithelioid osteoblasts, with surrounding osteoid, all of which was consistent with an ABC. The specimen was negative for reportable single nucleotide variants, as well as negative for EWSR1/FUS/SS18/STAT6 rearrangements. After having three separate pathology consultations, the final diagnosis of an osteoblastoma with secondary ABC was made.\nBased on the revised diagnosis of osteoblastoma, the decision was made to change her therapy to denosumab as surgery was still deemed to be too morbid. In May 2018, she was started on denosumab 120 mg every week for 4 weeks, and then monthly. At the time of writing this report, she has received 24 doses. Surveillance imaging throughout her treatment showed a decrease in the number and size of the cystic components, and an overall plain radiographic appearance of mineralization along with decreased FDG avidity on PET scan, suggestive of a treatment response ( and ). After nearly 24 months of treatment with denosumab, her latest MRI has continued to show stable to slightly decreased size of the lesion and loss of fluid level in the lesion (). Additionally, her pain control has drastically improved and she was able to completely titrate off all pain medications within 3 months of starting denosumab. |
An 18-year-old female track and field athlete with bradycardia was referred to a cardiologist by her family physician to rule out a cardiac defect. She underwent a 2D echocardiogram, which revealed what was thought to represent a poorly visualized aneurysm. More specifically, the abnormality was initially felt to be an aneurysm of a sinus of Valsalva; though, the lack of certainty required further imaging. A prospectively gated contrast enhanced multidetector CT (MDCT) scan of the heart was performed. CT imaging () confirmed the presence of an aneurysm; however, it was located at the membranous interventricular septum immediately caudal to the level of the right coronary sinus. The aneurysm measured 1.2 × 0.9 cm, stretching the septum into the right ventricle. No accompanying VSD was evident.\nFollowing the diagnosis, the patient remained asymptomatic, but persistently bradycardic. She denied any angina upon exertion (Canadian Cardiovascular Society class 0), allowing for her status as an elite track and field athlete, typically under cardiovascular stress. The patient denied symptoms of exertional dyspnea, orthopnea, paroxysmal nocturnal dyspnea, or ankle edema (New York Heart Association class I). There were no reports of palpitations or any other respiratory or cardiovascular symptoms. However, the patient recounted a history of 3 syncopal episodes since age 11, previously felt to reflect vasovagal syncope rather than manifestations of underlying structural heart disease. During physical examination, the patient had a regular heart rate of 45 beats per minute and blood pressures in a seated position measuring 95/65 mmHg and 100/65 mmHg in the left and right arm, respectively. Her right lower limb systolic blood pressure was measured at 110 mmHg. On auscultation, grade 1 systolic ejection and pansystolic murmurs were heard along the left sternal border. According to the patient's mother, heart murmurs were noted at birth, but no echocardiogram was performed at the time. Jugular venous pressure and carotid upstroke remained within normal limits, and no breathing abnormalities were identified. A transesophageal echocardiogram (TEE) was ordered to assess the resiliency of her aneurysm, which was subsequently found to be intact. There was no evidence of intracardiac shunting, right ventricular hypertrophy, pulmonary hypertension, or any other haemodynamic abnormalities. Essentially, the patient was entirely asymptomatic from a cardiac perspective. |
A 14-year-old girl presented with complaints of fatigue, inability to walk, urinary incontinence, dysarthria, and ataxia that had worsened in the last three weeks. She also had fever, weight loss and decreased appetite. In her past medical history she had been treated for seizures with valproic acid (15 mg/kg/day) for three years. Her seizures were documented by EEG findings.\nOn physical examination, her blood pressure was 110/60 mmHg and she had a pulse rate of 88/min, a respiratory rate of 14/min and oral temperature of 38.6°C. Her growth and development were normal, reaching menarche at the age of 13, followed by regular cycles until two months before admission. Her weight was 48 kg and her height was 154 cm. She had received all vaccinations since birth according to the national vaccination plan. Her family history was negative for any hereditary or metabolic disorders.\nHer consciousness level was normal and she had no neck stiffness. In spite of being awake, she could not communicate with anyone and only used indistinct words. The pupils were normal in size and equally reactive to light. Her sclera were pale.\nOn neurological examination, her upper limbs were mildly spastic, and her muscle strength was 2–3/5 as far as she could cooperate. Her upper limb reflexes, including the biceps, brachioradialis, and triceps, and also her sensory examinations were normal.\nHer lower limbs, however, were flaccid and atrophic; the muscle strength was 0/5 and the patellar and Achilles reflexes were absent. She showed an extensor plantar reflex. Her lower limb sensory examination showed lost senses of light touch, pain, temperature, vibration and joint position. This may have been unreliable due to her overall condition.\nAs she was not able to stand or walk, gait and cerebellar examinations were not performed. We also found a deep infected 5 × 4 cm bedsore in her sacral area, as a result of being bedridden for more than three weeks. Her abdominal, chest and heart examinations were normal. Her hemoglobin was 8.3 gr/dl (See Additional file : Table 1 for the CBC result). All other blood tests were normal (See Additional file : Table 2 for other blood tests). Her cerebrospinal fluid examination and chest X-ray were also normal.\nB12 deficiency was documented by serum vitamin B12 level <100 (normal, 160–970) pg/ml and peripheral blood smear that showed hypersegmented neutrophils (6 and 7 segments).\nEEG findings included some sharp activity that could indicate epileptogenic activity. The MRI demonstrated senile dilatation in the CSF space and sulci of both hemispheres; a finding compatible with mild atrophic changes (Fig. ).\nEMG/NCV showed prolonged distal latency of motor nerve, decreased amplitudes of compound muscle action potential, decreased conduction velocity, and increased F wave latencies. Sensory nerve action potentials were not detected. All above finding are compatible with mixed sensorimotor polyneuropathy.\nTreatment was begun with vitamin B12 (1 mg/IV/day) and folic acid (5 mg/oral/day). After a few weeks, her symptoms improved and she was able to speak and eat. Her upper limb mobility improved and she began to communicate with us. Her reticulocyte count also increased dramatically from 0.9% to 7.1% (on the 7th day of treatment) indicating a favorable treatment response. However, she was febrile in spite of being treated with vancomycin (1 gr/BID) and imipenem (500 mg/QID) which were chosen based on the infected bed sore cultures and antibiograms. After one month, her hemoglobin slightly rose and remained in the range of 8–9 gr/dl. Although her serial blood cultures were negative, she remained febrile, and we suspected an unusual source of infection that did not respond to broad spectrum antibiotics. Therefore, a bone marrow aspiration and biopsy was performed three weeks after initiating the treatment, and the specimen was examined for unusual germs such as tuberculosis and brucellosis.\nBone marrow examination (Fig. ) showed some degree of megaloblastic changes, giant metamyelocytes and nuclei/cytoplasm dissociation. The laboratory report showed there were acid fast bacilli in her bone marrow aspiration smear. Therefore, we started a six month antituberculosis treatment with isoniazid 5 mg/kg/day, rifampin 10 mg/kg/day, ethambutol 15 mg/kg/day, and pyrazinamide 20 mg/kg/day.\nThis treatment resulted in the termination of fever on the second day, further elevation of the hemoglobin level and improvements in her general health. Her upper-limb force became normal and she was able to eat by herself. Her lower-limb force improved and the plantar reflex was now flexor. To find the cause of megaloblastic anemia, we reassessed the case. Valproic acid was not the cause, she was not a vegetarian, and she had no sign or symptoms of malabsorbtion or malnutrition. Her growth and development were normal. We concluded that her vitamin B12 deficiency may be of a gastrointestinal origin. As the Schilling test was not available at our center, we conducted a small bowel series by barium examination and the results showed terminal ileum narrowing, irregularity and mucosal ulceration; all highly indicative of tuberculosis (Fig. ).\nColonoscopy showed a normal colon but a severely strictured and edematous ileocecal valve, obstructing the path to the terminal ileum for the endoscopic tube.\nWe concluded that M. tuberculosis had caused the terminal ileum disease and possibly vitamin B12 deficiency in our patient. At present, our patient has been treated for vitamin B12 deficiency and M. tuberculosis. After being treated with anti-tuberculosis drugs her fever terminated and her hemoglobin level returned to normal. Currently, 10 months after admission, she can eat, write, and speak normally as well as walk and ride a bicycle, but she has some degree of foot drop. Her latest tests showed a hemoglobin level of 13.7 gr/dl, hematocrit 39.4, and her MCV was 89.3 fl. The small intestine barium examination at the completion of the anti-tuberculosis treatment showed less narrowing and irregularity, which further confirmed the diagnosis. |
A 47 years old Asian lady was seen in the breast clinic in July 2004 for a rapidly increasing lump in the right breast which had been present for four months. There was no nipple discharge and no family history of breast cancer. He mother in law died of pulmonary tuberculosis about 10 years ago.\nClinical examination revealed a 6 cm × 8 cm mass in the right breast with nipple retraction. There was also a 2 cm × 2 cm palpable lymph node in the right axilla.\nMammogram showed asymmetric increased density in the right retro-areolar area with some skin thickening of the areola and some retraction of the nipple (Figure ). Foci of fine calcification were also noted in both breasts. Ultrasound of the right breast revealed widespread hypodense irregular areas extending from 7–10 O'clock in position close to the areola with some distal shadowing (Figure ), raising the suspicion of infiltrating ductal carcinoma. There was also a 1.3 cm × 1.9 cm lymph node with some cortical thickening at its distal pole which suggested some focal metastasis (Figure ).\nFine needle aspiration of the mass was inadequate. A tru-cut biopsy confirmed an invasive ductal carcinoma of no special type along with evidence of non-necrotising granulomatous inflammation containing multinucleated Langhans type giant cells. Subsequent Z-N staining for acid fast bacilli showed multiple bacilli within macrophages, confirming a tuberculous aetiology. Erythrocyte Sedimentation Rate was 25 mm/h. She was commenced on antituberculous treatment.\nShe underwent a right mastectomy with axillary node sampling which showed a 5.5 cm × 5.0 cm × 3.0 cm, grade-II invasive ductal cell carcinoma which was multifocal, with the largest focus measuring 33 mm. Florid lymphovascular invasion was seen along with low grade ductal carcinoma in situ. A striking granulomatous inflammation was seen within the surrounding stroma with multiple non-necrotising epithelioid containing granulomata (Figures &). Ten of the thirteen indentified lymph nodes showed metastatic carcinoma, and one lymph node showed multiple epithelioid granulomas. TNM classification was pT3, pN3a, pMx. Since the patient had already been commenced on antituberculous treatment prior to surgery, special stains for acid fast bacilli were negative in this specimen.\nChest X-Ray, abdominal ultrasound, small bowel follow through and isotope bone scan were normal. The patient received adjuvant eight courses of FEC (Fluorouracil, Epirubicin and Cyclophosphamide), and a course of radiotherapy to the right chest wall, supraclavicular fossa and axilla (40 Gy in 15 Fractions). The right chest wall was fitted with 8 MeV electrons, and the supraclavicular foaas and axilaa were fitted with 8 MeV photons. Subsequently, she had wide spread metastases with pleural and pericardial effusion which were drained. She was commenced on weekly Paclitaxel with three weekly Herceptin. Unfortunately, she died in April 2007 before finishing the treatment. |
A 43-year-old man was admitted to the hospital following an acute cerebellar hemorrhage. He complained of recent fever, chills, and left wrist pain. Past medical history included alcoholic cardiomyopathy, porcine mitral valve replacement, chronic atrial fibrillation, and multiple intracranial hemorrhages associated with warfarin toxicity. The chronic left wrist pain and swelling had been previously diagnosed as gout. Social history was notable for heavy ethanol abuse and intravenous drug abuse. Medications upon admission included warfarin, aspirin, metoprolol, and simvastatin. During the preceding four months, he was also treated with systemic corticosteroids following multiple intracerebral hemorrhages.\nOn admission, the patient was afebrile, and the left wrist was moderately swollen and tender to palpation. His white blood cell count was 16.6 × 103 cells. Blood cultures were negative, and transesophageal echocardiography revealed no vegetations or perivalvular abscess. He was started on intravenous dexamethasone, and 2 days later a craniotomy was performed to evacuate his intracerebral hemorrhage.\nTen days following admission, an arthrocentesis of the left wrist was performed after the patient developed increased swelling and marked worsening of pain. Gram stain revealed numerous white blood cells but no organisms or crystals, and cultures of the synovial fluid were negative. Intrarticular depomedrol was administered following the arthrocentesis.\nTwo days following the arthrocentesis, the patient complained of right eye pain with redness and blurry vision. Visual acuity was hand movements in the right eye and 20/20 in the left eye. Slit lamp examination revealed severe conjunctival injection, Descemet’s folds, and 3+ anterior chamber cells and flare in the right eye. Moderate vitreous haze with 3+ vitreous cells was noted. Fundus examination revealed multiple large, fluffy retinal and vitreous infiltrates in the right eye (Fig. ). Examination of the left eye was unremarkable.\nA presumptive diagnosis of endogenous endophthalmitis was established. A diagnostic vitrectomy was performed followed by injection of intravitreal cefazolin, vancomycin, and amphotericin B (5 μg). Topical ciprofloxacin, natamycin, and intravenous amphotericin were initiated thereafter. Cytology preparation of the vitreous fluid revealed a mixed inflammatory cell infiltrate with numerous fungal elements including hyphae and clusters of conidia (Fig. ). Intravenous amphotericin B was continued for the next 10 days; however, the vitritis persisted. During the same period, he complained of worsening left wrist pain and redness. Magnetic resonance imaging of the wrist revealed osteomyelitis of the distal radius, ulna, lunate, and triquetrium. Synovial fluid aspiration and biopsy of the radius was performed, and numerous white blood cells were seen on gram stain, but no bacteria or fungal elements were noted. Transesophageal echocardiography was repeated but again revealed no valvular vegetations.\nSubsequently, intravenous voriconazole was added to the treatment regimen but his vision continued to deteriorate to light perception in the right eye. Slit lamp examination showed 4+ cells and flare with a hypopyon, and severe vitritis obscured all fundus details. A repeat vitrectomy was performed, and intravitreal amphotericin B injection (10 μg) was repeated. Microscopic examination of the fluid revealed numerous white blood cells, hyphae, and numerous conidia.\nOn the following day, the patient died following a massive intracranial hemorrhage. An autopsy was performed, but examination of the eyes was not permitted. Small friable vegetations less than 5 mm in size were present on the insertion ring of the prosthetic mitral valve. Microscopic examination of the vegetations revealed fungal elements including branched septate hyphae and conidia. Multiple organs including the brain, kidneys, and spleen showed evidence of septic embolization. Fungal culture results from the vitreous biopsy were received from the reference laboratory after the patient’s death and were positive for P. richardsiae; however, no sensitivity testing was performed. |
A 57-year-old man presented in April 2017 with non-specific headaches and a short history of left facial numbness and pain. He had no other visual nor cranial nerve symptoms such as ophthalmoplegia nor paraesthesia. He had a longstanding history of stage 4 clear cell RCC. He underwent a left nephrectomy in 2008 with metastatic recurrence demonstrated in 2010. He was initially treated with a right adrenalectomy and stereotactic radiotherapy to a lung lesion. In terms of systemic therapy, he received first-line sunitinib from June 2011 and dendritic cell vaccine therapy from July 2011, followed by nivolumab.\nMagnetic resonance imaging (MRI) of his brain revealed a left-sided lesion centered in MC with extension into the cavernous sinus. It measured 17 x 12 x 18 mm and enhanced with gadolinium. There was no intra-axial involvement. Based on the clinical features and imaging, the provisional diagnosis was metastatic disease. There was no prior MRI available for comparison, and prior staging compute tomography (CT) scans including cranial imaging did not reveal the lesion. A four-week-interval MRI demonstrated progressive growth in keeping with metastatic disease (Figure ).\nThe patient was treated with Gamma Knife (GK) SRS to a dose of 20 Gy in one fraction prescribed to the 50% isodose in May 2017.\nClinical follow-up at two months post-GK SRS showed marked symptomatic improvement in facial pain and paraesthesia. The patient was able to cease analgesic medication. Radiological follow-up at six months post-GK SRS showed substantial tumour reduction on MRI; however, he had recently developed facial numbness and wasting of his muscles of mastication in keeping with a trigeminal nerve palsy. His lesion showed a partial response, reducing in size to 16 x 9 x 14 mm six months post-SRS with persistent residual enhancing changes seen one year following SRS.\nJust over 13 months post-GK SRS, he presented to clinic with a recurrence of facial pain, paraesthesia and new-onset diplopia consistent with a right sixth nerve palsy. MRI confirmed recurrent disease in the left MC with extension into the cavernous sinus (Figure ). CT staging revealed three metastases progressing in the left lower lung, right hilum and left gluteal soft tissue mass. These signified progressive systemic disease.\nThe patient was recommenced on sunitinib, which showed an initial partial response, but subsequently, his systemic disease progressed again in October 2019 with enlargement of the MC lesion to 24 x 20 x 18 mm (Figure ), a right lung mass and thoracic adenopathy. The result of a multidisciplinary team meeting was to retreat with SRS rather than surgery. He was treated with repeat GK SRS to the left cavernous sinus in November 2019 (30 months after initial SRS) to a dose of 30 Gy in five fractions at the 50% isodose line.\nAs of his MRI in August 2020 (nine months after repeat SRS), the MC metastasis was stable and post-treatment effect enhancement in the adjacent temporal lobe was noted (Figure ). From his latest clinical follow-up in March 2021, his facial pain had completely resolved, while his paraesthesia had significantly improved but was still present. He continued to experience diplopia, managed with prism glasses. A repeat MRI in March 2021 confirmed stability of the treated lesion with some enhancing post-radiosurgical change and oedema. |
We report a case of bilateral-altitude retinopathy with vitreous hemorrhage in the left eye in a 29-year-old boy. He presented to us with a history of floaters in the left eye after five days of climbing the Himalaya of 4760 meters in Nepal. He also complained of slight diminution of vision in the same eye. He disclosed that climbing mountain was his hobby and he was not using supplemental oxygen either. He denied a history of similar complaints in the past and did not give systemic symptoms like headache, giddiness, vomiting, or any discomfort.\nOn examination, the best-corrected visual acuity (BCVA) was 20/20 in the right eye and 20/30 in the left eye. Intraocular pressure was 12 mmHg in both eyes. Slit-lamp biomicroscopic examination of the anterior segment of both eyes revealed the normal finding. Dilated fundus examination revealed bilaterally dilated major retinal veins, and the retinal vein and artery ratio showed 3 : 1 in both eyes. The right eye revealed clear, quiet vitreous, healthy macula, and pink and smooth optic disc. There was fresh vitreous hemorrhage confined just one disc diameter away at the superior and inferior part of the optic nerve in the left eye. The macula and optic nerve head of this eye grossly looked normal. The peripheral retinal finding also revealed no tears or other abnormalities.\nColor fundus photographs were taken and are shown in . Macular optical coherence tomography (OCT) was not taken in this visit due to vitreous hemorrhage. Full blood count, haemoglobin, sugar, creatinine, urea, lipids, CRP, and serology for HIV, HCV, VDRL, and HBsAg were normal. The tuberculin test and chest X-ray also appeared normal. Consultation of an internal medicine specialist did not give any abnormalities.\nThe patient was counselled for a wait and watch schedule and asked to be followed up in 3 weeks or as soon as possible if flashes, curtain-like visual block, or poor vision was noticed.\nOn the 3rd week, his BCVA was 20/20 in both eyes. Vitreous was clear in both eyes; however, retinal venous tortuosities and dilation were still present in the left eye. The macular OCT scan showed normal-appearing fundus. The retinal vein and artery ratio changed to 3 : 2. Color photos and OCT are shown in . |
A 57-year-old male had a 3-year history of heartburn and a 1-year history of cough. The patient began to suffer heartburn, regurgitation, nausea, bloating, and vomiting 3 years ago. Acid could reflux to his mouth sometimes. He had to pad the head in order to reduce the incidence of reflux while sleeping. The patient began to cough while symptoms of regurgitation and heartburn aggravated 1 year ago.\nThe endoscopy result was esophagitis LA-C and atrophic gastritis with erosion. The patient was diagnosed as having GERD in a local hospital and received treatment of life adjustment and medicine including PPI and gastrointestinal prokinetic drug. The symptoms of cough, heartburn, and vomiting were relieved after taking drugs such as PPI in the beginning. However, the patient felt that symptoms were aggravated in the past 2 months and reoccurred after he stopping using drugs. He had a 5-kg weight loss during a period of 2 years. The body mass index of the patient was calculated to be 19.3 after hospital admission in our center. Physical examination revealed only abdominal slight tenderness.\nThe DeMeester score was 1.56 and the result did not meet the standard of acid reflux disease.\nThe lower esophageal sphincter pressure was lower than normal, all swallowing is invalid peristalsis.\nBecause the patient's 24-hour pH monitoring did not meet the standard of acid reflux disease, the patient completed upper gastrointestinal radiography to exclude other causes.\nThe results of the upper gastrointestinal radiography revealed esophageal reflux disease and possible gastritis; hence, SMA oppression syndrome (descending part of duodenal dilation, the contrast agent blocked through the tract, formation characteristic of penholder oppression) was considered (Fig. ). Abdominal CT scan revealed a 25° angle between the SMA and the aorta, a distance of 7.8 mm.\nThe patient was diagnosed as having SMA syndrome. We combined laparoscopic Toupet fundoplication with duodenojejunostomy to manage SMA syndrome complicating extraesophageal symptoms. |
A 38-year-old male patient received a renal transplant (RT) of a kidney from a donation after circulatory death donor. The right donor kidney had a single real artery on an aortic patch and the short renal vein which was elongated by using a segment of inferior vena cava. The kidney was implanted in the right iliac fossa by anastomosing the renal artery to the external iliac artery in an end-to-side fashion using continuous 5/0 prolene sutures (Ethicon Inc., United Kingdom) and renal vein to the external iliac vein in the similar fashion. An extravesical ureteroneocystostomy was performed as describe by Lich-Gregoir. The vascular anastomosis time was 45 min while the total cold ischaemic time was 15 h and 38 min. The patient received basiliximab (Sandoz, United Kingdom) and methyl prednisolone as induction therapy and tacrolimus, mycophenolate mofetil and prednisolone as maintenance immunosuppression.\nThe transplant had delayed graft function and required haemodialysis during the first week until renal function started to improve. The initial ultrasound scan of the transplant kidney showed a well perfused graft with no evidence of hydronephrosis or any collection and the resistive indices (RI) were within normal limits. The renal function was stable with a serum creatinine of 136 μmol/L and an estimated glomerular filtration rate (eGFR) of 51 mL/min per 1.73 m2 at 3 mo post-transplantation.\nAt five months post-transplantation, on routine outpatient review, deterioration in kidney renal function with a rise in serum creatinine to 633 μmol/L (eGFR 13 mL/min per 1.73 m2) was observed. A duplex ultrasound scan showed a well-perfused kidney with no evidence of hydronephrosis. An ultrasound-guided biopsy of the kidney, which was treated with three pulses of intravenous methyl prednisolone, showed features of acute cellular rejection. However, there was no improvement in renal function. A repeat duplex ultrasound scan showed damped flow signals on the intra-renal blood vessels with reduced RI ranging between 0.4 and 0.45. There were associated high velocities at the transplant artery origin which were suspicious of TRA stenosis. A computerized tomography (CT) scan was done which showed a 20 mm × 25 mm pseudoaneurysm arising from the aortic patch and the TRA origin lying adjacent to the pseudoaneurysm was tightly narrowed (Figures and ). After discussion in the departmental multidisciplinary team meeting and with patient’s informed consent, he underwent radiological intervention as described below.\nUnder ultrasound guidance, the right common femoral artery was punctured, and a 5Fr sheath was inserted. 7500 unit of heparin was administered intravenously. The dimensions of the TRA were confirmed and were found to be like those of CT scan findings. The TRA was catheterized using a size 4Fr Berenstein® catheter and Terumo® wire (Terumo Medical Corporation, United States), subsequently exchanged for a 0.014 Thruway wire (Boston Scientific Inc., Ireland) and was left in situ as a “safety wire”. The left common femoral artery was punctured and a 6Fr destination sheath was placed over the aortic bifurcation. Through the ipsilateral 6Fr sheath, a 10 mm percutaneous transluminal angioplasty (PTA) balloon was placed opposite the aneurysm neck. From the 6Fr sheath, a 4Fr Cobra (Cook Medical, United States) and 2/7 Progreat® microcatheter (Terumo Medical Corporation, United States) were used to gain access to the aneurysm sac. Within the right external iliac artery, the PTA balloon was inflated to reduce the risk of coil prolapse and migration and the aneurysm was embolized using two Azur® 20 mm Framing coils (Terumo Medical Corporation, United States), and packed with Azur® Hydrogel Coils. The pseudoaneurysm was filled with coils and hydrogel leading to its complete occlusion. Subsequently, the stenosed transplant RA was stented using a 6 mm × 20 mm Hippocampus stent (Medtronic, United Kingdom), which restored the patency of the stenosed renal artery and normal blood flow (Figure ).\nFollowing embolization of the pseudoaneurysm and stenting of the TRA, improvement in renal function occurred leading to a fall in the serum creatinine level to 159 μmol/L (eGFR 47 mL/min per 1.73 m2). A follow-up CT angiogram one month after the intervention showed a patent TRA with successful coiling of the pseudoaneurysm and satisfactory position of the stent with no evidence of TRA stenosis. The patient continues to be followed up in the routine RT clinic and has a serum creatinine of 150 μmol/L (eGFR 49 mL/min per 1.73 m2). |
A 44-year-old man with hypertension visited emergency department (ED) due to recurrent vertigo. Two weeks before admission, he visited the ED with a sudden onset severe headache in the retro-orbital area and right peripheral type facial palsy of House-Brackmann scale grade 4. No skin lesion was observed from the periauricular area and the external auditory canal. Computerized tomography was unremarkable. He was diagnosed as Bell’s palsy and received methylprednisolone 60 mg once daily for one week and then was under a scheduled tapering. He had history of hypertension and was under regular medication (amlodipine 5 mg and fimasartan 60 mg, once a day) for the last 2 years.\nThirteen days later, the patient felt headache aggravation after stretching himself and just after a few seconds the vertigo began. At the second visit to the ED, initial blood pressure was 184/105 mmHg. Neurologic examination revealed severe ataxia at left side. Sensory for pain and temperature was declined in the right arm and leg. Horner syndrome including miosis and ptosis of the left eye was observed. There also was gaze evoked nystagmus during left lateral gaze. The peripheral type right facial palsy remained. There was no family history, symptom or sign of any connective tissue disorder or a history of external trauma. There were no findings of secondary hypertension from an extensive evaluation.\nMagnetic resonance image (MRI) was taken from ED; an acute ischemic lesion was observed at the whole territory of left posterior inferior cerebellar artery at cerebellum with an additional lesion at left lateral medulla from diffusion weighted image (Fig. ). From the time-of-flight MR angiography (TOF-MRA), severe stenosis was observed from bilateral VAs and ICAs (Fig. ). TOF-MRA with high-resolution MR was followed up 2 days later; the stenosis at right distal ICA was improved (Fig. ) and, an intramural hematoma was observed from bilateral VA (Fig. and ) and an intimal flap was observed from bilateral distal ICAs (Fig. ). Digital subtraction angiography was performed; left VA was occluded. Right VA and left ICA demonstrated severe stenosis (Fig. and ). However, as dissection was multiple, located at eloquent area and not clinically worsening after the first stroke, intervention was not considered initially and antiplatelet agent was used. The patient was followed up 2 months after the initial event. There was no further ischemic event and the patient recovered to have mild ataxia and sensory change. The peripheral type facial palsy improved to a satisfactory level (House-Brackmann scale grade 2). Informed consent was obtained from the patient including the consent for the publication of all the personal, medical details and images. |
A 39-year-old woman visited at a provincial oncology center with a complaint of vaginal discharge and chronic pelvic pain for 4 months. The patient got married and was nulliparous. She had a history of intestinal multiple polyp resection by endoscopy 7 years ago, with no history of smoking, drug or alcohol use. Her father and her brother had been diagnosed with Peutz-Jeghers syndrome and died of colon cancer at the age of 64 and 45, respectively. Her older brother and his two children had experienced laparoscopic surgery for strangulated intussusception of the colon. She meets the diagnostic criteria for Peutz-Jeghers syndrome proposed by WHO criteria for diagnosis with gastrointestinal hamartomatous polyps (), characteristic, prominent, mucocutaneous pigmentation () and family history of Peutz-Jeghers syndrome. The patient did not have screening PAP smear before.\nGynecological examination revealed bloody vaginal discharge, a mass 3 × 4 cm in diameter was found on the cervix, not invading vagina and parametrium and no evidence of metastatic lymphadenopathy involvement. Initial biopsy was negative and colposcopy-guided cervical biopsy of the red ulcerated lesion was performed, and atypical glandular cells (AGC), endocervical type was recorded on histopathological examination.\nSonographic findings included a slightly enlarged uterus with a fluid filled uterine corpus (about 51–70mm). Endometrial biopsy was performed, which yielded endometrial hyperplasia. Pelvic magnetic resonance imaging (MRI) revealed a prominent uterine cervix with a diameter of 4 × 2.3 × 3.5cm (). Some pelvic lymph nodes about 8 × 9mm in size were found. The mass was confined to the cervix and did not invade the parametrium. No evidence of distant metastasis was identified. Laboratory data showed no blood, urine changes and serum tumor markers were within their normal ranges.\nThe patient was then diagnosed with clinical stage IB3 cervical cancer in accordance with the International Federation of Gynecology and Obstetrics 2018 staging system. Specific findings were 1) a tumor size of 4 cm in the greatest dimension, 2) depth of invasion of 3.5 cm, 3) no lymphovascular invasion, 4) no involvement of parametrium and 5) extension to the endometrium. Eventually, the patient underwent radical transabdominal hysterectomy, bilateral salpingo-oophorectomy and bilateral pelvic lymphadenectomy by a senior surgeon specialized in gynecological cancer surgery without delay. The final histopathological analysis of the specimen from radical surgery confirmed gastric type mucinous adenocarcinoma of the cervix (). Pathological stage IIIC was diagnosed with tumor dimension 5cm, myometrial invasion and metastasis to pelvic lymph nodes (2/7 positive nodes). The patient then had adjuvant chemoradiation therapy. After one year of follow up, the patient was stable and no recurrences were detected. |
A 67-yr-old Asian man who presented with intermittent melena and significant weight loss was diagnosed with small bowel GIST with multiple liver metastases and peritoneal seeding on May 25, 2010. He underwent jejunal resection and anastomosis with palliative intent. The excised jejunal GIST measured 7 × 5 × 5 cm and had a c-KIT exon 9 mutation (1,510-1,515 duplication). After surgery, the patient was treated with 400 mg/day imatinib. He had no concomitant medications or co-morbid diseases.\nTwo months later, the patient developed grade 3 edema, grade 3 ascites, and grade 2 vomiting, with chest radiography revealing layering of a moderate amount of pleural fluid. Because the patient had peritoneal seeding, it was hard to determine whether his ascites and pleural effusion were due to imatinib toxicity or the progression of GIST. Although the follow-up abdomino-pelvic computed tomography (CT) scan showed that the patient's liver metastases had not changed significantly, disease progression was suspected, which prompted a cessation in imatinib treatment and a commencement of sunitinib treatment. The patient was transferred to our hospital for a second opinion after he had been taking sunitinib for approximately 2 months. A thorough review of his previous serial CT scans showed no definitive evidence of disease progression whilst he was on imatinib treatment. We therefore decided to resume 400 mg/day imatinib and use 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT for accurate assessment of response to imatinib treatment.\nOne month after the patient restarted imatinib, a PET/CT scan revealed a significant decrease in maximum standardized uptake value from 4.2 to 2.9 in one of his liver metastases (), suggesting the tumor was responding to imatinib. However, the patient required paracentesis once weekly to control his ascites, and he complained of peripheral edema and dyspnea. Because imatinib blood level testing revealed that the patient had very high imatinib trough plasma exposure, 4,120 ng/mL and 4,600 ng/mL on two different days (), we decreased his dose to 300 mg/day, which resulted in a steady-state imatinib plasma trough concentration of 3,220 ng/mL (). However, the patient still had grade 2 edema, ascites, and dyspnea on exertion due to pleural effusion. As his imatinib trough plasma exposure was sufficiently high to achieve an adequate tumor response (), we further reduced his dose to 200 mg/day. At this dose, his fluid retention, including ascites, edema, and pleural effusion, was greatly improved, and he had no difficulties in daily life; in addition, his liver metastases remained stable ().\nFive months later, the patient expressed concerns that 200 mg/day of imatinib may be insufficient to control his tumor, as studies have shown that GIST patients with the c-KIT exon 9 mutation may benefit from higher than normal exposure to imatinib (, , ). We therefore increased his dose to 300 mg/day. He was able to tolerate ascites and dyspnea with the use of regular diuretics but had some limitation of activities. Follow-up CT scans to date showed the patient's disease remained stable (). |
Ms A, a 90-year-old woman with a known past medical history of hypertension, chronic obstructive pulmonary disease, lower extremity edema, cognitive deficit, and urinary incontinence, was admitted to the hospital for cellulitis in her right lower extremity, that initially responded to intravenous vancomycin. She was readmitted for recurrence and growth of the discolored skin area, consistent with cellulitis that did not recede after the administration of oral antibiotics (doxycycline and sulfamethoxazole). Following the last admission, she had developed an extensive area of red-purple discoloration surrounding a dark purple, raised round lesion that was 2 cm in diameter, with a smooth surface and puffy consistency. The lesion was located in the anterior aspect of her right shin.\nDespite extensive discoloration of the skin area, the patient was afebrile and did not have leukocytosis. Intravenous vancomycin was resumed, and the differential diagnosis was expanded to include vasculitis and neoplasia. A small fluid collection with internal echoes was identified on ultrasound of the dark purple lesion, leading to suspicion of organizing hematoma or bacterial abscess. However, needle aspirations of the lesions yielded only a few polymorphonuclear leukocytes and the absence of bacteria (an erythrocyte sedimentation rate [ESR] of 69).\nThe differential diagnoses were revised to include cutaneous malignancy. Given the purple discoloration and associated chronic lymphedema, consideration was also given to CA, and cutaneous B- or T-cell lymphoma. Wedge skin biopsy was recommended by the dermatologist. However, The patient would not consent to the surgical procedure owing to cognitive deficit (associated with Montreal Cognitive Assessment score of 11/30, with the identification of cerebral atrophy on head computed tomography).\nPreviously, the patient authorized one of her two daughters to be a surrogate medical decision-maker. The other daughter could not commit to a definitive decision because of her own medical history of traumatic brain injury. During discussions about the risks and the benefits of the procedure, the surgeon expressed significant concern about lack of healing at the surgical site, particularly in the context of chronic edema and the patient’s very frail skin.\nOncology was consulted for alternative diagnostic approaches. Biopsy remained the optimal diagnostic test but was not pursued given the likelihood of a nonhealing ulcer at the surgical site. Radiotherapy was recommended as the most suitable treatment for palliative purposes, based on suspicion of neoplasia.\nThe ESR increased from 69 to 86 and intravenous antibiotics were stopped after a 10-day course, by which time the C-reactive protein levels had normalized. The patient was discharged from the hospital, subject to clinical observation of the reported skin lesion, despite significant anxiety over the need to obtain a diagnosis and the inability to perform a biopsy. The patient’s daughter did not consent to bringing Ms A for radiation therapy as the daughter believed that it would be too difficult for the patient and that it would impact negatively on her quality of life.\nSubsequently, the dark purple lesion continued to grow and eventually ruptured. A biopsy was performed by the surgeon during a house call visit. Given her cognitive deficit, the patient was not fully able to understand her condition. In addition, despite learning about the poor prognosis attributed to the disease, the two daughters initially refused to admit their mother in hospice. At that stage, potential future clinical complications were envisioned, including infection, bleeding, and deep vein thrombosis.\nWound care was pursued using silver alginate to prevent infection at the lesion site. By then, the clinical course was complicated by bleeding and pain. Opiates were used for pain control and ambulation became progressively more difficult, despite the patient having assistance at home.\nA hemoglobin level of 10 g/dL was recorded on patient discharge. Once home, the bleeding increased in intensity. Ambulation was still possible, but with considerably more effort and increased shortness of breath. The surrogate decision-maker continued to refuse a hospice admission. During a subsequent house call visit, when asked if the patient had any chest pain, the daughter agreed that she did and that it was a new symptom. The physician then re-discussed the need for hospice care with the daughters and explained that it was likely that myocardial infarction (MI) would complicate the bleeding. It was explained that MI is associated with significant distress and an increased need for pain- and anxiety-reducing medication, which eventually helped to overcome the family’s resistance to the idea of admitting her to a hospice.\nThe patient continued to experience pain that increased in intensity once in hospice and the bleeding worsened to the point where the leg wound dressings were becoming saturated daily. The subsequent clinical course was complicated by delirium, at which point the daughters called 911 and had the patient was sent to the emergency room. The patient received a blood transfusion for a hemoglobin level of 4.6 g/dl Complications relating to the potential need for repeated blood transfusions were pivotal to discussions about patient treatment. Although blood transfusions would reduce the patient’s shortness of breath, chest pain, and delirium, they would also artificially prolong her life, thereby exposing the patient to additional complications, and, in particular, worsening pain. After careful and involved counsel with the patient and her family, a decision was taken not to subject her to further blood transfusions. Further treatment centered exclusively on symptom control and she died peacefully at home, surrounded by her family. |
A 65-year-old man came to our attention in October 2018 due to the appearance of a right frontotemporal swelling with progressive growth for about a month and a half. In anamnesis, he had a single kidney. The patient’s neurological exam was negative. Contrast-enhanced brain magnetic resonance imaging (MRI) revealed a large lesion (28 x 20 x 38 mm) in the right frontotemporal cranial theca. The lesion had caused complete bone lysis involving the full thickness of cranial planking and extending widely into the subcutaneous soft tissues while extending approximately 4 mm into the extradural space. In the T1 sequence, the lesion was homogeneously intense and, after administration of contrast, it was characterized by inhomogeneous enhancement (Figure ).\nAs metastasis was suspected, the patient preoperatively underwent a total body computed tomography (CT) scan, which showed the presence of diffuse thickening of the left postero-lateral bladder wall and a solid endoluminal protrusion at the ureteral outlet, characterized by irregular endoluminal margins and discrete enhancement (Figure ). Urinary cytology showed the presence of carcinoma cells (Figure ).\nThe patient gave informed consent for the surgery and to the use of the data for scientific purposes. During the operation, which took place on July 11, 2018, the patient underwent gross total removal (GTR) of the head lesion and subtotal resection (SR) of the bladder lesion. The cranial lesion was of a hard-elastic consistency and was resected en bloc, involving bone margins apparently without disease. The dura mater was infiltrated so it was removed keeping very wide edges. Dural plastic surgery using NeoDura and cranioplasty with titanium mesh were performed. The temporal fascia and epicranial tissue over the lesion were removed.\nDuring the transurethral cystoscopy, in the left postero-lateral bladder wall, a solid lesion of about 2 cm with a large implant base with significant periwound edema was identified. The lesion was transurethrally resected. The resection did not appear to reach healthy tissue.\nA histological exam of the cranial lesion revealed metastasis of poorly differentiated carcinoma with an immunophenotype compatible with urothelial origin (cytokeratin 7+, cytokeratin 20+, gata 3 -/+) and focal infiltration of the dura mater (Figure ).\nA histological exam of the bladder lesion revealed a high-grade urothelial carcinoma (gata 3+, chromogranin-, synaptophysin-) infiltrating the subepithelial connective tissue (t2 hg9). The results of molecular screening (fibroblast growth factor receptors (FGFR) mutation/translocation research) were negative for molecular alterations of FGFR3 (Figure ).\nThe patient was discharged in good clinical and neurological conditions six days after surgery.\nOncoradiological screening (scintigraphic bone scan [December 5, 2018], chest-abdomen CT scan [January 25, 2019], and positron emission tomography [PET] scan [February 11, 2019]) confirmed the presence of a bladder wall lesion and revealed the presence of lesions at the level of the left hemi-mandible, right ethmoid, the middle third of the left femur, as well as 2-mm lung focalities, and lymphadenopathy at the level of the left common iliac artery and retroaortic artery.\nTherefore, the patient underwent chemotherapy (carbo therapy and gemcitabine for four cycles) and radiotherapy to the left hemi-mandible (up to 30Gy in 10 fractions with the 3Dconf technique and 6MV photons) between March 3, 2019, and March 29, 2019, and to the middle third of the left femur (up to 20Gy in five fractions with the 3Dconf technique and 18MV photons) between March 18, 2019, and March 22, 2019, with an initial good response.\nRadiological examinations showed good tumor control. PET scans (performed on June 4, 2019, and October 30, 2019) showed a significant lowering of the pathological accumulation of the radio drug, and the chest-abdomen CT scan indicated a reduction in the size of the known lesions.\nIn December 2019, the patient reported motor impairment in his left hand. MRI scan revealed a metastatic cortico-subcortical right temporoparietal lesion (Figure ). On December 27, 2019, the patient underwent surgery to remove the brain lesion.\nA histological exam confirmed the known diagnosis (Figure ). The patient immediately showed an immediate improvement of the neurological deficit and was discharged three days after surgery in good clinical condition. The patient died two months later after suffering a heart attack. |
A 17-year-old male with no history of medical illness and a previously usual state of health presented to King Fahd Hospital of the University with acute facial numbness along the distribution of the right V1 (ophthalmic division of the right trigeminal nerve), followed by weakness in the right eye abduction (i.e., right abducent nerve palsy). Magnetic resonance imaging (MRI) of the brain was performed and it showed a right parasellar mass (iso- to hypointense in T1 and hyperintense in T2) []. Surgery was offered to the patient and his family, but it was not performed owing to the family's socioeconomic concerns. Subsequently, the patient was lost to follow-up until he revisited the hospital a year later complaining of acute right-sided ptosis associated with dilated nonreactive right pupil (i.e., right oculomotor nerve palsy). MRI of the brain was performed and it showed expansion of the right parasellar mass []. The patient was admitted for workup and was prepared for surgery. After 5 days, he had complete right ophthalmoplegia. MRI of the brain was repeated and it showed an expanding right parasellar mass with interval development of T1 high-signal intensity and multiple fluid-fluid level on T2, suggestive of internal hemorrhage. It also showed multiple internal locules and septations with peripheral enhancement []. Radiology report suggested differential diagnosis of aneurysmal bone cyst versus cystic meningioma. Magnetic resonance angiogram was negative.\nThe right temporal craniotomy with gross total excision of the right parasellar mass was done, and the intraoperative frozen section showed spindle cell proliferation. The final histopathology showed a typical benign schwannoma appearance showing random nuclear pleomorphism along with short spindled cells. Most of the tissue was of the Antoni A type (dense and organized), with very few Antoni B type tissue. No Verocay bodies were seen. S-100 protein immunochemistry showed strong, dispersed cytoplasmic and nuclear reactivity in the schwannoma [].\nPostoperative MRI of the brain was done after about 4 months and it showed complete mass excision with no residuals and no recurrence []. The patient showed gradual improvement in the pupillary size and reaction as well as in extraocular movements. During his last visit, the patient was found to have been completely cured with full extraocular movements and normal pupil size and reaction. |
A 63-year-old man with a history of hypertension, hyperlipidemia, type 2 diabetes mellitus, and cigarette smoking was admitted to the hospital following episodes of massive hemoptysis in the previous 24 hours. Admission laboratory work did not reveal a coagulopathy or thrombocytopenia. A chest radiograph demonstrated a radiodensity in the medial aspect of his left lower lung. A follow-up chest computed tomography (CT) scan was significant for a 4-cm spiculated cavitary mass in the superior segment of the left lower lobe that abutted the posterior mediastinum along with several subcentimeter nodules inferior to the lesion.\nHis presentation was highly suspicious for a primary bronchogenic carcinoma. The patient underwent bronchoscopy. The takeoff to the superior segment was significantly stenotic with induration and heaped-up mucosa. The FOB was advanced into the cavity. Forceps biopsies through the working channel of the bronchoscope were taken inside the cavity. This led to bleeding from the orifice of the superior segment that was initially controlled using a combination of recombinant thrombin and balloon bronchoplasty with a 4-Fr Fogarty balloon. Although the bleeding slowed, it, nevertheless, continued unabated.\nAt that point, two pieces of oxidized regenerated cellulose (ORC, Surgicell®, Johnson and Johnson's, London) approximately 15×15 mm were folded and placed into the jaws of a flexible biopsy forceps. The forceps were then withdrawn into the operating channel of the bronchoscope, and the scope was reinserted into the airways. The forceps were inserted into the cavity, and the ORC was deployed. No further samples were taken to prevent dislodgement of the ORC.\nThe pathology on the forceps biopsy was consistent with inflammatory changes. He then underwent thoracotomy 1 week later due to a continued concern for a malignancy. Prior to the thoracotomy, there were no further episodes of bleeding. The mass was noted to be quite fibrotic with involvement of a branch of the inferior pulmonary artery. Acid-fast bacilli were noted on pathology staining, and DNA probe was positive for Mycobacterium tuberculosis. |
A 40-year-old gentleman presented with low backache of 5 years duration. He was evaluated clinically and radiologically. A diagnosis of PDB was made. Bone scan revealed multiple site involvement (skull, sternum, dorsal and lumbar, pelvis, ribs, femur, and tibia) (, , , ). Bone biopsy from the iliac region revealed numerous multinucleated giant cells with the haphazard new bone formation and diagnosis of polyostotic Paget’s disease was confirmed. The patient was treated with intravenous (IV) bisphosphonate every 3 weeks (pamidronate 60 mg IV infusion) with vitamin D and calcium supplement at another institution. He was apparently alright for 3 years when he noticed a lump in the right gluteal region. It was associated with dull aching localized pain. Radiographs revealed a lytic lesion in right posterior ilium and a magnetic resonance imaging (MRI) of the pelvis showed a large lesion with an extraosseous soft tissue component involving the iliac bone and adjacent sacral ala. Blood investigations showed an elevated serum alkaline phosphatase. Computed tomography-guided trucut biopsy from gluteal mass was diagnosed as GCT. Serial angioembolizations () were attempted with intent to control the disease without surgery owing to the complex anatomy and associated morbidity of surgery, but the mass progressed in size. A decision for surgical excision was taken. Excision of soft tissue mass with curettage and cementation of the right sacroiliac component of the lesion was done. The final histopathology report confirmed GCT in case of PDB. 2 years after the surgery, he presented with a large local recurrence involving the posterior ilium and sacral ala. The recurrence was confirmed with a repeat biopsy. He was started on denosumab, a monoclonal RANK ligand inhibitor in an attempt to downstage the lesion and thus reduce the morbidity of surgery. Denosumab was administered at a dose of 120 mg subcutaneously at monthly intervals with loading doses at day 1, 8, and 15. A repeat MRI evaluation, after 6 injections of denosumab, revealed a good response with shrinkage of the soft tissue mass. He then underwent partial Type-1 internal hemipelvectomy and curettage of the sacral lesion (, , , , ). At a 15 months follow-up, the patient is asymptomatic and disease-free. |
A 33-year-old gentleman who was allegedly involved in a motor-vehicle accident was referred to our unit for further management of the suspected orbital fracture. Initial assessment in the Emergency Department (ED) showed that the patient was drowsy, not opening his eyes, or responding to questions, possibly due to alcohol intoxication. His conscious level improved gradually within 24 hours. Clinical assessment showed a right periorbital hematoma and markedly swollen right cheek. Step deformity both over the right supra- and infraorbital rim cannot be appreciated due to the swelling. Satisfactory mouth opening was also recorded. We reviewed the computed tomography (CT) done for the assessment of concomitant traumatic brain injury. The right temporal bone fracture with a fractured fragment in the temporomandibular joint (TMJ) space, the right zygomatic arch fracture, the right greater wing of the sphenoid fracture, the orbital floor fracture, and lateral and medial walls of the right maxillary sinus fracture were reported. The patient was subsequently observed in the neurosurgical ward for 48 hours prior to his discharge. Outpatient assessment in the oral and maxillofacial clinic a week after trauma noted grossly symmetrical malar prominence, with resolving right periorbital hematoma. No enophthalmos, diplopia, and eye motility restriction were clinically observed (). Mouth opening was, however, quite limited to 25 mm interincisal. Further ophthalmology assessment confirmed good eye movement with no diplopia. The patient continued to be reviewed weekly under maxillofacial follow-up for assessment of late enophthalmos. Cone-beam CT (CBCT) orbit carried out 3 weeks after trauma confirmed findings of the previous scan. The fractured lateral wall and the floor of the right orbit (involving the infraorbital foramen), the fractured right greater wing of the sphenoid, the lateral wall of right maxillary sinus involving the right alveolar process, and the undisplaced fracture of the right zygomatic arch were observed. Detailed radiographic analysis showed that although the fracture size is small involving less than 50% of the overall size of the orbital floor, some degree of herniation albeit without periorbital entrapment was observed (). Additionally, the orbital floor fracture occurred just immediately behind the equator of the globe, that is, at the main bulk of the inferior rectus muscle. However, its muscle height-to-width ratio remained intact. These detailed radiographic findings may suggest that the patient could develop late orbital fracture complications such as late enophthalmos, diplopia, and restriction in eye movements. These complications could occur secondary to the incarceration of periorbital tissues or muscle or later cicatrization in and around the inferior rectus muscle.\nHowever, it was also noticeable to us that the fractured lateral wall of maxillary sinus was displaced medially along with some lateral soft tissue components. All muscles especially the lateral and inferior extraocular muscles appeared normal. Close follow-up was advocated in this case. At one month after trauma, the patient's eye movements remained intact, and there were no diplopia and apparent enophthalmos. Given these clinical findings, he was subjected to conservative treatment with regular follow-ups. The interesting component of this case is that, despite the multitude of bony fractures, the patient did not develop enophthalmos, diplopia, or restricted eye movements. Upon close review of the CBCT of the right orbit, we believe that the medialization of the fractured lateral wall of maxillary sinus has somehow acted and has healed as a pillar, partly or wholly to maintain the integrity of the right orbital floor. |
A 25-year-old African American woman, Gravida 7, Para 1, Aborta 5, presented at 29 weeks and 2 days with threatened preterm labor. The patient initially sought care at an outside facility where she received 0.25 mg of terbutaline SC for tocolysis and 12 mg IM of betamethasone for lung maturation. The patient was transferred to our tertiary facility with strong, regular uterine contractions. She underwent a transvaginal ultrasonogram which showed a normal cervical length of 3.4 cm. The patient was placed on continuous cardiotocographic monitoring and started on nifedipine (Procardia) 20 mg every 4 hours, with subsequent administration of the second dose of 12 mg IM betamethasone. Her pregnancy was complicated by opioid abuse, normocytic anemia (hemoglobin on admission 9.9 g/dL), and history of low transverse cesarean section for breech presentation. During the course of her hospitalization, she complained of heart palpitations and chest pain that radiated to her neck. On examination, her pulse palpated as irregularly irregular and vitals revealed a tachycardia into the 140 s. A twelve-lead ECG confirmed atrial fibrillation with rapid ventricular response. Cardiology was consulted. The patient was transferred to the intensive care unit and began on diltiazem drip and intravenous metoprolol for rate control. She received a total of six doses of nifedipine during her admission before discontinuation of the medication. Her symptoms occurred within 20 hours from the first dose of nifedipine. Work-up included an echocardiogram, lower extremities venous Doppler, troponin levels, thyroid function test, electrolytes, liver function tests, and a repeat urine drug test. All results were normal apart from borderline magnesium of 1.7 mg/dL (see for further results). The patient converted to normal sinus rhythm in less than 24 hours with a CHA2DS2-VASc score of 1 and anticoagulation with 81 mg aspirin was started. After transfer out of the intensive care unit the patient remained in sinus rhythm for the remainder of the hospitalization. Discharge medications included metoprolol 25 mg twice daily for rate control with close outpatient follow-up with MFM and cardiology.\nThe pregnancy culminated with a repeat low-transverse cesarean section at 39-week gestation resulting in a live-born male infant with Apgar scores of 9 and 9 at 1 and 5 minutes, respectively, and birthweight of 3400 grams. Continuous cardiac monitoring for 24 hours following delivery showed sinus rhythm. Per cardiology recommendations she was continued on metoprolol for prophylactic rate control and 81 mg aspirin in the postpartum period until she was seen as an outpatient. During that visit, metoprolol was discontinued. |
A 72-year-old female was admitted to the operating theater for aortic valve replacement. During the operation and in the first hours in the intensive care unit (ICU) the patient had to undergo TEE in order to assess postoperative left ventricle performance status. Soon after, in the ICU, the patient presented hematemesis. Gastroscopy revealed a corkscrew esophagus along with a bend before the gastroesophageal junction. A deep 2 × 1.5 cm ulcer covered with blood thrombus was found approximately 2 cm above the gastroesophageal junction. Blood oozing was clearly seen from the ulcer edges. Despite thorough irrigation the thrombus could not be removed. Endoscopic therapy with 10 ml NaCl 0.9% solution successfully stopped bleeding. The rest of the endoscopy was unremarkable. Within the next 48 h, thoracic and abdominal CT scans were ordered twice to rule out esophageal perforation. Only a few blood clots were found in the area between the stomach and the left liver hilum, but no signs of mediastinitis or free intra-abdominal air. The patient remained in the ICU hemodynamically stable and on nil by mouth.\nFour days later, as the patient became febrile, a second gastroscopy was performed. A 2 × 1.5 cm perforation was seen at the same site of ulcer with no bleeding (fig. ). Abdominal X-ray confirmed subdiaphragmatic air. Due to the patient's clinical condition and the size of the gap, an endoscopic intervention was decided. A new 12-mm clip (OTSC®; Ovesco Endoscopy, Tübingen, Germany) was engaged. The clip is made of nitinol and approximates large perforation margins like a surgical clamp. It is preloaded over a transparent cap attached to the scope tip. The perforation size was endoscopically assessed with a biopsy forceps. The edges of the perforation were approximated by using a specific endoscopic double grasping forceps and applying suction through the cap. Thus, the tissue was pulled into the cap and the clip was released by rotating the wheel attached to the shaft of the endoscope. The result was inspected endoscopically (fig. ). A nasogastric Levin tube was uneventfully left in the stomach under direct vision for long-term enteral feeding. The whole procedure lasted approximately 14 min. Further, the patient was offered two intra-abdominal drainage catheters in the operating theater so that intra-abdominal air and blood clots be drained and high fever get under control. All intra-abdominal and peripheral blood cultures were sterile.\nThe patient was kept on intravenous antibiotics, proton pump inhibitors and parenteral nutrition over the next 10 days followed by enteral feeding through the Levin tube. Two weeks later she was transferred to the clinical ward, experiencing no dysphagia, and was discharged fully recovered 3 months after the operation. |
A 44 year-old male presented with 14 years of chronic infections and pelvic pain. Although subject to frequent respiratory and gastrointestinal infections since childhood, his pelvic pain began at age 30. Following initiation of antibiotic and corticosteroid treatment for acute sinusitis, he developed a painful erythematous scrotal rash. His core symptoms are presented in . He initially attempted treatment with antifungals with mild improvement, but his rash gradually worsened over time. After another course of antibiotics for acute sinusitis, his rash spread to the glans penis and he subsequently developed severe urethral, testicular, and pelvic pain. He also began having chronic purulent sinusitis, and since that time he has struggled to control his upper respiratory symptoms, pelvic pain, and scrotal rash. The dynamics of his symptoms over time is presented in .\nHe has undergone treatment with numerous immunologic and antifungal therapies including G-CSF, IFN-gamma, GM-CSF, IVIG, IL-2, fluconazole, amphotericin B, micafungin, itraconazole, caspofungin, voriconazole, with varying levels of success (–). He experienced substantial improvement of his pelvic pain and skin lesions and mild improvement of his upper respiratory symptoms with micafungin but had to forego therapy for financial reasons ( and ). Other regimens including GM-CSF () with fluconazole and amphotericin () with caspofungin () have also helped control symptoms, but to a lesser degree. He has consistently noted worsening of his pelvic pain, rash, and upper respiratory symptoms with antibiotic treatments. Unfortunately, medication costs and side effects have prohibited the establishment of a successful long-term regimen. His pelvic pain and the fatigue associated with his symptoms have significantly impacted his quality of life. His pelvic pain limits his ability to sit for long periods of time, and his sinusitis is associated with pharyngitis, headaches, fatigue, and malaise. These symptoms have limited his ability to work, exercise, maintain a social life, and enjoy dating or sexual activity.\nHe has undergone extensive work-up, which has failed to identify a unifying underlying diagnosis (, , and and , , ). Prostatic fluid and ejaculate cultures, however, have grown multiple Candida species (), and immunologic testing has demonstrated anergy to Candida antigen ().\nWe hypothesized that defects in the cellular immune response may underlie his clinical condition, as we have demonstrated previously in other chronic fungal infections. , The current study was approved by the Institutional Review Boards of Children’s Hospital Boston, Beth Israel Deaconess Medical Center in Boston and Radboud University in Nijmegen, the Netherlands. The individual in this manuscript has given written informed consent to publish these case details, as outlined in the PLoS consent form available at: .” |
The patient, a 45-year-old female, was diagnosed with an infiltrating ductal carcinoma in the right breast (cT3N1M0) by fine needle aspiration biopsy in June 2009. The patient subsequently received six cycles of neoadjuvant chemotherapy. A follow-up mammectomy was performed in November 2009. In June 2012, the patient presented with palpable masses on the right thyroid, isthmus and lateral neck. Subsequently, the patient underwent a bilateral subtotal thyroidectomy and lymphadenectomy. Histological assessment of the surgical thyroid specimens and the neck lymph nodes revealed multiple carcinoma foci. The microscopic and immunohistochemical findings confirmed that the thyroid masses were breast cancer metastases.\nThe primary lesion excised from the right breast was a 20×18×15-mm infiltrating ductal carcinoma. The metastases excised from the right and left thyroid three years later were 30×20×15 and 20×15×12 mm in size, respectively, and the lymph nodes contained sheets of metastatic carcinoma (4/9). The diagnosis of metastatic carcinoma was made based on immunostaining data for the estrogen receptor (ER), progesterone receptor (PR) and CerbB-2 in neoplastic cells. Additionally, staining for thyroglobulin (TG) and thyroid transcription factor 1 (TTF-1), which are expressed in the thyroid gland but not in breast cancer tissue, was performed to differentiate the thyroid component from neoplastic breast cells.\nHistologically, pleomorphic tumor cells from the breast carcinoma metastasis were intermingled with the thyroid follicles (, hematoxylin and eosin staining of the adenocarcinoma tissue in the breast and thyroid gland). In the thyroid gland, clusters of metastatic breast carcinoma cells were surrounded by a normal thyroid component. Tumor cells in metastatic foci exhibited negative staining for the ER and PR and strong positive staining for CerbB-2, similar to the staining pattern of the primary breast lesion. The adjacent normal thyroid tissue was positive for TTF-1 (, lower panel, region IV) and TG (, lower panel, region II); however, the metastatic tumor cells were negative for both markers (, lower panel, regions III and I). These two markers indicate that the cancer lesions did not originate from the thyroid component. |
A 58-year-old man presented with productive cough and fever. His medical history was significant for hepatitis C and a maternal family history of colon cancer. The patient had no past history of cancer or surgery. He was an ex-smoker (40 pack/year), and worked as a screenwriter and photographer. He had no history of asbestos exposure. He was initially treated with antibiotics, and his symptoms were resolved. However, due to persistent abnormal chest X-ray findings, a CT scan of the chest was carried out revealing a 5 cm × 4 cm paraspinal mass in the upper right chest, which was also intensely hypermetabolic on a corresponding positron emission tomography scan without evidence of lymph node metastasis. No pleural effusion was detected []. A radiologic differential diagnosis included a posterior mediastinal neurogenic tumor and a metastatic carcinoma. Because the mass was located adjacent to the esophagus, an esophagogastroscopy was arranged to rule out esophageal cancer, and this was normal. A magnetic resonance imaging of the brain and a bone scan were negative. Laboratory studies were all within the normal range. The mass was aspirated under CT guidance using coaxial technique and a 22-gauge needle. Air-dried and alcohol fixed smears were stained with Romanowsky and Papanicolaou method. A cell block was prepared from sample rinsed in saline, using the histogel method. Rapid on-site assessment provided by a cytopathologist was recorded as an adequate sample showing an epithelioid neoplasm.\nThe smears showed a hypercellular specimen consisting of loosely cohesive “lobules” of heavily vacuolated epithelioid cells displayed against a background of myxoid material, which was highlighted on Field's and Giemsa stained direct smears, suggesting the possibility of chordoma []. The vacuoles in the cells were filled with the same myxoid material seen in the background [] and were negative for mucicarmine stain. Occasional cells with intracytoplasmic vacuoles displacing their nuclei to the periphery resembling signet-ring cells were also seen within the lobules, expanding the differential diagnosis to include the possibility of adenocarcinoma []. Individual microcysts were fused, resulting in secondary cystic dilatation. The epithelioid cells showed moderate to marked nuclear pleomorphism out of keeping with chordoma, hyperchromatic nuclei, prominent nucleoli, a dense chromatin pattern, and abundant cytoplasm. Mitotic activity was easily identified []. There was no evidence of necrosis. The working differential diagnoses included chordoma, benign adenomatoid tumor, epithelioid hemangioendothelioma, adenocarcinoma, and epithelioid MM.\nImmunohistochemical studies performed on cell block sections showed tumor cells were strongly immunoreactive for calretinin, WT-1, D2-40, cytokeratin (CK) 7, and AE1/AE3; and moderately positive for high molecular weight keratin (CK5/6), vimentin, and epithelial membrane antigen, which supported a mesothelial origin. Negative stains included thyroid transcription factor-1, Ber-EP4, carcinoembryonic antigen, S100 protein, CK20, and CDX-2, which excluded adenocarcinoma and chordoma and further supported the diagnosis of mesothelioma []. In the context of radiologic findings, a diagnosis of localized MM, microcystic (adenomatoid) variant, was made. The patient subsequently underwent right pneumonectomy. Examination of the lung found a localized, pleural-based 4.8 cm tumor located adjacent to the right upper lobe. The tumor involved parietal and visceral pleura, and focally invaded the underlying lung parenchyma and overlying chest wall soft tissue, confirming the diagnosis of MM. The patient remains disease free 29 months after the pneumonectomy. |
A 55-year-old African American female with a past medical history of intravenous drug abuse (cocaine), chronic hepatitis B & C, chronic obstructive pulmonary disease (COPD) and hypertension presented with blurring of vision, and floaters. Best-corrected visual acuity was 20/25 and slitlamp biomicroscopy was unremarkable in both eyes. Intraocular pressure was 30 in the right eye and 28 in the left (Goldmann applanation tonometry). Fundus examination showed multiple, small, yellow, glistening crystals scattered throughout the posterior pole of both eyes. Crystals appeared to be located inside small retinal arterioles and venules of the macula with few crystals scattered outside the vessels within the retina (Fig. ). The optic nerve showed increased cup to disc ratio of 0.75 with no disc hemorrhage OU. There were no signs of neovascularization and retinal periphery was unremarkable in both eyes. SD-OCT demonstrated multiple hyper-reflective dots of varying sizes scattered among the nerve fiber layer, ganglion cell layer, inner plexiform and inner nuclear layer (Fig. ). Thinning of the inner retinal layers in temporal part of the macula, a finding similar to that usually seen with vascular occlusion was also noted in the right eye (Fig. ). AO imaging revealed multiple shiny refractile dots distributed both intravascularly and extravascularly corresponding to those seen in color photo. The high resolution of AO allowed detection of some tiny particles that were not detectable clinically (Arrow heads: Fig. ).\nDifferent diagnostic modalities have been used previously to characterize the retinal lesions in talc retinopathy. Fundus examination usually reveals a characteristic appearance of these crystals, which are found inside the small retinal vessels and throughout the fundus. Talc crystals should be differentiated from other conditions causing crystalline retinopathy and from other causes of retinal embolism. Classification of the detected materials was based on clinical diagnosis. Careful examination of these crystals have shown that they most commonly represent an accumulation or clumps of multiple talc particles rather than a single embolus of talc []. Absence of retinal granuloma as may be seen in the lungs of IV drug users might be due to the blood retinal barrier. A vascular filling defect is seen on fluorescein angiography when the embolus block the small retinal blood vessels causing capillary non perfusion and retinal ischemia []. With the introduction of SD-OCT, the locations of these crystals with respect to retinal layers have been demonstrated. They were found distributed among the inner retinal layers where retinal blood vessels reside []. |
A 50-year-old Chinese woman reported the presence of a cough associated with a small amount of sputum for the previous month, and a fever over the previous week. A physical examination and laboratorytests showed no abnormal findings.\nWhen a posteroanterior chest radiograph was obtained, a widened mediastinum, a mass protruding from the pulmonary segment were detected (Figure ). Using contrast-enhanced computed tomography (CT), a 9 × 11 cm2 lobulated mass exhibiting heterogeneous enhancement and punctate calcification with sharp margins was observed. The capsule of the mass was enhanced in the CT images. The mass was located in the pretracheal space and aortopulmonary window (Figure ), directly adjacent to, and displacing, the superior vena cava (SVC), ascending and descending aorta, pulmonary artery, left atrium, trachea, and primary bronchi. In addition, small amounts of pericardial and left pleural effusion were observed (Figure ).\nComplete surgical resection was performed via a thoracotomy approach. The resected intrapericardial tumor was large in size and firm. The tumor was situated under the ascending aorta, with its right edge adhered to SVC, and was compressing the SVC and right atrium. The upper edge of the tumor was adjacent to the aortic arch, the left edge was attached to the pericardium and left hilus of lung, the lower edge was adhered to left atrium and pulmonary artery, and the posterior edge was adjacent to the trachea, primary bronchi and the descending thoracic aorta, The tumor compressed the trachea and was pushing the primary bronchi toward the vertebral column. The tumor exhibited lobulation and capsulation (Figure A). Correspondingly, no infiltration of adjacent organs was observed.\nHistological examination revealed that the tumor was made up of two tissue types: Antoni A and Antoni B. The Antoni A type tissue was composed of spindle cells that were closely packed together and arranged in bundles and rows with palisading nuclei without mitoses (Figure B). The Antoni B type tissue had a low density of cells that were dispersed in a loose and random fashion; the tumor cells were polygonal with abundant cytoplasm, cytoplasmic lipids, and had round or oval nuclei (Figure C). The tumor cells were strongly immuno positive for S-100 protein (Figure D).Based on these results, a diagnosis of benign pericardial schwannoma was made.\nThe patient’s general health was good. She exhibited no evidence of recurrence at a follow-up CT scan performed 5 months after the surgical resection. |
A 73-year-old man underwent periodic surveillance for abdominal aortic aneurysm (AAA) and asymptomatic PAD at the Division of Cardiovascular Surgery in our hospital. The patient was a skilled, active dentist. His resting ankle–brachial index (ABI) was 0.55 and 0.52 for the right and left sides, respectively. Computed tomography (CT) indicated an AAA of 38 mm, PAD, the possibility of rectal arteriovenous malformation, and the increased wall thickness of the sigmoid colon with regional lymph node swelling (Fig. ). The IMA was well developed and measured more than 5 mm in diameter on CT angiography. Colonoscopy revealed advanced sigmoid colon cancer (Fig. ), and our initial diagnosis was cT4aN1bM0, cStage IIIB sigmoid colon cancer according to the 8th edition of the Union for Cancer Control TNM classification. The patient also had dilated cardiomyopathy. Echocardiograms revealed a left ventricular ejection fraction of 35–40% with regular administration of β-blockers, angiotensin-converting-enzyme inhibitors, and diuretics. Two major concerns were related to successful cancer treatment. First, ligation of the IMA or superior rectal artery (SRA), which is a vital procedure in CME for advanced sigmoid colon cancer, could result in insufficient blood flow and ischemia of the lower limbs. Second, the procedure could result in insufficient blood supply to the remaining rectum and increase the risk of leakage in the remaining rectosigmoid anastomosis. In the treatment of symptomatic PAD, CT angiography is a potentially less invasive and adequate technique to plan for the provision of additional blood supply during surgery. However, evaluation of blood distribution after shutting off the IMA blood flow was not sufficient, and further angiographic work-up using a balloon-occlusion catheter by interventional radiologists revealed that his lower limbs were receiving collateral blood flow from the internal iliac branches, with the flow being worse in the left limb. The flows of the internal iliac arteries were sustained by the prominent IMA blood flow through the collateral mid-rectal arteries and collateral lumbar arteries (Fig. ). In a multidisciplinary conference with interventional radiologists and cardiovascular surgeons, a left axillofemoral bypass graft was preferred to avoid ischemia of the lower limbs. Because of the low patency rate of an axillofemoral bypass graft [], a “Y-shaped” connection to the bilateral femoral artery was not proposed to preserve the right femoral artery in case of graft occlusion. Additionally, the possibility of conversion from laparoscopic surgery to laparotomy requiring a median hypogastric incision traversing the Y-shaped bypass could not be ruled out. The patient underwent surgical angioplasty of the left common to the superficial femoral artery and axillofemoral artery bypass (Fig. a). Another multidisciplinary conference was held among cardiovascular physicians, anesthetists, perioperative nurses, and gastrointestinal surgeons. Retrograde blood flow through the obturator artery to the remaining rectum can be expected after IMA ligation. However, taking the patient’s comorbidities into consideration, the cardiovascular physicians warned that his heart would not overcome the pan-peritonitis if an anastomotic leak occurred. Although indocyanine green fluorescence imaging (ICG-FI) was used intraoperatively to confirm blood supply to the remaining rectum, we proposed Hartmann’s procedure and obtained the patient’s consent. A month after the bypass, the patient underwent laparoscopic sigmoid colectomy and D3 lymph node dissection with IMA preservation, composed of preservation of the IMA to the left colic artery and ligation of the SRA (Fig. b). Anesthetists and perioperative nurses proposed oxygen saturation monitoring of both feet during laparoscopic surgery, which required extended lithotomy in the Trendelenburg position (Fig. ). The mobilization of the rectum was limited down to the peritoneal reflection so as not to damage the middle rectal artery (MRA), which provided collateral blood flow. The MRA fed the patient’s lower limbs until ligation of the SRA and provided retrograde flow to the remaining rectum after the sigmoid colectomy. Although ICG-FI revealed good perfusion of the remaining rectum, anastomosis was omitted as planned. The cardiovascular surgeon evaluated the blood flow at both feet by Doppler echo immediately after surgery in the operating room and confirmed sufficient blood flow to the lower limbs. The patient’s postoperative course was uneventful, and he was discharged 10 days postoperatively. Pathological examination of the specimen showed a pT3N0M0, pStage IIA tumor. The patient resumed work a month after the resection and was followed up for a year with no evidence of tumor recurrence. |
A 72-year-old male was admitted to the Department of General Surgery at the Second Affiliated Hospital of Dalian Medical University (Dailan, China) due to a tumor of the spleen that had been identified incidentally 1 year previously and which had grown in diameter over a 15-day period prior to the admittance. The splenic mass was detected in a routine ultrasound scan 1 year prior to admittance. At the time of identification, the diameter was 5.5 cm (), while at the time of admittance, 1 year later, the diameter was 7.7 cm. The patient remained asymptomatic. A physical examination revealed that the patient had no fever, or abdominal pain and distension. The patient’s abdomen was flat, with no tenderness. The biochemical and hematological investigations were all within the normal ranges. Magnetic resonance imaging (MRI; ) and computed tomography (CT) scans of the abdomen were performed and confirmed the presence of a mass within the spleen demonstrating diffuse heterogeneous enhancement. The tumor was suspected to be a splenic lymphoma or another type of malignant tumor. Consequently, the decision was made to proceed with surgery and the patient underwent a splenectomy.\nOn entering the abdominal cavity, the splenic tumor was visible and occupied the majority of the spleen, therefore, the patient underwent a splenectomy. The resected spleen weighed 385 g and the tumor size was 7.8×6.5×5.5 cm. When the spleen was placed into a pan, it was noted that the tumor was circumscribed, but not encapsulated, and contained a large amount of tan-white, necrotic tissue in the center ().\nOn histological examination, a large, irregularly-shaped necrotic focus was observed in the center, with a marked area of inflammatory infiltration. This was composed of an admixture of inflammatory cellular elements, predominantly plasma cells and lymphocytes with hyalinization, fibrosis, lymph follicles and multinuclear giant cells (). The final pathological diagnosis was of IPTS.\nThe patient thus far remains alive and asymptomatic at 4 months subsequent to surgery. |
A 74-year-old gentleman with coronary risk factors of diabetes mellitus and hypertension was electively admitted for PCI for CAD in UKMMC. Diagnostic coronary angiogram revealed severe CAD with stenosis at the bifurcation of the artery. Coronary angioplasty was performed using drug-eluting stent (DES) for right coronary artery (RCA), left main stem coronary artery (LMS) and left anterior descending artery (LAD). Good angiographic flow was obtained post stents dilatation. On top of the dual antiplatelet therapy (aspirin and clopidrogrel) started prior to the procedure, patient was loaded with clopidrogrel as well during the PCI. Electrocardiogram (ECG) showed sinus rhythm throughout and after the procedure. Bedside echocardiography showed good left ventricular ejection fraction without evidence of clot.\nHowever, immediately after it the patient complained of loss of vision in his right eye (RE), specifically involving the lower visual field. Visual acuity in the RE was 6/18 with an inferior visual hemifield defect. Fundus examination revealed BRAO involving the superior branches of the retinal artery with the presence of multiple emboli or Hollenhorst plaque in the branches of the superior retinal artery (Fig. ). The superior retina was swollen and pale (Fig. ). Complete neurological examination revealed no neurological deficit. No carotid bruit detected. ECG was stable without evidence of arrhythmia. Computed tomography (CT) scan of the brain was performed and no abnormalities were detected.\nDespite immediate conventional treatment for his BRAO was given, his RE inferior hemi-field loss was persistent. The condition remained stable and patient was informed for the guarded prognosis for further visual field recovery. Case was decided to continue on dual antiplatelet therapy without heparin therapy. He was monitored in CRW for another 48 h and discharged with stable vital sign and no evidence of new thromboembolic event.\nDual antiplatelet was continued and planned for lifelong aspirin with one year clopidogrel by his cardiologist for his underlying treated coronary artery disese. |
A 23-year-old Arabic gentleman from Syria had a positive family history of MS affecting two of his uncles (maternal and paternal uncles; no blood relationship existed between the parents) and idiopathic epilepsy affecting his sister. His past medical history was positive for chronic autoimmune uveitis since the age of 13. It was then treated with Methotrexate with poor response, and thus it was substituted for combination of Cyclosporine and Azathioprine which achieved a better control. A year ago, Azathioprine was suspended for a while because of the political situation and Adalimumab was initiated. Few days following the second dose of Adalimumab, the patient developed right hemibody anesthesia which lasted two weeks and was successfully treated with intravenous methylprednisolone. Adalimumab was then stopped and both of Azathioprine at a dose of 3 mg/kg and oral prednisolone were initiated. Five months later, brain magnetic resonance imaging (MRI) was requested and revealed multiple periventricular and juxtacortical lesions with high T2 signal intensity along with T2 hypersignal lesion in the spinal cord at the level of the fourth cervical vertebra C4; none of these lesions were contrast enhancing ().\nVisually evoked potentials (VEP) were also performed and were normal. Cerebrospinal fluid (CSF) analysis was normal and negative for oligoclonal bands (OCBs).\nEight months following Adalimumab infusion, the patient had right lower limb paresis which was also successfully treated with methylprednisolone, and normal strength was restored within three weeks. Six months after then, the patient was referred by a rheumatologist to a neurology clinic for consultation and followup though there has been no new attack since then.\nNeurological examination was normal except for anisocoria with slightly irregular pupils and bilaterally sluggish reaction to light (an abnormality that was explained by chronic uveitis) with normal fundoscopy and visual acuity. Mild right facial and body hemihypoesthesia, absent abdominal reflexes over the right side, generalized hyperreflexia (+3), and mild right lower limb spasticity were also noticed. To find out the relationship between the uveitis and the neurological attacks, the type of uveitis was reassessed and revealed hints of anterior and intermediate uveitis with no signs of active inflammation. An X-ray of sacroiliac joints and chest X-ray were then requested to rule out both subclinical ankylosing spondylitis and sarcoidosis and were both normal. Brain MRI with contrast was repeated and revealed no new lesions comparing with the previous one ().\nThe previous laboratory study was reviewed and showed normal ordinary labs including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Antinuclear antibodies (ANA), rheumatic factor (RF), and antineutrophil cytoplasmic antibodies C-ANCA and P-ANCA were all negative. Human leukocyte antigen (HLA) analysis revealed HLA-B27. CSF analysis showed acellular fluid with immunoglobulin G (IgG) index of 0.53, normal protein, lactate dehydrogenase (LDH), and glucose levels. Despite the lack of enhancement, diagnosis of Adalimumab induced MS was suspected in this patient with HLAB-27 associated uveitis depending on Mcdonald's criteria for space and time distribution and the temporal relationship between the first neurological attack and drug initiation besides the positive family history of MS. Since there were no active lesions on MRI, treatment plan was continued with no modification and regular clinical and radiological followup was suggested. |
A 35-year-old man was admitted to our emergency department with the chief complaint of right hemithoracic pain and respiratory distress. He reportedly slipped in his bathtub. Blood pressure, pulse rate, respiratory frequency, hemoglobin, and blood chemistry findings were all normal. On physical examination, decreased breath sounds on the right side were found. After assurance of the patient's hemodynamic stability, abdominal and thoracic imaging with CT scanning was obtained. A right-sided pneumothorax and a small liver laceration in segment VIII without any active bleeding or further intraperitoneal trauma were found. A chest tube for air removal was placed at that moment. The next day, the patient complained of abdominal pain which radiated to the right shoulder and had a fever of 39°C. A chest X-ray and a new abdominal/thoracic CT scan were obtained and the following findings were detected: rupture of the rectosigmoid, liver laceration in segment VIII, rupture of the right hemidiaphragm, and a foreign body in the thoracic cavity (Figures and ). On digital examination, the rectal ampulla was found without any blood clots, and laboratory findings were all normal. Repeated questioning led to the eventual discovery of the correct etiology of the trauma—the patient admitted that a broomstick was violently placed through his rectum that its plastic rounded end probably stayed in his body. The patient was brought to the operating room so that his injuries could be explored and operatively managed. Both thoracic surgeons and abdominal surgeons took part. Median sternotomy and exploratory laparotomy were performed. The broom had pierced the rectosigmoid junction and the transverse mesocolon (), had lacerated the segment 8 of liver, and had pierced the right diaphragm. In the right pleural cavity, an empyema and the rounded end of the broomstick were also discovered (). There were not any vascular injuries. The foreign object was removed (), and a lung decortication was done with simultaneous removal of pseudomembranes and pus. The diaphragmatic laceration was then repaired. Due to high suspicion of intraperitoneal sepsis because of peritonitis, a Hartmann's procedure with end colostomy was performed. Thoracostomy tubes were bilaterally placed and an intra-abdominal drainage as well. Postoperatively, the patient was treated with antibiotics and stayed in the intensive care unit for four uneventful days. He was hospitalized for 25 days and was discharged in good condition with a temporary colostomy. |
A 73-year-old female patient was diagnosed with spinal stenosis on L1-L5, and it was decided to perform posterior lumbar spinal fusion. The patient had height of 154 cm and weight of 72 kg. Except for hypertension, no other specific medical history was known. Prior to surgery, there were no abnormal findings in the complete blood count, and electrolyte and blood coagulation tests.\nBefore anesthetic induction, the patient's blood pressure was 150/70 mmHg, heart rate was 82 beats/min, and oxygen saturation was 98%. Anesthetic induction was conducted with injection of propofol 120 mg and rocuronium 50 mg, followed by endotracheal intubation. General anesthesia was maintained through sevoflurane (1-1.5 %)-O2(2 L/min)-N2O (2 L/min). For direct measurement of the arterial pressure, right radial artery cannulation was performed. It was planned that the patient would be managed in the intensive care unit for a few days after the surgery, and at the request of the orthopedic surgeon, a subclavian venous catheter, which is comparatively convenient for long term maintenance, was to be inserted. Due to a skin lesion in the right subclavian area, central venous catheter insertion was conducted in the left subclavian vein with a two-lumen CVC set (Arrow International, Inc., Reading, PA, USA). The catheter was fixed at a depth of 15 cm while the intravenous supply after blood regurgitation was confirmed. Afterwards, the patient was turned into the prone position on the Wilson frame (OSI, Union City, CA, USA) for the surgery (). After changing the position, the upper chest area from above the nipples and including the subclavian area showed overall compression. In order to prevent pressure by compression, a surgical cotton pad was applied to the applicable area, and free flow of fluid through the CVC was checked before commencing the operation. During the whole operating time of 4 hours and 45 minutes, the mean arterial blood pressure and the heart rate were maintained at 65-75 mmHg and 60-75 beats/min, respectively, showing stable vital signs. Total fluid input during the surgery amounted to 1,200 ml of normal saline, 500 ml of colloid, 5 units of packed red blood cell and 3 units of fresh frozen plasma, and the actual blood loss amounted to 1,500 ml and the urine output was 850 ml. After the operation, the patient was transferred to the intensive care unit. The patient was mentally alert, with a blood pressure of 130/80 mmHg and heart rate of 61 beats/min. In the blood coagulation test carried out immediately after the operation, the prothrombin time (INR) was measured at 1.13 and activated partial thromboplastin time was 42.3 seconds.\nOn the next day, there was a sudden occurrence of severe edema in the patient's left arm, and the fingertips of her left hand felt cold. The CVC was removed from the patient immediately. A blood test conducted on the day of the symptom showed that D-dimer had increased above 20 g/ml, which led to the suspicion of deep vein thrombosis. To suppress the swelling, the patient's left arm was wrapped with a pressure bandage, and ultrasonography and computed tomography scan were performed on the patient. On ultrasonography, extensive deep vein thrombosis was observed in the left subclavian vein and caudal branch () while on computed tomography, thrombosis was found in the left brachiocephalic vein and subclavian vein (). Immediately, thrombolysis was conducted in the upper left arm through a venogram (). Over the next 3 days after the thrombolysis, anticoagulation therapy was carried out by concurrently administering heparin 500 IU/h and urokinase 100,000 IU/h, and over the next 6 days, urokinase was stopped and only heparin was administered. The patient showed no more particular complications, and was discharged from the hospital 2 weeks after the treatment. |
A 43 year old lady presented in 2005 with wheeze, shortness of breath and pain in the right back. She had no other symptoms of note and was an ex-smoker. The patient had had an ovarian cystectomy in 2004 which was complicated by fistula formation requiring several laparotomies.\nAt bronchoscopy, obstruction by a large right middle lobe tumour was noted and biopsy confirmed an adenocarcinoma. Subsequent PET-CT scanning revealed extensive soft tissue abnormalities in the right paravertebral region posteriorly and lymphadenopathy in the subcarinal, contralateral and pretracheal regions. The final staging was T2N3M0 (IIIB).\nThe patient was enrolled in the AVAiL trial (AVAstin In Lung cancer – Trial No. BO17704) – a study in which the primary objective is to evaluate safety and efficacy of two doses of bevacizumab in combination with gemcitabine and cisplatin and determine the optimal dose of bevacizumab. The trial is a randomised, double-blind, multicentre, 2-stage, phase III study of bevacizumab and gemcitabine/cisplatin versus placebo and gemcitabine/cisplatin in patients with advanced or recurrent non-small cell lung carcinoma who have not received prior chemotherapy. The patient was randomised to receive gemcitabine/cisplatin and bevacizumab on the maintenance arm.\nFive cycles of treatment (carboplatin was substituted for cisplatin at cycle 3 due to toxicity) were completed in March 2006 with partial response. At cycle 6 (eighteen weeks into treatment), marked gum recession was noted (fig ). The patient was then unblinded and found to be on the continuation arm of bevacizumab. Treatment was continued with no interventions and the patient remained entirely asymptomatic.\nIn September 2006 (ten months after commencing treatment), the patient was noted to have worsening periodontal disease (fig ). She had completed 18 cycles of bevacizumab at this stage. The patient completed treatment in December 2006 and the periodontal disease has since remained stable.\nThis case is, to the best of our knowledge, the first reported of a patient developing periodontal disease whilst receiving bevacizumab. Although it would be difficult to exclude all other risk factors in this patient, the onset of periodontal disease on commencement of bevacizumab and the fact that the disease remained stable on discontinuation of the drug points to this as the cause. |
A 23-year-old African American woman in her third pregnancy presented at 26-week gestation for prenatal care. She reported a 10-year history of psoriasis, which flared with her prior pregnancy but was otherwise well controlled with topical corticosteroids. She described worsening of her psoriatic lesions with the current pregnancy, with an increase in the number of burning, pruritic, scaly plaques to which she had only been applying white petrolatum. She also described new flaccid blisters overlying her psoriatic plaques with this recent flare. She denied any joint pain or other systemic symptoms and otherwise felt well. Both she and her partner denied a history of herpes simplex virus or other sexually transmitted infections.\nOn examination, she was in no acute distress, and vital signs were stable and within normal limits. Cutaneous examination revealed erythematous scaly plaques with peripheral hyperpigmentation involving the scalp, face, arms, abdomen including the umbilicus, and pretibial legs, with scattered ~1 cm erosions within the plaques ().\nPunch biopsy of a representative skin lesion revealed psoriasiform acanthosis, epidermal necrosis, and acantholysis, as well as multinucleate keratinocytes with margination of chromatin and nuclear molding (). Immunoperoxidase staining by monoclonal antibody for HSV type I and type II was positive, in keeping with the frequent cross-reactivity encountered with these antibody preparations (). Direct immunofluorescence of perilesional skin was negative. These histologic and immunohistochemical findings were consistent with a diagnosis of herpes simplex infection within psoriatic plaques. HSV serology was positive for HSV IgM. Her HSV-1 IgG titer was negative and HSV-2 IgG was positive. HSV type 1 and 2 viral cultures obtained from the cervix were reported as negative.\nShe was started on a 14-day course of valacyclovir therapy and then switched to a daily prophylaxis regimen, with improvement of the blistering component of her skin lesions. She underwent a 1-week course of outpatient daily triamcinolone ointment wraps and narrow-band UVB phototherapy, as well as daily topical corticosteroids, with improvement of her cutaneous psoriasis.\nAt a follow-up prenatal visit, the patient complained of generalized itching and was switched to acyclovir prophylaxis. At 35-week and 4-day gestation, the patient self-discontinued antiviral therapy due to continued generalized itching, resulting in recurrence of the intralesional herpetiform vesicles. The Dermatology Service was again consulted for treatment recommendations of erythroderma secondary to psoriasis flare with superimposed HSV infection. The patient was treated with daily inpatient triamcinolone wet wraps until she was no longer erythrodermic. Oral antihistamine therapy was initiated to improve acyclovir tolerability related to her reported pruritus.\nAt 37-week and 1-day gestation, the patient presented to labor and delivery with premature rupture of membranes. Upon admission, no cutaneous or mucosal HSV lesions were noted. Labor was augmented with oxytocin. The patient delivered a healthy female infant without any observable HSV lesions. As such, neonatal HSV cultures were not obtained and prophylactic antiviral therapy was not initiated. At the infant's one-week pediatric visit, the baby was noted to be doing well. |
A 33-year-old Indian female presented with loss of vision in the right eye for one month with associated headache and ocular pain, which increased with eye movement. The best corrected visual acuity (BCVA) was hand motions close to the face in the right eye, and 20/20 in the left eye. Extraocular movements were full but painful. Relative afferent pupillary defect was noted in the right eye, and remaining anterior segment examination was within normal limits. Examination of the right fundus revealed a hyperemic and swollen optic disc with blurring of disc margins []. The macula and the retinal periphery were unremarkable. Examination of the left eye showed no abnormality. Systemic evaluation results, including the results of detailed neurological examinations, were within normal limits.\nB-scan ultrasonography of the right orbit showed elevation at the optic nerve head, the definitive nature of which could not be discerned. Contrast enhanced magnetic resonance imaging (MRI) of the brain and orbits demonstrated a swollen and tortuous right optic nerve with a well-defined T2 hyperintense cystic lesion with peripheral hypointensity in the optic nerve substance at the orbital apex [Figures and ]. Post gadolinium contrast sequences revealed diffuse optic nerve enhancement with peripheral rim enhancement of the lesion. The brain and left orbit appeared to be normal. Based on the MRI findings, and the high prevalence and endemicity of cysticercosis in the region, a diagnosis of optic nerve cysticercosis was made. Pattern visual evoked potential (VEP) showed prolonged implicit time and decreased amplitude of the P100 wave. Hemogram revealed an elevated erythrocyte sedimentation rate, but no eosinophilia. An enzyme-linked immunosorbent assay (ELISA) test for cysticercosis was positive, which further confirmed the diagnosis.\nThe patient was started on a course of oral albendazole (15 mg/kg body weight in two divided doses) in combination with prednisolone (1 mg/kg body weight) for 4 weeks. BCVA showed a dramatic improvement to 20/30 in the right eye, which was associated with resolution of the disc edema []. Oral steroids were tapered over the following month. An MRI scan taken 6 weeks after initiation of Albendazole revealed a complete resolution of the cyst []. The clinical picture remained stable at 6-month follow up. |
A 30-year-old nulligravida was referred to our hospital because of an uterine anomaly in pregnancy. She presented at 5+4 weeks of pregnancy without abdominal pain or vaginal spotting.\nThe patient attained menarche at 14 years of age, and had regular periods and a history of severe dysmenorrhea. She had been given a diagnosis of left renal agenesis at birth. However, she had not visited any hospital for evaluation of her dysmenorrhea with unilateral renal agenesis. There was no other past history. A systemic examination was normal. The abdomen was soft and nontender on examination. Speculum examination revealed a blind bulging pouch at the left side of the vagina. A small cervix in the right was seen lateral to the bulging pouch.\nTwo-dimensional (2D) ultrasonography (US) revealed a complete septate double uterus with diverging cornua. An 8-mm sized gestational sac was seen in the right horn. A dilated hypoechogenic left vagina was evident (). The right ovary was visualized normally, but the left ovary was not visualized. Three-dimensional (3D) US revealed the complete septate uterus with a tiny gestational sac in the right uterus and decidual reaction in the left uterus. The markedly dilated left vagina bulged into the right vagina (). The left maternal kidney was absent and the right kidney was normal. Our diagnosis was a complete septate uterus with obstructing hemivagina and ipsilateral adnexal and renal agenesis. Intermittent vaginal spotting had been present since about 14 weeks of gestation. However, the patient and her fetus were otherwise normal.\nShe visited the St. Vincent's Hospital for spontaneous rupture of membrane at 38+2 weeks of gestation. She gave birth by cesarean section because the left bulging vaginal pouch made a vaginal birth difficult. During the cesarean section, we confirmed that the uterine anomaly was a complete septation that continued to the vagina. There was thickening and obliteration of the cul-de sac and adhesion between the posterior wall of the uterus and descending colon. The right ovary and tube were normal, but the left ovary and tube were absent. The septum of the uterus was resected and the left cervix was able to be palpated and visualized. Through a vaginal approach, the vaginal septum was incised and a window of about 3 cm×3 cm in size was made by suturing the incision site. About 200 mL of chocolate colored sticky fluid was drained.\nThe baby's weight was 2.98 kg and the 1- and 5-minute Apgar score was 8 and 9, respectively. The patient was discharged after 4 days without complications. She was followed-up for 1.5 years, with no complaints of dysmenorrhea and abnormal vaginal bleeding after the first postpartum menstruation. The vaginal septum window has remained intact with no fluid collection in the left vagina. |
A 56-year-old female with a history of Grave's disease 20 years ago presented to Banbuntane Hospital, Nagoya, on January 26, 2016, with incidental findings of multiple intracranial aneurysms. She was diagnosed to have basilar tip, internal carotid-anterior choroidal, and middle cerebral artery aneurysms since 5 years prior to the referral. She was asymptomatic of Grave's disease since she has been on regular medications and follow-up by the endocrinologist. Initial MRA done on September 25, 2012, and DSA done on November 14, 2012, revealed basilar tip saccular aneurysm measuring 4.2 mm × 6.2 mm. She was not keen for any treatment at that moment. Follow-up CTA done on January 26, 2016, revealed enlarging superior pointing aneurysm measuring 5.8 mm × 6.2 mm []. A CFD was performed showed high wall pressure and low WSS pressure within the aneurysmal sac []. She was admitted on February 12, 2017. She underwent left anterior temporal craniotomy and clipping of basilar tip and left MCA aneurysms []. The surgery was uneventful with duration of 6 h. MEP monitoring and ICG (DIVA) and neuroendoscope were used during the surgery []. However, she had prolonged hospitalization due to respiratory dysfunction caused by her underlying disease. She was discharged 34 days later. She was asymptomatic for any neurological issue during 3-month follow-up on November 5, 2017. Postoperative MRA on June 16, 2017, and CTA on January 15, 2018, did not reveal any residual basilar tip aneurysm []. CFD study revealed improvement in the streamline and WSS vectors at the bifurcation with no area and high wall pressure despite the WSS pressure remained low at the bifurcation []. There was a reduction in the ratio between velocity in basilar artery and the average velocity within both P1 []. She was readmitted recently on May 3, 2018, for elective clipping of the MCA aneurysm. The surgery was uneventful and she was discharged well. |
A 43-year-old female patient with localized intrahepatic bile duct dilatation was found by physical examination for 20 years. For recent 3 years, the patient felt repeated right upper abdominal pain and distension, and aggravated for half a year, without fever and jaundice. She was admitted to our hospital for further examination and treatment. The patient's physical examination and hematological tests, including tumor markers Ca-199 were unremarkable. Enhanced CT and magnetic resonance cholangiopancreatography (MRCP) suggested that the intrahepatic bile duct dilatation in segment 4(S4) with an extent of 2.5*3.0 cm (Fig. a, b). Hepatobiliary ultrasound revealed localized dilatation of bile duct with local acoustic enhancement in S4 of the liver (Fig. c). On the basis of these findings, a diagnosis of S4 localized bile duct dilatation with lithiasis was made. Given the recurrent and persistent symptoms, the patient opted for surgical resection of biliary dilatation. Correspondingly, we selected to perform a combined resection of segment 4b and part of 4a with the guidance of subsegmental ICG staining, so as to facilitate the laparoscopic operation more simply and accurately, as well as to retain normal tissues in a certain extent.\nIntraoperatively, after mobilization of the liver, relationships of the pathological segment with the hepatic vessels were confirmed by LUS, which was consistent with the preoperative CT imaging (Fig. d). ICG fluorescence positive staining method was performed to define the exact anatomical location of the lesion. Portal vein branch of the target hepatic segment was super-selected and visualized longitudinally, followed by punctured with a 18G needle by LUS guidance, then an injection of 0.05 mg ICG was performed gently (Fig. a, Additional file ). The specific configuration method of dilution was as follows: 25 mg ICG was dissolved in 10 ml sterilized water, and then taken 1 ml of which into 100 ml saline. Significantly, special attention must be paid to avoid the dye entering to other hepatic lobes. After injection of ICG, fluorescence imaging could highlight the segmental resection line, the non-fluorescent hepatic tissues were also identified (Fig. b). Fusion fluorescence images were obtained using the PINPOINT imaging system (Stryker, Kalamazoo, USA). The imaging was switched to the fluorescent mode when predicting that the surgical boundary of the lesion would be exposed, then enabled visualization of the lesion as a clearly distinguished region of green fluorescence during operation (Fig. c). Since the lesion was located between S4a and S4b, indicating the need for partial resection of the segment 4 rather than removing the whole S4. There were no complications during the procedure, with an operation time of 90 min and a bleeding volume of 100 ml, and the size of the resected hepatic segment was 9*4 cm (Fig. a). Final pathology revealed presence of chronic and fibrosing inflammation with dilatation and cholelithiasis in local bile duct (Fig. b), and post-operative enhanced CT confirmed the integrity of the resection (Fig. c). The patient was recovered soon, then discharged 6 days after operation, and she has not experienced similar symptoms as before during the first three month of follow-up. |
A 47-year-old male presented to us with features suggestive of obstructive jaundice for three months duration. He did not have cholangitis. Abdominal examination revealed hepatomegaly and a palpable gallbladder. The esophagogastroduodenoscopy revealed an ulcerated lesion in the ampullary region. Biopsy from the lesion had features of adenocarcinoma. His CECT scan showed a lesion in the ampullary region with dilatation of the common bile duct and pancreatic duct. There were no distant metastases. The portal and superior mesenteric veins and the superior mesenteric artery were free from the lesion. There was no abnormal arterial anatomy.\nClassical pancreatoduodenectomy (Whipple resection) was planned. During the uncinate dissection, we found that the uncinate process was extending posterior to the portal vein and was communicating with the body of pancreas to the left side of the portal vein (Figure , ). We dissected the aberrant pancreatic tissue from the posterior surface of the portal vein, and then transected the pancreatic tissue anterior and posterior to the portal vein. After transection, we had two pancreatic stumps. The pancreatic duct was identified in the stump anterior to the portal vein. No duct was present in the posterior pancreatic stump. We performed "duct to mucosa" pancreatico-jejunostomy in the pancreatic stump anterior to the portal vein and closed the posterior pancreatic stump with interrupted polypropylene sutures. On reviewing the preoperative CECT scan, we were able to identify the pancreatic tissue encasing the portal vein superior to the splenic vein. The main pancreatic duct (MPD) was anterior to the portal vein. No duct was identified in the aberrant pancreatic parenchyma posterior to the portal vein (Figure , ). He had secondary haemorrhage on postoperative day 7. On exploration, we found that there was bleeding from a tributary of the middle colic vein, which was secured. Subsequently, he had an uneventful recovery and he was started on adjuvant chemotherapy. |
A 69-year-old Japanese man presented with pain and a polypoid mass on the lower left gingiva for which he consulted in our hospital. He had received chemoradiotherapy for SCC of the buccal mucosa 15 years prior to this consultation at the age of 54 years. At that time, he had an ulcerative lesion with a reddish and rough surface in the buccal mucosa including the corner of the mouth (Fig. A). The patient was a plasterer, and asbestos exposure was not apparent. He had no relevant medical history, including no history of smoking and alcohol consumption. The lesion was diagnosed as moderately differentiated SCC following a biopsy (Fig. B, C). Radiological examinations suggested a cancer metastasis in the left submandibular lymph node, and he received chemotherapy with cisplatin (total 110 mg) and 5-fluorouracil (total 2925 mg) via peripheral venous route and concomitant radiotherapy: the primary tumor and neck were irradiated at the dose of 70 Gy, performed in the hospital for 4 months. The treatment resulted in a complete clinical remission of the primary tumor (Fig. D) and a complete remission of the neck lesion, which was pathologically confirmed as metastasis composed of nonviable SCC cells (data not shown). No recurrence was detected in subsequent years; however, he developed mandibular osteonecrosis 6 years after chemoradiotherapy and was treated with oral roxithromycin.\nOn examination at the age of 69 years, his vital signs were within normal ranges. He developed Parkinson’s disease a year before this consultation, and the disease was well controlled with oral levodopa (300 mg/day) and benserazide hydrochloride (85.5 mg/day). He showed trismus; however, he had no other abnormal physical and neurological findings. On intraoral inspection, a polypoid mass measuring 10 mm in diameter was found in the left molar region of the lower gingiva (Fig. A). Its surface was rough and covered by a whitish pseudomembrane. Panoramic radiography showed bone resorption, exhibiting a moth-eaten appearance that involved the base of the mandible (Fig. B). Contrast-enhanced computed tomography revealed a soft-tissue mass lesion accompanied by extensive bone resorption of the mandible (Fig. C, D).\nA malignant tumor was suspected, and a biopsy specimen from the polypoid lesion was obtained. The polypoid mass was composed mainly of loose granulation tissue (Fig. A), where atypical spindle or pleomorphic cells were scattered in the fibrin-rich edematous or pale stroma together with neutrophils, lymphocytes, and capillary endothelial cells (Fig. B). The surface of the lesion was mostly ulcerated but partially covered by squamous epithelium with no obvious atypia; only slight nuclear enlargement and disordered basal cell polarity were noted (Fig. C). In the subepithelial tissue, atypical spindle cells were arranged haphazardly and exhibited large, basophilic cytoplasm and bizarre nuclei (Fig. B, C). Some of them exhibited mitotic figures, some of which were atypical (Fig. D, E). Neutrophils were frequently incorporated within their cytoplasm (Fig. E–G), some of which appeared to be degraded with missing nuclei (Fig. E, G, arrows).\nImmunohistochemistry revealed that atypical spindle cells were positive for vimentin (Fig. A) and α-smooth muscle actin (Fig. B), but not for pan-cytokeratin (Fig. C). No epithelial markers other than p63 were detected (Table ). The spindle cells were partially positive for p63 (Fig. D), and most were positive for p53 (Fig. E). Ki-67 staining was observed in approximately half of the cells (data not shown). Furthermore, granular CD68 staining was observed in the cytoplasm (Fig. F). These findings were highly suggestive of a malignancy, and SCSCC and post-irradiation sarcoma were considered in the differential diagnosis, given that the patient had a history of chemoradiotherapy for treating SCC of the buccal mucosa 15 years before.\nAlthough the final diagnosis had not been determined, the patient was admitted to our hospital for surgery. On admission, his height and body weight were 145 cm and 45.7 kg, respectively, and vital signs were within normal range (blood pressure 127/79 mmHg, pulse 68 beats/minute, and body temperature 36.0 °C). Complete blood count showed mild anemia (red blood cells 4.07 × 1012/L, hemoglobin 128 g/L, hematocrit 36.0%, mean corpuscular volume 90.4 fL, mean corpuscular hemoglobin 31.4 pg, mean corpuscular hemoglobin concentration 348 g/L, white blood cells 5.49 × 109/L, and platelets 130 × 109/L). The biochemical test results of blood and urine were within normal ranges with the exception of a mild elevation in C-reactive protein (8.7 mg/L), which was presumably caused by the gingival tumor. He underwent hemimandibulectomy pursuant to a clinical diagnosis of a malignant tumor. The postoperative course was uneventful.\nThe hemimandibulectomy specimen had a protruding tumor that exhibited an ulcerated surface and that measured 23 × 15 mm from the gingiva to buccal mucosa (Fig. A). The location of this tumor was not the same site of the previous tumor. On the cut surface, there was a whitish and solid lesion invading the mandibular bone (Fig. B, left). Histologically, most of the lesion was composed of conventional SCC, measured 45 × 25 × 15 mm in size, and invaded the mandibular bone extensively (Fig. B, right and Fig. C). The spindle cells were distributed near the SCC foci, mostly in the remaining polypoid segment (Fig. D). Because the atypical spindle cells were concomitant with the conventional SCC component, we finally diagnosed the lesion as SCSCC. The tumorous spindle cells frequently contained neutrophils within the cytoplasmic vacuoles (Fig. E, F), forming cell-in-cell figures. Such figures were much rarer in the conventional SCC cells. Intense staining for lysosomal-associated membrane protein 1 (LAMP-1) and cathepsin B, which are both lysosomal markers, were observed around the vacuoles containing the neutrophils (Fig. G, H). This suggested that such cell-in-cell figures may result from the phagocytosis of neutrophils rather than from emperipolesis. The patient has had no evidence of recurrence 5 years after the surgery. |
While driving a van and wearing a seatbelt, a 19-year-old Caucasian woman, was involved in a head-on vehicle collision (speed about 40 km/hour), followed by a rear-end hit from another vehicle. When rescue services arrived at the scene, the patient was found sitting in her car with her head immobilized in a left rotation. She was transferred onto a spinal board. The application of a stiff neck collar was not possible as her head was fixed in the rotated position. After admittance to a regional hospital, the physician in charge tried to reposition her head but she reported painful paresthesia in the left arm. She was transferred to our spine and trauma center. Upon admittance, the woman complained about strong, immobilizing pain in the upper cervical spine with torticollis to the left side. A computed tomography (CT) scan revealed an atlantoaxial rotation of 46° to the left without any signs of osseous lesions (Figure ). The neck was then reduced by cautious rotation under traction with the cervical spine in flexion thus avoiding harm by potential posttraumatic disc lesions. During this process, the patient was awake and did not report any new paresthetic sensations during the procedure. There were no clinical signs of neurological sequelae before or after reduction. However, a fluoroscopic control still showed signs of atlantoaxial pathology (Figure ) and magnetic resonance imaging (MRI) of the cervical spine was done (Figure ). It showed the integrity of the transverse and the alar ligaments and a traumatic discus protrusion on level C5/6 (Figure ). After three days of immobilization and analgesic therapy, a CT (with maximum bilateral head rotation) showed no persisting atlantoaxial fixation (Figure ). Subsequently, she was discharged three days after admittance and immobilized in a soft collar for six weeks. At a follow-up examination six weeks after the trauma, the pain and paresthesia in the left arm had receded completely and the patient had a full range of motion. A follow-up MRI of the cervical spine showed only slight persistent atlantoaxial rotational displacement of C1/2. |
A 28-year-old gravida 2, para 1 was transferred to our institution 19 days post operation with symptoms of excessive bleeding per vaginum. She had undergone an elective c-section for suspected macrosomia. She was apparently asymptomatic for 15 days post operation. She later developed excessive bleeding per vaginum, high grade fever, and was readmitted. On abdominal examination, the c-section scar was found to be healthy and no abnormality was detected. She was bleeding per vaginum. The uterus was bulky and the cervical os was closed. Her hemoglobin level was 3.8 g/dl. She was stabilized with crystalloids, five units packed red blood cells, and started on broad spectrum antibiotics.\nTransvaginal ultrasonography with color Doppler confirmed a postpartum uterus. There was no evidence of residual placental tissue in the uterine cavity and endometrial thickness was 7 mm. A hypoechoic lesion measuring 2 cm × 1.5 cm [] was detected in the isthmic region of uterus and power Doppler revealed blood flow within it []. Color flow Doppler sonography showed yin and yang blood flow pattern within the body of pseudoaneurysm.\nComputed tomography angiogram was done with non-ionic contrast showed early contrast filling of the lesion within the uterus [Figure , ]. Maximum intensity projection (MIP) and Volume rendered (VR) images nicely demonstrated the pseudoaneurysm in relation to the uterine artery [Figure , ].\nTo preserve the fertility in this young patient, a transcatheter arterial embolization of this pseudoaneurysm was planned. She underwent digital subtraction angiography. Arteriography revealed a pseudoaneurysm from the terminal part of the left uterine artery, in addition the left uterine artery was tortuous and was hypertrophied [Figure , ]. Left uterine artery was selectively embolized with mixture of gelfoam and contrast media followed by 2 stainless steel coils 4 mm in diameter []. The right uterine artery was also tortuous and hypertrophied and was embolized with gel foam. A post embolization angiographic study was performed to ensure the complete occlusion of the vessels []. Follow-up color Doppler US showed aneurysmal cavity filled with echogenic content with no evidence of blood flow [], except for pain in the abdomen that was managed with analgesics. |
A 73-year-old man with a history of chronic obstructive pulmonary disease (COPD) and hypertension, who had a pacemaker due to 2nd degree AV block, was admitted due to sudden-onset severe dizziness, diplopia, and nausea after an intense episode of coughing. He had difficulty in walking and could not stand without support. There was no history of neck manipulation, trauma, or pain. The patient had a history of difficulty in swallowing solid food for the last year and stated that he had to cough often to make the airway free.\nOn arrival, the patient was conscious with a Glasgow coma score of 15. He was examined in sitting position due to dizziness. The patient was slightly aphonic. Cranial nerve examinations revealed slight anisocoria right > left, although it was uncertain if he had had this symptom before. There was both vertical and horizontal nystagmus. He had diplopia while looking straight and upwards, and there was improvement in dizziness while lying down with eyes closed. There was paresthesia in the left arm with left-sided positive finger-nose test, positive left-sided dysdiadochokinesis, and deviation of the left arm to the left in the stretched arm test. He had difficulty in standing up from a sitting position. Gait function could not be examined due to dizziness. Computed tomography (CT) of the cerebrum (Fig. ) showed a calcified process measuring 2.6 × 1.5 cm, ventral to the medulla oblongata, in the junction between the VA and the basilar artery; presumably a large aneurysm. This process seemed to compress the medulla oblongata. Later, the patient was transferred to our hospital for neurosurgical intervention. After arrival, CTA of the cerebrum was performed, which showed a gigantic VA aneurysm on the left side in the V4 segment with complete thrombosis and partial calcification (Fig. ). Since the aneurysm was found to be completely thrombosed, no further indications for coils or clips were found.\nThe patient was then transferred to the neurological department for further observation and rehabilitation for his gait dysfunction and diplopia, since no neurosurgical intervention was required and performed. There was improvement in diplopia and dizziness over the following days. The patient continued to have intermittent nausea with constant cough and signs of dysphagia. As mentioned above, the patient was slightly aphonic during admission, and there was gradual weakness in his voice. Chest X-ray examinations were normal, and there was normal auscultation of the lungs. Later, FEES was performed, which showed normal rhino-, oro-, and hypopharynx but complete paralysis of the left-sided recurrent nerve. Few spontaneous swallowing was observed with normal sensation in the epiglottis and hypopharynx. The patient had been admitted 2 months ago due to an exacerbation of COPD, where a chest CT showed no sign of malignancy. So, recurrent nerve palsy due to the aneurysm was confirmed. The patient was later discharged with follow-up after 3 months in the Phoniatric Department and was also referred to the laryngologist. There was slight improvement in motor functions with moderate discoordination of the left arm with imbalance, and the patient is in rehabilitation now. There was also improvement in his gait and he could walk without support. |
An 18-year-old female (45 kg, 152 cm), a known case of Stage IV metastatic sigmoid colon cancer, post 4 cycles of chemotherapy (CAPOX regimen), was admitted in the palliative care unit with complaints of pain abdomen for 10 days, multiple episodes of vomiting, inability to pass stools, and flatus for 3 days. There was no history of fever, cough, and difficulty in breathing at the time of presentation. She resided in the containment zone of the national capital which was the hotspot for coronavirus. X-ray erect abdomen was done which revealed multiple air–fluid levels []. Initially patient was managed conservatively and kept nil per oral. Nasogastric tube was inserted. Injection paractemaol alongwith injection fentanyl infusion was given for managing her pain. After 24 h of conservative management, there was no improvement in signs and symptoms; it was decided to go for contrast-enhanced computed tomography (CECT) abdomen, which revealed single-level obstruction at the level of sigmoid colon with the proximal bowel dilated and the distal segment collapsed []. Keeping in mind the ESMO and ACS guidelines for cancer care prioritization during the COVID pandemic, it was decided to take the case for surgical intervention.[] However, at the same time, the patient developed fever, so, along with infection control department, it was decided to test for severe acute respiratory syndrome (SARS)-coronavirus before taking up for surgery as the patient came from a COVID-19 hotspot area and fell into the category of immunocompromised patient as well as developed fever despite giving round-the-clock paracetamol. The patient and the family members were explained about the pros and cons of the surgery, and written informed consent was taken. Once the reports of SARS coronavirus came out to be negative, then, the surgical oncologists proceeded with palliative diversion transverse loop colostomy with both the anesthesiologists and the surgeons using full precautions and personal protective equipment. Post procedure, the patient was shifted to the ward, slowly injection fentanyl was tapered, and she got discharged on tablet paracetamol only. One week post surgery, she was called up for follow-up in our outpatient department and reported complete pain relief with stoma functioning well. |
A 42-year-old female patient reported with a chief complaint of pain in the right mandibular back tooth region for 3 days. Medical history of the patient was noncontributory. On clinical examination, the right mandibular first molar presented with mild attrition. Cervical abfraction was noticed for teeth 44, 45, and 46 []. There was a deep disto-occlusal carious lesion in relation to tooth 17, and teeth 36 and 21 were missing. Dental history revealed that the patient visited a dentist 8 years ago with a complaint of severe pain in relation to tooth 36 for which the tooth was extracted. The patient does not remember the cause for loss of tooth 21 as the tooth was missing for many years for which she was wearing a removable partial denture. There was no history of any orthodontic treatment. Intraoral periapical radiograph of the mandibular right posterior region revealed extensive external resorption of the mesial root of tooth 46 []. Upon cold test (RC Ice; Prime Dental Products Pvt. Ltd., Mumbai, India) and electric pulp testing (EPT) (Parkell Electronics Division, Farmingdale, NY, USA), tooth 46 gave an exaggerated painful response. EPT reading for tooth 46 was 6 (early response); for tooth 47, it was 25, and as the contralateral tooth was missing, tooth 37 was tested which responded at 28. A probing depth of 2–4 mm was recorded, and the clinical attachment of the gingiva was considered to be normal with no mobility. There was no swelling, discharge, or sinus opening in relation to tooth 46. The periodontal sulcus was probed using a standard periodontal probe. Hematological investigations including complete blood count as well as calcium, phosphorus, and alkaline phosphatase were within the normal range, so endocrine diseases such as hyperparathyroidism, hypoparathyroidism, hypophosphatemia, hyperphosphatemia, and Paget's disease were ruled out. The patient was informed about the questionable prognosis of tooth 46 due to resorption of mesial root, and written informed consent was taken. Provisional diagnosis of irreversible pulpitis was made, and to relieve the patient from pain, emergency endodontic treatment was planned.\nAfter administration of local anesthesia (2% lignocaine with 1:80,000 adrenaline), access opening was done under rubber dam isolation. Three root canal orifices were located (mesiobuccal [MB], mesiolingual [ML], and distal [D]) []. Working length was determined using radiographs (Ingle's method) and confirmed with an apex locator (Root ZXII, Morita, Tokyo, Japan). MB and ML canals were measuring only 4 mm. Cleaning and shaping was done for distal root with ISO 2% taper files up to size 40 (MANI Inc., Tochigi-Ken, Japan). MB and ML canals were enlarged till #3 GG drills (MANI Inc., Tochigi-Ken, Japan). Irrigation was done using 3% sodium hypochlorite solution (Prime Dental Products Pvt. Ltd., Maharashtra, India) and 17% EDTA (Prime Dental Product Pvt. Ltd., Mumbai, India). Final rinsing of the canal was performed using 2% chlorhexidine digluconate (Neelkanth Healthcare Pvt. Ltd., Rajasthan, India). Then, the canals were dried with paper points (Prime Dental Products Pvt. Ltd., Maharashtra, India), and a calcium hydroxide dressing was placed (Ultracal; Optident, Skipton, UK). To rule out the possibility of resorption involving other teeth, an orthopantomogram was taken which confirmed localized idiopathic apical resorption (LIAR) involving tooth 46 []. After 1 week, temporary restoration was removed and canals were cleaned and dried. The distal canal was obturated by lateral compaction of gutta-percha (Maillefer, Dentsply) and AH Plus resin sealer (Maillefer, Dentsply). The mineral trioxide aggregate (MTA) cement (ProRoot MTA; Dentsply Tulsa Dental, Tulsa, OK) was mixed according to the manufacturer's instructions and placed into the MB and ML canals using amalgam carrier and condensed vertically with hand pluggers (Maillefer, Dentsply) []. The tooth was then restored with a posterior composite resin core (Filtek Z250 3M ESPE, A2 shade/USA) followed by a full-coverage porcelain crown in the subsequent visit. Five-year follow-up revealed satisfactory result as the tooth was completely asymptomatic clinically with no mobility, and radiographically, there was no evidence of further furcal breakdown and resorption []. |
A 45-year-old male patient presented with the chief complaint of the palpable mass in the right cheek area which was found 3–4 years before he came to the hospital. The mass was found to be solid on palpation, and the patient did not complain of other symptoms such as tenderness and did not have any trauma history (). On facial magnetic resonance imaging, a relatively well-circumscribed mass sized 1.7×2.1×2.3 cm was observed in the right cheek area. The image showed peripheral enhancement of the mass, and the inside included a necrotic portion showing low signal intensity (SI) on T1 weighted image and high SI on T2 weighted image ().\nIn addition, involvement of adjacent orbicularis oculi muscles was observed, and there were restrictions on the evaluation due to the air of the right maxillary sinus, but the findings suggestive of involvement of adjacent maxilla were not definite. Based on physical examination and imaging findings, we planned to perform resection through a subciliary approach, with a higher possibility of a true mass than vascular malformation in mind.\nUnder general anesthesia, the orbital floor and zygoma were exposed through the subciliary incision, the tumor was carefully dissected, and the mass was completely excised paying heed to the margins along the boundary with surround tissues. No evidence of the invasion of the tumor tissue into neighboring tissues was observed. We were careful to spare nerve from excision and used small blunt scissors and bipolar electrocautery. We opened epineurium and separated mass from the nerve fascicles.\nIntraoperative frozen section biopsy results were suggestive of schwannoma. On final histologic examination, the tissue specimen was positive for s-100 protein immunohistochemical staining, confirming the diagnosis of schwannoma (). The patient has been followed up for 2 years after surgery and no other symptoms such as sensory abnormality are reported without any findings of recurrence (). |
A 78-year-old man was admitted to the pulmonary hypertension service at Stanford Hospital in December, 2010 with elevated pulmonary arterial pressures and progressive right-sided heart failure. He reported progressive shortness of breath over a period of two months, culminating in dyspnea at rest. This was associated with a 20-pound weight gain, abdominal bloating, and leg edema.\nHe was first evaluated for dyspnea several years prior to admission, where he was diagnosed with bilateral pleural effusions and extensive pericardial calcification on chest computed tomography (CT). Echocardiography and cardiac catheterization were consistent with constrictive pericarditis. He subsequently underwent uneventful complete pericardiectomy in 2008. The pericardium was stripped from phrenic nerve to phrenic nerve and the diaphragmatic and posterior pericardium was removed as well.\nThe patient enjoyed symptomatic improvement for approximately one year but then developed recurrent dyspnea. An echocardiogram demonstrated right ventricular (RV) pressure overload and a right ventricular systolic pressure of 66 mmHg. A trial of sildenafil was initiated but was discontinued after the patient developed a marked worsening of his dyspnea.\nHis medical history was remarkable for pericarditis diagnosed in 1978, which was presumed to be viral in origin. He had permanent atrial fibrillation/atrial flutter which did not respond to an attempted atrial flutter ablation in 2003. He had a history of chronic hypertension and gradually progressing stage 3-4 chronic renal impairment.\nOn admission, his jugular veins were distended to angle of his jaw when upright. Pitting edema was noted up to the mid thighs bilaterally. Crepitations were heard on auscultation of his lungs. N-terminal pro-brain natriuretic peptide was measured to be greater than 30,000 pg/mL. Chest x-ray showed blunting of the costophrenic sulci with coarse reticular linear basilar parenchymal opacities ().\nPrior to admission, ventilation-perfusion scan was low probability for pulmonary embolism. Neither pulmonary function tests nor screening blood work revealed any other potential cause for his pulmonary hypertension.\nEchocardiogram on admission was technically difficult. Moderate RV enlargement and dysfunction were identified. Right ventricular systolic pressure was severely elevated at 82 mmHg. No findings of pericardial constriction were reported.\nCardiac catheterization was performed two days post admission after the patient had received intravenous diuresis. Pulmonary artery pressures were elevated at 70/13 mmHg with a mean of 35 mmHg. The pulmonary capillary wedge pressure was 13 mmHg. Cardiac output as calculated by the Fick equation was preserved at 5.3 L/min and pulmonary vascular resistance was calculated to be 4.3 WU. The pulmonary pressures did not respond to vasodilator challenge with inhaled nitric oxide at a dose of 20 ppm. The right atrial pressure tracing () showed abrupt x and y descents, and no fall in right atrial pressure with inspiration (positive Kussmaul's sign). Simultaneous pressure measurements of the RV and left ventricle (LV) revealed an early diastolic “dip and plateau” and equalization of end diastolic pressures (). However, no evidence of discordant change in RV and LV pressures was seen with respiration. Thus, the catheterization was supportive but not diagnostic for pericardial constriction. Volume challenge was not performed. Cardiac biopsies were performed at the time of the catheterization and showed only nonspecific changes.\nCardiac magnetic resonance imaging (MRI) was performed on a 1.5 T GE scanner, with no contrast given secondary to the patient's renal insufficiency. The main pulmonary artery, inferior vena cava and right atrium were found to be dilated. Residual thickened pericardium was identified in the basolateral and basal posterolateral segments (), with adhesive segments, most prominent in the basal anterior segment of the RV and the basal posterolateral segment of the LV. Septal flattening occurred on inspiration () and resolved completely on expiration (), revealing heightened ventricular interdependence. These findings confirmed the diagnosis of recurrent pericardial constriction.\nGiven his significant comorbidity and the high-risk nature of repeat pericardiectomy, the patient was managed medically with diuresis. He responded well to furosemide and was clinically euvolemic with improvement in his dyspnea on discharge. |
In 2012, a 24-year-old systemically healthy female patient presented to Department of Periodontics, SRM Dental College, Chennai, India with the chief complaint of pain for past one day and swelling in relation to gums of the inner aspect of upper front teeth region, associated with impaired pronunciation of words for past the 2 weeks.\nDetailed history of the patient revealed that she was undergoing orthodontic treatment for past one year. Patient noticed swelling 2 weeks back, which slowly started increasing in size in her anterior palatal region associated with disturbance in her speech. Presence of pain for past one day was elicited.\nOn clinical examination, palatal gingiva was edematous, presence of small pustule with fluctuant gingival enlargement extending from right maxillary canine to left maxillary canine on the palatal aspect involving interdental papillae, marginal gingiva, covering up to two-third of palatal surface, with 7 mm of pseudo pocket, along with the presence of bleeding on probing [Figures and ]. Subjective evaluation was done with visual analogue scale (VAS) score to analyze the degree of speech disturbance (VAS score: Good pronunciation – 0, poor pronunciation – 10) VAS score before treatment was 7.\nBased on history and clinical examination, patient was provisionally diagnosed as “gingival abscess with inflammatory enlargement in relation to right maxillary canine to left maxillary canine on the palatal region.”\nPatient was treated nonsurgically by scaling and root surface debridement to facilitate abscess drainage and to reduce inflammation. Antibiotic coverage for 5 days was prescribed. On recall of patient after 7 days anterior palatal gingiva appeared fibrotic with resolved inflammation.\nGingivectomy was done under 2% lignocaine with 1:80,000 adrenaline with no. 15 blade. External bevel incisions were placed from 12 to 22, and a large wedge of epithelium with connective tissue was excised [] and periodontal dressing was given and analgesics and chlorhexidine mouth was prescribed.\nOne month following the surgery, wound healing was uneventful, improved gingival contour was achieved []. VAS score was 2 after treatment, there was an improvement when compared with the baseline value.\nStrict oral hygiene instructions were given to patient for plaque control on brushing habits and usage of mouthwash.\nPatients’ closest associates also revealed that her enunciation of sounds such as “t”, “th”, “d”, “l”, “n”, “nth” were improved following gingivectomy procedure. |
Our patient was a 23-year-old unmarried, unemployed woman graduate from a middle socioeconomic status and urban Asian background. She had a six-year history of patchy hair loss from her scalp. She had earlier consulted a dermatologist who referred her to a psychiatrist.\nShe walked into our consultation room with her head covered with a scarf. She had a short haircut and there were many bald patches, 5 cm to 10 cm in area, on her scalp. The hair, particularly in the parietal region of both sides of the head, was very thin, brittle and uneven.\nOur patient had an uncontrollable, irresistible, repetitive urge to pull out her scalp hair. The hair-pulling behavior had first appeared when she was 17 years of age, had completed school and was entering college. There was no evidence of any significant stressors in her life. The hair pulling had become particularly distressing and problematic over the past six months and she described her present condition as 'tremendously out of control'. There was a significant deterioration in her academic performance and social functioning.\nThe hair pulling spells occurred on a daily basis, just before bedtime, when she was alone in her room listening to the radio, or watching television. The hair pulling used to be more severe when she was stressed. She reported a feeling of mounting tension before the act of hair pulling, with an accompanied sensation of itching. The tension was alleviated when she pulled the hair out. Pulling of hair involved twisting each hair around a finger, mostly from the sides of her head near her ears. She reported a sense of satisfaction in hearing the sound of the hair being pulled out, accompanied by pleasurable sensations. She would pull out countless hairs over few hours, resulting in a bald patch on the scalp. After the hair was pulled, she would examine the root of the hair. Later, she would secretly dispose of the plucked out hair after neatly packing them in a cover.\nShe felt guilty and embarrassed by her hair-pulling behavior and often wore her hair tied back. She was more self-conscious about the bald patches behind the ears. She used scarves to camouflage her disfigured scalp while going out. She had good insight into her illness, and recognized that her mental state was contributing to her symptoms.\nOur patient was pre-morbidly well adjusted with no significant medical or family history. There was also no history of substance use or suicide attempts. She was diagnosed to have TTM (impulse control disorder not otherwise specified) as per DSM-IV criteria []. There was no comorbid Axis I psychiatric disorders, including mood disorder. OCD was ruled out in our patient as the hair pulling (i) used to occur in leisure time only, (ii) was not in response to obsessive thoughts, but rather because of irresistible urges, (iii) was followed by a sense of satisfaction, (iv) was not associated with any other OCD symptoms, and (v) was not associated with depression.\nAt various time points, different instruments were applied to measure her treatment response (Table ). TTM behavior was measured with The Massachusetts General Hospital Hair Pulling Scale (MGHHPS) and National Institute of Mental Health Trichotillomania Symptom Severity and Impairment Scale (NIMH-TSS and NIMH-TIS) [,]. MGHHPS is a self-report measure of the severity of hair pulling urges and behavior, efforts to resist urges, control over the problem, and associated distress []. Higher scores reflect greater severity. The NIMH-TSS and NIMH-TIS are clinician rating scales []. The NIMH-TSS is a five-item measure assessing average hair-pulling episode duration during the past week and on the previous day, thoughts preceding the pulling episode, resistance to urges, distress, and interference. The NIMH-TIS is an 11-point scale assessing overall impairment in a patient's life due to TTM. Clinical Global Impression - Severity and Improvement scales (CGI-S and CGI- I) are clinician-rated instruments to assess the baseline severity and improvement in a psychiatric disorder []. Presence of depression was assessed with the Hospital Anxiety Depression Rating Scale - Depression subscale (HADS-D) [].\nOur patient was initially started on fluoxetine 20 mg/day (gradually increasing the dose up to 80 mg/day by week eight), clonazepam 0.5 mg/day (tapered off by week three) and continuous cognitive behavior therapy (CBT). By week eight, despite the reasonably adequate dose and treatment duration, there was minimal improvement in hair-pulling behavior. Hence, at week eight, bupropion sustained release (bupropion-SR) was initiated at 150 mg/day, and increased to 300 mg/day at week 10. Fluoxetine was tapered off by week 11. By week 12, there was a mild improvement in our patient's hair pulling behavior, and the dose of bupropion-SR was increased to 450 mg/day, in divided doses. At this dose, at week 13, our patient experienced significant clinical improvement in TTM. She continued to show sustained improvement during the 6 and 12-month follow-ups (Figure ). CBT was started at the beginning of treatment, and was continued throughout the one-year follow-up period. Bupropion-SR was well tolerated, except for few mild adverse effects such as nausea and nervousness during the initial two weeks, which resolved without any intervention. |
A 32-year-old woman, gravida 5 para 2, had no regular prenatal care before 24 weeks of gestation. An anterior thoracic defect with an extrathoracic two-chamber heart was recognized at 24 gestational weeks of uneventful pregnancy during the first ultrasonographic evaluation. There was no family history of congenital anomalies, genetic abnormalities, or history related to ectopia cordis. During pregnancy, the mother was smoking 15 cigarettes per day. An unfavorable prognosis to the fetus was predicted and conservative management of prenatal care had been chosen.\nThe woman was admitted to the tertiary level obstetrics and gynecology clinic during active stage of labor, at gestational age of 42 weeks. Diagnosis of congenital heart anomaly was heart ectopia, and septal ventricular defect was confirmed by ultrasound. Multiple anomalies were observed: wide anterior thoracic defect with extrathoracic four-chamber heart, rounded apex of the heart, high ventricular septal defect, the major blood vessels transposition, narrow pulmonary artery, and pericardium covering only ventricles (Figures and ). Despite unfavorable prognosis to the fetus, mother had chosen intrapartum fetal heart monitoring. Due to fetal distress at a cervical dilation of 6 cm, cesarean section was performed. The newborn was a female of 3300 g weight and 44 cm height who scored 8 (1 min) and 8 (5 min) on Apgar scale (, Supporting Information Video 1 in Supplementary Material available online at ). At birth, the infant had hypotonia, weak cry, and generalized facial cyanosis. The physical examination revealed split sternum with complete thoracic ectopia cordis, the defect followed by anterior abdominal wall defect, supraumbilical omphalocele. Ectopic heart with partial absence of the pericardium was beating outside the thoracic cavity, at a rate of 130/min with remittent bradycardia. After birth, the infant's heart was covered with warm saline-soaked sterile dressing. The newborn girl was transferred to the specialized cardiac surgery centre, children's intensive care unit. She died within 24 hours. The parents declined postmortem newborn's autopsy. |
A 54-year-old Caucasian woman was referred to our unit in March 2014 because of a suspected thyroid nodule. The diagnosis was formulated in the emergency department, where the patient was admitted because of headache, as an incidental finding during US of the carotids and vertebral arteries. The US examination was therefore extended to thyroid; the physician described a unique, solid, hypoechoic nodule, with shell calcification, with maximum diameter of 10 mm, located in the posterior part of the left thyroid lobe.\nThe patient was on levothyroxine replacement therapy to treat hypothyroidism due to thyroiditis. A second thyroid US was performed by an experienced endocrinologist, using a MyLab25Gold scanner and a linear 5–10-MHz probe (Esaote SpA, Genoa, Italy), showing an atrophic thyroid gland, without nodules (). The previously reported thyroid nodule was characterized as an oval, hypoechoic lesion with echogenic foci and peripheral vascularity, measuring 8.9 AP × 15.8 T × 23.4 L mm and located outside of the thyroid capsule, corresponding to an abnormal esophagus (). Therefore the patient was referred for esophagography that showed a 2 cm oval wall irregularity on the back slope of the distal cervical esophagus (). The patient did not complain about symptoms of dysphagia or pain. The endoscopic exploration confirmed a solid lesion at the level of the upper esophageal orifice. A biopsy examination was performed and a squamous cell papilloma was suspected. Magnetic resonance imaging confirmed the localization and the extension of the mass, while positron emission tomography demonstrated the absence of intralesional metabolic activity. Considering the biopsy, the patient was referred to surgery. The pedunculated lesion was excised through an anterior pharyngotomy. Surprisingly, the final histological diagnosis was fibrovascular polyp of the esophagus. Unluckily, the postoperative course was complicated by the formation of a pharyngoesophageal fistula, treated through a second surgical intervention.\nThis case report was written according to ethical standards. The patient gave her written consent for publication. |
A 40-year-old female presented to orthopedics-out-patient department with complaints of shoulder pain for 6 months and treated for cervical spondylitis, shoulder arthritis and given short wave diathermy and physiotherapy. The pain only worsened. In due course of treatment, she noticed an axillary mass, which increased gradually and further referred to surgery out-patient department for further management. Local examination revealed a 6 × 4 cm oval tender, firm, mobile mass in the left axilla deeper to pectoralis major muscle. A provisional diagnosis of peripheral nerve sheath tumor was made and the case was subjected for fine needle aspiration cytology (FNAC).\nFine needle aspiration from the left axillary mass yielded 30 ml straw colored fluid. The size of the swelling markedly reduced after aspiration and no residual lump was palpated. The smears showed occasional lymphocytes in the proteinaceous background. The cytological diagnosis of benign cystic lesion possibly lymphatic cyst was made and the case was asked for biopsy.\nUltrasonogram showed a cystic lesion. On magnetic resonance imaging, a well-defined, oval shaped, hyperintense cystic lesion, of 6.6 × 4.8 cm in anterolateral compartment of arm was noted []. The lesion was parallel to long axis of neurovascular bundle predominantly in subcutaneous plane with an extension into the inter-muscular plane. The case underwent surgery and the excised mass was sent for the histopathological examination.\nGross examination revealed a cystic mass measuring 6.5 × 4.5 × 2 cm. External surface was smooth and grey white. Cut section revealed uniloculated cyst filled with gelatinous material and the inner lining of the wall was smooth []. Sections from the cyst wall showed hypocellular and hypercellular areas. Hypercellular areas show spindle shaped cells having wavy nuclei arranged in fascicles with focal palisading of nuclei []. Hypocellular areas showed large number of foamy macrophages and loose myxoid stroma []. There was mild nuclear pleomorphism, but no mitotic activity was noted. On IHC tumor cells showed diffuse cytoplasmic positivity for S-100 protein [] and was negative for KI67 []; confirming the diagnosis of schwannoma. |
A 14-year-old male patient presented with complaints of vague abdominal pain and fullness on the right side of abdomen. He also had history of intermittent episodes of low-grade fever since 4 months. Clinical examination revealed mild distension on the right side of abdomen without a palpable mass. Laboratory investigations were normal except for anemia with Hb of 6.8 g% (normal range 13.5–17 g% for males). However, there was no history of any overt gastro-intestinal (GI) bleeding. Abdominal radiograph showed a large gas shadow in the right hypochondrium along with few enteroliths on the right side of abdomen []. An ultrasound of the abdomen revealed a large unilocular cystic lesion showing air–fluid level in the right lumbar and iliac region. Contrast-enhanced computed tomography (CECT) of the abdomen revealed similar imaging findings with the cyst showing air–fluid level and appearing to be in continuity with the ileum, suggesting dilated ileal loop [Figures and ]. However, no cause for obstruction could be found on CT. Barium meal follow-through (BMFT) examination was carried out later which proved that the cystic lesion was an aneurysmally dilated ileal loop with normal caliber of ileum proximal and distal to the dilated segment. The dilated segment showed normal peristalsis without evidence of any obstruction and was oriented along the long axis of the ileum [Figures and ].\nIntraoperatively, there was large cystic dilatation of the mid-ileum, measuring about 15 × 14 cm, located about 90 cm proximal to the ileo-caecal junction causing postero-lateral displacement of the ascending colon []. The cut section showed normal mucosal folds in the dilated segment with 3 × 2 cm irregular mucosa. Segmental resection with end-to-end anastomosis of the involved ileum was done. Postoperative course was uneventful. Biopsy showed normal bowel musculature and ganglion cells. An island of ectopic gastric mucosa was found corresponding to the irregular mucosa on gross examination. Final diagnosis of segmental dilatation of ileum (SDI) was made. |
This 67-year-old male man has had a progressively worsening pain on the left cranium over 2 weeks that intractable to some analgesics. There was no recent head trauma or other medical disease in his history. On admission, the general physical and neurologic investigations were not remarkable. Routine laboratory evaluations including coagulation profiles and platelet function were within normal limits.\nBrain computed tomography (CT) scans revealed an isodense left-sided CSDH with marked cerebral shifting (). There was no evidence of source of this hemorrhage with temporal predilection on the CT angiogram. On magnetic resonance (MR) image subsequently obtained, the abnormal intensity within the subarachnoid space and the brain parenchyma was not visible. The patency without steno-occlusion in both transverse and sigmoid sinuses was clearly delineated on T2-weighted sequences (). This patient has received a trephination and SDH drainage, after that he was sent home with resolution of headache. Approximately 2 weeks later, however, he developed an excruciating pain in the temporal and parietal regions with recurrence of subdural collection. The site and density of hematoma was similar to the first presentation (). He was immediately returned for subdural irrigation and decompression through the prior burr-holes. The patient's clinical course was not eventful, but he complained of a mild headache again. Follow-up CT scanned just prior to discharge was strikingly for the newly-formed thin hematoma at the operative site (). Another evacuation of this subacute subdural clot was not deemed to be necessary.\nAt this time, an active intervention was sought for this patient who had an intractably recurring CSDH. On the 7th day after the second surgery, angiography was performed to rule out an occult vascular lesion. A flow-guided type microcatheter (Prowler 10™, Cordis Neurovascular, Miami Lakes, FL, USA) was positioned in the main trunk of the MMA for selective angiography. The frontal and parietal branch of the MMA was appeared normally, and the abnormal membrane staining on the affected side was not detected. A left external carotid angiogram disclosed a dural AVF between the petrosquamosal branch of the MMA and the transverse-sigmoid sinus without retrograde cortical venous draining. The AV shunt had no connection to the internal carotid artery and its branches. It was suggested that the bleeding from the draining venous system of the dural AVF led to refractory CSDH. The microcatheter was introduced into the petrosquamosal branch of the MMA, thereafter polyvinyl alcohol particles ranging 150 to 250 µm were distally injected (). After trans-arterial obliteration of the feeder and fistula, the AV shunt disappeared. The recurrent hematoma of this patient did not increase, and his complaints of headache gradually subsided. The brain CT at one year following the embolization therapy revealed complete regression of the subdural hematomas (). |
A 24-year-old, right-handed male, machinist, with no pathological personal or family history, was admitted to evaluate a right forearm mass that has slowly increased in the past 4 years. The patient described vague forearm pain, weakness and an oval mass (of approximately 7/4 cm) of hard-elastic consistency that was found on palpation of the extensor part of the forearm. No numbness or functional impairment of the hand were present and the neurovascular examination revealed a normal distal extremity. No history of trauma or other significant systemic diseases was found. Routine laboratory tests were in between normal range. The anteroposterior (AP) radiograph of the distal forearm showed the presence of lamellar periostosis of the radius bone associated with diffuse changes in the bone structure and an aria of reduced bone density in the distal radius (Figure ). The ultrasound performed described a highly vascularizated mass situated over the radius, poorly delimitated from the surrounding tissues. Magnetic resonance imaging (MRI) was obtained to delineate bone and soft tissue involvement (see details in Figure and ).\nThe patient was scheduled for elective surgical resection. Intrasurgically we found an oval mass that was delimitated by the extensor digitorum muscle, and the extensor brevis of the thumb muscle, and the tendinous portion of the extensor carpi radialis brevis muscle. (Figure ). The tumor was not dissociable from the radius, the direct continuity with the bone substance being observed. This observation was interpreted as being a continuous tumor growing. Tumor invasion into the adjacent radius being confirmed, the lower third of the radius was scraped out.\nThe resected mass measured 6.8 cm × 3.5 cm and was surrounded by healthy tissue with grossly negative margins.\nThe submitted material was fixed in 10% formalin, paraffin-embedded and cut at 5 μm. Slides were stained with hematoxylin and eosin (H&E stain), Masson trichrome, with monoclonal antibody against CD31 (clone M0823, Dako Corporation), as well as the standard avidin-biotin complex immunostaining method for Ki67.\nMicroscopic examination revealed the proliferation of venous channels with dilated lumina, partially filled with thrombi. Papillary endothelial proliferation was focally present. On this background, a particular finding was the presence of a solid intraluminal nodule, formed by spindled cells that infiltrated the venous wall and extended into the surrounding fibrous and adipose tissue, focally reaching the bone surface (Figure ). The tumoral nodule showed many small angulated lumina, delineated by flattened cells, positive for CD31 (Figure ). On higher magnification, the tumoral nodule was formed by spindled and epithelioid cells with mild nuclear atypia and scant mitotic activity. Prominent clear vacuoles were seen within the cytoplasm of some epithelioid cells (Figure ).\nThe patient was referred to the Oncology Centre for local adjuvant radiotherapy having a total dose of 60 Gray/23 fractions/43 days. The tolerance of the treatment was good.\nNo regional or distant metastasis was found. No local recurrence has been observed at the 6, 12 and 24 month follow-up. |
A 45-year-old man visited our clinic with the complaint of a persistent hole in the 27-socket area. He explained that he had had tooth 27 extracted five years earlier and subsequently noted an opening at the socket which had never closed. He recalled that the extraction procedure itself had been uncomplicated. Subsequently, he had occasionally developed symptoms of chronic sinusitis such as nasal congestion and nasal discharge, but he had never sought treatment for it. There was also an occasional fluid discharge from the opening, but it never troubled him too much. Otherwise, he had no underlying medical illnesses. Upon examination, it was noted that there was a fistula of 1.5 cm × 1 cm in the area of the extracted 27. Tooth 16 was present, but 18 was missing. No fluid discharge was seen. Further investigation with cone beam CT confirmed an oroantral fistula measuring 9.4 mm × 7.7 mm (Figures and ). The sinus lining was noticeably thickened.\nHe underwent closure of the oroantral fistula under local anesthesia. A buccal mucoperiosteal flap was elevated around the fistula. Subsequently, the periosteum under the flap was incised to give access to the BFP. The BFP was mobilized and advanced to cover the fistula. Thereafter, the buccal mucoperiosteal flap was advanced over the BFP to achieve two-layered closure of the fistula. The advancing flaps were approximated with vertical mattress suturing. Bleeding was minimal throughout the procedure, and he was discharged with antibiotics, antihistamine, and nonsteroidal anti-inflammatory analgesia. He was advised on nasal precautions including instructions to sneeze with the mouth open and avoid blowing the nose, forceful spitting, and use of straws.\nAround 2 hours postoperative, he contacted the clinic to inform us that he had a sudden facial swelling that developed over a few seconds (). The swelling occurred immediately after he tried to clear his throat. There was no associated pain or bleeding during the development of swelling. He was asked to come back to the clinic immediately. Upon examination of the swelling, crepitation was felt on palpation. Intraorally, the surgical wound was intact, and no indication of bleeding was noted. Diagnosis of emphysema was made on the basis of the sudden progression of swelling after a trigger and clinical findings. He was reassured, and reemphasis was placed on the sinus precaution. Besides the already prescribed medications, no additional intervention was made. The swelling was slightly reduced 8 hours after its initial appearance (). Otherwise, he was well with minimal bleeding and pain.\nUpon review, 3 days later, the swelling was significantly reduced (). At 7 days postoperative, the swelling had completely resolved (). He was well throughout the postoperative period. Upon examination of the intraoral wound, no dehiscence was seen, and the oroantral fistula was completely closed. He was followed up again a few weeks later, and no reoccurrence of swelling or OAF was seen. |
A 13-year-old African-American female presented to our pediatric emergency department (ED) with fever for eight days, as high as 103°F at home. Her fevers responded to antipyretics but would return without an identifiable pattern, often within a few hours, sometimes longer; but the fever would always return by the next day. She also complained of odynophagia, headache, fatigue, and dizziness. The patient had been seen in another ED two days prior for these symptoms. She had been diagnosed as having streptococcal pharyngitis because of a positive rapid test and was prescribed amoxicillin. She came into our ED because her symptoms had worsened despite compliance with her antibiotics. Specifically, the patient had developed gradually worsening joint pains in her ankles, knees and shoulders. Her mother noted the patient’s joints had become swollen that day and she had developed a facial rash. The patient denied any nausea, vomiting, diarrhea, abdominal pain, or respiratory symptoms such as coughing or wheezing.\nHer past medical history included seizures of an unknown etiology occurring between birth and three years of age. She saw her pediatrician regularly. While she lacked a current influenza vaccination, all of her other immunizations were up to date. Her only medication was the amoxicillin she had been prescribed two days prior to presentation. She had no known drug allergies. Her family medical history was significant for a sibling with idiopathic pancreatitis and several family members with type 2 diabetes and hypertension. The patient had not travelled recently, she was post-menarchal, and was not sexually active. Her last menstrual period was two weeks prior to presentation. When questioned without family present, the patient denied any form of abuse.\nAt the time of her ED evaluation, the patient was afebrile (37.2°C) with a heart rate of 80 beats/minute, respiratory rate of 20 breaths/minute, blood pressure of 118/73 mmHg, and she had an oxygen saturation of 99% on room air. She was 5 feet 1 inch tall (1.35m) and weighed 90 pounds (41kg), giving her a body mass index of 17. This placed her at the 25th percentile for height and weight for her age.\nPhysical examination revealed a well-developed and nourished adolescent female in no acute distress who appeared fatigued. Her head was normocephalic and atraumatic with bilateral periorbital edema. Her conjunctiva and sclera were normal. Her mucous membranes were moist and she had no nasal discharge. The posterior oropharynx was erythematous without exudates. Her tongue was normal and no intra-oral lesions were seen. Her neck was supple with bilateral cervical lymphadenopathy, the largest node measuring 1.5cm in length. Her lungs were clear to auscultation bilaterally. Her heart was regular without murmurs, rubs or gallop. Her abdomen was soft and non-tender without organomegaly.\nShe had mild joint pain with passive movement of her extremities, but she had full range of motion except for pain-limited plantar and dorsiflexion of her ankles. There was bilateral pedal edema. Examination of her skin revealed an erythematous midface rash with nasolabial and perioral sparing. No rashes were present elsewhere. Her cranial nerves were intact. The patient had full motor strength throughout all of her extremities. Her coordination, balance, speech, and comprehension were all normal.\nHer initial laboratory results are shown in and . Based on the suspicion of the clinician, an additional laboratory test was sent that confirmed the diagnosis. |
A 51-year-old female patient with diabetes visited the Hepatobiliary Surgery Department of our hospital to address a persistence of chills and fever that had lasted for over 2 wk.\nThe patient reported that her symptoms had started 15 d prior to presentation, with her highest recorded body temperature during that time reaching 38.8 °C. She had visited another local hospital and had been given anti-infection treatment, which had not resolved the symptoms. The repeat fever prompted her to visit our hospital.\nThe patient had been diagnosed with diabetes 1 year prior but was not monitoring her glucose levels. She also had a history of appendectomy and no known drug allergies. She denied any history of hypertension or coronary heart disease. She recalled no experience of physical discomfort. She has not had physical examinations for an extended period of time.\nThe patient denied any relevant family history.\nEvaluation of the vital signs showed blood pressure of 115/71 mmHg, heart rate of 90 beats per min, temperature of 36.7 °C, and respiratory rate of 18 breaths per min. The height was 160 cm and weight was 65 kg (being classified by body mass index as ‘slightly overweight’).\nPhysical examination showed a slight tenderness under the xiphoid process and upper abdomen. No palpable lymphadenopathy or mass was found. There were no remarkable findings with respect to other clinical signs (e.g., McBurney's point tenderness, rebound tenderness and muscle tension, and abnormalities of the cardio-pulmonary system).\nAfter being admitted, the patient underwent laboratory examinations including routine blood test, stool analysis with fecal occult blood test, liver and kidney function tests, electrolyte panel, blood coagulation factor tests, and tumor markers tests. Endoscopy and the preoperative examinations eliminated the possibility of syphilis, hepatitis B, hepatitis C, and human immunodeficiency virus infection. Routine blood test showed elevated leukocytes (24.67 × 109/L; normal range: 4.00-10.00 × 109/L) and neutrophils (91.24%; normal range: 50%-70%). The biochemical tests showed decreased albumin (28.2 g/L; normal range: 35.0-54.0 g/L) and increased alanine aminotransferase (84 IU/L; normal range: 0-40 IU/L), aspartate aminotransferase (67 IU/L; normal range: 0-46 IU/L), and D-dimer (14.68 mg/mL; normal range: 0-0.50 mg/mL). The tumor markers carcinoembryonic antigen (CEA) (0.8 ng/mL; normal range: 0-5.0 ng/mL) and CA199 (5.45 U/mL; normal range: 0-40 U/mL) were within the normal range. There was no remarkable finding obtained from any of the other hematologic tests.\nPlain computed tomography (CT) scanning of the liver and gallbladder, performed in another hospital, had displayed a low-density mass in the patient’s hepatic porta, and the possibility of malignant tumor cannot be ruled out. We augmented the imaging examination by performing color Doppler ultrasound of the liver and gallbladder, and found a hypoechogenic mass in the caudate lobe of the liver (6.5 cm × 4.3 cm in size) which was suspected as a malignant tumor (Figure ). Plain plus enhanced magnetic resonance imaging of the liver and gallbladder also showed a space-occupying lesion on the caudate lobe of the liver (7.6 cm × 4.4 cm × 5.0 cm), which was suspected as cystadenocarcinoma (Figure ) and was considered in differential diagnosis (from hepatic abscess) along with the patient’s clinical manifestations. A new plain CT scan of the liver, gallbladder, and spleen suggested a foreign body (fishbone) in the upper abdomen, which had perforated the gastric wall and reached the hepatic hila, and consequent abscess formation in the caudate lobe and superior head of the pancreas (7.8 cm × 6.0 cm × 5.0 cm) (Figure ).\nGastroscopy was ordered and revealed chronic gastritis and a sinus tract located in the anterior wall of the duodenal bulb (Figure ). |
A 34-year-old, para 1, Korean woman underwent emergent cesarean section at 34 weeks of gestation in the Korea University Medical Center Anam Hospital in March 2012 because of preterm premature rupture of the membrane and breech presentation. Her body mass index (BMI) was 22.1 kg/m2. She was diagnosed with a uterus didelphys and vaginal septum but had no other medical or surgical history. A transverse suprapubic (Pfannenstiel) incision was performed during the cesarean section. A 2.42-kg male baby was delivered, without perioperative complications. After closure of the subcutaneous layer, the skin was closed with subcuticular running sutures by using a 3-0 nonabsorbable monofilament nylon suture (AILEE Inc., Busan, South Korea). The distal part of that string was snapped during the wound dressing on postoperative day 4, and the resident ordered the discharge of the patient on the next day without removal of the remaining string. Five months after the patient was discharged, she presented to the outpatient clinic with a fine, blue object visible on the left lower quadrant of her abdomen. A 10-cm thread-like foreign body showed through her skin. It was situated transversely; the medial end was located approximately 5 cm horizontally from the scar of the previous cesarean section, and the lateral end reached the upper lateral margin of the anterior superior iliac spine (Figures [arrows] and ). No pain or any other sign of inflammation was observed around the region. We discovered that the foreign body was the nonabsorbable suture material that had not been removed completely during the hospital stay. We thought that the remnant suture material had migrated laterally approximately 15 cm in dermis layer during the previous 5 months without any symptoms. Under local anesthesia with lidocaine, the material was removed easily through a 2-mm incision (Additional file : Video 1). The nylon suture material was confirmed, without the knot portion (Figure ). The patient was discharged without any complications. |
A 39-year-old African American male patient with NF-1 presented to the Orthopaedics Department of Saint Louis University with the chief complaint of a 30-year history of left knee instability. He was diagnosed with NF-1 after minor trauma resulted in left knee edema at age 9 and subsequent testing revealed NF-1. As he grew, this swelling also increased. He had progressive difficulty walking, only ambulating one block without aid. He could neither run nor squat. His leg was very uncomfortable and painful, and it required his constant readjustment and reengagement due to recurrent subluxations. He had two osteotomies, one per leg between ages 10 and 15. The patient had no history of any types of fractures, but minor trauma caused recurrent subluxations throughout the years. His most recent trauma was two years ago, when minor stumbling on stairs resulted in excessive subluxation of the tibia. He had no other relevant past medical history. Other relevant family history included NF-1 in his father.\nOn examination, left leg was significantly larger than the right due to hemihypertrophy and lymphedema. The knee was very unstable, and crepitus with range of motion was present. A large effusion was present posteromedially, and there was minimal ligamentous support (). Bilateral knee radiographs revealed a significantly dysplastic and sclerotic left distal femur and left proximal tibia with visible varus deformity. Significant joint space narrowing and medial and lateral compartment bone-on-bone contact were present. provides evidence of a Kellgren-Lawrence grade 4 OA of the left knee. MRI found extensive soft tissue swelling. Two effusions were present: one large suprapatellar effusion and one posterior to the knee measuring 8.2 × 7.3 × 6 cm (). Also present were chronic bony deformities of the distal femur and proximal tibia (). The posterior cruciate ligament was thinned yet intact. The quadriceps and patellar tendons were intact. The medial collateral ligament was stretched but also intact.\nThe operation was performed using a classic transfemoral/supracondylar approach. There were no complications or difficulties during the procedure. The following were notable findings discovered during the operation: most muscles were visibly atrophic, a large neurofibroma was visualized in the tibial nerve prior to dissection, and the patient's lymphedemic leg had resulted in extensive vascular anastomoses and hypervascularization. The left femur postamputation is seen in . As of 6 month followup, patient reports minimal discomfort, improved functional capacity and ambulation of more than 2 blocks with no physical aids (other than prosthetic device), and no complications due to surgery other than slight stump bleeding occurring soon after operation, which has not recurred. depicts patient during initial physical therapy 2 months after amputation. |
A 38-year-old Asian woman was referred to the clinic with the chief complaint of sinus tract on maxillary left permanent central incisor and unpleasant aesthetics of her maxillary anterior crowns. Patient had crowns performed on both maxillary permanent central incisors about five years ago. The patient reported no history of dental pain or swelling. She had RCT completed on maxillary left permanent central incisor a month ago by her dentist, but the sinus tract did not resolve after the RCT. Both teeth were asymptomatic. Patient denied history of dental trauma.\nClinical examination revealed that both teeth presented with marginal discrepancy of PFMs. They were not tender to percussion and palpation, and had no gingival swelling. However, a sinus tract was detected on the buccal gingivae, and a palatal accessed cavity was observed on the PFM crown of maxillary left permanent central incisor. Maxillary right permanent central incisor did not respond to the cold test. A deep pocket more than 10 mm was observed on the mesial aspect, and 5 mm was observed on the distal aspect of maxillary left permanent central incisor. A deep pocket more than 6 mm was observed on the mesial aspect of maxillary right permanent central incisor. Maxillary right and left permanent central incisors presented with Miller grade I and II mobility, respectively.\nThe PA radiograph () showed previous RCT on maxillary left permanent central incisor with acceptable length and density. A halo-shaped periradicular radiolucency was observed, and the gutta-percha size #30/02 (Dentsply Maillefer, Ballaigues, Switzerland) traced the sinus tract to the periradicular radiolucency associated to this tooth. Besides, a few radiopaque hard-tissue-like materials were also observed around the apical half of the root. In regards with maxillary right permanent central incisor, no RCT was performed, and a periradicular radiolucency was observed. Vertical bone loss was detected on the mesial aspect of the tooth. An irregular radiopaque mass was attached on the mesial aspect of the root of maxillary right permanent central incisor at mid-root region, suggesting possible calculus deposition. A partially detached radiopaque material was observed at the distal aspect of the root at the apical third. CBCT views (Veraviewepocs 3D R100, J. Morita, Osaka, Japan) () showed extensive periradicular bone loss around both teeth. The radiopaque fragments were found partially detached on the root surface of maxillary right permanent central incisor at apical third and at the mesial surface of the mid-root region. A radiopaque fragment was also found completely detached from the root of maxillary left permanent central incisor at mesio-palatal part of apical part of the root. A partially detached radiopaque fragment was also found on the disto-buccal aspect of the root at apical half of maxillary left permanent central incisor.\nMaxillary right permanent central incisor was diagnosed as pulp necrosis with asymptomatic periradicular periodontitis. Maxillary left permanent central incisor was diagnosed chronic periradicular abscess with a secondary periodontal affection. Both teeth presented with the cemental tear. The suggested treatment plan and options were:\nRCT on maxillary right permanent central incisor prior to surgical debridement of cemental tear and apicoectomy with retrograde filling on both teeth. Extraction of maxillary right permanent central incisor and replacement of the missing tooth with implant, bridge, or denture.\nAfter discussing with the patient the risks and benefits, the procedures involved, the cost, and the possible prognosis of the treatment, the patient decided to have both the teeth extracted. The extraction was performed by the patient’s dentist, and the extracted teeth, curetted granulation soft tissue, and prickle-like hard tissue were sent for biopsy.\nCalculus and plaque were observed especially on the mesial mid-root region of the extracted maxillary right permanent central incisor (). A cemental line could be seen on the root surface, which indicated of the history of cemental tear. A prickle-like hard tissue was observed on extraction of maxillary left permanent central incisor (). A partially detached hard tissue was observed on the buccal surface of the maxillary left permanent central incisor, and a few cemental lines were observed on the root surface suggesting that cementum was detached from the root surfaces.\nThe histopathological examination of the soft tissue lesion () showed inflamed fibrous connective tissue wall lined by non-keratinized stratified squamous epithelium, confirming a diagnosis of radicular cyst. The decalcified section of the hard tissue fragment that was detached from the tooth root consisted of dentine with attached cementum (). Whereas, the decalcified section of the extracted teeth showed cemental tear involving multiple sites along the root surface (), corroborating a diagnosis of combined cemental and cementodentinal tears. Deep periodontal pocket was reflected by the presence of microbial deposits along the root surface (). |
A 51-year-old man who had an excavator managing job at a construction field, as a head of the household of a multicultural family, had been steadily gaining weight since his 30s. The patient suffered from a herniated disc, gonarthritis, and sleep apnea as his weight reached 106 kg. He lost approximately 13 kg through exercising during 1 year prior to the surgery. When the weight loss slowed down, he visited the Bariatric Surgery Center at Soonchunhyang University at Bucheon Hospital in June 2013 for a surgical consult. At the time of the surgery, his weight was 93.0 kg and BMI was 35.8 kg/m2, which was corresponding to severe morbid obesity. Medical comorbidities included fatty liver and hyperlipidemia.\nThe patient underwent laparoscopic sleeve gastrectomy on July 11, 2013. He followed up at the hospital before surgery, 1 month, 3 months, and 6 months after the obesity surgery. He underwent examinations for anthropometry, body component, blood test, and dietary survey on each visit. Abdominal computed tomography (CT) was performed 6 months before and after the surgery. Dietary counseling included nutritional assessment, changes in eating behavior, nutrient considerations, weight-loss strategies, motivation, telephone consultation, and nutrition diagnosis. All physicians and a professional nutritionist provided patient education on every visit along with the feedback the test results.\nChanges in the patient's anthropometry, body fat, and blood test measurements during the 6 months following the surgery are shown in . The patient's weight decreased by approximately 20 kg from 93 kg to 73.3 kg, close to his target weight of 70 kg. His body fat decreased from 32.4 kg to 22.2 kg, and body fat percentage decreased from 35.5% to 33.0%. His waist circumference decreased by approximately 20 cm from 114 cm to 93.4 cm. His liver function test decreased to normal levels, which was proven by the decrease in aspartate aminotransferase level to 17 IU/L and an alanine aminotransferase level to 19 IU/L at 6 months after the surgery. His triglyceride level decreased from 215 mg/dL to 104 mg/dL at 6 months after the surgery, while high-density lipoprotein cholesterol level increased from 39 mg/dL to 56 mg/dL.\nThe abdominal CT scans before the surgery and 6 months after the surgery are shown in . The area of the fat has been represented by the green color. The area of subcutaneous fat of patient decreased 28.3% from 293.7 cm2 (A) to 210.5 cm2 (B) after operation. While the area of visceral fat decreased 59.0% from 317.8 cm2 (C) to 130.4 cm2 (D), showing remarkable improvement compared the change of area subcutaneous fat. The visceral-to-subcutaneous fat ratio (VSR) was changed from 1.08 to 0.62 and remained stable after the surgery.\nThe patient's nutrient intake was investigated using a food recording method and was analyzed using a specialized nutrition analysis program (CAN program ver. 3.0, The Korean Nutrition Society, 2005). The major nutrient intake and fat types are shown in . Prior to the surgery, the patient consumed excessive amounts of alcohol and meat nearly every day during the week, while his weekend diet mainly consisted of fast food, fried chicken, and fruit. The patient also expressed that he had difficulty of controlling his gluttony. During the first month after the surgery, the patient consumed rice liquid, porridge, and high-protein powder before returning to a regular diet. Three months after the surgery, the patient was able to control his meal portions. As a result, the patient's caloric intake, which was approximately 3,700 kcal before the surgery, was reduced to approximately 900 kcal 6 months after the surgery; and his intake of carbohydrate, protein, and fat also showed dramatic decreases. Cholesterol intake was decreased from 578 mg to 121 mg after the surgery; and his intake of total fatty acid, monounsaturated fatty acid, and polyunsaturated fatty acid was also decreased noticeably. The intake ratio of total fatty acid to monounsaturated fatty acids to polyunsaturated fatty acids showed a positive change with 1:1.1:0.9 at 6 months after the surgery.\nAfter the surgery, the patient no longer had sleep apnea, and no complications or eating disorder were observed during the follow-up care. Moreover, the patient's family members were satisfied and were very supportive to accommodate to his changed body type and lifestyle after the surgery. The patient is still maintaining the effort to keep his current weight by controlling his food intake and exercising. |
A 49-year-old woman was investigated for intestinal dysmotility with symptoms of gradual weight loss, postprandial epigastric bloating, a sense of repletion and vomiting over an 8-month period. Since her teenage years, she had maintained a steady weight of 48 kg, but had lost 6 kg over the last 8 months. Her past medical history was remarkable for symptoms suggestive of Raynaud's syndrome, multi-joint arthralgia and an episode of anorexia 4 years previously. She started to experience symptoms of Raynaud's syndrome nearly 5 years ago when she changed her occupation and started working in the food catering industry. There was no deterioration of the symptoms of Raynaud's syndrome associated with the weight loss. There was no history of recent trauma, surgery, prolonged immobilisation or neurological illness. Her weight loss was gradual. A gastroscopy demonstrated a large residue of fluid and undigested food in her stomach with a dilated duodenum extending approximately 10 cm distal to the pylorus. Duodenal and gastric biopsies were negative. Symptoms of presumed delayed gastric emptying failed to resolve after a trial of prokinetics and proton pump inhibitors and she was admitted with hypoalbuminaemia, hypokalaemia and continued weight loss.\nA CT scan of the abdomen and pelvis revealed a hugely dilated stomach extending to the pelvis. The second part of the duodenum (D2) was dilated proximal to a point of sharp obstruction at the level of the third part of the duodenum (D3). At this point of obstruction, the aorto-mesenteric distance was reduced to 6 mm (Figure ). A CT scan also confirmed the impingement of the left renal vein between the aorta and superior mesenteric artery (SMA; see Figure ). There was no thickening of the wall of the duodenum or extrinsic mass lesion. Sagittal reconstruction of CT images through the mid-abdomen (Figure ) showed that the angle between the SMA and the aorta (SMA-aorta angle) was reduced to 16°. A diagnosis of Wilkie's syndrome was made on the basis of clinical suspicion and CT findings. The potential cause of the syndrome was unclear and the only obvious precipitating factor was her gradual loss of weight over 8 months.\nA 7-day trial of conservative management with a nasogastric tube, enteral feeding supplements and prokinetics failed. The patient's symptoms worsened, and she experienced more frequent vomiting and lost a further 1 kg. In view of the continued weight loss and worsening symptoms, the conservative management was abandoned and the patient chose to have Strong's procedure and open surgery.\nAn attempted laparoscopic duodenojejunostomy and mobilisation of the duodenum (Strong's procedure) was abandoned as the massively distended stomach did not allow a satisfactory assessment of the third part of the duodenum. During the open procedure (laparotomy), the duodenum was Kocherised and the duodenojejunal flexure was mobilised to perform a side-to-side duodenojejunostomy. There was no evidence of alternative causes of obstruction. Her symptoms gradually improved after the operation, she steadily gained weight and her biochemical markers returned to normal. The patient returned to work 3 weeks after surgery.\nA subsequent CT scan showed less gastric distention with evidence of continued poor gastric emptying secondary to an enlarged redundant stomach, suggesting persistent gastroparesis. The symptoms of vomiting and poor gastric emptying returned 5 months after surgery. A gastroscopy confirmed an enlarged redundant stomach with a patent duodenojejunostomy and poor gastric emptying. Her symptoms improved with supplement feeding for 3 days and correction of her hypokalaemia. Endoscopic biopsies of the duodenum and stomach failed to indicate any alternative diagnosis such as intestinal myopathy.\nShe was also reviewed in our hospital and a tertiary hospital 14 months after the initial operation. She still had a few episodes of vomiting especially after eating a large meal and her weight remained unchanged. Again endoscopic and radiological investigation confirmed the diagnosis of gastroparesis related to Wilkie's syndrome. |
A 65-year-old woman with a history of morbid obesity status post-LAGB nine years before admission presented to the emergency department with shortness of breath for one week and acute onset of pleuritic chest pain for one day. She was afebrile but tachycardiac and had an elevated white count of 20,600 white blood cell/mcL. The patient was transferred from an outside hospital after computed tomography (CT) scan demonstrated a pericardial effusion, pneumoperitoneum, pneumopericardium, pulmonary embolism, and a dilated esophagus [].\nThe patient underwent gastric band decompression in the emergency room, was admitted to the intensive care unit, and was started on antibiotics. An upper gastrointestinal (UGI) examination performed at our hospital demonstrated an increased phi angle concerning for gastric band slippage but no definitive evidence of leak, perforation, or fistula []. The phi angle is defined as the superior angle formed by the longitudinal axis of the gastric band and the spinal column. A normal phi angle is 4–58°. Any angulation greater than 58° is consistent with gastric band slippage and will result in eccentric gastric pouch dilation.[]\nEchocardiogram showed a pericardial effusion and evidence of impending tamponade with a preserved ejection fraction. The patient subsequently had a pericardiocentesis. Approximately, 340 mL of serous fluid was removed and a drain was placed. On day three, the patient underwent an esophagogastroduodenoscopy that showed erosion of the gastric band into the stomach just distal to the lower esophageal sphincter [].\nOn day four, patient went to the operating room for a laparoscopic gastric band removal. A large abscess was noted adjacent to the lap band which was drained intraoperatively. An omental patch was used to close off the abscess cavity and gastrostomy site. Postoperatively, the patient continued to complain of the left upper quadrant pain, left pleuritic chest pain, and shortness of breath. At this time, she was noted to have muddy brown output from her pericardial drain. A CT chest and abdomen were performed. This demonstrated a fistulous connection between a perigastric collection that was contiguous with the gastric body and the pericardium. Oral contrast was noted layering in the pericardium [].\nA left pleural drain was placed for a persistent left pleural effusion associated with shortness of breath. This provided symptomatic relief, improved oxygen saturation, and decreased the patients oxygen requirements. An additional UGI was performed to confirm a gastropericardial fistula []. Fistula was confirmed and managed nonoperatively. Pericardial drain output was monitored as diet was slowly advanced until output was minimal and the pericardial drain was removed. The patient was discharged on the 37th hospital day to a long-term acute care facility. |
We present the case of a 53-year-old Caucasian male who was admitted to the hospital owing to fatigue and black stools. His vital signs on the admission were: temperature 36.8 °C, heart rate 70 beats per minute, respiratory rate 17 breaths per minute, and blood pressure 120/80 mmHg.\nThe patient was pale but with good nutrition status [body mass index (BMI) 23.1 kg/m2]. He did not consume alcohol or tobacco. He was married and had two children aged 15 and 19 years.\nHe was taking no medication and had no other underlying disease. Moreover, his medical history was free of any previous surgical interventions. During the clinical examination, there was no sensitivity or tenderness in the abdomen and the bowel sounds were normal. Digital rectal examination revealed the presence of black stools. Clinical examination of cardiopulmonary and urogenital systems showed no abnormal signs. Apart from fatigue, there were no other findings on physical and neurological examination.\nThe blood tests depicted anemia (hemoglobin 6.0 g/dl with normal values between 13.4 and 17.4 g/dl, and hematocrit 20% with normal values between 41% and 53.8%). The results of all other markers were within normal range (Table ).\nDuring his admission to the hospital, the patient was transfused with 4 units of blood and 1 unit of fresh frozen plasma, and a Computerized Tomography (CT) scan of the abdomen was performed. The CT scan of the abdomen revealed a mass with vague limits (dimensions 6.8 × 5.7 cm) between the pylorus of the stomach and the first part of the duodenum. Around that mass, there were several lymph nodes with diameter up to 1.6 cm. Furthermore, the presence of appendiceal mucocele was indicated as an incidental finding.\nOn the third day of his hospital stay, the patient underwent EUS and upper gastrointestinal endoscopy, in which the biopsies showed morphologic and immunochemical features compatible with glomus tumor. Twelve days after his admission, the patient underwent open laparotomy with a midline incision. An antrectomy with Roux-en-Y anastomosis and appendicectomy were performed owing to suspicion of appendiceal mucocele from the CT scan (Figs. , ).\nThe postoperative period was without any incident, and the patient was released in good condition 5 days after the operation. After the surgery and during his hospital stay, he was receiving intravenously 3 g cefoxitin/day for 3 days, 1.5 g metronidazole/day for 3 days, 4 g paracetamol/day for 4 days, and 200 mg tramadol/day for 3 days. The initial postoperatively intravenously administration of fluids was followed by oral feeding after 3 days.\nPathology examination showed a 5.5 × 5 × 4.2 cm intramural gastric mesenchymal neoplasm compatible with glomus tumor. Although there was no prominent nuclear atypia, and no mitotic activity or any atypical mitosis, because of the tumor’s size, > 2 cm, and the location, deep in the layers of gastric wall, the final diagnosis was glomus tumor with uncertain malignant potential (Figs. , , ). Also, the examination showed appendiceal mucocele with elements of previous rupture.\nAfter discharge from the hospital, the patient underwent regular follow-up meetings with clinical examination, blood tests, and computerized tomography of the brain, the chest, and the abdomen. His last follow-up was in January 2021, 20 months after his admission to the hospital, in which he was found in good clinical status and with no signs of recurrence. There were no findings of pathological entity on the CT of the chest and the abdomen. He was scheduled for a meeting again after 6 months. |
A 14-year-old boy was diagnosed with osteosarcoma of the left distal femur. He underwent 4 courses of chemotherapy with cisplatin (CDDP) (167 mg) and doxorubicin (42 mg). He was also administered a 5-HT3 receptor antagonist to prevent nausea and vomiting and a regimen of the histamine-2-receptor antagonist to prevent gastroesophageal reflux disease; despite this, he complained of severe nausea and vomiting. The patient experienced hearing impairment because of CDDP, therefore, after the first 4 courses of chemotherapy, the regimen was changed to comprise ifosfamide (4200 mg), etoposide (84 mg), and methotrexate (28 mg). Two courses of this new regimen were administered. Five months after the diagnosis, the patient underwent extensive tumour resection and total knee replacement with a custom-made extendable knee replacement system (Growing KotzProsthesis;Stryker, Germany).Pathological examination showed that the tumour tissue was almost necrotic, which suggested that the preoperative chemotherapy was effective. After the operation, the patient was administered 6 courses of the second chemotherapy regimen over a period of 5 months. During this treatment period, he experienced nausea and vomiting. Four weeks after the postoperative 6-course chemotherapy, he complained of dysphagia and could not eat solid foods. A barium-swallowing examination showed significant narrowing of the distal oesophagus (). Upper gastrointestinal endoscopy revealed stricture of the distal oesophagus, demonstrating slightly reddish and inflammatory mucosa at the distal oesophagus (). The distal oesophagus was narrow, and the entire oesophageal wall was thick, so the endoscope could not pass beyond the stricture of the oesophagus. A biopsy of the strictured part of the lesion was not performed. He was diagnosed with chemotherapy-related oesophageal stricture, and balloon dilatation for the oesophageal stricture was performed 10 times over a period of 2 months. Finally, the oesophagus was dilated till its diameter increased by 12 mm. During this period, omeprazole (proton pump inhibitor, PPI) was administered. After ballooning, his dysphagia improved, and he could ingest solid food. At the final follow-up, which was conducted 20 months after the ballooning, the oesophagus was found to be normal with no strictures, and osteosarcoma of the left distal femur had also not relapsed and metastasized. |
A 10-month-old developmentally normal boy, second born of third degree consanguineous parents, presented with right focal seizures. Postictally, the child had altered sensorium along with right hemiparesis. He was hospitalized for the same and was later referred to us on day 6 of illness.\nAt admission, the child was in altered sensorium with a Glasgow coma score (pediatric) of 12. He had significant pallor but was hemodynamically stable. All peripheral pulses were well- palpable. Clinical diagnosis of left middle cerebral artery territory AIS was kept and MRI brain confirmed the same []. MR angiography revealed a sudden cut-off in left internal carotid artery just after the origin with complete occlusion of the same but dissection could not be ruled out. Intracranial left internal carotid artery and its branches were also not visualized and there were no significant collaterals []. On sonography and Doppler of neck vessels, left internal carotid artery could not be visualized. Echocardiography was essentially normal.\nThe child was extensively evaluated for all possible risk factors contributing to stroke, detailed in . His blood investigations revealed a normocytic normochromic anemia (MCV = 94 μl) with a normal platelet and total leucocyte count. By the fourth day of hospitalization, he developed thrombocytopenia with elevated lactate dehydrogenase (LDH). Peripheral smear examination showed schistocytes (4%-5%) with a low reticulocyte count. His serum vitamin B12, folate, plasma homocysteine, and C3 levels were within normal limits. Subsequently, a gradual decline of hemoglobin levels and platelet count was noted. In addition to the evolution of bicytopenia, he also developed facial puffiness with hypertension. A working diagnosis of congenital thrombotic thrombocytopenic purpura (TTP)/hemolytic uremic syndrome (HUS) was considered and clinical exome sequencing was ordered. He was started on daily fresh frozen plasma transfusion followed by Plasma Exchange (PEX). Once platelet counts improved to >1 lac/mm3 with normalization of blood pressure records, the child was switched to oral steroids which were continued for about a month and gradually tapered monitoring the platelet count.\nExome sequencing revealed a novel homozygous nonsense variation in exon 2 of the SLC19A2 gene (chr1:169446604; G>C; Depth: 119x) that results in a stop codon and premature truncation of the protein at codon 199 (p. Ser199Ter; ENST00000236137). The region is conserved across species. The detected variant was predicted to be damaging by SIFT, LRT and Mutation Taster and was likely pathogenic.\nAt 3-month follow-up, the child developed sensorineural hearing loss. Bone marrow aspirate revealed cellular marrow with adequate megakaryocytosis and erythroid series showed megaloblastic maturation. HbA1c was 6.7 g/dl (normal < 6.5%). Child was initiated on thiamine in supratherapeutic doses (500 mg/day). Currently, at 2-year follow-up, hematological parameters are within normal limits. There has not been any recurrence of stroke. He failed a hearing-aid trial and has been planned for cochlear implant surgery. Informed consent was obtained from parents for publication.\nThiamine responsive megaloblastic anemia (Roger syndrome) is characterized by a clinical triad of megaloblastic anemia, deafness, and diabetes. Megaloblastic anemia and diabetes may manifest from infancy to adolescence while deafness usually manifests in the toddler age group. This entity was first described in 1969 by Roger et al.[] It is caused by a mutation in SLC19A2 gene, which encodes the high-affinity thiamine transporter. Besides classical triad, other features include abnormalities of retina and optic nerve, stroke-like episodes, congenital heart disease, arrhythmias, aminoaciduria, tri-lineage myelodysplasia, short stature, and situs inversus.\nThiamine deficiency is known to affect respiratory chain complex I activity. The neurological features of TRMA, resembling those of mitochondrial complex I deficiency, may be caused due to a secondary defect in mitochondrial energy production.[] But, the association with stroke and pathophysiology of stroke in TRMA is unclear.\nTill date, there are two patients with TRMA reported with AIS and one with cerebral sinus thrombosis.[] Possible pathogenic mechanisms include: Mapping of human coagulation factor V and antithrombin III precursor to nearby region as SLC19A2 gene (a complex mutational event in this region may also involve coagulation cascade genes, causing susceptibility to thrombosis)[] and prothrombotic tendency due to hyperglycemic state (increased levels of plasminogen activator inhibitor-1 and the decreased endogenous fibrinolysis activity).[] Besides these, the clinical spectrum of TRMA is still evolving due to the rarity of this disorder; microvascular and coagulation pathway affection are still ambiguous. Also, the role of a particular genotype causing disease and stroke as a presenting feature is also unclear.\nOur initial clinical possibility in the index patient was congenital HUS/TTP in view of thrombocytopenia, hypertension, consanguinity and early infantile onset, and exome sequencing was done suspecting the same. But it revealed a likely pathogenic variation in the gene for TRMA. Anecdotal reports suggest that TRMA may be associated with stroke however its association with TTP/HUS is speculative. There have been rare reports of atypical HUS associated with cobalamin C defect which presents with megaloblastic anemia, methylmalonic acidemia, and homocystinuria.[] However, no direct link between TRMA and HUS/TTP have been mentioned. Both acquired HUS and TRMA seem to have coexisted in the index case and this probable unusual association merits documentation.\nThe basis for responsiveness of thrombocytopenia to plasma exchange in the index case is uncertain. Possibilities include superadded consumption coagulopathy, acquired hemolytic uremic syndrome/TTP, thiamine repletion, and prothrombotic state due to disease per se which also might have responded to plasma exchange.\nThis patient had two uncommon manifestations of this rare disorder: AIS and thrombocytopenia. Also, the child had a novel nonsense mutation in SLC19A2 gene. History of consanguinity, in this case, was another pointer towards a genetic disorder. Later, clinical features such as sensorineural hearing loss and slightly elevated HbA1c confirmed the clinical phenotype.\nThis case brings forth association between TRMA, prothrombotic state, and AIS; although the exact physiological basis still remains indeterminate. TRMA as a possible risk factor for stroke still remains a mystery. Evolving phenotypic spectrum of this disease might make the intricate pathways more lucid.\nThe authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.\nNil.\nThere are no conflicts of interest. |
A 35-year-old G3P2002 with two prior uncomplicated vaginal deliveries and no significant medical history presented at 15 3/7 weeks gestation by last menstrual period. She was brought in by emergency medical services due to a syncopal episode at home. Her history was notable for one-month of progressive abdominal pain and associated nausea, vomiting and diarrhea. She reported having regular follow-up with her obstetrician and multiple recent emergency department visits. She had no prior surgeries. Her vitals showed a heart rate in the 130s and her blood pressure was 76/43. On physical exam her abdomen was diffusely tender with peritoneal signs. Her labs were significant for a leukocytosis to 28,700, hemoglobin of 7.6, creatinine of 1.07 mg/dL and lactate of 5.7 mmol/L. Ultrasonography showed free fluid in the abdomen. Both the obstetrics and trauma surgery teams were immediately consulted as the source of her hemorrhagic shock was unknown. The patient became hemodynamically stable with resuscitation; so, the decision was made to proceed with a computed tomography (CT) scan of the abdomen and pelvis with contrast.\nConcurrently, outside hospital records were made available and reviewed by both teams. They revealed that the patient had ultrasounds performed at 8 5/7, 12 4/7, and 13 0/7. Her ultrasound at 12 4/7 noted a possible arcuate uterus with the pregnancy located more on the patient’s right. An additional scan at 13 0/7 reported suboptimal visualization of the pregnancy on both transabdominal scan and transvaginal scan. These records also showed that one week prior to presentation at our facility, the patient had gone to an outside emergency department with right lower quadrant pain. There she had an elevated white blood cell count to 18,000, a transabdominal ultrasound and abdominal MRI. Neither the ultrasound nor the MRI were interpreted as showing any acute abnormalities. Both specifically commented on there being an intrauterine pregnancy. The patient was discharged home from the outside emergency department with a presumed viral illness. A timeline of events is presented in Fig. .\nAt our facility the patient’s CT scan demonstrated a large volume hemoperitoneum, and the pregnancy appeared to be superior to the uterine corpus. This raised concern for a ruptured interstitial ectopic pregnancy (Fig. ). The patient was consented for emergency laparotomy with possible hysterectomy.\nA midline laparotomy incision was made below the umbilicus. Upon entering the abdominal cavity, hemoperitoneum was encountered and more than 2 L of blood were evacuated from the abdomen. Inspection of the uterus showed an enlarged right cornua with the amniotic sac actively rupturing through the uterine serosa. Gentle manipulation of the uterus resulted in spontaneous rupture of the amniotic sac and expulsion of the pregnancy (Fig. ). The patient was hemodynamically stable as resuscitation was ongoing. Further inspection of the operative field showed brisk bleeding from placental tissue densely adherent to the uterus and what remained of the right uterine cornua. On close examination, there was no remaining tissue in the right cornua which could be used for re-approximation and no clear plane for dissection or removal of the remaining placental tissue (Fig. ). Thus, the decision was made to proceed with supracervical hysterectomy which was uncomplicated. Intraoperative blood loss was 5000 mL. The patient received 6000 mL intravenous fluid, 6 units packed red blood cells, 4 units fresh frozen plasma, 1 unit platelets and 500 mL albumin. Preoperative hemoglobin was 7.6 g/dL and was 8.4 g/dL on postoperative day 1. The surgical pathology report was significant for right cornual changes consistent with placenta percreta.\nHer postoperative course was complicated by the need for brief use of supplemental oxygen on postoperative night 0. This was thought to be due to atelectasis versus volume overload secondary to large volume resuscitation. The patient and her spouse received emotional support and counseling services while in the hospital and the patient was started on sertraline. She was discharged on postoperative day 3 with plans for a two-week follow-up. At her two-week follow-up the patient had recovered from a surgical perspective. However, emotionally, the patient had developed significant anxiety as a result of the prior events. |
A 37-year-old woman patient who is a homemaker was accepted to our hospital with the complaint of a 10-day pain in her right upper limb. She mentioned that the pain first involved the elbow and then the forearm, particularly the medial part of it. Nearly 10 days before, while she was cleaning and shaking the rug, she developed a discomforting pain in her right elbow. She explained that the pain in her elbow had become worse and in 24 hours spread through her whole forearm. She added that, previously, the pain had been partially responding to NSAI drugs, but subsequently, it continued to progressively increase.\nThere was a pain in her medial forearm and elbow. She felt abnormal when she was palpated on her medial forearm. During her examination, she was able to describe the point where her pain started in the proximal part of her elbow. On detailed neurological examination, a region of dysesthesia which extends from the elbow to the medial forearm was detected (Figure ). The patient had no history of polyneuropathy, chronic systemic disease, injection or surgical intervention at the elbow. Range of motion, motor, and reflex examinations of both upper extremities were normal. Cervical spine examination was normal. Varus-valgus stress test for the elbow was normal. Medial epicondylitis test and tinel test for the ulnar nerve were negative.\nX-ray views of the elbow, including oblique views, appeared normal. Electromyography showed normal findings in the right biceps, triceps, flexor digitorum sublimis, pronator quadratus, interosseous and abductor pollicis brevis muscles, and nerve conduction studies in both upper limbs except for the right MACN were found normal. The MACN is stimulated antidromically at the lateral border of the biceps brachii tendon in the cubital fossa. An active surface recording electrode is placed on the anteromedial surface of the forearm 14 cm from the active stimulating electrode. Sensory action potential (SAP) amplitude of the right anterior branch of the MACN was detected to be lower in proportion to the left. The sensory conduction velocity (SCV) was normal. On both right and left sides, the posterior branch of the MACN was normal and symmetrical for amplitude and velocity (Table ). On magnetic resonance imaging of the elbow, no lesion was detected which may cause MACN neuropathy.\nAs well as NSAI drug treatment, physical therapy of 15 days (TENS, ultrasound, ROM exercises) was applied to the patient. The complaint of pain was totally relieved. Two months later, the dysesthesia disappeared. No recurrence occured after a follow-up of 14 months. |
A 61-year-old female presented to our department with a seven-year-history of involuntary movements of the abdominal wall. The movements were initially intermittent. However, they gradually evolved over time to occur more frequently. There was no pain but some discomfort was accompanying the movements. The movements were not voluntarily suppressible and disappeared during sleep. The rate and the amplitude of the involuntary movements were increased by stress. Five years prior to presentation, she underwent cholecystectomy, hysterectomy, and oophorectomy. One year later she was diagnosed with depression and irritable bowel syndrome. She was treated with lorazepam, fluoxetine, chlorodiazepoxide, and clidinium bromide. The patient is a chronic smoker with chronic obstructive pulmonary disease. She had no family history of movement disorders.\nHer neurological examination was unremarkable. Semirhythmic writhing wavy movements involving abdominal wall and paravertebral muscles producing displacement of umbilicus were observed. These movements occurred exclusively in the sitting and standing positions, and abolished on lying on her sides and significantly decreased when lying supine (Additional file 1: video, segment 2). The movements did not show any variation in intensity or frequency during respiration.\nLaboratory, radiological, and fluoroscopic investigations were normal similarly to case I. Abdominal ultrasound depicted fatty liver. A diagnosis of belly dancer’s dyskinesia was made and she was started on trihexylphenydil and clonazepam, but without noticeable benefit.\nUtilizing the same aseptic technique and under ultrasound guidance, a total of 240 units of BTX was injected into the rectus abdominus muscles bilaterally, in which 40 units/site in a total of 6 sites were administered. The patient reported marked improvement and she was able to sit on the chair and walk independently 3 weeks after the injections with no side effects. She has been on regular follow ups every 6 months, to which she received BTX reinjection over a period of 48 months. On her last follow up, the patient documented maintained therapeutic effects of the injections with no adverse events. |
A Forty five-year-old previously healthy female presented with complaints of swelling and pain in bilateral feet for eight years and history of recurrent episodes of multiple nodules over bilateral legs and feet, which burst to form ulcers for a period of seven years. There was no history of any preceding drug intake. There was no history of similar complaints in family members and no history suggestive of systemic illness. On examination, there was pitting pedal edema, multiple ulcers over bilateral lower limbs (one over medial malleolus of right foot and two over lateral aspect of the left foot) measuring about 1-2 cm in size, slightly tender, irregular margins, sloping edges, floor of the ulcers were covered with slough with erythema and hyperpigmentation of the surrounding skin. Multiple scars of previously healed wounds were present [Figure and ]. There was no palpable thrill or bruit, no clinical evidence of varicose veins and no significant lymphadenopathy. All the peripheral pulsations were well felt. Systemic examination was within normal limits. Routine investigations including complete blood counts, liver and renal function tests, urinalysis, chest radiographs were within normal limits. Enzyme linked immunosorbent assay (ELISA) for HIV 1 and 2 was non reactive. The result of Mantoux test was negative. Bleeding time, clotting time and prothrombin time were within normal limits. Color Doppler study of bilateral lower limbs was within normal limits. Based on the history and investigations, differential diagnosis of chromoblastomycosis, mycetoma, vasculitis and erythema induratum was kept. Histopathological examination of a biopsy sample from the edge of the lesion showed hyperplastic epidermis with an increased number of thick walled capillaries in the papillary, mid and reticular dermis. Moderate amount of hemosiderin deposits were seen around these vessels with perivascular lymphocytic infiltrate and mucin []. Based on the history, examination and histopathology findings, a final diagnosis of Acroangiodermatitis of Mali was made. |
A 35-year-old woman in her third pregnancy was referred to our tertiary institution at 27-week gestation for investigation of unexplained fetal hydrops. She had originally presented to her family practitioner with bilateral pedal edema and hypertension. Her obstetric history is significant for one previous uninvestigated male stillbirth followed by an uncomplicated pregnancy. She has chronic hypertension treated with labetalol. The woman and her partner are both of East Indian ethnic background and there is no history of consanguinity.\nObstetric ultrasound assessment showed severe fetal hydrops with increased abdominal circumference due to ascites and elevated Middle Cerebral Artery Peak Systolic Velocity. Subsequent amniocentesis and maternal blood tests excluded common chromosomal and infectious causes of fetal anemia. Cordocentesis confirmed fetal anemia (hemoglobin level 46 g/dL) and thrombocytopenia (platelet count: 9 × 109/L). The white cell count was normal (13.5 × 109/L). Two intrauterine blood transfusions were administered in an attempt to improve fetal hemoglobin levels. However, no significant improvement in the anemia or hydrops was observed. Intrauterine fetal death occurred at 28 weeks and 3 days.\nParental consent for partial autopsy examination that excluded the brain was obtained. Post mortem weight of 1650 g (normal range 1144.3 ± 163.8 g) was higher than expected at 28 weeks and is attributable to the anasarca. The abdominal circumference was also increased and all other external fetal measurements were within normal range for stated gestational age. External examination showed subtle facial anomalies including a high arched palate with no clefting, flat broad nose with hypertelorism, and rounded face. Elbow and knee joints were stiff with fixed flexion deformities and pterygia on the flexor surfaces. There was no collodion or ichthyotic changes of the edematous skin. Internal autopsy examination showed 30 mls of pleural fluid bilaterally together with smaller amounts of pericardial and peritoneal effusions. The lungs and kidney weights were below the 5th percentile for gestational age with a lung: body weight ratio of 0.01 (pulmonary hypoplasia). The liver was average sized at 64.0 g and the spleen was enlarged 18.4 g (1.32–4.10 g). All other internal organ weights were within interquartile range for 28-week gestation.\nMicroscopic examination of the liver, spleen, bone marrow, and intra-abdominal lymph nodes showed infiltration by mono- and multinuclear histiocytic cells containing ample cytoplasm with delicate fibrillary appearance (). The cells displaced native hematopoietic cells in these organs and were CD68 positive on immunohistochemistry. The intracytoplasmic material was PAS-D and luxol fast blue (LFB) positive. Alcian blue was negative. Attempt at electron microscopy was unsuccessful due to marked post mortem degenerative change of the Gaucher cells. Histopathological sections of the skin showed subcutaneous oedema. Light microscopy of other internal organs examined was normal for stated gestational age.\nPlacental examination showed placentomegaly with villous hydrops, fetal erythroblastosis, and infiltration of villous stroma by aforementioned histiocytic cells (). Hypertrophic decidual vasculopathy, attributable to the chronic maternal hypertension, was also demonstrated. Post mortem skeletal survey excluded skeletal dysplasia, microbiology studies were negative, and cytogenetics analysis using Affymetrix CytoScan™ 750 kb array showed a normal male karyotype with no copy number aberration.\nFetal DNA extracted from cord blood, fetal liver, thymus, and spleen, together with parental DNA, was submitted. Targeted exome sequencing of 88 genes associated with fetal hydrops and lysosomal storage disorders was performed. Next generation sequence analysis was performed using the 4813 gene Illumina TruSight One™ panel on the Illumina NextSeq (R) Sequencing System™. Variant calls were analysed using Variant Studio v2.2. Variants with MAF > 1% in population databases and variants found previously in unrelated individuals were excluded from the analysis. Interpretation of variant pathogenicity was based on the American College of Medical Genetics and Genomics (ACMG) and Association for Clinical Genetic Science (ACGS) guidelines.\nThis analysis revealed that the fetus was homozygous for the RecNcil recombinant mutation ([c.1448T>C;1483G>C;1497G>C]) in the GBA (glucocerebrosidase) gene. Sanger sequencing was used to confirm the presence of this pathogenic variant and testing of the parents showed that both were heterozygous for the same variant. A diagnosis of Gaucher disease was confirmed on correlation with autopsy findings. Testing of the parents showed that both were heterozygous for the same variant. |
A 48-year-old female patient was diagnosed with ileocolonic CD after experiencing months of abdominal discomfort, loose stools and bloating. Her initial colonoscopy demonstrated an inflamed intestinal mucosa with a cobblestone appearance and longitudinal ulcerations throughout the colon extending into the terminal ileum with rectal sparing. Based on these findings she was commenced on oral prednisolone. Her symptoms improved initially however upon weaning the prednisolone, she developed recurrence of symptoms. She was subsequently commenced on 6-mercaptopurine 25 mg daily and allopurinol 100 mg daily. Despite therapeutic thiopurine metabolites and 3 months of therapy, she continued to have abdominal pain and loose stools, opening her bowels 5 to 6 times a day, there was no rectal bleeding.\nThere was no family history of IBD, no prior use of NSAID, oral contraceptive pill or illicit drugs and no recent travel overseas. She was a current smoker with no other risk factors for cardiovascular disease and had a normal BMI. She did not display any extraintestinal manifestations of CD on history or physical examination.\nShe underwent a second colonoscopy which showed mild patchy colitis throughout the colon with rectal sparing (). Biopsy revealed patchy mild active chronic colitis. There were rare small clusters of histiocytes without well-formed granulomata ().\nFollowing this, she had an MRI of the small bowel to assess the extent of disease. This demonstrated a 25 cm segment of distal and terminal ileum with bowel wall thickening and enhancement. There was no proximal dilatation. A decision was made to commence adalimumab.\nTwo weeks later and just prior to the commencement of anti-TNF therapy, she presented to the local emergency department with severe abdominal pain, vomiting, and watery diarrhea. On examination, she was hemodynamically stable, afebrile and her abdomen was soft with a mildly tender epigastrium but no signs of peritonism. Blood tests revealed anemia with a hemoglobin 113 g/L, a mild leukocytosis 14.2×109/L, CRP 6 mg/L, ALT 23 IU/L, GGT 31 IU/L, ALP 81 IU/L, albumin 36 g/L, lactate 1 mmol/L, and lipase of 394 IU/L.\nAn initial CT scan of the abdomen and pelvis did not show any evidence of colitis or bowel obstruction. However, the spleen was heterogenous and showed features consistent with splenic infarction ().\nOn day 2 of her admission, she developed worsening abdominal pain and rectal bleeding with no relief from opioid analgesia. A thrombophilia and vasculitis screen did not identify a cause and she was treated empirically with therapeutic enoxaparin. By day 4, she had on-going pain refractory to opioid analgesia so a CT angiogram was performed which revealed a nearly occlusive thrombus within the superior mesenteric artery and diffusely thickened loops of small bowel suggestive of ischemia, plus focal areas of nonenhancement within the liver suggestive of hepatic infarction (). There were no features to suggest aortitis.\nLactate was 2 mmol/L with a pH of 7.44. White cell count had risen to 18.8×109/L and hemoglobin had dropped to 95 g/L, CRP peaked at 13 mg/L, ALT 179 IU/L, GGT 58 IU/L, ALP 88 IU/L and albumin 16 g/L. Extensive investigation did not show any evidence of an underlying thrombophilia including protein C or S deficiency, antithrombin III, factor V Leiden, antiphospholipid syndrome and JAK2 gene testing. Serial electrocardiography demonstrated sinus rhythm consistently (no atrial fibrillation or other arrhythmias detected) and transesophageal echocardiogram was unremarkable with no evidence of valvular, atrial or ventricular thrombus and no atrial septal defect or cardiac shunt. There were no clinical features of deep venous thrombosis.\nShe was subsequently commenced on a heparin infusion and underwent a laparotomy, which revealed a dilated, ischemic gallbladder, ischemic caecum, and distal small bowel. An embolectomy of the superior mesenteric artery was performed and a resection of small and large bowel starting 140 cm from the pylorus to the hepatic flexure leaving 140 cm of viable small bowel remaining. She was commenced on intravenous antibiotics and 2 days later had a repeat laparotomy showing healthy small and large bowel and a small bowel to transverse colon anastomosis was performed.\nHistopathology from the resected specimens was consistent with ischemic colitis (). Her postoperative course was uncomplicated apart from on-going diarrhea likely secondary to short gut syndrome. She required a short period of total parenteral nutrition. Her prednisolone was gradually weaned and long-term warfarin therapy was commenced. |
A 59-year-old morbidly obese lady with a history of essential hypertension for ten years and hypothyroidism was referred to the urology department because of an incidental finding of a huge left suprarenal mass on the abdominal CT scan. The patient had no other complaints or clinical signs that may be attributed to adrenal (medullary or cortical) overactivity, and the physical examination had no abnormality. Laboratory tests, including urine tests for 24-hour fractionated metanephrine, were within normal limits.\nPre- and postcontrast abdominal CT scans () revealed a large heterogeneous solid left adrenal gland mass with internal calcifications and central necrosis. Postcontrast showed peripheral nodular discontinuous enhancement (; B + D). The mass caused a significant mass effect on the left kidney, which is pushed inferiorly.\nThe mass was removed through an open approach via a supraumbilical midline incision. It was well-circumscribed and has no signs of local invasion to the adjacent structures. The gross pathological examination showed an intact mass covered by a smooth and glistening capsule, measuring 16 × 15 × 10 cm and weighing 1.73 kg. On cut-section, the mass showed cystic areas with extensive hemorrhage and yellow areas, suggestive of necrosis. The residual adrenal gland could be identified ().\nOn histological examination, the specimen consisted mainly of infarcted, necrotic tissue with cyst-like vascular spaces that were expanded due to collection of blood and were lined by a single layer of mature endothelial cells, without atypia or mitosis. The stroma showed extensive fibrinous material in which many Liesegangs rings were observed. The rings were assembled in a lamellar fashion similar to psammoma bodies or a parasite egg (commonly, kidney worm). With an amorphous material in the middle, the central core was surrounded by a two-layered wall that stained negative with PAS and von Kossa (for calcium). The annular structure was nonbirefringent when viewed under polarized-light microscopy, and on immunohistochemistry, the cystic lining tested positive for CD34 and CD31 (Figures and ).\nThe excision surgery was uneventful with no postoperative complications. The patient stayed in the hospital for three days postoperatively without any untoward incident. The six-month follow-up CT scan of the abdomen and pelvis showed no signs of any distant metastasis or recurrence. |
In April 2016, a 44-year-old premenopausal woman was admitted to the Breast Surgery Department, the Second Hospital of Dalian Medical University, with a 12-month history of a huge lump in the right breast. Initially, the patient just had a small fist-sized lump in the right breast, without any pain or discomfort. It grew so slowly that she did not pay much attention on it. Then, the lump started to grow rapidly and became very large within 2 months, and her right breast was entirely covered with a 8-inch ball-sized mass. The breast skin appeared dark and had ulceration.\nThe patient had undergone breast lump resection twice in 2001 and 2008, and both pathological diagnoses were benign PT. The patient had no history of cardiovascular or respiratory disease. Moreover, she had no family history of breast cancer.\nPhysical examination revealed that the left breast was normal, without any palpable lump. The right breast was obviously large, nearly 25×25 cm in size, with a circumference of nearly 80 cm. The skin on the surface of the lump was dark, with ulceration area of 7×5 cm and without bleeding or discharge (). On palpation, the size of the lump was 18×20 cm, hard with local tenderness and poor mobility. Axillary lymph nodes were not palpable. Mammography showed a huge right breast, about 23×23 cm, with dermal ulceration around nipple (). Ultrasonoscopy showed the right mammary gland that was enlarged obviously, with inset showing echo intensity disorder and blood flow signal probed (). Several hypoechoic focuses were seen on the area of right axilla, of which the largest was 1.5×0.9 cm in size. The left breast and axilla had no abnormal signal. Biochemical examination revealed no significant abnormalities. The chest computed tomography (CT) revealed that the lump had not broken into the chest cavity, and the lungs were normal (). The patient’s other relevant examinations such as the abdomen CT, the brain CT, and the bone ECT were also normal.\nNeedle core biopsy of the breast lump was suggestive of PT of the borderline subgroup. Then, the patient underwent mastectomy of the right breast () and sentinel lymph node biopsy. During surgery, surgeons detected that the pectoralis major was invaded, and so the partial muscle adhered to the tumor was resected. The intraoperative frozen section showed no metastatic cancer cell in sentinel lymph nodes. Finally, the latissimus dorsi muscle flap was graft to remedy for the tissular defect on the chest. Postoperative treatment was uneventful, and she was discharged after 6 days.\nThe cut surface demonstrated a leaf-like pattern. The scope of dermatic ulceration or necrosis was 13×10 cm. Postoperative paraffin-based histopathology showed fast cellular proliferation, mitosis about 9/10 high-power fields (HPFs) (). The tumor did not invade the skin. Immunohistochemistry (IHC) was as follows: spindle cells AE1/AE3, smooth muscle actin (SMA) (+), Desmin (−), CD34 partial (+), BCL-2 (−), p53 partial (+), ki-67 15%, tumor cells, estrogen receptor (ER) <1% weakly positive, progesterone receptor (PR) <1% weakly positive, and Ki67 40% ().\nThe patient had recovered well after the surgery until March 2017. The patient came to our hospital again because of a local recurrence. Her palpation and imaging examinations indicated three large masses on the right chest, the largest one of about 6.7×4.0 cm (). Then, she had lumpectomy, and the tumor was diagnosed as malignant PT (). After the second surgery, we followed up her by telephone, and knew that she had underwent radiotherapy and chemotherapy. Up until now (February 2018), she is still alive and undergoing chemotherapy. |
This female patient is now 47 years old and works as a fitness instructor. She is married and has two children and has no family history of psychiatric disorder. She presented to Accident and Emergency with florid symptoms of mania necessitating admission to a locked psychiatric ward under the Mental Health Act. She was disinhibited, garrulous, with flight of ideas, sleep disturbance and grandiosity. During her stay she sustained an injury to her leg while jumping off a bed.\nTwo days prior to her presentation she had been administered intramuscularly the gonadotrophin-releasing hormone antagonist (GnRH) analogue leuproreline acetate. This was to reduce the vascularity of endometrial fibroids in preparation for surgery.\nThe only past psychiatric history of note was a very similar presentation of florid mania 14 years previously necessitating admission to a psychiatric hospital under the Mental Health Act. This was 5 days postpartum after her second child and was described as a puerperal psychosis, and from which she fully recovered and had no need for long-term psychiatric support or medication.\nThe patient was stabilized on Semi Sodium Valproate 500 mg twice a day and Olanzapine 17.5 mg daily and had been discharged after 8 weeks euthymic. She subsequently went on to have a hysterectomy with preservation of the ovaries, and was discharged from psychiatric follow-up 2 years later.\nFive years later she was re-referred from her general practitioner (GP). She had noticed early warning signs and thought she may be relapsing. She also noticed racing thoughts and an inability to concentrate and had some sleep disturbance. She was also suffering from hot sweats and was biochemically menopausal. She was recommenced on Semi Sodium Valproate and decided after discussion with her GP to commence hormone replacement therapy (HRT). She has subsequently made a full recovery, and is now back at work and back to her ‘normal self’. |
A 14 year old Chinese boy presented to a district hospital with a 5 day history of non-specific right hypochondriac pain. There was no history of jaundice, fever, anorexia or weight loss. He did not have any other associated symptoms. He had no significant past or family history, and no history of drug intake or allergies. His general physical examination was unremarkable. Yellowish discoloration of skin or sclera, spider naevi and palmar erythema all were absent. Systemic examination revealed massive hepatomegaly. His blood count and liver function tests were normal. Alpha fetoprotein was also normal. Ultrasonography revealed a large mass in the right lobe of the liver. He was referred to our hospital for further management. Contrast enhanced CT Scan revealed a large, hypodense mass of 14 × 15 × 15 cm in the right lobe of the liver. (Figure ). Exploratory laparotomy was performed and revealed a large mass in the right lobe of the liver and part of the left lobe with ruptured capsule and the ruptured part adhered to pleura. Tumor resection was performed and about 70% of the total liver was resected. Pathologic review of the specimen revealed an 1150 gm right hepatic lobe and part of the left hepatic lobe with a 14 × 15 × 15 cm tumor mass. The histological examination showed malignant sarcomatous tissue with giant neoplastic cells and residual hepatocytes suggestive of Undifferentiated Liver Sarcoma. (Figure ). Sarcomatous tissue with severe atypia of the neoplastic cells and focal presence of giant cells, partially with myoblastic characteristics, was also present. Immunohistochemical staining was positive for Phosphoenolpyruvate Carboxy Kinase (PCK), Vimentin and Alpha 1 Antitrypsin and negative for Epithelial Membrane Antigen (EMA).\nOur patient's postoperative recovery was uneventful. He was given 6 cycles of adjuvant chemotherapy, AIM Regimen (Adriamycin, Ifosfamide and Mesna), which was well tolerated. A post-chemotherapy CT scan showed no signs of recurrence and our patient is alive and well 6 months after surgery. |
D.N. is a 75-year-old Caucasian man with a history of malignant melanoma of the left shoulder resected in 2011, who presented with progressive dysphagia. An upper endoscopy revealed a gastric mass, and biopsy specimens demonstrated a poorly differentiated malignant neoplasm. Staging CT of the chest, abdomen, and pelvis demonstrated numerous pulmonary nodules bilaterally and scattered liver lesions consistent with metastatic disease. Biopsy of a liver lesion confirmed metastatic melanoma, negative for BRAF mutations. A brain MRI was negative for intracranial involvement. Pertinent medical history included acetylcholine receptor (AChR) autoantibody positive myasthenia gravis (MG) diagnosed at age 64. At that time, he had primarily ocular symptoms and AChR binding antibodies were positive at a titer of 2.1 nmol/L (normal range = 0.0-0.4 nmol/L). Over the years, his MG progressed and he developed other symptoms such as dysphagia, chewing fatigue, dyspnea on exertion, and neck and limb weakness. He responded well to prednisone and was eventually transitioned to mycophenolate mofetil. Just before the metastatic melanoma diagnosis, his MG was asymptomatic with mycophenolate mofetil 750 mg daily and pyridostigmine 60 mg 3 times daily.\nFor lack of a more robust treatment option, and after discussing the potential side effects with the patient and the neurologist, pembrolizumab was started at 2 mg/kg every 3 weeks. The dose of mycophenolate mofetil was decreased to 500 mg daily and pyridostigmine was continued unchanged. After his second dose, the patient developed symptoms of diplopia and worsening bilateral ptosis concerning for an exacerbation of MG. Pembrolizumab was held, and pyridostigmine was titrated up to 180 mg 3 times daily, along with prednisone 30 mg daily. The patient’s condition continued to deteriorate, and he was admitted to the intensive care unit with respiratory distress and worsening dysphagia, where he required noninvasive ventilation and gastrostomy tube placement. The patient’s AChR binding antibody titer was measured during his hospitalization and was elevated at 0.77 nmol/L (normal range <0.02 nmol/L).\nHis MG symptoms improved with 7 treatments of plasma exchange, 2 doses of intravenous immunoglobulin, and 4 doses of rituximab. On discharge from the intensive care unit, the patient underwent a restaging PET/CT scan, which showed resolving lung nodules, consistent with a partial response. Due to the grade 4 toxicity, pembrolizumab therapy was not resumed. Temozolomide was started at 75 mg/m2 daily. The patient was readmitted 1 month later for pneumonia secondary to aspiration and Escherichia coli bacteremia. He quickly succumbed to the infection. |
A 62-year-old woman presented a pulsatile mass on the right side of the neck with thrill and murmur. Except for being a smoker for a long time clinical history was unremarkable. CT angiography confirmed a saccular aneurysm at the middle third of the extracranial internal carotid; larger diameter was 3 cm; no significant stenosis was detected.\nEndovascular treatment was planned. The procedure was carried out under general anesthesia and systemic anticoagulation (nonfractioned heparin). Right femoral access was used. Angiography of the aortic arch and selective catheterization of its branches excluded significant stenosis and intracranial aneurysms.\nA vertebral catheter and a hydrophilic guidewire were placed at the distal right internal carotid; there materials were exchanged for an Amplatzer guidewire and an 8-French sheath to enhance support.\nUnder road map and using a 0.018-inch guidewire a 5 mm × 2.5 cm Viabahn stent graft (Gore®) was deployed sealing the aneurysmal neck; no protection device for distal embolization was used. Control angiography showed persistence of blood flows inside the aneurysm because of an intense leak directly through the stent graft coating. After 15 minutes a control angiography showed no diminishment of the leak intensity (a video showing the leak is available as Supplementary Material, available online at ).\nThe only stent graft available was a Viabahn 5 mm × 5 cm. A second 0.018-inch guidewire was placed in parallel to first guidewire in order to enhance support for the second stent graft navigation. Under road map this second stent was deployed inside the previously implanted one. demonstrates the preoperative CT and intraoperative angiographies.\nControl angiography showed complete aneurysm exclusion, patency of the carotid artery, and no signs of distal embolization.\nAfter the procedure patient received 200 mg of aspirin and 300 mg of clopidogrel.\nPatient remained in the ICU for 24 hours and was discharged after 48 hours.\nAspirin (100 mg/day), clopidogrel (75 mg/day), and atorvastatin (40 mg/day) were prescribed for continuous use.\nPatient has been followed up for 18 months; she gave up smoking and the neck bulging disappeared four months after surgery. Duplex scan after 3 months and angio-CT after 6 months and 12 months showed persistence of aneurysm exclusion and carotid artery patency with minimum signs of intimal hyperplasia. shows the immediate angiographic control after the second stent deployment and the control after one year by CT. |
A 29-year-old woman presented with superior visual field defect of 30 minutes’ duration in her left eye which had suddenly appeared after swimming.\nOn ocular examination, visual acuity was 20/20 and 20/32 in her right and left eyes, respectively. A weak relative afferent papillary defect was identified on the affected side. No remarkable finding was observed in the anterior segment of either eye. Funduscopic examination revealed a calcified white embolus at the first bifurcation of the inferior temporal artery of the left retina. Except for mild retinal edema in the left eye, no other ocular abnormality was noted.\nWith a diagnosis of BRAO, the patient was treated with oral acetazolamide, topical timolol, ocular massage, followed by anterior chamber paracentesis. Visual field loss partially recovered within 15 minutes after initiation of treatment, meanwhile the embolus moved to the third bifurcation level.\nVisual field testing (Humphrey Field Analyzer; Carl Zeiss, Jena, Germany) was performed two days after the onset of symptoms and disclosed a superior visual field defect in the left eye (). At the same time, fluorescein angiography depicted no embolus and delayed filling of the affected artery ().\nAn initial review of the patient’s medical history revealed no systemic disease. The patient underwent an extensive diagnostic work-up consisting of neurology, rheumatology, cardiology, and hematology consultation, carotid color Doppler ultrasound evaluation, and complete laboratory testing to determine the source of embolization. These studies however, could not identify the cause of the condition. Accordingly, further echocardiographic studies were performed in search of a probable cardiac source which identified a small PFO which was 1 to 2 mm in diameter (A). TEE illustrated abnormal passage of agitated saline microbubbles through the PFO; following peripheral administration of agitated saline, about 10 micro-bubbles were detected to pass from the right to the left atrium via the PFO with Valsalva maneuver (B). The inter- atrial septum was not aneurysmal and all other tests were normal.\nSubsequently, the patient received long- term oral aspirin and developed no other embolic event over a two-year follow-up course. |
A 50-year-old obese ex-smoker male suffers from chronic uncontrolled type two diabetes mellitus and dyslipidemia for more than 10 years. He presented to the emergency department (ED) with a progressive, painless right-eye decrease in vision over the previous two weeks. He primarily complained of worsening central vision and color vision of the affected eye. At presentation, he had a bilateral faint maculopapular rash covering both palms and soles (Figures , ) and had a mucus patch on the inside of his left cheek (Figure ) and a genital skin rash over his right testicle (Figure ). He also had onychomadesis in some nails on both hands (Figure ). He denied any other relevant symptoms like fever, headaches, or red eyes. He reported a single event of unprotected casual sexual encounter during a trip to Indonesia 11 months before his ED presentation.\nOn physical examination, his vital signs were within normal limits. A complete neurological examination was unremarkable; however, an ophthalmological examination revealed some interesting findings that are summarized in Table .\nThere were no signs of uveitis or any inflammation. The fundus examination is shown in Figure . Fundus fluorescein angiography (FFA) showed leakage in the right optic disc only. The patient was started on Ceftriaxone 2 g IV once daily for four days till benzylpenicillin became available, for which he received 2.4 g (4 megaunits) IV every four hours for 10 days. Adjunctive therapy with high-dose steroids was started with tapering over two weeks with adjustments of his diabetic control medications. One month after discharge, he reported no improvement of his vision but complete resolution of the rashes all over his body. At three months, there was a slight improvement in his visual acuity of the right eye, but dilated fundus examination showed a pale right optic disc (Figure ). Five months after discharge, he did suffer another episode of painless loss of vision of the left eye that was sudden and not associated with other signs or symptoms. At a one-year follow-up, there were no significant changes in his ophthalmological examination (Figure ).\nBrain CT and MRI revealed no enhancement in the post-contrast images, including the spine. The optic nerves were unremarkable bilaterally with no enhancement in the fat-suppressed images at any part of the nerve. Visual evoked potentials (VEP) showed absent NPN complexes in the right eye while the left eye had deformed NPN complexes with low amplitude and P100 latency at 109 ms. The cerebrospinal fluid (CSF) analysis and other investigations were suggestive of secondary syphilis with ocular/neurosyphilis, as shown in Table . A biopsy was taken from the rash on his palm showing hypergranulosis with band-like inflammation and saw-tooth rete ridges, supporting the diagnosis of secondary syphilis.\nUnfortunately, due to the COVID-19 pandemic, he was reluctant to visit the hospital and presented to the hospital after two months. There were no rashes during his physical examination nor any new neurological symptoms (Figure ). Brain MRI was repeated; it was unremarkable. This time, VEP revealed absent NPN complexes in both eyes.\nA neuro-ophthalmologist evaluated the patient; the final diagnosis was NA-AION (Figure ). Behavioral counseling and management of other controllable risk factors such as smoking and diabetes were explained to the patient to minimize/prevent disease progression. |
A-25-year-old female presented to the emergency department with history of palpitations, sweating, syncope and breathlessness of sudden onset. There was history of intermittent similar episodes of breathlessness and palpitations over the last two years at rest. There was no history of drug intake or joint pain. There was no history of chest pain, hypertension or diabetes mellitus. The patient was previously diagnosed as hypertrophic cardiomyopathy by transthoracic echocardiography six months prior to admission. There was no history of significant medical illness.\nOn examination the pulse rate was 200 beats per minute, respiratory rate was 24 per minute, and blood pressure was 80/ 60 mmHg. The patient was immediately shifted to the coronary care unit for further management. The oxygen saturation (SpO2) was 77%. On auscultation, the heart sounds were soft and there was gallop rhythm with fine rales at bases of both lung fields. JVP was raised with mild hepatomegaly and mild pedal edema. Electrocardiogram showed sustained ventricular tachycardia with rate of 230/ minute. Chest X-ray showed an enlarged cardiac shadow and pulmonary congestion. Laboratory investigations: Hemoglobin was 11.4 gm%. The total WBC count was 7800 with normal differentiation. Renal and liver functions were within normal limits. Serum electrolytes (sodium, potassium, calcium and magnesium) were within normal range. Arterial blood gas analysis (ABGA) was suggestive of mild respiratory acidosis.\nHospital course: Ventricular tachycardia was treated with emergency electrical cardioversion. Considering the hemodynamic instability and life threatening arrhythmia with respiratory insufficiency, the patient was intubated and put on artificial ventilator. Supportive treatment was given in the form of inotrophic agents, antibiotics and proton pump inhibitors. After two hours, the patient was weaned from the ventilator and extubated.\nAfter stabilization, the patient underwent transthoracic Doppler echocardiographic examination, with findings suggestive of isolated left ventricular non-compaction cardiomyopathy. The overall LVEF by Simpson's method was 55%. There was grade I diastolic dysfunction by Pulse Wave Doppler and tissue Doppler imaging. There was mild mitral regurgitation and mild tricuspid regurgitation. Pulmonary artery pressure (PAP) was normal (14.5 mmHg).\nA 2-layered structure of the left ventricular wall with an end-systolic ratio of the noncompacted to the compacted layer was >2. The segments involved in left ventricular non-compaction were the left ventricular (LV) apex. (LV) lateral wall, anterior wall and mid-ventricular areas. The direct blood flow was from the ventricular cavity into the deep intertrabecular recesses, as assessed by color Doppler echocardiography with six prominent trabaculae. There was severe concentric type LV hypertrophy (LVPW: 17.2 mm and IVSd: 17.1 mm).\nColor Doppler was used to establish the continuity of flow between the chamber and the intertrabecular recesses and to evaluate the distribution of the prominent trabeculae in the left ventricle. There was no resting regional wall motion abnormality (RWMA), no vegetations, no clot and no pericardial effusion. The great arteries were normal. Interatrial septum (IAS) and interventricular septum (IVS) were intact. There were no associated cardiac abnormalities. [Figures –].\nThe patient was put on amiodarone and diuretic treatment. After seven days of hospitalization the patient was discharged and advised to continue amiodarone, ACE inhibitors and diuretic. The prognosis, complications, and need of cardioverter-defibrillation implantation was explained to the patient. We also advised echocardiography screening of first relatives considering the familial association of IVNC. |
Subsets and Splits
No community queries yet
The top public SQL queries from the community will appear here once available.