Πέμπτη 9 Αυγούστου 2018

Post-traumatic thrombotic microangiopathy following pelvic fracture treated with transcatheter arterial embolization: a case report

Thrombotic microangiopathy is caused by various conditions, but few cases secondary to trauma have been reported. We present the rare case of a patient with thrombotic microangiopathy-induced high-impact traum...

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A 12-Year-Old Girl with Bilateral Coats Disease and ABCA4 Gene Mutation

A 12-year-old girl with bilateral stage 2B Coats disease was screened meticulously for a possible underlying systemic disease as she was female and the disease was bilateral. Full systemic workout turned out to be unremarkable. However, an ABCA4 gene mutation was found in the genetic analysis. NDP and TINF2 gene mutations were not present. She was successfully treated with a bilateral, single intravitreal injection of dexamethasone implant and a single session of indirect laser photocoagulation with a relatively good anatomic and functional result. To the best of our knowledge, the present case is the only reported case of Coats disease with an ABCA4 gene mutation.
Case Rep Ophthalmol 2018;9:375–380

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Unilateral Multifocal Intraocular Lens Implantation in a Patient with Adie’s Pupil

Purpose: To report a case of a patient with unilateral Adie's pupil who underwent bilateral cataract extraction with multifocal and monofocal posterior chamber intraocular lens (IOL) implantation. Methods: A 74-year-old woman presented to our institution complaining of worsening near vision. Gross examination revealed a 6-mm fixed pupil on the right eye (OD) and a 5-mm pupil reacting to 3 mm with light on the left eye (OS). Slit lamp examination revealed a tonic pupil with an exaggerated pupillary constriction to dilute pilocarpine OD. Dilated exam revealed 2–3+ nuclear and cortical lens changes bilaterally. The patient's active lifestyle, personality, and biometry measurements made her a good candidate for multifocal IOL (MfIOL) implantation OS. Femtosecond laser-assisted cataract extraction with a ReSTOR +3 ADD (SN60D1) implantation was performed OS. Monofocal lens implantation (SN60WF) was performed OD 6 months later. Results: One year postoperatively, our patient had an uncorrected visual acuity (VAsc) of 20/15 for distance and J10 for near OD. Her VAsc was 20/25 +1 for distance and J1 for near OS. Visual acuity when using both eyes was 20/15 for distance and J1 for near. Conclusion: Optimizing success for MfIOL implantation is a multifactorial process. Large pupils preoperatively are of particular concern, as this may lead to increased dysphotopsia with pupil-dependent MfIOLs. Thus, patients with unilateral mydriasis, such as Aide's pupil, may have a beneficial outcome combining multifocal-monofocal implantation after bilateral cataract extraction, especially if they are not a candidate for monovision but desire spectacle independence.
Case Rep Ophthalmol 2018;9:369–374

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Hydatid cyst of the neck mimicking a branchial cleft cyst

Here we report a rare case of primary hydatid cyst with involvement of soft tissue of the neck in a child. A 4-year-old girl presented with gradual swelling of the right side of her neck over a period of several months. The lump was fairly soft and painless with no significant inflammation. There was no other abnormal finding in the physical examination. Imaging was performed by ultrasonography followed by neck CT scan which demonstrated a simple cyst as the cause for the neck bulging. With the presumed diagnosis of a branchial cleft cyst, surgical resection was performed. Hydatid cyst was confirmed in pathology. Isolated soft-tissue hydatid cyst is an uncommon finding which should be considered in the differential diagnosis of soft-tissue cystic lesions especially in endemic regions. This is of more significance in children in whom congenital cysts of head and neck are more common.



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Non-invasive treatment of pyogenic granuloma by using Nd:YAG laser

Pyogenic granuloma is a 'reactive lesion' in the oral cavity caused due to hormonal imbalance or poor oral hygiene. There are different methods to excise this lesion but most successful is treatment with lasers. Currently, there are different lasers available commercially and are used by clinicians to excise this lesion. In this case report, a 20-year-old female patient reported to the department with a complaint of overgrowth of gingiva on lower canine. It was excised by using neodymium-doped yttrium aluminium garnet laser and the patient was asked for regular follow-up after 1 week and 6 months. The excised lesion was sent to pathology where the lesion was confirmed to be pyogenic granuloma. The patient reported no pain, no blood loss during or postsurgery. Laser is a useful technique for excisional surgeries; it is safe, effective and reduces time of treatment as well as time of healing.



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Acupuncture treatment for dysfunctional uterine bleeding in an adolescent

A 17-year-old girl with a history of dysfunctional uterine bleeding (DUB) and severe dysmenorrhoea was treated with different conventional hormonal therapies for 16 months without improvement.

Treatment with traditional Chinese acupuncture was started while she was taking oral contraceptives. She received a total of 27 treatments in 17 weeks. Her menstrual cycle normalised after 4 weeks (10 treatments). She continued to be regular during the rest of treatments and to date, 6 months after the treatment was discontinued. This report summarises the acupuncture treatment for DUB in this adolescent girl.



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Eosinophilic angiocentric fibrosis : a sino-orbital masquerader

A 58-year-old Caucasian male presented with left periorbital oedema extending to the nasal area for 1 year along with nasal discharge for 1 month. Lab work was significant for positive cytoplasmic antineutrophil cytoplasmic antibodies. CT scan showed solid mass along the nasal soft tissue with bony nasal destruction. A CT scan of the thorax was performed to rule out granulomatosis with polyangiitis and showed multiple pulmonary nodules. Biopsies of the nasal mass and lung nodule were performed which showed fragments of fibrosis with spindle cell proliferation consistent with eosinophilic angiocentric fibrosis (EAF). EAF is a very rare disease, recently described as a subtype of immunoglobulin G4-related disease. A few rare cases of EAF involving the structures of the orbit have been reported in the literature.



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Acute serous tamponade after paraesophageal hernia repair reoperation

Cardiac tamponade is a common complication after cardiac surgery and is usually caused by bleeding or thrombus in the early postoperative period. Postoperative serous tamponade is more rare and usually of gradual onset. We report an unusual and life-threatening case of serous tamponade occurring on postoperative day 1 following a third-time paraesophageal hernia repair.



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Arthrogryposis, renal dysfunction and cholestasis (ARC) syndrome: a rare association with high GGT level and absent kidney

We report a case of a term baby presenting with neonatal cholestasis and upper limb flexion deformity on day 4 of life. On further evaluation, high gamma glutamyl transpeptidase (GGT) levels and absent left kidney were found. A diagnosis of arthrogryposis, renal dysfunction and cholestasis (ARC) syndrome was made which is a rare autosomal recessive disorder with primarily clinical diagnosis. Outcome of this condition is dismal. It has a large spectrum of clinical manifestations, but association with high GGT levels and absent kidney is quite rare. No single case report has observed such an association, and this is the first case of ARC syndrome reported from India to the best of our knowledge.



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Adult Henolch-Schonlein purpura: multiorgan failure in the setting of a purpuric rash

We report a 66-year-old man with a history of congestive heart failure, atrial fibrillation on warfarin therapy and chronic kidney disease that presented with acute dyspnoea. He had multiple palpable purpuric lesions on his bilateral lower extremities. Laboratory findings supported acute anaemia with no obvious bleeding source, supratherapeutic international normalised ratio and acute on chronic kidney injury. Oesophogastroduodenoscopy and colonoscopy initially suggested ischaemic colitis. The patient's legs were treated symptomatically with topical steroids. He later developed acute large volume bloody diarrhoea that made him haemodynamically unstable. Punch biopsy of the skin was consistent with leucocytoclastic vasculitis and direct immunofluorescence demonstrated immunoglobulin A and C3 deposits consistent with Henoch-Schonlein purpura. The patient was treated with oral steroids. Bleeding stabilised and rash resolved. Steroids were successfully tapered. The patient was discharged on haemodialysis but ultimately this was able to be discontinued.



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Renal germ cell metastatic tumour with rupture, bleeding and syncope: an unusual clinical presentation in a young patient

The most common malignancy in young men is a germ cell tumour of the testes. Metastatic renal disease is relatively uncommon and usually manifests in the retroperitoneal lymph nodes. Visceral metastases to the liver, and metastases to the lungs and the brain are more common. Few large studies characterising patients with germ cell tumours and associated renal metastases have yet been published. We report an unusual case of successful management, through demolitive surgery, of a 22-year-old male patient affected by spontaneous intrarenal rupture of metastases secondary to testicular cancer. The patient was admitted to our urology department due to recurrent episodes of haematuria and clinical symptoms of persistent hypotension.



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A rare case of incarcerated femoral hernia containing small bowel and appendix

An 81-year-old woman was admitted under the acute medical team with a significant acute kidney injury secondary to presumed gastroenteritis, following a 5-day history of diarrhoea and vomiting. She continued to deteriorate despite resuscitative efforts. Subsequently, a non-contrast CT scan revealed likely small bowel obstruction second to a Richter's hernia in the inguinal canal. At diagnostic laparoscopy, both small bowel and appendix were identified to be incarcerated within the right femoral canal. The patient recovered uneventfully and was safely discharged several days following a laparoscopic appendicectomy and right femoral hernia repair.



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Follow-up of water-only fasting and an exclusively plant food diet in the management of stage IIIa, low-grade follicular lymphoma

Description  

Here we present a 3-year follow-up report of a case that was originally published in BMJ Case Reports in December 2015.1 Briefly, a 42-year-old woman presented to her primary physician with a palpable mass in her right inguinal region and was subsequently diagnosed with stage IIIa, grade 1 follicular lymphoma (FL). In November 2014, the patient arrived to TrueNorth Health Center (TNHC) and elected to undergo a 21-day medically supervised, water-only fast, after which she refed on an exclusively whole plant food diet free of added salt, oil and sugar (SOS-free), including refined carbohydrates, for 10 days. Over the course of treatment, her enlarged lymph nodes became impalpable. Follow-up CT scans confirmed reduction in size. She did not undergo standard cancer treatment, maintained the SOS-free diet, and was symptom-free at 3-month and 6-month follow-up visits.1

In November 2017, the patient returned to TNHC to...



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Hepatic angiosarcoma as a cause of acute liver failure

Hepatic angiosarcoma is an extremely rare disease entity that accounts for approximately 0.1%–2% of primary liver malignancy. It is three times more common in men than women and usually affects the former in their sixth or seventh decade of life. Risk factors for the development of hepatic angiosarcoma include the use of oral contraceptives, exposure to anabolic steroids, radiation, thorium dioxide, arsenic and vinyl chloride. The prognosis of hepatic angiosarcoma is extremely poor which is attributable to early metastases to other organs, resistance to traditional chemotherapy and radiotherapy regimens and rapid progression of the tumour. Optimal management of patients is poorly demarcated due to the rarity of the tumour. We present a case series of two patients: one who passed away due to acute hepatic failure secondary to hepatic angiosarcoma and the second who underwent a liver transplantation and was subsequently diagnosed with hepatic angiosarcoma based on his explant histology.



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Spontaneous coronary artery dissection in a man with intense coughing spasms

Spontaneous coronary artery dissection (SCAD) is a rare cause of acute myocardial infarction of emerging significance, which occurs predominantly in young women without coronary artery disease risk factors. Valsalva-like activities such as coughing have been identified as potential triggers for the development of SCAD. We report a case of SCAD in a man in whom the only identifiable predisposing factor was excessive coughing. He presented with atypical chest pain. Troponin I peaked at 29 ng/mL, and ECG showed no evidence of ischaemic changes. He underwent cardiac catheterisation via the radial approach, which revealed a linear second obtuse marginal dissection. He was managed conservatively with medical therapy with a good outcome.



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Spontaneous ovarian heterotopic pregnancy

Heterotopic pregnancy is a simultaneous intrauterine and ectopic pregnancy. We report a case of a spontaneous ovarian heterotopic pregnancy. A 36-year-old woman, gravida 7 para 4-1-1-5 at 4 weeks gestation (spontaneous conception), presented to the emergency department with vaginal spotting, lower abdominal cramps with human chorionic gonadotropin(hCG) 10 772 mIU/mL (hCG at T0). Abdominal and pelvic examinations were benign. Transvaginal sonogram (TVS) showed an intrauterine gestational sac and yolk sac, no fetal pole visualised. She was discharged home with a diagnosis of threatened abortion. The patient returned to the emergency department 3 weeks later (T1) at 7 weeks gestation with recurrent vaginal bleeding and lower abdominal pain. Her TVS showed an empty uterus with small amount of free fluid in the cul-de-sac. A small 2 cm round mass noted in the adnexa with hCG of 4663 mIU/mL (hCG at T1). Laparoscopy revealed normal fallopian tubes bilaterally and a ruptured right ovarian ectopic pregnancy. Pathology was consistent with ectopic pregnancy. Abnormal hCG patterns should raise suspicion for heterotopic pregnancy.



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Synchronous colon cancer with pulmonary metastasis and follicular variant of papillary thyroid cancer metastasising to kidney

Thyroid malignancies are one of the fastest growing cancers in the world, with the majority being papillary thyroid cancer. Follicular variant of papillary thyroid cancer accounts for about 10%–20% of papillary thyroid carcinomas. The usual sites for metastases of these tumours are lungs and bones with renal metastases being extremely rare. We describe a case of a 64-year-old woman who presented with abdominal pain. On subsequent imaging, she was found to have a colonic mass with metastatic lesions in the lungs and tumour involving left kidney. On biopsy and immunohistochemical staining, the renal mass showed positivity for thyroid cancer markers. Thyroid scan was noted to be negative and the patient was placed on active surveillance after undergoing chemotherapy for colonic adenocarcinoma.



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Innocent PET avidity in patient with metastatic melanoma

Description

A 53-year-old man presented for a surveillance 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT following recent resection of a melanoma of his right leg and metastasis to his right inguinal region. The scan was acquired approximately 60 min after intravenous injection of FDG. The FDG-PET/CT scan demonstrated no FDG uptake in the right inguinal region. However, intense FDG uptake was observed in skeletal muscles, specifically in the left foot (figure 1A) and around the shoulder, bicep and pectoral regions bilaterally (figure 1B,C).

Figure 1

18F-fluorodeoxyglucose positron emission tomography showed intense uptake within the left foot (A) and around the shoulder (B), bicep and pectoral regions bilaterally (C).

FDG-PET/CT is an integral part of modern-day practice in oncology imaging and has been shown to have high sensitivity and specificity for the detection of melanoma metastasis.1 Concerned by the...



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Auto-amputation of penis due to advanced carcinoma penis

Description

A 82-year-old Indian male patient was referred with complaints of the wound in right inguinal region for the last 15 days. He initially presented to a primary care centre with complaints of a progressive ulcer over penis for 12 months and swelling in right inguinal region for the last two months. He also revealed sloughing of penile tissue 1 month back. The discharged card mentioned that the patient received antibiotics and underwent incision/drainage of the inguinal abscess under anaesthesia. He also had difficulty in micturition. The patient denied any history of sexually transmitted disease or local trauma. Clinical examination revealed almost complete loss of phallus. (figure 1). There was evidence of abscess in right inguinal region and hard matted nodes palpable in the left inguinal region. Metastatic workup revealed evidence of distant metastases. Biopsy from the base of residual penile stump revealed squamous cell carcinoma (figure 2). The...



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Peek through the smoke: a report of moyamoya disease in a 32-year-old female patient presenting with ischaemic stroke

Moyamoya disease is a vasculopathy causing chronic progressive stenosis and occlusion of the large arteries of the circle of Willis that could lead to brain ischaemia. The condition may also present with haemorrhagic strokes. This is a case report of moyamoya disease in a 32-year-old woman presenting with ischaemic stroke. The report describes her inpatient stay and investigations and findings.

The report reviews the main aspects of moyamoya disease definition, epidemiology, clinical features, diagnosis, classification and treatment. This case is interesting because her first presentation occurred after 3 months of her second delivery. Whether the different physiological stresses of pregnancy, child birth and puerperium have had some effect in accelerating the pathogenesis of her moyamoya disease remains unknown.1



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Abdominal compartment syndrome secondary to megarectum and megasigmoid

A 31-year-old male patient with chronic constipation of unknown aetiology presented emergently with worsening nausea, vomiting and abdominal distension of one week duration. On examination, his abdomen was distended with minimal tenderness. A plain film of the abdomen demonstrated severe faecal loading. The patient was haemodynamically unstable on admission and appeared sick. An urgent CT abdomen and pelvis was conducted showing extensive rectal dilatation and associated proximal colonic stercoral perforation. The patient proceeded straight to theatre for laparotomy as his general condition was deteriorating rapidly. On transfer to the operating table, the patient suffered cardiopulmonary arrest. Resuscitation was immediately commenced. Abdominal compartment syndrome was suspected. Cardiac output was re-established following a midline laparotomy which acted relieve the abdominal pressure. The rectosigmoid faecal content was decompressed via an enterotomy. The perforated segment of transverse colon was resected and an end colostomy fashioned. A year later, the continuity of the bowel was re-established.



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Spontaneous bone regeneration in a large haemophilic pseudotumour of mandible

Pseudotumours of haemophilia (PTH) are locally expansile destructive haematomas which result in varying morbidity among haemophilic patients. Adequate haematological treatment and prophylaxis helps in preventing these haematomas. Currently, there is no uniform standard management protocol for this entity due to rarity of these lesions. PTH are seen in 1%–2% of the severe haemophilic patients. They may also be seen in moderate cases when adequate factor coverage is not provided or in cases with factor VIII inhibitors. We report a rare case of mandibular pseudotumour in a patient with moderate haemophilia and Glanzmann's thrombasthenia, treated successfully with decompression of the haematoma. Postdecompression, sequential radiography revealed spontaneous bone regeneration at the site of the lesion. With 2 years follow-up, the mandible had no residual lesion. This reveals the role and potential of conservative decompression even in cases with severe osteodestruction secondary to developing haematoma of the mandible in haemophilic patients.



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Delayed Presentation of Isolated Adrenocorticotropin Insufficiency after Nivolumab Therapy for Advanced Non-small-cell lung carcinoma (NSCLC)

We describe a 73-year-old man who developed adrenal insufficiency 7 months after completing nivolumab therapy for advanced non-small cell lung cancer. He presented with non-specific symptoms of malaise and fatigue with an insidious 13.6 kilogram weight loss, prompting an evaluation for disease progression, which was negative. Subsequent evaluation revealed isolated adrenocorticotropin insufficiency as the aetiology, attributed to a delayed side effect of nivolumab therapy.



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Beer anaphylaxis due to coriander as hidden allergen

Beer is one of the most widely consumed alcoholic beverage all over the world. Although the production and consumption of this beverage is diffuse, allergic reactions are very uncommonly reported, mainly due to wheat, yeast or hops allergy. More recently, many foods and drinks have been flavoured with spices, with a reported increase in allergic reactions. We report on a case of a young woman who experienced anaphylaxis due to coriander-flavoured beer. This is the first case of beer anaphylaxis due to coriander, which had been added to the beer as aromatising substance. As the presence of flavours is not always reported, they may be considered hidden allergens, whenever they are the cause of anaphylaxis to foods or beverages, the patient usually tolerated. In conclusion, allergic reactions to spices have to be considered in the patients with 'idiopathic' anaphylaxis induced by common foods, where spices had been hidden.



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Retroperitoneal schwannoma misdiagnosed as an ovarian malignancy

A 55-year-old woman presented to the hospital with abdominal discomfort over 3 months. Contrast-enhanced abdominal CT showed a 10x9x6.5 cm sized well-defined solid-cystic enhancing mass in the right pelvic cavity. Under general anaesthesia, exploratory laparotomy was performed on suspicion of ovarian malignancy. Pathological examination revealed a retroperitoneal schwannoma with highly ordered cellular component (Antoni A) and hypocellularity with predominantly loose myxoid component (Antoni B). On immunohistochemical staining, the sample showed typically positive result for S-100 in the cytoplasm of the tumour cells. Schwannomas are consisting only of Schwann cells and are most often non-malignant tumors. It is relatively slow growing and usually located in the head, neck and the extremities. Schwannomas are quite rare in the retroperitoneal region. The diagnosis of retroperitoneal schwannomas is often delayed and misdiagnosed as an adnexal tumour, especially locating in the pelvic cavity. Surgical complete resection of tumour is the treatment of choice and recurrence is unusual after complete resection.



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Trauma-induced dual vascular lesions in the liver-hepatic pseudoaneurysm with arteriohepatic venous fistula

Description 

A 17-year-old boy presented to the emergency triage with severe abdominal pain following blunt abdominal trauma. The patient was conscious, hypotensive with blood pressure 60/30 mm Hg, pulse rate 110/min and per abdomen examination revealed abdominal distension, tenderness, rigidity and free fluid. FAST (Focused Assessment with Sonography in Trauma) imaging revealed liver lacerations and haemoperitoneum. The patient was initially stabilised with inotropes, intravenous fluids and adequate analgesics and had to be directly taken up for emergency laparotomy as the ideal protocol of performing an emergency CT was forgone due to technical issues with the CT machine. Laparotomy revealed grade V liver injury with active bleeding and haemoperitoneum of about 2 L. Haemostatic agent (AbGel) and multiple intra-abdominal packs were used to stop bleeding, and massive blood transfusion protocol was activated. The patient was transferred to intensive care unit where he improved with conservative treatment. Before removing the intra-abdominal packs by relaparotomy,...



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