Portal hypertension: An uncommon presentation of Caroli's syndrome.

Portal hypertension is not a classical presentation of Caroli's syndrome. However, some young children can present with overt signs and symptoms indicative of advanced disease state despite the improvement in imaging technology which has decreased its diagnostic age. High index of clinical suspicion can help in timely diagnosis and management.

Recurrent nonbacterial thrombotic endocarditis and stroke on anticoagulation.

We present a rare case of recurrent nonbacterial thrombotic endocarditis (NBTE) and stroke despite anticoagulation. A 48-year-old man with history of antiphospholipid syndrome, prior nonbacterial aortic valve endocarditis status post valve replacement and prior stroke was found to have acute ischemic stroke while on apixaban and nonbacterial thrombotic endocarditis of mitral valve. This was initially managed conservatively with therapeutic dose of enoxaparin, but the patient later underwent mitral valve replacement. Unfortunately, the patient later passed away with hemorrhagic stroke while on enoxaparin.

Clinical Characteristics and Management of the Hydatid Cyst of the Liver: A Study from a Tertiary Care Center in Nepal.

A hydatid cyst of the liver is a significant yet neglected public health problem in Nepal. The present study was carried out to evaluate the demographic characteristics, clinical presentations, and management of the patients of the hydatid cyst of the liver in the setting of a developing country. It was a retrospective, descriptive analysis of 53 patients admitted in the department of surgery with the diagnosis of hydatid cyst of the liver based on clinical manifestations, imaging studies, or serology between 2016 and 2019. The median age of the patients was 36 years, with the age group of 25-45 years being the most commonly affected (23, 43.4%). 58.5% of the patients were female. Abdominal pain (49, 92.5%) and a palpable liver (17, 32.1%) were the most common complaint and physical finding in our study population, respectively. Abdominal ultrasonography and computed tomography scan were the major imaging studies used to establish a diagnosis. A unilocular and anechoic cystic lesion was the most frequent imaging finding. The right lobe of the liver harbored the cysts in the maximum number of patients. Surgery was the preferred modality of treatment (23, 43.4%), with pericystectomy being the most common form of surgical intervention. The hydatid cyst of the liver is a common cause of morbidity in Nepal. Clinical evaluation supplemented by imaging studies is required for diagnosis, and surgery remains the treatment of choice in most cases. To explain the epidemiological pattern of the disease, multicentric studies involving a larger sample of patients should be conducted.

Correction to: Atrial myxoma with cerebellar signs: a case report.

In the publication of this article [1], there is an error in the name of one of the contributing authors.

Atrial myxoma with cerebellar signs: a case report.

BACKGROUND: Atrial myxoma remains a rare clinical entity with an incidence of surgically resected cases of 0.5-0.7 per million population and prevalence of < 5 per 10,000. It typically manifests in woman after third decade of life; symptoms vary greatly and may present with arrhythmia, intracardiac flow obstruction, embolic phenomenon, and associated constitutional symptoms. Neurological complications associated with atrial myxoma most frequently include cerebral infarct due to embolus. Cerebellar involvement is very rare and only a few cases have been reported in the literature. CASE PRESENTATION: A 55-year-old Brahmin man with no history of diabetes mellitus and hypertension, presented with complaints of dizziness, headache, vomiting, double vision, and unsteadiness of gait for 2 weeks. His headache was sudden in onset, of a pulsating type and localized on left temporal side. Vomiting was projectile and bilious. Double vision was present in all directions of gaze and he had uncoordinated movement of his body and tilting to the left side. On examination, his cerebellar functions were impaired. He was thoroughly investigated for the cause of stroke after abnormal magnetic resonance imaging results with normal computed tomography angiography of his brain. Echocardiography and computed tomography of his chest showed a mass attached to intra-atrial septum and prolapsing through mitral valve, which was suggestive of left atrial myxoma. Five days following admission, he developed abdominal pain due to thromboembolism causing splenic and renal infarct. CONCLUSION: Although rare, atrial myxoma has to be considered a cause of stroke and other embolic phenomenon causing multiorgan infarctions. Early and timely diagnosis of the condition can prevent further recurrence and inappropriate anticoagulant therapy. It would be pertinent to have echocardiography done in patients who present with a stroke, arrhythmias, and other constitutional symptoms. The tumor once detected must be removed surgically as early as possible, which not only reduces serious thromboembolic complications but can be potentially curative.

Dystrophic Epidermolysis Bullosa.

Epidermolysis bullosa is a rare inherited blistering disease with an incidence of 8-10 per million live births. Dystrophic epidermolysis bullosa is a type of epidermolysis bullosa caused by mutation in type VII collagen, COL7A1. There are 14 subtypes of dystrophic epidermolysis bullosa and 400 mutations of COL7A1. Electron microscopy is the gold standard diagnostic test but expensive. Immunofluorescence study is a suitable diagnostic alternative. Trauma prevention along with supportive care is the mainstay of therapy. Squamous cell carcinoma develops at an early age in epidermolysis bullosa than other patients, particularly in recessive dystrophic epidermolysis bullosa subtypes. Regular follow-up is imperative in detecting and preventing complications. Gene therapy, cell therapy and bone marrow transplantation are the emerging novel therapeutic innovations. Preventing possible skin and mucosal injury in patients requiring surgery should be worked on. Here, we present a case of dystrophic epidermolysis bullosa in a 26-year-old male. Keywords: blister; dystrophic epidermolysis bullosa; epidermolysis bullosa; knee disarticulation; surgery.