Budd-Chiari syndrome associated with congenital afibrinogenaemia reversed after orthotopic liver transplant.

A woman in her mid-20s, a known case of congenital afibrinogenaemia, presented with abdominal pain and distension. She was diagnosed with decompensated liver cirrhosis due to Budd-Chiari syndrome. She underwent deceased donor liver transplantation. Preoperatively, her serum fibrinogen level was undetectable and prothrombin time and international normalised ratio (INR) were unrecordable. Intraoperatively, she was given thromboelastography-guided human fibrinogen concentrate. Postoperatively, her fibrinogen, prothrombin time and INR normalised rapidly. This report summarises the rare occurrence of a complication of hypercoagulability (Budd-Chiari syndrome) in the setting of congenital hypocoagulability (congenital afibrinogenaemia). In this report, we discuss the simultaneous management of these two clinical problems and the curative role of liver transplantation.

Gallbladder Neuroendocrine Tumors: Is There a Need for a Specific Approach?

Neuroendocrine tumors (NETs) of the gallbladder or the biliary tree are rare. Most of the current guidelines and protocols are derived from the experience of managing lung small cell neuroendocrine carcinoma or gastrointestinal NETs. But, the overall outcome of gallbladder NETs (GB-NETs) seems worse than similarly staged lung NETs and adenocarcinoma of the gallbladder. This may be due to its rarity and lack of literature for a focused approach toward its treatment. Hence, the need for a specifically designed approach might help improve results of treatment for these rare tumors. We share our experience of 2 patients with GB-NETs and their 5-year outcome.

Idiopathic CD4 Lymphocytopenia Presenting as Cryptococcal Meningitis.

A 54-year-old male presented to our center with a 3-month history of headache, giddiness, and blurring of vision. Cerebrospinal fluid examination revealed him to be having cryptococcal meningitis. He was worked up for probable causes of immunosuppression including HIV and other infections and had an autoimmune profile as well as a bone marrow examination, none of which revealed any abnormality. Lymphocyte flow cytometry revealed low counts of CD4 T lymphocytes, likely secondary to idiopathic CD4 lymphocytopenia. He was treated for cryptococcal meningitis. Due to marked immunosuppression, the disease progressed rapidly with deterioration in neurological and hemodynamic status, leading to his demise.

Ileal cavernous haemangioma in an adult presenting as a rare cause of small bowel obstruction.

Haemangiomas of the small bowel are a very rare entity and rarely considered as an aetiology for an intestinal obstruction. Contrast-enhanced CT is the investigation of choice but the lesion can be confused with malignancy or rarely a neuroendocrine tumour. Commonly it presents as abdominal pain with anaemia and/or melaena. With patients presenting without obstruction or acute gastrointestinal bleed, capsule endoscopy has shown to be a useful diagnostic tool.We present here our experience of managing a case of a patient with ileal haemangioma who presented with a subacute small bowel obstruction and underwent a laparoscopic-assisted ileal segmental resection with side-to-side anastomosis. The lesion was a cavernous haemangioma on histopathological analysis.

Handlebar sign: a bruise with underlying visceral injury.

A 14-year-old boy presented with a painful swelling topped by a bruise overlying the skin of the right inguinal region without peritonitis. This was the area of impact of bicycle handlebar while riding 6 days ago. On contrast-enhanced CT scan, we found a traumatic abdominal wall hernia (handlebar hernia) near the right deep ring without any solid organ, bowel or urinary bladder injury. Inguinal exploration revealed a defect in transversus abdominis and internal oblique muscle which was repaired and meshplasty was done.Delayed presentation and ignorance towards 'handlebar sign' is associated with visceral injury (haematoma/perforation) will incur the risk of rising morbidity and mortality. With CT scan we can assess the abdominal cavity to rule out associated visceral or vascular injury. Surgical repair for restoring disrupted anatomy with or without meshplasty is the preferred approach.

Dysautonomia and hyponatraemia as harbingers of Guillain-Barre syndrome.

A 56-year-old woman with a medical history of hypertension presented to our hospital with back pain, abdominal pain, vomiting and elevated blood pressure. The laboratory parameters including evaluation for secondary hypertension were within normal ranges at the time of presentation. During her hospitalisation, fluctuations in her blood pressure and pulse were observed which were attributed to autonomic disturbances, the cause of which was unknown. On the seventh day after presentation to the hospital, the patient developed focal seizures and slurred speech which was believed to be secondary to hyponatraemia detected at that time. Hyponatraemia improved with hypertonic saline and she experienced no further seizures. On the eighth day of her admission, she developed acute flaccid paralysis of all her limbs and respiratory distress. We concluded this to be secondary to Guillain-Barre syndrome (GBS). She responded to plasmapheresis.The presence of dysautonomia and hyponatraemia before the onset of paralysis makes this a rare presentation of GBS.

'Little old lady's hernia' (obturator hernia): A deceptive encounter.

Obturator hernia (OH) is rare which not only carries high mortality amongst all abdominal hernia, but also known for the difficulty in diagnosing it. Howship-Romberg sign is a clinical sign to diagnose OH, but due to the lower-limb muscle contractures, it was not possible in our case. Computed tomography scan becomes the investigation of choice in this situation. A laparoscopic approach can be used safely.

Successful treatment of extensive splanchnic vein thrombosis in a patient with mycosis fungoides.

A 33-year-old man of a Middle Eastern origin presented to us with abdominal pain and distension secondary to refractory ascites of 1-month duration. The patient had a history of taking oral retinoic acid 25 mg for 4 months for mycosis fungoides. Investigations revealed thrombosis of hepatic veins with extensive thrombosis of the porto-mesenteric axis. A combination of transjugular intrahepatic portosystemic shunt, balloon angioplasty and thrombolysis with recombinant tissue plasminogen activator was successfully used to treat his condition.

Left-sided sinistroposition of the gall bladder: incidental presentation during elective cholecystectomy.

A 59-year-old male patient presented with mild gallstone pancreatitis. He underwent laparoscopic cholecystectomy during the same admission, where we encountered a left-sided gall bladder (GB). This was managed during laparoscopic surgery by modifying the laparoscopic port positions, and we did not encounter any other variations in the biliary anatomy. Thorough knowledge regarding anatomical variations of the GB will help in managing rare cases and avoid injuries to vital structures.

Primary hepatic hemangioendothelioma in a patient with Budd-Chiari syndrome.

A 36-year-old woman was diagnosed with compensated cirrhosis of liver secondary to Budd-Chiari syndrome (BCS) and had undergone stenting of a thrombosed left hepatic vein. Eight months later, she presented with jaundice and right upper quadrant pain. CT revealed multiple focal lesions in the liver, which on biopsy proved to be hepatic hemangioendothelioma (HHE). Her liver disease and ascites progressively increased. Four months later, magnetic resonance cholangiopancreatography showed an advanced stage of HHE with infiltration of the common bile duct and vascular invasion with a blocked stent, with metastasis to the spleen and dorsolumbar vertebrae. We believe this is the first reported case of an HHE developing in the background of BCS.