Idiopathic non-cirrhotic intrahepatic portal hypertension: common cause of cryptogenic intrahepatic portal hypertension in a Southern Indian tertiary hospital.

Background and aim Patients with intrahepatic portal hypertension and negative etiological work-up for liver disease are often labeled as having cryptogenic cirrhosis. The aim of this study was to evaluate causes of liver disease in patients with unexplained intrahepatic portal hypertension. Methods We retrospectively analyzed cause of liver disease in all patients with cryptogenic intrahepatic portal hypertension who underwent liver biopsies between June 2005 to June 2007 in our center. Results Five hundred and seventeen patients underwent liver biopsies of whom 227 had portal hypertension. Of these, the cause of liver disease could not be detected prior to liver biopsy in 62 patients. Causes of liver disease identified after liver biopsy in these 62 patients were: idiopathic non-cirrhotic intrahepatic portal hypertension (NCIPH) (30 patients, 48 ), cirrhosis (14), fatty liver disease (7) and other causes (11). Initial presentations in idiopathic NCIPH patients were splenomegaly and anemia (18 patients), variceal bleed (9) and ascites (3). Median age (range) of patients at first presentation was 32 (15-57) years, and 19 were male. Majority (90 ) were in Child’s class A. Hepatic vein pressure gradient was <5 mmHg in 2 of 7 NCIPH patients tested. Conclusions We identified 30 patients with idiopathic NCIPH at our center over the 2 year study period. The clinical presentation and investigations of NCIPH closely mimic cryptogenic cirrhosis. Idiopathic NCIPH should be considered as a differential diagnosis of cryptogenic cirrhosis in India.

Changing profile of Budd Chiari syndrome in India.

There is increasing evidence that Budd Chiari syndrome occurs when acquired predisposing factor(s) affect a susceptible individual with one or more underlying thrombophilic conditions. Geographical variations in disease pattern of Budd Chiari syndrome exist, which may reflect differing predisposing factors. We review a change in disease profile of Budd Chiari syndrome in India over the past three decades. While earlier studies from India reported isolated inferior vena cava (IVC) obstruction as the commonest disease type, this is a minority in more recent reports where a combination of IVC and hepatic vein obstruction is the commonest type. Longer duration of illness has been shown to be associated with IVC obstruction and the recent change in disease profile in India may reflect earlier diagnosis of Budd Chiari syndrome. Poverty, malnutrition, recurrent bacterial infections and filariasis have been previously suggested as predisposing factors for IVC obstruction. Improvement in hygiene and sanitation may partly explain the recent change in disease profile of Budd Chiari syndrome in India.

Isolated fourth ventricular cysticercus cyst: MR imaging in 4 cases with short literature review.

We describe the magnetic resonance imaging (MRI) signal characteristics of isolated (solitary lesion) intra fourth ventricular cysticercus cyst in 4 patients who clinically presented with obstructive hydrocephalus. All patients had routine MRI sequences (T1, T2, & proton density-weighted imaging), Fluid Attenuation Inversion Recovery (FLAIR), and post-gadolinium imaging followed by cerebrospinal fluid (CSF) flow study. It revealed a CSF signal intensity (on all pulse sequences), intra fourth ventricular cyst with a nidus (scolex), and wall enhancement. On T1-weighted and FLAIR images, the cyst wall and nidus (scolex) were seen in 3 cases, which were not seen in other routine sequences. The CSF flow study showed the intraluminal nature of the cyst. The MRI features suspected a cysticercus cyst, and per-operative findings and histopathological examination confirmed the diagnosis. The review of literature of the intra fourth ventricular cyst is briefly discussed.

Giant splenic artery mycotic aneurysm presenting with massive hematemesis.

We report a 40-year-old man with rheumatic heart disease who presented with abdominal pain for three weeks and hematemesis for 24 hours. CT scan showed a large splenic artery aneurysm without evidence of pancreatitis. Mycotic aneurysm due to infective endocarditis was considered and confirmed by echocardiogram, which showed aortic and mitral valve regurgitation and vegetations. He was managed successfully with coil embolization of the aneurysm and antibiotics.

Vesical varix in cirrhosis of liver.

Vesical varices in portal hypertension are rare. We report a patient with portal hypertension who developed recurrent painless hematuria. Cystoscopy was normal. Doppler ultrasound and MR angiography showed a dilated paraumbilical vein within the falciform ligament coursing down to the urinary bladder wall and draining into the right internal iliac vein. He underwent liver transplantation for decompensated chronic liver disease. He is in good health and has not had further episodes of hematuria.