__________________________ E xtrahepatic portal venous obstruction (EHPVO) is a common cause of portal hypertension in the developing countries, and constitutes up to 40% of all patients with portal hypertension. 1 2 EHPVO is a common cause of major upper-gastrointestinal bleeding among children.2-4 The most common presentation in children is well-tolerated variceal bleeding and splenomegaly. In adults, EHPVO is often recognised when evaluating for other disorders or with uncommon presentations such as jaundice, pruritus, acute cholecystitis-like syndrome, ascites and so on, resulting from prolonged portal hypertension. [5][6][7] The portal vein in EHPVO is transformed into a cavernoma, which is a bunch of multiple collateral veins around the obstructed portion of portal vein (fig 1). Marked improvements in the management of variceal bleeding in patients with EHPVO have resulted in an improved survival, thus presenting with unusual symptoms in adulthood.The reasons for EHPVO are obscure in approximately half of the patients. Omphalitis and intraabdominal sepsis are the common causes in neonates and children. Adults develop EHPVO due to increased blood coagulability, local inflammation, intra-abdominal sepsis, myeloproliferative disorders, underlying cirrhosis, or tumours in the liver, bile ducts or pancreas. 7-10Gibson et al 11 first reported the relationship between EHPVO and jaundice in 1965. Since then, several cases of obstructive jaundice due to common bile duct (CBD) obstruction caused by cavernomatous transformation of portal vein (portal cavernoma) have been described. Williams et al 12 were the first to report cholangiographic changes caused by choledochal varices. We, for the first time, describe abnormalities on endoscopic retrograde cholangiography (ERC) in a prospective study.13 These abnormalities were similar to those of primary sclerosing cholangitis and the term ''pseudosclerosing cholangitis'' was then coined to describe them. However, unlike primary sclerosing cholangitis, biliary strictures in patients with EHPVO were smooth rather than irregular (figs 2, 3A). Since then, several case series have described biliary abnormalities among patients with portal hypertension on ERC.14-22

BACKGROUNDPrimary hyperparathyroidism (PHPT) has a variable clinical expression. Symptomatic PHPT is still the predominant form of the disease in many parts of the world, especially developing countries. Because the clinical profile of the disease has changed from that described in the past, we sought to improve our understanding of the disease in patients in north India.METHODSWe summarized the clinical presentation, biochemical and radiological features, and operative findings from the case records from the last 13 years of 52 patients at a tertiary care centre in north India who had documented PHPT.RESULTSThe male: female ratio was 1: 3.3 with ages ranging from 6 to 60 years (mean±SD, 36.38±12.73). Bone disease (46%), recurrent renal stones (21%) and body aches and pains (21%) were the most common modes of presentation. The lag time varied ranged from 1 month to 16 years. Common clinical manifestations included bone pain (67%), weakness/fatigue (56%), fracture of the long bones (48%), abdominal pain (39%), polyuria (37%) and psychiatric manifestations (23.1%). Hypertension was observed in 42% and a palpable nodule in the neck in 19%. Biochemical features included hypercalcemia (86.5%), hypophosphatemia (65.4%) and hyperphosphatasia (67.3%). Mean intact PTH (±SD) was 809.0±696.3 ng/L with levels significantly lower in patients who had only kidney stone disease as compared with those with bone disease (P=0.017). A single parathyroid adenoma was localized in 50 (98%) patients. Hungry bone disease was seen in 59% patients.CONCLUSIONPHPT in India continues to be a symptomatic disorder with skeletal and renal manifestations at a much younger age.

Portal cavernoma cholangiopathy (PCC) is defined as abnormalities in the extrahepatic biliary system including the cystic duct and gallbladder with or without abnormalities in the 1st and 2nd generation biliary ducts in a patient with portal cavernoma. Presence of a portal cavernoma, typical cholangiographic changes on endoscopic or magnetic resonance cholangiography and the absence of other causes of these biliary changes like bile duct injury, primary sclerosing cholangitis, cholangiocarcinoma etc are mandatory to arrive a diagnosis. Compression by porto-portal collateral veins involving the paracholedochal and epicholedochal venous plexuses and cholecystic veins and ischemic insult due to deficient portal blood supply or prolonged compression by collaterals bring about biliary changes. While the former are reversible after porto-systemic shunt surgery, the latter are not. Majority of the patients with PCC are asymptomatic and approximately 21% are symptomatic. Symptoms in PCC could be in the form of long standing jaundice due to chronic cholestasis, or biliary pain with or without cholangitis due to biliary stones. Endoscopic retrograde cholangiography has no diagnostic role because it is invasive and is associated with risk of complications, hence it is reserved for therapeutic procedures. Magnetic resonance cholangiography and portovenography is a noninvasive and comprehensive imaging technique, and is the modality of choice for mapping of the biliary and vascular abnormalities in these patients. PCC is a progressive condition and symptoms develop late in the course of portal hypertension only in patients with severe or advanced changes of cholangiopathy. Asymptomatic patients with PCC do not require any treatment. Treatment of symptomatic PCC can be approached in a phased manner, coping first with biliary clearance by nasobiliary or biliary stent placement for acute cholangitis and endoscopic biliary sphincterotomy for biliary stone removal; second, with portal decompression by creating portosystemic shunt; and third, with persistent biliary obstruction by performing second-stage biliary drainage surgery such as hepaticojejunostomy or choledochoduodenostomy. Patients with symptomatic PCC have good prognosis after successful endoscopic biliary drainage and after successful shunt surgery. ( J CLIN EXP HEPATOL 2014;4:S2-S14)

The patterns of presentations, diagnosis and management of gall bladder perforation are changing. But there is scope for improvement.

Among the 3 imaging techniques tested simultaneously, FCH PET/CT was superior for accurate preoperative localization of parathyroid adenomas, especially for ectopic or small parathyroid lesions.

There does not appear to be a significant difference in the rates of pancreatic fistula following either method of reconstruction. However, performance of an isolated Roux loop pancreaticojejunostomy entails a prolongation of operative time and more intraoperative requirement of blood transfusions.

Ligation and excision of pseudoaneurysms without revascularization is safe for drug addicts provided it is based on the presence of a postligation Doppler signal.

Internal pancreatic fistulas should be treated initially non-operatively; if this is not effective, operative therapy should be considered without delay.