Obstructive jaundice caused by cavernous transformation of the portal vein.

Abstract

Extrahepatic portal obstruction developing in the neonatal period usually presents with manifestations of portal hypertension. Obstructive jaundice has not previously been described as a complication of this condition. A 22 year-old white male presented with jaundice, decreased appetite and tiredness for six months. He was known to have portal hypertension which followed neonatal omphalitis and portal vein thrombosis. At age four, splenectomy and lieno-renal shunt was done because of bleeding from oesophageal varices. Two further, self-limiting episodes of gastrointestinal bleeding occurred in his teens. He was normally developed and showed no other abnormalities apart from jaundice. Total bilirubin was 10.4 mgm % with a direct fraction of 7.2 mgm%. Alkaline phosphatase was elevated to 344 K.A. units, but liver function tests were otherwise normal. Liver biopsy was unsuccessful, but was complicated by puncture of the gall-bladder with haemorrhage into it, necessitating laparotomy. The wall of the gallbladder was found to contain varices, one of which was oversewn to control bleeding and a cholecystostomy tube was inserted. Serum bilirubin fell to 3.6 mgm%. Angiography showed oesophageal varices, non-patent lieno-renal shunt, thrombosis of the proximal superior mesenteric vein and cavernous transformation of the portal vein, with varices in the region of the common bile duct (Fig. 1). The patient was re-explored because tube cholecystography revealed marked narrowing and displacement of the distal common bile duct. Varices were found in the region of the common bile ductand also in the gall-bladder wall, with no other lesion. Portal pressure was 45 mg Hg.