Extrahepatic portosystemic venous shunt presenting as galactosaemia

Abstract

A newborn screening program identified a 23-day-old asymptomatic girl with elevated serum galactose (>18.3 mg/dl) after breast feeding. The enzymes that metabolise galactose showed normal activity, thereby excluding hereditary galactosaemias. Three-dimensional CT venography (Fig. 1) showed direct portocaval communication between the inferior mesenteric vein and the left internal iliac vein. Inferior mesenteric venous portography via the shunt confirmed that the flow was hepatofugal through the communication (Fig. 2). Intrahepatic