Diagnosis and follow‐up of congenital intrahepatic portosystemic venous shunt by ultrasounds

Abstract

Intrahepatic portosystemic venous shunt (IPSVS) is mostly observed in adults in association with portal hypertension or cirrhosis and diagnosed by angiography. The congenital form of IPSVS occurs less frequently, without primary liver disease, and can lead to hepatic encephalopathy(HE). Usually, small portosystemic shunts, less than 2 mms. in size, are present in patients with cirrhosis, while the largest ones have a congenital origin. The etiology of the congenital form is controversial, but it is likely due to an incomplete fetal development of vitellin veins that give rise to portal venous system. Literature reports that in infants the disease is usually discovered casually either through routine metabolic screening for galactosaemia (1) or through follow up of a cystic liver detected by prenatal sonogram (2). With improvements in diagnostic imaging techniques, an increasing number of infants and children with asymptomatic IPSVS has been diagnosed. To date, the natural course and indications regarding surgical closure of the shunt have still not been well established (3). Now we are reporting a case of a 5-month-old infant admitted to our department for poor weight gain and diarrhoea. Physical examination and laboratory data including liver function tests were normal. Only lactose tolerance test showed lactose maldigestion. Abdomen ultrasonography showed an anechoic lesion in the anterior segment of the right lobe of the liver that was supplied by a branch of the right portal vein and drained by an enlarged branch of the right hepatic vein, without evident