Extrahepatic portal vein thrombosis is associated with an increased incidence of cholelithiasis.

BACKGROUND/PURPOSE: The effect of portal flow deprivation to the liver on bile composition and the biliary system remains undefined in children. This report catalogues the authors' experience with biliary tract problems in children with extrahepatic portal vein thrombosis (EHPVT).

METHODS

Twenty-nine children with symptomatic idiopathic EHPVT were evaluated for the Rex shunt procedure (mesenterico-left portal bypass) over a 4-year period. The authors retrospectively reviewed all operative reports and pre- and postoperative abdominal ultrasound findings with regard to associated congenital anomalies and abnormal biliary tract findings.

RESULTS

Seven of the 29 patients with EHPVT (24%) had associated nonbiliary congenital abnormalities. Twenty-four of 29 (83%) patients had detectable biliary tract pathology by ultrasound examination. Biliary symptoms developed in 3 of the 9 (33%) patients with either stones or sludge (10.3% of all patients). Two patients were treated by cholecystectomy. There was no statistical correlation between biliary tract pathology and the age of presentation, symptoms of portal hypertension, gender, or underlying medical condition.

CONCLUSIONS

The authors have noted a high incidence of biliary tract pathology in patients with EHPVT compared with the normal population and a 10% incidence of symptomatic biliary pathology in this series.