Patterns of Cholangiographic Changes in Patients With Symptomatic Portal Cavernoma Cholangiopathy

Abstract

the long-term outcome of patients undergoing radiological intervention for the treatment of BCS. Methods: 32 children diagnosed with BCS between 2005 and 2014 were included. Retrospective data on the course of disease, response to intervention, complications and outcome of the disease was collected. Results: Details of 25 patients who had a regular follow up were analysed in this study. The median age at presentation was 9 months (4.5–214). The median follow up was 44 months (5–132). Sixteen patients initially received anticoagulation alone. It was associated with high failure rate of 66%. Twenty patients underwent radiological intervention. Failure rate with angioplasty was 57%. Hepatic vein stenting was successful in 66%. TIPS was needed in 14 patients. Immediate complications were seen in four patients (28.5%). TIPS patency rate at 2 years was 75%. Five of the 14 patients required interventions in form of restenting or revision. Four patients developed hepatopulmonary syndrome after a median period of 3 years (1.5–5.25) and 1 developed a hepatocellular carcinoma. TIPS decreased portal hypertension and hepatic dysfunction and improved child growth in all patients. Based on our experience we propose an algorithm for management of pediatric BCS. Conclusions: BCS commonly presents during infancy. Anticoagulation alone and angioplasty of the hepatic veins are associated with high failure rate. Hepatic vein stenting or TIPS is feasible and efficacious in improving liver function, portal hypertension and growth. It is associated with good long term outcome and delays the need for liver transplantation, but may not prevent complications like hepatopulmonary syndrome and hepatocellular carcinoma.