The efficacy of surgical shunts to treat severe portal hypertension after a Kasai procedure for biliary atresia

Abstract Background To assess the outcome of patients with biliary atresia (BA) who underwent a surgical shunt (SS) for severe portal hypertension (PH) following a Kasai procedure. Methods We collected and analyzed the data and outcomes of patients with BA who underwent SS for severe PH following a Kasai procedure between 1974 and 2014, focusing on complications related to the procedure, overall survival (OS), and transplant-free survival (TFS). Results SS was performed at a median age of 5.5 years [2–13.5] in 38 patients. Conjugated bilirubin level (cBL) was ≤ 20 μmol/l in 24 patients at time of SS. Median follow-up was 15 years [1–32]. OS at 5 and 10 years was 91% and 87% respectively. TFS at 5 and 10 years was 84% and 70% respectively. Long-term complications included hepatic encephalopathy in 9 patients, and hepatopulmonary syndrome in 3. At last follow-up, 10/14 patients without LT and 18/ 24 who had a delayed LT at a median delay of 11 years [1.5–22] were alive. Conclusion Surgical shunt for severe portal hypertension in biliary atresia may delay the need for liver transplantation. However complications are indications for transplantation. Level of evidence Type of study: Therapeutic. Level of evidence III.