Bile Leakage after Liver Transplantation Owing to Stricture of Afferent Jejunal Loop Caused by an Intussusception Valve after Biliary Atresia Surgery: A Case Report.

introductionBiliary atresia (BA) is a progressive cholangiopathy in neonates that results in biliary cirrhosis and liver failure without early intervention. Hepatic portoenterostomy (Kasai operation) remains the standard treatment, significantly improving survival rates. However, postoperative cholangitis is a major determinant of prognosis. To prevent cholangitis, various surgical modifications, including anti-reflux procedures such as intussusception anti-reflux valves (IAV), have been introduced. Although IAV has been widely adopted, some reports suggest that long-term survivors may develop afferent jejunal limb stenosis, leading to complications such as cholangitis and intestinal obstruction. Herein, we report a case of afferent jejunal loop stricture caused by IAV, which became symptomatic after liver transplantation (LT). CASE PRESENTATION: A 34-year-old man with a history of BA underwent Kasai operation with IAV and spur valve at 77 days of age. Despite experiencing recurrent cholangitis in adulthood, he survived with his native liver until developing liver cirrhosis and porto-pulmonary hypertension, necessitating deceased donor LT. Preoperative imaging revealed portal vein obstruction and dilated collateral circulation. During LT, severe adhesions and afferent limb dilation were observed, requiring a 30 cm resection of the jejunal limb. Postoperatively, he developed cholangitis, and imaging on postoperative day 16 revealed an anastomotic leak with an intra-abdominal abscess. Retrospectively, CT image before LT demonstrated the dilatation of the afferent limb and the stricture due to IAV was highly suspected. Double-balloon endoscopy confirmed complete afferent limb obstruction due to IAV-related stenosis. Surgical reconstruction with resection of the obstructed Roux-en-Y limb and creation of a new hepatojejunal anastomosis was performed. The patient recovered well and was discharged on postoperative day 45 without further complications.

conclusionsThis case highlights the possibility for late-onset afferent jejunal stricture due to IAV in BA patients undergoing LT. The narrowing likely results from long-term fibrotic changes after 34 years from BA operation. Given the increasing number of BA survivors receiving LT, awareness of IAV-related complications is crucial. In cases with suspected afferent limb stenosis, preoperative assessment and consideration of jejunal limb resection during LT may help prevent postoperative complications.