Portosystemic shunt for portal hypertension after Kasai operation in patients with biliary atresia
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ORIGINAL ARTICLE
Portosystemic shunt for portal hypertension after Kasai operation in patients with biliary atresia Toru Shimizu1,2 · Albert Shun1 · Gordon Thomas1 Accepted: 22 October 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Purpose Many biliary atresia (BA) patients will eventually develop liver failure even after a successful Kasai portoenterostomy. A common complication of long-term BA survivors with their native liver is problematic portal hypertension. The aim of this study was to defend the view that portosystemic shunts can delay or negate the need for transplantation in these children. Methods A retrospective single center review of the efficacy of portosystemic shunts in BA patients after a successful Kasai portoenterostomy was conducted. Results From 1991 to 2017, 11 patients received portosystemic shunts. Median age of Kasai operation was 48 (36–61) days. Shunts were performed at the median age of 6.2 (4.1–6.8) years. Three of these eleven patients required subsequent liver transplantation. OS at 5 and 10 years were 90.9% and 81.8%, respectively. TFS at 5 and 10 years were 90.9% and 72.7%, respectively. Long-term complications included mild encephalopathy in 2 patients, hypersplenism in 3, and cholestasis in 1. Conclusion Portosystemic shunt for the treatment of portal hypertension in carefully selected BA patients is an effective option in delaying or negating the need for liver transplantation. Keywords Portosystemic shunt · Portal hypertension · Liver transplant · Children · Biliary atresia · Esophageal varices
Introduction Biliary atresia (BA) is an obliterative condition of the bile ducts which may be amendable to treatment with Kasai portoenterostomy with a relatively satisfactory long-term outcome. Unfortunately, most of these patients will eventually develop liver failure and will require a liver transplant. It has been widely known that BA is the most common indication for pediatric liver transplant in Australia, accounting for 54% of cases [1] and other centers worldwide [2]. A common complication of BA after a successful Kasai procedure is the development of portal hypertension (PHT). * Toru Shimizu [email protected] Albert Shun [email protected] Gordon Thomas [email protected] 1
Children’s Hospital at Westmead, Sydney, Australia
Nagano Children’s Hospital, Nagano, Japan
2
Clinical manifestations of PHT include gastric/esophageal/ Roux loop varices that cause gastrointestinal (GI) bleeding. To date, a common method to manage this major complication is to either band or inject the visible varices with a sclerosant via an upper endoscopic procedure. If the repeated endoscopic procedure fails to control the bleeding, the next option in most centers would be to perform a liver transplant. Beta blockers to prevent recurrent bleeding have not been validated to be of reliable therapeutic use in children [3]. Given the potential morbidity and mortality associated with liver transplantation and the shortage of suit
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