Portosystemic shunt for portal hypertension after Kasai operation in patients with biliary atresia. 2021

Toru Shimizu, and Albert Shun, and Gordon Thomas
Children's Hospital at Westmead, Sydney, Australia. toru.shimizu.md@gmail.com.

OBJECTIVE 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. CONCLUSIONS 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.

UI MeSH Term Description Entries
D006975 Hypertension, Portal Abnormal increase of resistance to blood flow within the hepatic PORTAL SYSTEM, frequently seen in LIVER CIRRHOSIS and conditions with obstruction of the PORTAL VEIN. Cruveilhier-Baumgarten Disease,Cruveilhier-Baumgarten Syndrome,Cruveilhier Baumgarten Disease,Cruveilhier Baumgarten Syndrome,Disease, Cruveilhier-Baumgarten,Portal Hypertension,Portal Hypertensions,Syndrome, Cruveilhier-Baumgarten
D008297 Male Males
D011171 Portoenterostomy, Hepatic Operation for BILIARY ATRESIA by anastomosis of the BILE DUCTS into the JEJUNUM or DUODENUM. Hepatic Portoenterostomy,Hepatoportoenterostomy,Kasai Procedure,Hepatic Portoenterostomies,Hepatoportoenterostomies,Portoenterostomies, Hepatic
D011183 Postoperative Complications Pathologic processes that affect patients after a surgical procedure. They may or may not be related to the disease for which the surgery was done, and they may or may not be direct results of the surgery. Complication, Postoperative,Complications, Postoperative,Postoperative Complication
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001656 Biliary Atresia Progressive destruction or the absence of all or part of the extrahepatic BILE DUCTS, resulting in the complete obstruction of BILE flow. Usually, biliary atresia is found in infants and accounts for one third of the neonatal cholestatic JAUNDICE. Atresia, Biliary,Biliary Atresia, Extrahepatic,Biliary Atresia, Intrahepatic,Extrahepatic Biliary Atresia,Familial Extrahepatic Biliary Atresia,Idiopathic Extrahepatic Biliary Atresia,Intrahepatic Biliary Atresia,Atresia, Extrahepatic Biliary,Atresia, Intrahepatic Biliary
D012189 Retrospective Studies Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons. Retrospective Study,Studies, Retrospective,Study, Retrospective
D016896 Treatment Outcome Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series. Rehabilitation Outcome,Treatment Effectiveness,Clinical Effectiveness,Clinical Efficacy,Patient-Relevant Outcome,Treatment Efficacy,Effectiveness, Clinical,Effectiveness, Treatment,Efficacy, Clinical,Efficacy, Treatment,Outcome, Patient-Relevant,Outcome, Rehabilitation,Outcome, Treatment,Outcomes, Patient-Relevant,Patient Relevant Outcome,Patient-Relevant Outcomes

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