Long‐term Outcome of Children With Biliary Atresia Who Were Not Transplanted After the Kasai Operation: >20‐year Experience at a Children's Hospital
- 1 April 2009
- journal article
- Published by Wiley in Journal of Pediatric Gastroenterology and Nutrition
- Vol. 48 (4), 443-450
- https://doi.org/10.1097/mpg.0b013e318189f2d5
Abstract
Sequential strategies combining the Kasai operation as a first-line treatment and liver transplantation as a second-line option, if necessary, have been accepted for patients with biliary atresia (BA). To understand the role of the Kasai operation in the treatment of BA, it is necessary to analyze the long-term outcome of the operation alone and to evaluate the present status of survivors retaining their native livers. A retrospective chart review was carried out for a group of 80 patients who had undergone the Kasai operation between 1970 and 1986 at the Kanagawa Children's Medical Center. The 5-, 10-, and 20-year survival rates of patients with their native livers were 63%, 54%, and 44%, respectively. The survival rates varied significantly depending on the type of BA, age at initial Kasai operation, era of surgery, and surgical method. By age 20, nearly half of the adult survivors had already developed liver cirrhosis and its sequelae. Episodes of cholangitis and gastrointestinal bleeding occurred after 20 years of age in 37% and 17% of the adult patients, respectively, and 20% of the adult patients died of liver failure or underwent living-related partial liver transplantation in their 20s. Five female patients gave birth to a total of 9 children, and 1 male patient fathered a child. Although increasing numbers of patients with BA survive 20 years or more after the Kasai operation, meticulous lifelong postoperative care should be continued for the survivors because of the possibility of hepatic deterioration.This publication has 23 references indexed in Scilit:
- Long‐Term Prognosis of Patients with Biliary Atresia: A 25 Year SummaryJournal of Pediatric Gastroenterology and Nutrition, 2006
- Unraveling the Pathogenesis and Etiology of Biliary AtresiaPediatric Research, 2005
- Outcome in adulthood of biliary atresia: A study of 63 patients who survived for over 20 years with their native liverHepatology, 2005
- Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese biliary atresia registryJournal of Pediatric Surgery, 2003
- Survival patterns in biliary atresia and comparison of quality of life of long-term survivors in Japan and EnglandJournal of Pediatric Surgery, 2001
- Long-term postsurgical outcome of biliary atresiaJournal of Pediatric Surgery, 1999
- Biliary atresia: The King's College Hospital Experience (1974–1995)Journal of Pediatric Surgery, 1997
- Long-Term Survivors in Biliary Atresia. Findings for a 20-year Survival Group.The Tohoku Journal of Experimental Medicine, 1997
- Biliary atresia registry, 1976 to 1989Journal of Pediatric Surgery, 1990
- The therapy of biliary atresia combining the Kasai portoenterostomy with liver transplantation: A single center experienceJournal of Pediatric Surgery, 1990