Long term results of Kasai portoenterostomy for the management of Biliary Atresia Academic Article uri icon

abstract

  • Biliary Atresia (BA) is a rare disorder. It is the leading cause of cholestasis in the newborn period, and the single most frequent indication for Orthotopic Liver Transplantation (OLT) in children. Early diagnosis of BA is critical for optimal intervention. Our objective in the current study is to compare the results of our center to those reported in the literature, in terms of incidence of BA, age at diagnosis, rates of OLT and long term survival after operation. A secondary objective is to seek differences between the Jewish and non-Jewish population. The study included newborns with BA who were treated in Soroka University Medical Center between the years 1980-2007. The sources of information included medical charts, operation reports, and follow-up tests and visits. The comparison between the results of our study and those accepted in the western world, and between the two populations, was made using proportion test for two independent samples. The long term results were analyzed using the Kaplan Meier method. Overall, 29 infants with BA were treated: 16 were Jewish and 13 non-Jewish; 15 (52%) males and 14 (48%) females. The incidence rate of BA was 0.9 per 10,000 live births. The average age at operation was 58 + 23 days (20 to 115). Fifteen (51%) of the patients are alive today: 10 with their native liver and 5 after OLT. Two children who underwent OLT died 1-3 months after the transplantation. Nine (31%) patients died due to end stage liver disease and its complications, without OLT. Three of the patients were lost to follow-up. The OLT rates in the Jewish population were 31% compared to 15% in the non-Jewish population (p = 0.41). The long term results of our center meet the reported results from other parts of the world, with no significant difference between the two populations.

publication date

  • April 1, 2009