BILIARY ATRESIA: FROM 95% MORTALITY TO 94% SURVIVAL

Abstract

4 Twenty years ago biliary atresia (BA) was untreatable with a two year mortality of 95%. Kasai portoenterostomy (PE) and liver transplantation (OLT) have radically improved outcome but their relative roles have been debated. At our centre (KCH) a multidisciplinary team including Paediatricians, Paediatric and Transplant Surgeons provides the complete range of treatment for hepato-biliary disorders. The aim of the study was to document the results of this comprehensive approach to BA. Between 2.12.1992 and 25.1.1995, 50 infants referred to KCH for neonatal cholestasis were found to have BA. PE was performed in all at median age 54 (range 14-120) days. A successful operation was defined as serum bilirubin less than 30umol/l. At median 56 (range 47-73) months, one was lost to follow up at 17 months, 8 have normal liver function tests (LFT) including aspartate amino-transferase and gamma glutamyl transpeptidase and no clinical or ultrasound evidence of port hypertension (PHT), 8 have abnormal LFT with only ultrasound evidence of splenomegaly, 9 have clinical evidence of PHT, 5 have significant PHT of whom 3 have impaired nutrition by arm anthropometry, 2 have serum bilirubin >30umol/1, 3 are listed for OLT, 14 have undergone OLT successfully, all except 1 with normal LFT. One child died after OLT and 2 while listed. Thus with expert surgery PE has 62% 5 yr successful survival rate and actuarial 5 year survival including with OLT is 94%. These results indicate that an integrated service leading to low PE failure rate with reduced need for OLT, less competition for organs and excellent OLT outcome is the optimum management of BA.