Incidence and prognostic factors associated with biliary atresia in western India

To estimate the incidence of biliary atresia(BA) amongst neonatal cholestatic syndromes (NCS) and determine prognostic factors in BA patients who have undergone Kasai's portoenterostomy. Study design- Retrospective analysis.

Pediatric Hepatobiliary Clinic at B.J. Wadia Children's Hospital, Mumbai.

32 patients diagnosed with BA referred to the clinic from May 2005 to July 2007 were included in the study. All patients underwent a detailed history, clinical examination and were tested for liver function tests (LFT), USG abdomen, liver biopsy, intra-operative cholangiogram and CMV tests. Patients were followed up for a period of 1 month to 7 years post operatively and complications such as cholangitis, progress to liver cell failure and cirrhosis was noted.

Incidence of BA amongst NCS (n = 88) was 36.4%. 8 patients of BA (25%) were lost to follow up. Out of the remaining, 10 (41.7%) improved and 14 (58.3%) did not improve. The mean age of presentation was 89 + 55.8 days. 1 patient (25%) out of 4 with bile duct size of < 100 microns showed an improvement whereas 3 (37.5%) out 8 patients with bile duct size 100-200 microns showed improvement and 4 (50%) with bile duct size of > 200 microns had improvement post Kasai surgery. Those with bile duct sizes > 200 microns had better prognosis than those with sizes 100-200 microns (Odd's ratio = 1.8) and < 100 microns (Odd's ratio = 3). 12 patients (50%) were operated before 3 months of age and 50% of them responded to surgery. The remaining 12 patients were operated after 3 months of age and only 33% showed any improvement. (Odd's ratio = 2). Other parameters like SGOT (P = 0.598), SGPT (p = 0.901), total Bilirubin (p = 0.349), Direct Bilirubin (p = 0.429), Alkaline Phosphatase (p = 0.605) and GGTP (p = 0.480), cirrhosis (p = 0.417), degree of fibrosis (p = 0.384), degree of inflammation (p = 0.964) and Cholangitis (P = 0.388) had no effect on the outcome.

Biliary Atresia is a common cause of NCS in India. Children with Bile duct size > 200 microns have a good prognosis. Portoenterostomy before 3 months of age has a better outcome.