Management of biliary atresia in Nigeria: the ongoing challenge.

BACKGROUND

In developed countries, the outlook for patients with biliary atresia has improved remarkably owing to early referral, good diagnostic facilities and the availability of liver transplantation. In Nigeria and most of sub-Saharan Africa, there is little information on the current outlook for these children.

METHODS

A retrospective review of 14 patients with biliary atresia managed from 1991 to 2004 at Ahmadu Bello University Hospital, Zaria.

RESULTS

Six girls and eight boys presented at between 6 and 24 weeks of age (median 16). Seven presented to another hospital with persistent jaundice in the neonatal period. Eleven at presentation already had liver cirrhosis and deranged coagulation and could not have corrective surgery. Three patients aged 8, 10 and 16 weeks had Kasai's porto-enterostomy. Facilities for diagnosis were limited. Ten patients were lost to follow-up and their outcome is unknown. Of the three patients who had porto-enterostomy, one died at 2 years from gastro-enteritis, one from cholangitis after 8 weeks, and one did not recover from anaesthesia. One patient who had a laparotomy and liver biopsy died from peritonitis.

CONCLUSION

Owing to late presentation, delayed referral and difficulties in diagnosis, the outlook for infants with biliary atresia in this environment is poor. A concerted effort to encourage primary practitioners to detect and refer these infants at an earlier age is critical to outcome.