Biliary Atresia

Conclusion

Between 1953 and 2001, a total of 321 patients with biliary atresia underwent surgery in our hospital. Among them, 43 patients underwent double-valved hepatic portoenterostomy (the valved procedure). Bile drainage after surgery was obtained in 95% of the cases. The incidence of post-operative cholangitis was 56% in the valved procedure. Of the cases treated by this procedure, 49% are still alive without jaundice after hepatic portoenterostomy alone, and 30% are alive after subsequent liver transplantation.

The combination of hepatic portoenterostomy with subsequent liver transplantation is the treatment of choice for patients with biliary atresia. It is important, however, to attempt to keep the patient’s own organ by continuing efforts to achieve the best possible results with hepatic portoenterostomy.

The authors’ current strategy for surgical treatment for patients with this disease include (1) early diagnosis, including prenatal diagnosis, (2) hepatic portoenterostomy without stoma formation, (3) close postoperative care, especially for prevention of postoperative cholangitis, (4) revision of hepatic portoenterosotmy only in selected cases who showed good bile drainage after the first procedure, (5) early liver transplantation in patients with absolutely failed hepatic portoenterostomy, (6) avoidance of laparotomy for the treatment of oesophageal varices and hypersplenism (injection sclerotherpy and splenic artery embolization), and (7) consideration of exploratory laparotomy or primary liver transplantation for patients with advanced liver disease at the time of referral.