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Outcome of surgery for biliary atresia
S F Dorney1, A W Middleton, H C Martin
1Department of Gastroenterology, Children's Hospital, Camperdown, New South Wales, Australia.
Insights
Early hepatic portoenterostomy for biliary atresia significantly improves long-term survival and jaundice-free outcomes. Timely surgery, ideally before 120 days, is crucial for successful treatment and normal development.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Background:
- Biliary atresia is a severe neonatal liver disease requiring surgical intervention.
- Hepatic portoenterostomy is a primary surgical procedure for biliary atresia.
- Outcomes for biliary atresia surgery vary, with early intervention being a key factor.
Purpose of the Study:
- To evaluate the long-term outcomes of hepatic portoenterostomy in infants with biliary atresia.
- To determine the impact of age at surgery on survival and jaundice resolution.
- To assess the role of liver transplantation in relation to portoenterostomy.
Main Methods:
- Retrospective analysis of 47 infants with biliary atresia who underwent hepatic portoenterostomy between 1971 and 1987.
- Data collection included age at surgery, survival rates, jaundice status, and long-term follow-up.
- Correlation of surgical timing with patient outcomes.
Main Results:
- 55% of patients survived 1-17 years post-surgery, with 45% achieving jaundice-free status.
- Optimal outcomes were observed in patients operated on before 120 days of age (60% survival).
- Patients operated on after 125 days had a mortality rate of 100%.
Conclusions:
- Early referral and timely hepatic portoenterostomy are critical for achieving long-term survival in biliary atresia.
- A majority of patients can achieve normal lives without liver transplantation if treated early.
- Liver transplantation should be considered secondary to portoenterostomy, except in specific late-presenting cases.
Abstract:
Forty-seven infants (26 male, 21 female) with biliary atresia under- went hepatic portoenterostomy during the 16-year period 1971-87. Twenty-six patients (55%) are alive 1-17 years after surgery, with 21 (45%) being jaundice-free. For children who became jaundice-free, the mean age at surgery was 78 days (range: 34-125 days), compared with 97 days (range: 48-224 days) for those who did not. Of 39 patients operated on at less than 120 days of age, 24 (60%) are alive. All four patients operated on after 125 days of life died. Of 31 patients operated on more than 5 years ago, 12 (39%) have survived, the oldest being 17 years. Ten (32%) have normal serum bilirubin concentrations, have non-active cirrhosis on liver biopsy, have had normal growth and development, and lead normal lives. The oldest two patients suffered variceal haemorrhage in their teenage years. In our recent experience, 11 of 16 patients (69%) have had complete clearing of jaundice, lead normal lives and do not currently require assessment for liver transplantation. It is believed that early referral of children with biliary atresia to experienced surgical units for portoenterostomy will lead to long-term survival, without the need for liver transplantation in a majority of cases. Liver transplantation should be offered in infancy only after failed portoenterostomy, except for patients presenting after 120 days in whom transplantation may be considered primary therapy.