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Biliary atresia and the polysplenia syndrome
F M Karrer1, R J Hall, J R Lilly
1Department of Surgery, University of Colorado School of Medicine, Denver.
Journal of Pediatric Surgery
|May 1, 1991
Summary
The Kasai operation and liver transplantation can be successful in infants with biliary atresia and polysplenia syndrome. These complex cases have survival rates comparable to non-polysplenia patients, challenging previous poor prognoses.
Area of Science:
- Pediatric Surgery
- Hepatology
- Developmental Biology
Background:
- Biliary atresia is a serious condition in infants.
- Polysplenia syndrome is a complex congenital anomaly.
- Previous beliefs suggested poor outcomes for biliary atresia with polysplenia.
Purpose of the Study:
- To evaluate the efficacy of the Kasai operation and liver transplantation in infants with biliary atresia and polysplenia syndrome.
- To compare survival rates in these patients with those without polysplenia.
Main Methods:
- Retrospective review of 131 infants with biliary atresia from 1975 to 1989.
- Analysis of 16 infants (12%) with polysplenia syndrome components.
- Assessment of surgical outcomes and long-term survival for Kasai operation and liver transplantation.
Main Results:
- 12 of 15 polysplenia patients achieved biliary drainage after surgery.
- Four children survived: two with Kasai operation, two with liver transplantation.
- Actuarial 5-year survival was 44% for polysplenia patients, not significantly different from 48% in non-polysplenia patients.
Conclusions:
- The polysplenia syndrome in biliary atresia likely results from an early embryonic insult.
- Surgical reconstruction (Kasai procedure) and liver transplantation are viable options.
- Congenital anomalies in polysplenia do not preclude successful treatment outcomes.