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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.
Insights
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.
Abstract:
There is a widely held but unsubstantiated belief that in infants with biliary atresia and coexisting polysplenia syndrome, the Kasai operation fails. An equally poor prognosis has been forecast for patients with this complex treated by liver transplantation. From 1975 to 1989, 16 of 131 infants with biliary atresia (12%) had one or more components of the polysplenia syndrome: polysplenia (8), intestinal malrotation (12), preduodenal portal vein (6), absent inferior vena cava (6), aberrant hepatic artery (4), or abdominal heterotaxia (4). Although technically more demanding, 12 of the 15 polysplenia patients achieved biliary drainage after operation. (One patient had exploration only, because of parental preference). Four children are alive; two are anicteric and well at ages 5 and 8 following Kasai's operation, and two by virtue of liver transplantation done at ages 4 and 7. Three of the five patients who had liver transplantation died. Acturial survival was 44% at 5 years, not significantly different from that of the 115 nonpolysplenia patients (48%). When associated with the constellation of anomalies known as the polysplenia syndrome, biliary atresia is most likely caused by an early (at approximately the fifth week) embryonic insult. The anomalies do not preclude successful biliary reconstruction using the Kasai procedure or successful liver transplantation.