Elsevier

The Journal of Pediatrics

Volume 148, Issue 4, April 2006, Pages 467-474.e1
The Journal of Pediatrics

Original article
A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000

https://doi.org/10.1016/j.jpeds.2005.12.054Get rights and content

Objective

To determine the prognostic factors and optimal approaches to the diagnosis and management of biliary atresia, the leading indication for liver transplantation in children.

Study design

A retrospective study was performed of all children who underwent hepatoportoenterostomy (HPE) for biliary atresia between 1997 and 2000 at 9 centers in the United States. Outcome at age 24 months was correlated with demographic and clinical parameters.

Results

A total of 104 children underwent HPE; 25% had congenital anomalies, and outcome was worse in those with biliary atresia splenic malformation syndrome. Diagnostic and clinical approaches varied, although specific approaches did not appear to correlate with outcome. The average age at referral was 53 days, and the average age at HPE was 61 days. At age 24 months, 58 children were alive with their native liver, 42 had undergone liver transplantation (37 alive, 5 dead), and 4 had died without undergoing transplantation. Kaplan-Meier analysis of survival without liver transplantation revealed markedly improved survival in children with total bilirubin level < 2 mg/dL at 3 months after HPE (84% vs 16%; P < .0001).

Conclusions

Outcome in the study centers was equivalent to that reported in other countries. Total bilirubin in early follow-up after HPE was highly predictive of outcome. Efforts to improve bile flow after HPE may lead to improved outcome in children with biliary atresia.

Section snippets

Methods

A retrospective study was undertaken to analyze the results of a large cohort of patients with biliary atresia from a diverse group of US institutions with expertise in management of children with liver disease. As a first step, BARC embarked on a retrospective study of the clinical presentation and current management of biliary atresia and the surgical results after HPE. This analysis was particularly important because most of the literature on outcome of HPE from the United States are reports

Associated Congenital Anomalies

Among the 104 patients, 78 (75%) had no congenital anomalies identified, 13 (12.5%) had 1 anomaly, and the remaining 13 (12.5%) had more than 1 anomaly. The most common anomaly was splenic malformation (12 subjects); the second most common was interrupted inferior vena cava (11 subjects). Eleven of the 13 infants with more than 1 anomaly had BASM (1 asplenia, 10 polysplenia). Nine subjects had a cardiac malformation, and 9 had intestinal malrotation. The most common grouping included 7 subjects

Discussion

This study describes a population of 104 infants with biliary atresia diagnosed and treated between 1997 and 2000 at 9 centers participating in the BARC. Thus, the patient population and outcomes are likely to reflect the current standard practice against which future changes in management and clinical trials can be measured. At present, HPE is the only effective therapy for biliary atresia short of liver transplantation. Thus, the findings of most interest were the rate of success of HPE and

References (17)

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Supported by the National Institutes of Health (grants U01DK062436, U01DK062445, U01DK062452, U01DK062453, U01DK062456, U01DK062466, U01DK062481, U01DK062497, U01DK062500, and U01DK062503).

The initial draft of the manuscript was written jointly by Benjamin Shneider, Ronald Sokol, Morton Brown, Barbara Haber, and Peter Whitington. No honorarium, grant, or other form of payment was rendered for the preparation of this manuscript.

A list of Consortium institutions and members is available at www.jpeds.com.

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