Keywords:Neonatal cholestasis, Biliary atresia, Kasai portoenterostomy, Liver transplantation
AbstractBiliary atresia (BA) remains a puzzling medico-surgical conditionmore than 100 years after its original description. Itis the most important disease in paediatric hepatology andthe most frequent indication for liver transplantation in childrenand young adults. BA occurs sporadically in around 1/17,000 live births in Europe and North America and thereis no risk of recurrence within the same family. In patho-anatomicalterms, BA is characterised by a progressive ascendingobstruction of the biliary tree which evolves into biliarycirrhosis and end stage chronic liver disease within infancy.Corrective surgery – portoenterostomy or the “Kasai operation”can be effective in up to 50-60% of timely operated children,whilst the others should be considered for early livertransplantation. Medical treatment is important to provideoptimal nutrition during chronic cholestasis and ensure thenormal physical and neurological development of the affectedinfants. Early and precise diagnosis is of critical importancesince delaying surgery may reduce the chances of a successfuloutcome, while unnecessary surgery for unrecognised otherinfantile cholestatic conditions is deleterious. Diagnostic algorithmsvary depending on the local expertise, but mostcentres use expert ultrasonography and percutaneous liverbiopsy, as clinical examination and biochemical findings arenon-specific. Most children with prolonged neonatal conjugatedhyperbilirubinaemia will have pale stools, dark urine, adegree of soft hepatosplenomegaly and mildly elevated transaminases.Acholic stools point strongly in the direction of asurgical problem. In ambiguous cases, direct cholangiography,preferably endoscopic retrograde cholangiopancreatography(ERCP), performed at specialized centres, will be diagnosticand spare unnecessary laparotomy, if not indicated. The aetiologyof BA remains uncertain. Its inflammatory nature is undoubtedand many centres use steroids routinely post-Kasaiportoenterostomy in the hope of reducing the inflammatorycomponent and minimising ensuing fibrosis and progressionto cirrhosis. The scientific evidence for the benefit of steroidsis lacking and there is a lively ongoing research debate aboutfurther means of improving the postoperative outcome includingthe use of steroids at different doses and regimens,and proinflammatory cytokine-blocking agents in the future.
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