View source for Biliary atresia

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==Background== <!--T:1-->

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[[File:Bilatresia.jpg|thumb|Intraoperative view of complete extrahepatic biliary atresia.]]
*Rare, but leading indication for liver transplantation in children<ref>Mieli-Vergani G1, Vergani D. Biliary atresia. Semin Immunopathol. 2009 Sep;31(3):371-81. PMID: 19533128</ref>
*Exact etiology unclear, but inflammation leads to destruction of intra/extrahepatic bile ducts
*Prognosis ''significantly'' improved by early diagnosis<ref>Wadhwani SI1, Turmelle YP, Nagy R, Lowell J, Dillon P, Shepherd RW. Prolonged neonatal jaundice and the diagnosis of biliary atresia: a single-center analysis of trends in age at diagnosis and outcomes. Pediatrics. 2008 May;121(5):e1438-40. PMID: 18443020</ref>
*Two forms:
**Peri/postnatal (90% of cases), possibly related to infection
**Fetal/embryonic (10%)
***Often with other associated malformations (e.g [[Special:MyLanguage/congenital heart disease|congenital heart disease]], polysplenia or [[Special:MyLanguage/asplenic|asplenia]], malrotation, situs inversus, portal vein or hepatic artery malformations)

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{{Gallbladder disease types}}
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==Clinical Features== <!--T:3-->

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*Presentation often subtle
*[[Special:MyLanguage/Neonatal jaundice|Neonatal jaundice]]
**High index of suspicion for jaundice that persists beyond 1st week of life or includes conjugated [[Special:MyLanguage/hyperbilirubinemia|hyperbilirubinemia]]
*[[Special:MyLanguage/Hepatomegaly|Hepatomegaly]]
*Acholic stools
*Dark urine
*Late findings: [[Special:MyLanguage/failure to thrive (peds)|failure to thrive]], [[Special:MyLanguage/ascites|ascites]], splenomegaly


==Differential Diagnosis== <!--T:5-->

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{{Neonatal jaundice differential diagnosis}}
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==Evaluation== <!--T:6-->

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[[File:PMC3016234 247 2010 1858 Fig3 HTML.png|thumb|Persistent neonatal cholestasis and biopsy-proven biliary atresia in a 1.5-month-old boy. US shows the triangular cord sign (arrow in a) and increased diameter of the hepatic artery (27 mm, arrow in b)]]
*Evaluate for other causes of [[Special:MyLanguage/neonatal jaundice|neonatal jaundice]], [[Special:MyLanguage/liver failure|liver failure]]
*Assess for complications of liver disease (e.g. [[Special:MyLanguage/coagulopathy|coagulopathy]])
*[[Special:MyLanguage/RUQ US|RUQ US]] to screen for other abnormalities (though cannot definitively diagnose biliary atresia<ref>https://www.niddk.nih.gov/health-information/liver-disease/biliary-atresia/diagnosis</ref>)
*Definitive diagnosis made by biopsy or surgical pathology


==Management== <!--T:8-->

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*Surgery
**Kasai procedure or [[Special:MyLanguage/liver transplant|liver transplant]] for those who fail kasai
*[[Special:MyLanguage/Coagulopathy|Coagulopathy]] (if present) responds to [[Special:MyLanguage/vitamin K|vitamin K]] supplementation


==Disposition== <!--T:10-->

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*Admit


==See Also== <!--T:12-->

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*[[Special:MyLanguage/Neonatal jaundice|Neonatal jaundice]]


==External Links== <!--T:14-->


==References== <!--T:15-->

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https://pedemmorsels.com/biliary-atresia/
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[[Category:Pediatrics]] [[Category:GI]] [[Category:Surgery]]
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