An unusual presentation of extra-hepatic biliary atresia

J. Rosado, A. R. DeFelice, F. Zapata, R. Arnon, N. Sharma, P. G. Kazlow

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It is believed that extra-hepatic biliary atresia (EHBA) is typically not found in premature infants. In addition, children with EHBA classically present with light-colored, or acholic, stools. The patient, a 32 week gestation, twin A, was delivered via caesarean section. Birth weight was 1170 g. Apgars were 7 and 8. The patient was fed enterally and never received hyperalimentation. The neonatal course was complicated by sepsis, which responded to antibiotic treatment. The infant was noted to have conjugated hyperbilirubinemia (6.0/2.3 mg/dl) on day 2 of life. The direct bilirubin peaked at a level of 9.2/3.7 mg/dl, on day 5 of life, then began to decrease. However, the bilirubin then reached a plateau of 9.3/2.0 mg/dl. Liver function tests were elevated, including alkaline phosphatase 231 u/l, AST 55 u/l, ALT 53 u/l and GGTP 2074 u/l. Work-up for infectious and metabolic etiologies was negative. The patient was then placed on Phenobarbital. An initial DISIDA scan revealed no excretion of isotope into the duodenum. During this entire time, the child continued to produce stools that were greenish-yellow in color. The child was then placed on Actigal, and a repeat DISIDA scan again showed no excretion. A percutaneous liver biopsy, at age 11 weeks, revealed bile ductule proliferation, inflammation, and mild fibrosis. The child was taken to the operating room, where he underwent a Kasai (hepatoportoenterostomy) procedure. This resulted in a fall of the serum bilirubin and an improvement of the liver function tests. EHBA is the result of a progressive, inflammatory process that leads to the obliteration of the biliary tract. Cases in still-births, or in premature infants, are rare. Stools are generally acholic at the time of presentation. The etiology of this condition is unknown. Many theories have been proposed, including infectious, ischemic, immunologic and toxic causes. Our patient was intriguing in that he was premature and maintained pigmented stools throughout the course of his work-up. The Kasai procedure is most successful when performed before 60 days of age. We therefore suggest an aggressive work-up of all infants with neonatal jaundice, even those born prematurely and having pigmented stools, to rule out EHBA as a diagnostic entity.

Original languageEnglish (US)
Pages (from-to)169A
JournalJournal of Investigative Medicine
Issue number2
StatePublished - Feb 1999

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)


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