Open AccessUnusual Clinical Course for Untreated Malformative Biliary Atresia Infant
Author(s) -
Di Dato Fabiola,
Ranucci Giulia,
Ville de Goyet Jean,
Alberti Daniele,
Iorio Raffaele
Publication year - 2021
Publication title -
journal of pediatric gastroenterology and nutrition
Language(s) - English
Resource type - Journals
eISSN - 1536-4801
pISSN - 0277-2116
DOI - 10.1097/mpg.0000000000002932
Subject(s) - medicine , biliary atresia , portal hypertension , ascites , cirrhosis , congenital hepatic fibrosis , biliary cirrhosis , gastroenterology , liver function , portal venous pressure , surgery , liver transplantation , disease , transplantation , autoimmune disease
In biliary atresia, infants left untreated, and in those with unsuccessful porto‐enterostomy, hepatic condition and function worsen rapidly towards cirrhosis, malnutrition, portal hypertension with ascites, and variceal haemorrhage; many die within the first 3 years of life unless they benefit from liver replacement. We describe a girl with biliary atresia splenic malformation syndrome, who had portal vein cavernoma and microsplenia; she did not undergo porto‐enterostomy. She survived with her native liver over the age of 3 years. Remarkably, she remained in satisfactory condition in absence of ascites or severe hepatic dysfunction, when 4 other similar patients—managed during the same period of time—all had the usual clinical deterioration and ascites, with the need for liver replacement. To our knowledge, there is no similar report in literature. Possible pathogenetic mechanisms and the role of portal hypertension as important factors are discussed.