Does "cystic" biliary atresia represent a distinct clinical and etiological subgroup? A series of three cases.
We describe a unique series of 3 cases of biliary atresia (BA) associated with a choledochal cyst. All 3 children presented with jaundice at birth and had no other abnormalities. Although these children had a fetal form of BA, their presentation and outcome differed from those of biliary atresia splenic malformation syndrome (BASM), the well-described form of early onset BA. Unlike those with BASM, these children had no other associated malformations, had a normal birth weight, and did not yet require a liver transplant. We believe that the present series of patients and their associated pathology may represent a distinct phenotype with a common, prenatally acquired etiology that is different from other fetal forms of BA, such as BASM, and from patients who present with the classic perinatal form of BA.
De Matos
Vera
V
Divisions of Gastroenterology and Nutrition and Anatomical Pathology, The Children's Hospital of Philadelphia, 34th and Civic Center Boulevard, Philadelphia, PA 19104-4399, USA.
Erlichman
Jessi
J
Russo
Pierre A
PA
Haber
Barbara A
BA
D001656:Biliary Atresia / Q000150:complications* / Q000473:pathology*; D002648:Child; D015529:Choledochal Cyst / Q000150:complications* / Q000473:pathology*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007565:Jaundice / Q000209:etiology; D011247:Pregnancy