Does “Cystic” Biliary Atresia Represent a Distinct Clinical and Etiological Subgroup? A Series of Three Cases

Abstract

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.

Excellent long-term outcome of hepaticojejunostomy for biliary atresia with a hilar cyst

“…When a cystic dilatation of the common bile duct is accompanied with type I atresia, it is called a type I cyst. The prognosis of type I BA is usually much better than that of type II/III [8][9][10][11][12].…”

Screening and outcomes in biliary atresia

“…Cystic variants of BA, in which a bile ductal cyst is present, may prove to have etiologic and prognostic implications. 38 Similarly, the potential value of the histological findings of ductal plate malformation in predicting the outcome is controversial and requires further investigation. Hepatic histology does not differentiate patients with the embryonic and perinatal forms of BA.…”

Biliary Atresia: A Multidisciplinary Approach to Diagnosis and Management

“…9 In addition, cystic dilatation of biliary remnants may be seen in a small minority of cases of fetal-type biliary atresia (approximately 5%-10% of cases). 10,11 These cases are referred to as cystic biliary atresia and, likewise, have been postulated to form a distinct subgroup of patients whose prognoses were found to be more favorable in 1 study 11 but, as seen in BASM, may be more dependent on age at Kasai procedure than perinatal biliary atresia. 10 …”