Cholecystohepatic duct (type IIIA) - fifth report of aberrant biliary pathology in a paediatric case of oesophageal atresia and imperforate anus with review of literature.

Abstract

It is known that the presence of associated anomalies has become one of the most critical prognostic factor for infants born with oesophageal atresia (OA). Associated VACTERAL group of abnormalities are common, but other associations are rare. Anomalies of the hepatobiliary system are even more rare among other groups. There are only a few such cases in the literature including adults and paediatric population. We describe the findings and management of one infant born with OA with tracheoesophageal fistula (TEF) and Ano rectal malformation (ARM), who was later shown to have features of obstructive jaundice. The biliary pathology was due to a preoperative diagnosis of Type 1c choledochal cyst, but in reality it was associated with an aberrant form of biliary drainage in the form of cholecystohepatic duct (CCHD). In the English literature, there are only four reported cases of cholecystohepatic duct (CCHD) in children associated with complex congenital anomalies [1-3]. The association of these multiple complex congenital anomalies with aberrant biliary duct is a rare feature. Here, in this case, this aberrant cholecystohepatic duct is the only drainage pathway of bile from liver into gallbladder.