Biliary Atresia and Choledochal Malformations

Biliary atresia (BA) is best characterised as a cholangiodestructive disease affecting all parts of the biliary tract. Infants will invariably present with persisting conjugated jaundice and acholic stools within the first few weeks. There are a number of different variants which are aetiological dissimilar encompassed within the phenotype “biliary atresia”. These can include a primary failure of biliary development as seen in cases of cystic BA and those with the biliary atresia splenic malformation (BASM) syndrome. In others there may be perinatal destruction of a formed biliary tree, with those showing anti-CMV IgM+ve antibodies as the likeliest group. The others may be referred to as isolated BA. Management for all remains palliative with the Kasai portoenterostomy (KPE); and this remains an operation still best done as an open procedure. In large centres with high referral volumes, 5- and 10-year native liver survivals of 45-50% have been achieved with an overall survival of ~90% at 10 years. Choledochal malformation (CM) may be best defined as abnormal dilatation of the biliary tract in the absence of any acute obstruction and can be broadly separated into two extrahepatic phenotypes: fusiform (type 1f) and cystic (type 1c). Both can be complicated by additional intrahepatic dilatation (type 4). Most CMs present within childhood either in the first few years with jaundice or later on with recurrent abdominal pain (usually associated with pancreatitis). The principal aim of surgery is excision of the dilated extrahepatic biliary tract with proximal reconstruction (usually hepaticojejunostomy) using a Roux loop. Open surgery is still very much the standard in most North American and European centres, although laparoscopic excision and reconstruction and most recently robotic CM’s resection and reconstruction have certainly become an option particularly in some high-volume Asian centres.