ABSTRACT
Choledochal cyst is a rare condition characterised by congenital dilatation of the biliary tree. Commonly seen in the oriental countries, patients usually present with a varying combination of abdominal pain, jaundice, lump or cholangitis. Untreated patients or incomplete removal of the cysts usually leads to portal hypertension and cholangiocarcinoma. Almost one-third of the cases have intrahepatic cyst, making complete cyst excision often impossible. We are reporting a 9-year-old girl with type IVa choledochal cyst presented to us with recurrent pain abdomen and intermittent jaundice for 1 year. Excision of extrahepatic cyst, left hepatectomy, mucosectomy of the residual cyst wall of right lobe of the liver and a wide bilioenteric anastomosis was done. Patients followed up with an magnetic resonance cholangiopancreatography (MRCP) 2 months later showed shrinkage of the residual cyst and good bile drainage. Mucosectomy of intrahepatic cyst may prevent recurrent cholangitis, calculus formation and cholangiocarcinoma in the long run.
