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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.

Type IVa choledochal cyst: Results following haemihepatectomy and mucosectomy of intrahepatic cyst