Abstract
INTRODUCTION: Choledochal cyst (CC) is an uncommon congenital disease of the biliary tract. There are five main types of CC with several recognized sub-types. However, occasional variants with a difficulty in diagnosis and management do occur. PRESENTATION OF THE CASE: We report a case of a nine years old female child diagnosed with CC who presented with right quadrant abdominal pain with unremarkable physical findings. Investigation using abdominal CT scan suggested type II choledochal cyst. The intraoperative finding revealed an unusual site of the cyst that is at the confluence of common hepatic duct (CHD) posteriorly. The cyst was successfully excised and the child is doing well on her follow ups. DISCUSSION: In the management of choledochal cyst the anatomy should be clearly defined with detailed investigations like Abdominal CT Scan or cholangiography before surgical excision as abnormal variants which usually do not fit into the known classification types and subtypes. This might confuse with other differentials like gall bladder duplication. Surgical excision is the gold standard management option. CONCLUSION: This case report will alert surgeons that there are different anatomic variant of choledochal cysts out of the known classifications and with meticulous dissection will help proper excision and avoid unnecessary complications.