Situs inversus totalis is the mirror image of situs solitus, the normal position of abdominal and thoracic viscera. Many associated intraabdominal anomalies have been described with this condition. However, association of choledochal cyst with situs inversus has never been reported. Diagnosis and surgical procedures for abdominal pathology in patients with situs inversus totalis are technically more complicated and pose unique challenges because of left-right transposition of the visceral organs. The choledochal cyst is usually diagnosed in the neonatal period or during childhood. The clinical symptoms are nonspecific and usually include pain in the right upper quadrant and jaundice. The condition may also present with biliary colic, cholangitis, cholelithiasis or pancreatitis. In our case, the abdominal ultrasonography performed on a 55-year-old female who presented to us with jaundice, fever and pain in the left upper abdomen revealed presence of situs inversus with fusiform dilation of the common bile duct. Based on the magnetic resonance cholangiopancreatography (MRCP) images diagnosis of type I choledochal cyst was made. The aim of this case report is to illustrate an extremely rare association of situs inversus totalis with choledochal cyst along with the challenges faced in diagnosis and surgical management of this condition.
Keywords: Cholangiography; Choledochal cyst; Hepaticojejunostomy; Situs inversus totalis.