Gallbladder agenesis with a primary choledochal stone in a patient with situs inversus totalis

Patient: Female, 68 Final Diagnosis: Gallbladder agenesis with situs inversus totalis Symptoms: Epigastric pain • jaundice Medication: — Clinical Procedure: — Specialty: Surgery OBJECTIVE: Rare disease BACKGROUND: Situs inversus totalis is an inherited condition characterized by the mirror-image transposition of thoracic and abdominal organs. Gallbladder agenesis, which has normal bile ducts, is a rare congenital condition that occurs in 13 to 65 people out of 100 000. A common bile duct (CBD) stone or choledocholithiasis in patients with gallbladder agenesis is even rarer. CASE REPORT: We report the case of a 68-year-old woman who presented with epigastric pain and jaundice. She was not known to have situs inversus totalis. Abdominal ultrasound showed a large stone in the CBD, which could not be extracted by endoscopic retrograde cholangiopancreatography (ERCP), necessitating exploration. The gall-bladder and cystic duct were found to be absent. Incisional exploration of the CBD was performed, and a large stone was removed. A choledochoscope was used to identify the remnants and exclude the presence of ectopic gallbladder, and a T-tube was placed into the CBD. CONCLUSIONS: Gallbladder agenesis in a patient with situs inversus totalis is extremely rare, with no single reported case identified in the literature. In addition, our case showed a rare complication of ERCP – a failure to extract the CBD stone – and illustrates a way to overcome this complication.