Left-sided cholecystitis in a patient with situs inversus totalis, complicated by portal venous malformations

Abstract Situs inversus totalis (SIT) is a rare autosomal recessive anomaly in which the thoracoabdominal viscerae are laterally transposed, introducing unique challenges in surgical scenarios. Only a few reports have demonstrated the treatment of cholecystitis in situs inversus, much less so in the context of portal vascular anomalies. We present the case of a 41-year-old female presenting to the emergency department with right upper quadrant pain, and subsequently found to have left-sided cholecystitis complicated by SIT with portal venous malformations on magnetic resonance cholangiopancreatography and abdominal computed tomography. Initially, she was referred for open cholecystectomy however due to the lack of symptoms and the presence of a tortuous recanalized portal vein presenting multiple thrombotic complications, an expectant approach was adopted. Thus, imaging remains the gold-standard to diagnose SIT and consideration of all congenital risk factors to cholecystectomy is crucial to avoid post-operative complications.