Double rarity: malignant masquerade biliary stricture in a situs inversus totalis patient

Abstract

Background Situs inversus totalis is a rare anatomical variation of both the thoracic and the abdominal organs. Common bile duct strictures can be caused by malignant and benign diseases as well. 7–18% of the latter ones are 'malignant masquerade’ cases, as pre-operative differentiation is difficult. Case presentation We present the case of a 68y male patient with known situs inversus totalis and a recent onset of obstructive jaundice caused by a malignant behaving common bile duct stricture. Technically difficult endoscopic retrograde cholangiopancreatography, brush cytology, magnetic resonance cholangiopancreatography, endoscopic ultrasound, and percutaneous transhepatic drainage with stent implantation were performed for proper diagnosis. Cholecystectomy, common bile duct resection with hilar lymphadenectomy, and hepatico-jejunostomy have been performed following multidisciplinary consultation. The final histology report did not confirm any clear malignancy, the patient is doing well. Conclusion In situs inversus patients, both diagnostic and therapeutic procedures can lead to various difficulties. Benign biliary strictures are frequently misdiagnosed preoperatively as cholangiocellular carcinoma. Surgery is usually unavoidable, involving a significant risk of complications. The co-existence of these two difficult diagnostic and therapeutic features made our case challenging.

Situs Inversus Totalis: A Clinical Review

“…Our work group's recent paper presents a patient with a biliary stricture who underwent multiple biliary procedures. 69 Compared to traditional percutaneous biliary drainage procedures in our institute, we placed the patient heading the opposite direction in order to stay on the usual side to work. The operator and the IR nurse changed their position and the ceiling-hanged monitor was moved behind the IR nurse to allow a clear view.…”

“…110,111 We previously published a case of malignant masquerade biliary stricture in a situs inversus totalis patient (who gave his written consent to that) when distal resection of the common bile duct, cholecystectomy and hilar lymphadenectomy were performed. 69 After encountering the initial disorientation, experienced surgeons can perform these procedures successfully and safely.…”