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Common bile duct adenocarcinoma in a patient with situs inversus totalis: report of a rare case

DOI: 10.1186/1756-0500-5-681

Keywords: Situs inversus totalis, Bile duct cancer, Preoperative staging, Surgical management

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Abstract:

We describe a 33-year old Guinean man with situs inversus totalis who presented with obstructive jaundice. Imaging and endoscopic modalities demonstrated a mass of distal common bile duct which biopsy identified an adenocarcinoma. The patient was successfully treated by cephalic pancreaticoduodenectomy followed by adjuvant chemoradiation and he is doing well without recurrence 8 months after surgery.The occurrence of bile duct adenocarcinoma in patient with situs inversus totalis accounts as a rare coincidence. In this setting, when the tumor is resectable, surgical management should be considered without contraindication and must be preceded by a careful preoperative staging.Situs inversus totalis (SIT) is a rare congenital condition characterized by a mirror-image transposition of both the abdominal and thoracic viscera, its incidence accounts for 1/8000 to 1/25,000 of the normal population [1]. This condition may cause difficulties in the diagnosis and therapeutic management of abdominal pathology [2,3]. An increased risk of cardiac, splenic and hepatobiliary malformations are found in patients with SIT [4]; this abnormality is not considered to be a premalignant entity, however rare malignant neoplasms have been reported. A case of successfully treated common bile duct adenocarcinoma in a 33-year old man with SIT, an association described for the fourth time in the literature, is described.A 33-year old previously healthy man, with known SIT presented with 3 months history of obstructive jaundice and 10 Kg weight loss. Computed tomography (CT) confirmed the complete transposition of viscera as follows: dextrocardia, right subphrenic gastric bubble and spleen with a small accessory spleen, left sided liver and reversed superior vena cava, brachiocephalic artery and hepatic artery; CT also revealed a dilatation of intra and extra hepatic bile ducts secondary to stenosis of the distal common bile duct (CBD) (Figure 1). Magnetic resonance cholangiopancreatography co

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