Extrahepatic cholangiocarcinoma in a patient with situs inversus totalis diagnosed by endoscopic ultrasound
14 August 2013 (online)
Situs inversus totalis (SIT) is a rare congenital abnormality characterized by a left–right reversal of the positions of the internal organs. A 67-year-old man with SIT came to our attention with a recent onset of jaundice.
Computed tomography (CT) scanning showed dilatation of the biliary tree and truncation of the common bile duct (CBD) just above the papilla; no evidence of an ampullary or pancreatic mass was seen ([Fig. 1]).
Although to overcome potential technical limitations, it is advocated that patients with SIT lie in the right decubitus position, we performed radial scanning endoscopic ultrasound (EUS; Olympus GF UE160) without difficulties, keeping our patient in the left decubitus position and under conscious sedation ([Fig. 2 a, b]). The papilla of Vater appeared enlarged with normal overlying mucosa. EUS showed significant wall thickening and a 2-cm hypoechoic mass in the distal CBD, with infiltration of the papilla and of the pancreas ([Fig. 2 c]); the proximal CBD was dilated ([Fig. 2 d]). Because of the anatomical inversion, the echoendoscope had to be rotated clockwise rather than counterclockwise to explore the CBD from the papilla to the liver hilum ([Fig. 2 e]).
Subsequently, linear scanning EUS (Olympus GF UCT140) with fine needle aspiration (FNA) was carried out in the same fashion and without difficulties. Cytological examination of the periampullary lesion revealed cholangiocarcinoma ([Fig. 3]). On the basis of the results from the EUS, the patient initially underwent endoscopic retrograde cholangiopancreatography (ERCP) with biliary stenting for palliation of jaundice. He then underwent a Whipple’s procedure 2 weeks later, from which he made an uneventful recovery.
EUS is a very accurate technique for diagnosis and staging of most etiologies of extrahepatic biliary obstruction. However, reported cases of extrahepatic biliary tumor in patients with SIT have been investigated only by extracorporeal imaging    . A fear of technical difficulties due to the altered anatomy may account for the relative underutilization of EUS in this setting. To the best of our knowledge, EUS has been described only once before in a patient with SIT and extrahepatic cholangiocarcinoma but technical details were not provided .
In conclusion, using EUS performed under routine conditions, we were able to diagnose a small cholangiocarcinoma that had been missed on CT scanning. EUS proved to be feasible and accurate in this patient despite the associated SIT.
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