Cholangioscopy and electrohydraulic lithotripsy for the management of large bile duct stones in situs ambiguous: The first documented case report

Situs ambiguous (SA) is a rare genetic defect in embryogenesis characterised by failure to generate normal left-right asymmetry, leading to a spectrum of abnormalities. This anatomical variation creates additional challenges when performing endoscopic intervention.

We present the case of 65-year-old with SA who was admitted with cholangitis. Imaging confirmed large bile duct stones and unconventional anatomy with a right-sided stomach, third part of duodenum crossing the midline and a central liver. She underwent ERCP with cholangioscopy, electrohydraulic lithotripsy (EHL) and ultimately complete bile duct clearance, negating the need for surgical intervention [1] [2].

She was sedated with propofol/remifentanil with anaesthetist support. The ampulla appeared relatively more proximal in the duodenum and in a 1 o'clock position rather than the more conventional 11 o'clock. Maintaining a stable scope position was the largest challenge and only achieved with a second operator applying significant clockwise torque. When the duodenoscope position was threatened with falling back, stability was regained with additional clockwise torque and downward tip angulation.

Review of imaging with GI radiologists greatly assisted procedural planning. Patient consent should include discussion of the additional complexity and the risk of a prolonged procedure. The procedure should be optimised by ensuring adequate deep sedation/anaesthetic as well as sufficient time allocation and consideration of a second operator. A gastroscope was initially used to assess anatomy and technical factors. We have demonstrated that clearance of large bile duct stones using EHL is achievable in patients with SA.

Publication History

Article published online:
27 March 2025

• References

• 1 Situs ambiguous. Orphanet. Available at https://www.orpha.net/ (Accessed: 10 November 2024).
• 2 Eitler K, Bibok A, Telkes G.. Situs Inversus Totalis: A Clinical Review. Int J Gen Med 2022; 15: 2437-2449 PMID: 35264880; PMCID: PMC8901252