Endoscopy | 2021
Intraoperative digital single-operator cholangioscopy - extra-anatomical biliary access and insights into severe sclerosing cholangitis in a pediatric patient.
Abstract
A 3-year-old boy with Downʼs syndrome, born prematurely with atrial septal defect type II and stenosis of the pulmonary artery, presented with hepatopathy and fibrosis. Magnetic resonance imaging (MRI) showed diffuse dilations of the biliary system, with large cystic changes suspicious for Caroli syndrome (▶Fig. 1). Endoscopic retrograde cholangiography (ERC) presented signs of secondary sclerosing cholangitis of the right liver lobe with missing contrast enhancement of the left biliary system (▶Fig. 2). Because there was no communication of the left liver lobe with the central biliary system, the patient underwent surgery to drain the left liver lobe by peripheral hepaticojejunostomy. Access to the biliary system was gained by the open biliary system of segment II and intraoperative cholangioscopy was performed using the newly developed, short digital system (SpyGlass Discover; Boston Scientific Corp., Marlborough, Massachusetts, USA) (▶Video 1, ▶Fig. 3). Intraoperative cholangioscopy revealed cystic dilated bile ducts, partly filled with biliary casts in accordance with the MRI imaging (▶Fig. 4). These findings further supported the diagnosis of systemic sclerosis due to severe postnatal respiratory insufficiency and circulatory problems. Intraoperative cholangioscopy enables direct visualization of the biliary tract and is evaluated for stone clearance during cholecystectomy [1, 2]. Single-operator cholangioscopy has developed as a standardized procedure during ERCP for E-Videos