Reporting a Case on Congenital Agenesis of the Gallbladder with the Hypogenetic Cystic Duct Definitely Diagnosed by Peroral Cholangioscopy

Congenital gallbladder agenesis (CAGB) is a rare congenital disorder that is most commonly diagnosed serendipitously by autopsy or exploratory laparotomy in adults. A 59-year-old Japanese woman had a history of stomach pain, nausea, vomiting, jaundice, and low-grade fever for a few days. Her family history and background were non-contributory. Elevated serum hepatic and pancreatic enzymes, hyperbilirubinemia, and leukocytosis have been shown in laboratory data. Computed tomography (CT) showed the dilated biliary tree with enhanced peripancreatic adipose tissue obstructed by a stone in the distal part and the swollen pancreas. Ultrasonography (US), CT, or magnetic resonance cholangio-pancreatography have not detected entopic or ectopic gallbladder (MRCP). Vater’s papilla was patent-orificed. Although endoscopic retrograde cholangiography (ERC) failed to visualise the gallbladder, it showed a distal stone and a small thin, blind tube emerging from the middle extrahepatic biliary tree. She underwent endoscopic sphincterotomy (EST) and choledocholithotomy with a hypogenetic cystic duct complicated with choledocholithiasis causing acute cholangitis and pancreatitis under the preoperative diagnosis of congenital gallbladder agenesis (CAGB). The tube was discovered by peroral cholangioscopy to be a crescent-folded cul-de-sac running parallel to and draining into the common hepatic duct, the abortive primordium vestige that should have formed into the full-fledged cystic duct and gallbladder, confirming the diagnosis. She pursued an uneventful post-surgical path. This is the fifth case of hypogenetic cystic duct CAGB and the first case in the world in which peroral diagnosis has been identified. The critical task of peroral cholangioscopy and ERC and the importance of knowledge of the anomaly to prevent excessive, potentially dangerous exploration in the search for the phantom gallbladder through the abnormal orifices are emphasised. This study highlights the significance of CAGB knowledge and highlights the vital role of peroral cholangioscopy in diagnosing the anomaly and thus preventing unnecessary, potentially hazardous exploration in the search for the phantom gallbladder through the non-natural orifices.

Author(s) Details


Kenji Sasaki
Department of Internal Medicine, Midtown Medicare Clinic, Ohsaki, Miyagi, Japan.

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