Agenesis of the Gallbladder Diagnosed by Magnetic Resonance Cholangiography: Report of a Case and Review of the Literature

Abstract

Abstract:Gallbladder agenesis (GA) is a rare congenital anomaly of the biliary system often associated with other congenital abnormalities. Patients become symptomatic in 23% of cases (Richards et al., 1993). GA is often misinterpreted as other diseases, therefore, leading to unnecessary surgery. Many of these patients develop a typical symptomatology of cholelithiasis that leads them to operating theatre. If an operative procedure is done, it is better to remain at the level of laparoscopy because further surgical investigation may lead to detrimental biliary tract injuries (Waisberg et al., 2002). We present a case of GA, diagnosed by magnetic resonance with cholangiopancreatography.

Agenesis of the Gallbladder: A Phantom Menace

“…Interestingly, the frequency of primary stones in the common bile duct in patients with GBA (23%) is much higher than the post-cholecystectomy rate (5%). This finding may support the theory that a sphincter of Oddi dysfunction leads to both symptoms and stasis in patients with GBA, and thus to choledocholithiasis (3,4).…”

“…Patients with symptomatic GBA represent over 50% of cases. They often present, interestingly, with biliary colic, dyspepsia or jaundice (2,3). It is in this group that diagnosis becomes a challenge.…”

“…Hence, in a number of cases of symptomatic GBA, the diagnosis is made intraoperatively (4). Congenital defects often associated with GBA include cardiac, gastrointestinal, and genitourinary abnormalities such as duodenal atresia, intestinal malrotation, pancreas divisum, imperforate anus, hypoplasia of the right hepatic lobe, duplication cysts of the hepatic flexure, ventricular septal defect, renal agenesis, undescended testes, and syndactyly (1,3). It can also be associated with genetic syndromes such as trisomy 18 (1).…”

“…It is theorized that symptoms in symptomatic GBA may arise from sphincter of Oddi dysfunction, since sphincter spasms may mimic the symptoms of biliary colic (3,4). Interestingly, the frequency of primary stones in the common bile duct in patients with GBA (23%) is much higher than the post-cholecystectomy rate (5%).…”

Dwuczęściowy pęcherzyk żółciowy z dalszą częścią wypełnioną kamieniami i zmienioną zapalnie, a bliższą zdrową – opis przypadku

“…przypadków [1,2,3]. Pęcherzyk może być położony w nietypowym miejscu (wrośnięty w wątrobę, schowany w fałdzie otrzewnowym) [4], może być zanikowy, a mimo tego może dawać objawy takie jak w przebiegu kamicy i/lub zapalenia [5], zdwojony, lub może mieć nietypowy kształt. Ponadto, pęcherzyk może być podzielony przegrodami na części [6].…”