Kasi Pashtoon Murtaza, Ramirez Raymund, Rogal Shari S, Littleton Kailey, Fasanella Kenneth E
International Scholars Program, University of Pittsburgh Medical Center (UPMC), Pittsburgh, Pa., USA.
Case Rep Gastroenterol. 2011 Sep;5(3):654-62. doi: 10.1159/000334988. Epub 2011 Dec 2.
Gallbladder agenesis is a rare entity with an estimated incidence of 10-65 per 100,000. Females are more commonly affected (ratio 3:1), typically presenting in the 2nd or 3rd decade of life. Despite an absent gallbladder, half of patients present with symptoms similar to biliary colic, which is poorly understood. Clinicians should have a strong index of suspicion if nonvisualization is suggested by an ultrasound. HIDA scans are typically not helpful since nonvisualization of the gallbladder remains typical of cystic duct obstruction as well as of agenesis. While there are no specific guidelines for management of gallbladder agenesis, conservative management with smooth muscle relaxants is preferred. Sphincterotomy also has been reported in severe cases. Here, we report a case of a 21-year-old woman who presented with recurrent biliary colic and was diagnosed to have gallbladder agenesis on magnetic resonance cholangiopancreatography. A comparison with other cases and a review of the literature are presented.
胆囊缺如是一种罕见疾病，估计发病率为每10万人中有10 - 65例。女性更易受累（比例为3:1），通常在生命的第二个或第三个十年出现症状。尽管没有胆囊，但半数患者会出现类似胆绞痛的症状，对此了解甚少。如果超声提示胆囊未显影，临床医生应高度怀疑。由于胆囊未显影在胆囊管梗阻以及胆囊缺如中都很常见，因此肝胆动态显像扫描通常并无帮助。虽然对于胆囊缺如的治疗没有具体指南，但首选使用平滑肌松弛剂进行保守治疗。在严重病例中也有报道行括约肌切开术。在此，我们报告一例21岁女性，她反复出现胆绞痛，经磁共振胰胆管造影诊断为胆囊缺如。并与其他病例进行了比较，同时对文献进行了综述。
