Naim Huda, Hasan Syed Askari, Khalid Sameen, Abbass Aamer, DSouza Jason
Internal Medicine, Dow Medical College, Karachi, Pakistan.
Internal Medicine Residency, Florida Hospital-Orlando.
Cureus. 2017 Nov 10;9(11):e1834. doi: 10.7759/cureus.1834.
The congenital absence of the gallbladder (CAG) is a rare condition with an incidence of 13-65 cases/ 100,000 in the general population. This occurs when the gallbladder and the cystic duct fail to bud from the common bile duct during the fifth week of gestation. Most commonly, the patients with congenital absence of the gallbladder are asymptomatic. When symptomatic, they present as biliary colic, dyspepsia, jaundice or very rarely as acute cholecystitis. We present a case of a 27-year-old female who presented with acute right upper quadrant abdominal pain. Further evaluation with an ultrasound revealed a contracted gallbladder with stones. The hepatobiliary iminodiacetic acid scan was significant for non-visualization of the gallbladder, consistent with cystic duct obstruction. The laparoscopic cholecystectomy was attempted, however, the gallbladder was not visualized, and the procedure was aborted. The post-operative magnetic resonant cholangiopancreatography was consistent with the diagnosis of congenital absence of gallbladder.
先天性胆囊缺如(CAG)是一种罕见病症,在普通人群中的发病率为每10万人中有13 - 65例。这是在妊娠第五周时胆囊和胆囊管未能从胆总管芽生出来所导致的。最常见的情况是,先天性胆囊缺如的患者没有症状。出现症状时,表现为胆绞痛、消化不良、黄疸,极少数情况下表现为急性胆囊炎。我们报告一例27岁女性,她因急性右上腹疼痛前来就诊。进一步的超声检查显示胆囊收缩并伴有结石。肝胆亚氨基二乙酸扫描显示胆囊未显影,符合胆囊管梗阻。尝试进行腹腔镜胆囊切除术,但未见到胆囊,手术中止。术后磁共振胰胆管造影结果与先天性胆囊缺如的诊断相符。