Simma W, Wayand U, Lederer B, Brücke P
Wien Klin Wochenschr. 1979 Apr 13;91(8):257-61.
Two patients with symptoms of progressive obstructive jaundice, a history of vague pains in the right upper quadrant and laboratory evidence of biliary obstruction underwent laparotomy. A stone-free, but extremely thick-walled gallbladder was found in both patients. Intraoperative cholangiography showed diffusely-stenosed extrahepatic bile ducts suggestive of chronic inflammatory changes in the biliary system. The correct diagnosis was made only on histological examination, which revealed primary sclerosing cholangitis with secondary cholestatic changes of the liver. Postoperative treatment included long-time corticosteroid therapy. Both patients have remained jaundice-free for periods of one and two years, respectively, to date, but the eventual prognosis is poor. The diagnosis, which can be made only surgically, therapy and prognosis are discussed.
两名患有进行性梗阻性黄疸症状、有右上腹隐痛病史且有胆道梗阻实验室证据的患者接受了剖腹手术。两名患者均发现胆囊无结石但壁极厚。术中胆管造影显示肝外胆管弥漫性狭窄，提示胆道系统存在慢性炎症改变。仅通过组织学检查才做出正确诊断，结果显示为原发性硬化性胆管炎伴肝脏继发性胆汁淤积性改变。术后治疗包括长期使用皮质类固醇疗法。迄今为止，两名患者分别已保持无黄疸状态一年和两年，但最终预后不佳。本文讨论了只能通过手术做出的诊断、治疗方法及预后情况。
