Frick H J, Blank W, Braun B, Pawlowski Z
Medizinische Klinik, Kreiskrankenhaus Reutlingen, Bundesrepublik Deutschland.
Z Gastroenterol. 1992 Oct;30(10):739-42.
Diffuse cholangiofibromatosis is a rare and asymptomatic abnormality. Its clinical significance results from problems that occur in differential diagnosis to granulomatous hepatitis, sclerosing cholangitis, multiple microabscesses or diffuse tumour infiltration of the liver, when it is incidentally found in ultrasound, ERCP or laparoscopy. The characteristic findings in these examinations are described in two case reports. The ultrasonic pattern of multiple intrahepatic double structures, microcystic areas and surrounding hyperechoic reflexes are in accordance with multiple intrahepatic microcystic and hazy areas in ERCP x-rays and multiple white-yellowish areas that retract liver surface in laparoscopy. Final diagnosis is confined by the characteristical microscopic finding of von Meyenburg-complexes in liver biopsy.
弥漫性胆管纤维瘤病是一种罕见的无症状异常。其临床意义在于,当它在超声、内镜逆行胰胆管造影(ERCP)或腹腔镜检查中偶然发现时,在与肉芽肿性肝炎、硬化性胆管炎、多发性微脓肿或肝脏弥漫性肿瘤浸润进行鉴别诊断时会出现问题。两篇病例报告描述了这些检查中的特征性发现。肝内多发双结构、微囊区及周围高回声反射的超声表现,与ERCP X线片中肝内多发微囊及模糊区以及腹腔镜检查中使肝表面回缩的多发黄白色区域相符。最终诊断取决于肝活检中具有特征性的冯·迈恩伯格复合体的显微镜检查结果。