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先天性肝内胆管错构瘤的临床表现。

The clinical presentation of Von Meyenburg complexes.

作者信息

Sinakos E, Papalavrentios L, Chourmouzi D, Dimopoulou D, Drevelegas A, Akriviadis E

出版信息

Hippokratia. 2011 Apr;15(2):170-3.

Abstract

Von Meyenburg Complexes (VMCs) is a rare clinicopathologic entity, consisting of small (<1.5cm), usually multiple and nodular cystic lesions. VMCs typically cause no symptoms or disturbances in liver function and thus in most instances they are diagnosed incidentally. We present four VMCs cases, each with a distinct clinical presentation. In two of our cases, VMCs caused mild, non-specific abdominal symptoms, including diffuse abdominal pain and discomfort. In the other two cases, in a 60-year-old woman and a 25-year-old man, the clinical presentation was implicative of an infectious hepatic process reminiscent of cholangitis and liver abscesses respectively. In each case the diagnosis was based on magnetic resonance imaging and magnetic resonance cholangiopancreatography findings showing multiple hyper-intense cystic nodules not communicating with the biliary tree. Physicians should be aware of the entire clinical spectrum of VMCs and its unique radiologic features in order to differentiate VMCs from other cystic liver lesions.

摘要

梅耶尔堡复合体(VMCs)是一种罕见的临床病理实体,由小的(<1.5厘米)、通常为多发的结节性囊性病变组成。VMCs通常不引起症状或肝功能紊乱,因此在大多数情况下是偶然诊断出来的。我们报告4例VMCs病例,每例都有独特的临床表现。在我们的2例病例中,VMCs引起轻度、非特异性腹部症状,包括弥漫性腹痛和不适。在另外2例病例中,一名60岁女性和一名25岁男性的临床表现分别提示有类似胆管炎和肝脓肿的感染性肝脏病变。在每例病例中,诊断均基于磁共振成像和磁共振胰胆管造影结果,显示多个不与胆管树相通的高信号囊性结节。医生应了解VMCs的整个临床谱及其独特的放射学特征,以便将VMCs与其他肝脏囊性病变区分开来。

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