Iwamura K, Itakura M
Hepatogastroenterology. 1980 Jun;27(3):189-94.
The article reports on a patient with Cruveilhier-Baumgarten disease. The anamnesis of the 59-year-old housewife revealed previous anemia, splenomegaly and abnormal liver function 21 years ago. Three years ago, esophageal varices were found. A tortuously distended vein was seen in the falciform ligament at the time of laparoscopy, and this finding was confirmed by angiography and ultrasonography. Esophageal varices and hypersplenism were also noted. Despite these findings, liver biopsy specimens of both lobes showed only slight fibrosis with minimal lymphocyte infiltration in some portal areas, and no evidence of cirrhosis. Patency of the umbilical vein and portal hypertension without significant histologic change of the liver, are both in keeping with the features of this disease. Dilatation of the umbilical vein seemed to be congenital and did not contribute to active blood flow of portal hypertension in this patient. In Japan, development of "caput medusae" in portal hypertension is rather rare, whereas esophageal varices and splenomegaly are more frequent. Venous hum is also seldom found. 14 cases of Cruveilhier-Baumgarten disease and 28 cases of Cruveilhier-Baumgarten syndrome have been reported from Japan in the literature since 1911.
本文报道了一名患有克吕韦耶 - 鲍姆加滕病的患者。这位59岁家庭主妇的既往史显示，21年前曾有贫血、脾肿大及肝功能异常。三年前发现食管静脉曲张。腹腔镜检查时在镰状韧带处可见一条迂曲扩张的静脉，血管造影和超声检查证实了这一发现。同时还发现了食管静脉曲张和脾功能亢进。尽管有这些发现，但两个肝叶的肝活检标本仅显示轻度纤维化，部分门静脉区域有少量淋巴细胞浸润，无肝硬化证据。脐静脉通畅以及存在门静脉高压但肝脏无明显组织学改变，均符合该病的特征。脐静脉扩张似乎是先天性的，且在该患者中并非门静脉高压的主要血流原因。在日本，门静脉高压患者出现“海蛇头”较为罕见，而食管静脉曲张和脾肿大更为常见。静脉嗡鸣声也很少见。自1911年以来，日本文献中已报道了14例克吕韦耶 - 鲍姆加滕病和28例克吕韦耶 - 鲍姆加滕综合征。
