Bjøro K, Schrumpf E, Elgjo K, Kolmanskog F
Dept. of Medicine A, Rikshospitalet, Oslo, Norway.
Scand J Gastroenterol. 1995 Jan;30(1):92-4. doi: 10.3109/00365529509093242.
Hepatic involvement in hereditary haemorrhagic telangiectasia (HHT) consisting of fibrosis, telangiectases, and cirrhosis, has been reported as a relatively frequent finding.
A 50-year-old man with HHT presented with monstrous ascites. Liver biopsy demonstrated multiple dilated sinusoids but not cirrhosis. There were no findings indicative of portal hypertension or malignant disease. Portal pressure, recorded in hepatic vein wedge position, was normal. Arteriography showed numerous hypervascular lesions throughout the liver. The clinical course has been stable for more than 2 years.
No other reason for the monstrous ascites could be found. We thus hypothesize that this case of monstrous ascites is due to hepatic involvement in HHT, presenting as numerous vascular lesions throughout the liver.
遗传性出血性毛细血管扩张症（HHT）累及肝脏，表现为纤维化、毛细血管扩张和肝硬化，据报道这是一种相对常见的表现。
一名50岁的HHT男性患者出现大量腹水。肝脏活检显示多个扩张的血窦，但无肝硬化表现。未发现提示门静脉高压或恶性疾病的迹象。在肝静脉楔压位置记录的门静脉压力正常。动脉造影显示肝脏内有许多高血管病变。临床病程已稳定超过2年。
未发现导致大量腹水的其他原因。因此，我们推测该例大量腹水是由于HHT累及肝脏，表现为肝脏内大量血管病变。
