Inokuma Takamitsu, Eguchi Susumu, Tomonaga Tetsuo, Miyazaki Kensuke, Hamasaki Koji, Tokai Hirotaka, Hidaka Masaaki, Yamanouchi Kosho, Takatsuki Mitsuhisa, Okudaira Sadayuki, Tajima Yoshitsugu, Kanematsu Takashi
Department of Surgery, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan.
Dig Dis Sci. 2009 Jul;54(7):1597-601. doi: 10.1007/s10620-008-0504-7. Epub 2008 Oct 31.
Case reports of severe idiopathic portal hypertension (IPH) requiring liver transplantation are very rare. We report the case of a 65-year-old woman who was diagnosed as having IPH. At the age of 60 years, her initial symptom was hematemesis, due to ruptured esophageal varices. Computed tomography of the abdomen showed splenomegaly and a small amount of ascites, without liver cirrhosis. She was diagnosed as having IPH and followed-up as an outpatient. Five years later, she developed symptoms of a common cold and rapidly progressive abdominal distension. She was found to have severe liver atrophy, liver dysfunction, and massive ascites. Living donor liver transplantation was then performed, and her postoperative course was uneventful. Histopathological findings of the explanted liver showed collapse and stenosis of the peripheral portal vein. The areas of liver parenchyma were narrow, while the portal tracts and central veins were approximate one another, leading to a diagnosis of IPH. There was no liver cirrhosis. The natural history of refractory IPH could be observed in this case. Patients with end-stage liver failure due to severe IPH can be treated by liver transplantation.
需要肝移植的严重特发性门静脉高压（IPH）病例报告非常罕见。我们报告一例65岁女性被诊断为IPH的病例。60岁时，她的初始症状是因食管静脉曲张破裂导致的呕血。腹部计算机断层扫描显示脾肿大和少量腹水，无肝硬化。她被诊断为IPH并作为门诊患者进行随访。五年后，她出现了普通感冒症状并迅速进展为腹胀。发现她有严重的肝萎缩、肝功能障碍和大量腹水。随后进行了活体供肝移植，术后过程顺利。切除肝脏的组织病理学检查结果显示外周门静脉塌陷和狭窄。肝实质区域狭窄，而门静脉分支和中央静脉彼此靠近，从而诊断为IPH。无肝硬化。该病例可以观察到难治性IPH的自然病程。因严重IPH导致终末期肝衰竭的患者可通过肝移植治疗。
