title: Acute deterioration of idiopathic portal hypertension requiring living donor liver transplantation: a case report.
abstract: 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.
authors.affiliation: Department of Surgery, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan. tainokuma-gi@umin.net
authors.forename: Takamitsu
authors.initials: T
authors.lastname: Inokuma
authors.forename: Susumu
authors.initials: S
authors.lastname: Eguchi
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Tomonaga
authors.forename: Kensuke
authors.initials: K
authors.lastname: Miyazaki
authors.forename: Koji
authors.initials: K
authors.lastname: Hamasaki
authors.forename: Hirotaka
authors.initials: H
authors.lastname: Tokai
authors.forename: Masaaki
authors.initials: M
authors.lastname: Hidaka
authors.forename: Kosho
authors.initials: K
authors.lastname: Yamanouchi
authors.forename: Mitsuhisa
authors.initials: M
authors.lastname: Takatsuki
authors.forename: Sadayuki
authors.initials: S
authors.lastname: Okudaira
authors.forename: Yoshitsugu
authors.initials: Y
authors.lastname: Tajima
authors.forename: Takashi
authors.initials: T
authors.lastname: Kanematsu
mesh_terms: D000368:Aged; D001284:Atrophy; D003251:Constriction, Pathologic; D018450:Disease Progression; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D008099:Liver / Q000098:blood supply / Q000473:pathology; D017093:Liver Failure / Q000209:etiology* / Q000601:surgery*; D016031:Liver Transplantation; D011169:Portal Vein / Q000473:pathology; D012039:Regional Blood Flow