@article{ajmcr20261414,
author={{Suto, Yusuke and Umemura, Akira and Nitta, Hiroyuki and Shiohata, Takeshi and Tagane, Seiya and Yanagawa, Naoki and Nishiya, Masao and Sato, Ayaka and Sasaki, Akira},
title={A Case of Extrahepatic Portal Vein Obstruction Complicated by Idiopathic Portal Hypertension Treated with Living-Donor Liver Transplantation and Second-Stage Splenectomy},
journal={American Journal of Medical Case Reports},
volume={14},
number={1},
pages={18--27},
year={2026},
url={https://pubs.sciepub.com/ajmcr/14/1/4},
issn={2374-216X},
abstract={We report the case of a 12-year-old boy with extrahepatic portal vein obstruction (EHPVO) complicated by idiopathic portal hypertension (IPH) who underwent living-donor liver transplantation (LDLT) and second-stage splenectomy. He presented with tarry stools, severe fatigue, and anemia. Computed tomography (CT) revealed EHPVO with cavernous transformation, and endoscopy revealed gastroesophageal varices with red signs. Brain magnetic resonance imaging and spectroscopy revealed portal systemic shunt encephalopathy, and liver biopsy confirmed IPH. LDLT was performed using his father¡¯s right lobe graft, with resection of the cavernous transformation and portal vein reconstruction. His postoperative course was uneventful, and he was discharged on day 54. Pancytopenia and splenomegaly persisted, so a second-stage splenectomy was performed. Five years after transplantation, he remains well without graft complications or recurrent portal hypertension. The Rex shunt is a common surgical strategy for EHPVO, although IPH can limit its effectiveness. LDLT is a practical treatment option for EHPVO complicated by IPH},
doi={10.12691/ajmcr-14-1-4}
publisher={Science and Education Publishing}
}