A Case of Extrahepatic Portal Vein Obstruction Complicated by Idiopathic Portal Hypertension Treated with Living-Donor Liver Transplantation and Second-Stage Splenectomy

Yusuke Suto^1, , Akira Umemura¹, Hiroyuki Nitta¹, Takeshi Shiohata², Seiya Tagane³, Naoki Yanagawa⁴, Masao Nishiya⁴, Ayaka Sato⁴ and Akira Sasaki¹

¹Department of Surgery, School of Medicine, Iwate Medical University

²Shiohata Family Clinic

³Department of Pediatrics, School of Medicine, Iwate Medical University

⁴Department of Molecular Diagnostic of Pathology, School of Medicine, Iwate Medical University

Cite this paper:
Yusuke Suto, Akira Umemura, Hiroyuki Nitta, Takeshi Shiohata, Seiya Tagane, Naoki Yanagawa, Masao Nishiya, Ayaka Sato and Akira Sasaki. A Case of Extrahepatic Portal Vein Obstruction Complicated by Idiopathic Portal Hypertension Treated with Living-Donor Liver Transplantation and Second-Stage Splenectomy. American Journal of Medical Case Reports. 2026; 14(1):18-27. doi: 10.12691/ajmcr-14-1-4

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

Keywords:
extrahepatic portal vein obstruction idiopathic portal hypertension living-donor liver transplantation splenectomy portosystemic encephalopathy non-cirrhotic portal hypertension

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References:

[1]	Khanna, R. and Sarin, S.K., "Idiopathic portal hypertension and extrahepatic portal venous obstruction.", Hepatology International, 12 (Suppl 1), 148-167, Feb 2018.
[2]	Furuichi, Y., Kage, M., Ohta, M., Ohfuji, S., Sasaki, H., Hidaka, H., Yoshida, H., Kanto, T., Kusano, H., Akahoshi, T., Obara, K., Hashizume, M., Kuniyoshi, Y., Kawaguchi, T., Okubo, H., Ishikawa, T., Hirooka, M., Iwakiri, Y., Nio, M. and Tanaka, A, "Guidelines for the diagnosis and treatment of idiopathic portal hypertension, extrahepatic portal obstruction, and Budd-Chiari syndrome in Japan.", Hepatology Research, 54 (11), 952-969, Nov 2024.
[3]	Tamura, Y., Tamura, Y., Taniguchi, Y. and Atsukawa, M, "Current clinical understanding and effectiveness of portopulmonary hypertension treatment", Frontiers in Medicine.
[4]	Tanaka, T., Sugawara, Y. and Kokudo, N., "The current clinical aspects of idiopathic portal hypertension.", Intractable Rare Disease Research, 2 (3), 94-97, Aug 2013.
[5]	Jinshan Z. and Li, L., "Rex Shunt for Extra-Hepatic Portal Venous Obstruction in Children", Children, 9 (2), 297, Feb 2022.
[6]	Satai, M., Vaidya, A., Rathod, K., Singh, A., Harindranath, S., Patra, B.R. and Shukla, A., "Partial Splenic Artery Embolization for the Management of Symptomatic Hypersplenism in Portal Hypertension: Clinical Insights from a Case Series.", Journal of Clinical and Experimental Hepatology, 14 (5), 101435, 2024.
[7]	Zhang, J. and Li, L., "Surgical ligation of a portosystemic shunt for the treatment of type Ⅱ Abernethy malformation in 12 children", Journal of Vascular Surgery, Venous Lymphatic Disorder, 2021.
[8]	Murai, Y., Ohfuji, S., Fukushima, W., Tamakoshi, A., Yamaguchi, S., Hashizume, M., Moriyasu, F. and Hirota, Y., "Prognostic factors in patients with idiopathic portal hypertension: Two Japanese nationwide epidemiological surveys in 1999 and 2005.", Hepatology Research, 42 (12), 1211-1220, Dec 2012.
[9]	Bernard, P.H., Le Bail, B., Cransac, M., Barcina, M.G., Carles, J., Balabaud, C. and Bioulac-Sage, P., "Progression from idiopathic portal hypertension to incomplete septal cirrhosis with liver failure requiring liver transplantation.", Journal of Hepatology, 22 (4), 495-499, Apr 1995.
[10]	Dumortier, J., Bizollon, T., Scoazec, J.Y., Chevallier, M., Bancel, B., Berger, F., Ducerf, C., Claudel-Bonvoisin, S., Paliard, P., Boillot, O. and Trepo, C, "Orthotopic liver transplantation for idiopathic portal hypertension: indications and outcome.", Scandinavian Journal of Gastroenterology, 36 (4), 417-422, Apr 2001.
[11]	Krasinskas, A.M., Eghtesad, B., Kamath, P.S., Demetris, A.J. and Abraham, S.C., "Liver transplantation for severe intrahepatic noncirrhotic portal hypertension.", Liver Transplant, 11 (6), 627-34; discussion 610-1, Jun 2005.
[12]	Ohta, M., Shimada, T., Matsufuji, H., Yukizane, T., Yamada, H. and Sugimachi, K, "Surgical treatment of a patient with idiopathic portal hypertension and hepatic encephalopathy", Hepato-gastroenterology, 48 (41), 1461-3, Sep-Oct 2001.
[13]	Terada, T., Nakanuma Y., et al. "Expression of HLA-DR antigen on hepatic vascular endothelial cells in idiopathic portal hypertension", Clinical and Experimental Immunology, 84 (2), 303-7, May 1991.
[14]	Yasunori, S. and Yasuni N., "Role of endothelial mesenchymal transition in idiopathic portal hypertension", Histology and Histopathology, 28 (2), 145-54, Feb 2013.
[15]	Nakanuma, Y., Sato, Y. and Kiktao, A., "Pathology and pathogenesis of portal venopathy in idiopathic portal hypertension: Hints from systemic sclerosis.", Hepatology Research, 39 (10), 1023-1031, Oct 2009.
[16]	Maria, D., Dimitrios, F., et al. "2021 Guidelines: For the Management of Transfusion Dependent Thalassemia (TDT) ", 4th edition, 2023.
[17]	Jugenburg, M., Haddock, G., Freedman, M.H., Ford-Jones, L. and Ein, S.H., "The morbidity and mortality of pediatric splenectomy: does prophylaxis make a difference.", Journal of Pediatric Surgery, 34 (7), 1064-1067, Jul 1999.
[18]	Kawachi, S., Chiba, N., Nakagawa, M., Kobayashi, T., Hikita, K., Sano, T., Tomita, K., Hirano, H., Abe, Y., Obara, H. and Shimazu , M, "Living donor liver transplantation for idiopathic portal hypertension with extrahepatic portal vein stenosis and splenic artery aneurysms: a case report and review of the literature.", BMC Surgery, 20 (1), 257, Oct 2020.