REVIEW article
Front. Pediatr.
Sec. Pediatric Surgery
Volume 13 - 2025 | doi: 10.3389/fped.2025.1627388
Management of Pediatric Portal Vein Cavernous Transformation: A Seven-Case Single-Center Study
Provisionally accepted
Zhida Chen
Hui Liu
Li Wanfu*Gulimiremu MaimaitiAyiguzaili Maimaijiang
Yeliaman JiayilawuAerxin HabudingRunqi XiHaoyu WangHalimulati HuxitaerFangjuan Song- First Affiliated Hospital of Xinjiang Medical University, Urumqi, China
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Background：Portal vein cavernous transformation (CTPV) is a leading cause of prehepatic portal hypertension in children, accounting for approximately 40% of cases[1]. The Rex shunt, by reconstructing an intrahepatic portal venous pathway, not only effectively reduces portal pressure and restores physiological hepatic blood flow, but also promotes normal growth and development, making it the treatment of choice for CTPV. In contrast, traditional non-selective shunting procedures primarily alleviate symptoms of portal hypertension without restoring hepatic perfusion, thereby compromising growth potential. For patients unsuitable for the Rex shunt, living donor liver transplantation provides a definitive cure, achieving five-year survival rates exceeding 90%[2]. Overall, both the Rex shunt and liver transplantation improve long-term outcomes in children with CTPV by reestablishing physiological portal circulation. Purpose：This study aims to summarize the clinical efficacy and institutional experience in the management of pediatric portal vein cavernous transformation.Methods：A retrospective analysis was conducted on seven children with portal vein cavernous transformation treated at the Department of Pediatric Surgery, First Affiliated Hospital of Xinjiang Medical University, between December 2021 and April 2024. The cohort included four boys and three girls, with ages ranging from 5 years and 10 months to 12 years. All patients had a history of esophagogastric variceal bleeding and hypersplenism. Preoperative evaluations included portal vein color Doppler ultrasonography, abdominal computed tomography angiography (CTA) to assess the portal venous system anatomy and blood flow dynamics. Following a rigorous assessment, six patients underwent living donor liver transplantation, and one patient underwent Rex shunt surgery.Results：All seven surgeries were successfully completed. During a follow-up period ranging from 12 to 46 months, no episodes of gastrointestinal bleeding were observed in any patient. Among the six patients who underwent liver transplantation, no cases of graft rejection, arterial complications, or biliary complications were reported. Postoperatively, all seven patients demonstrated a significant reduction in portal vein pressure and improvement in pancytopenia compared to preoperative values (P < 0.05).Conclusions：While the Rex shunt remains the gold standard for the treatment of portal vein cavernous transformation , living donor liver transplantation provides a viable alternative for patients unsuitable for Rex shunt reconstruction.
Keywords: Portal vein cavernous transformation, Liver Transplantation, Rex shunt, Vascular reconstruction, pediatric
Received: 12 May 2025; Accepted: 27 Jun 2025.
Copyright: © 2025 Chen, Liu, Wanfu, Maimaiti, Maimaijiang, Jiayilawu, Habuding, Xi, Wang, Huxitaer and Song. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Li Wanfu, First Affiliated Hospital of Xinjiang Medical University, Urumqi, China
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