Xu MJ, Wei X, Huang Y, Lu Y, Sun L, Xie Y, Li MH. Congenital porto-sinusoidal vascular disorder-induced portal hypertension: A comprehensive review based on a classic clinical case. World J Hepatol 2026; 18(5): 117184 [DOI: 10.4254/wjh.v18.i5.117184]
Corresponding Author of This Article
Ming-Hui Li, Department of Hepatology II, Beijing Ditan Hospital, Capital Medical University, Peking University Ditan Teaching Hospital, No. 8 Jingshun East Street, Chaoyang District, Beijing 100015, China. wuhm2000@sina.com
Research Domain of This Article
Gastroenterology & Hepatology
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review-article
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Xu MJ, Wei X, Huang Y, Lu Y, Sun L, Xie Y, Li MH. Congenital porto-sinusoidal vascular disorder-induced portal hypertension: A comprehensive review based on a classic clinical case. World J Hepatol 2026; 18(5): 117184 [DOI: 10.4254/wjh.v18.i5.117184]
World J Hepatol. May 27, 2026; 18(5): 117184 Published online May 27, 2026. doi: 10.4254/wjh.v18.i5.117184
Congenital porto-sinusoidal vascular disorder-induced portal hypertension: A comprehensive review based on a classic clinical case
Meng-Jiao Xu, Xin Wei, Yu Huang, Yao Lu, Lei Sun, Yao Xie, Ming-Hui Li
Meng-Jiao Xu, Xin Wei, Yao Lu, Yao Xie, Department of Hepatology II, Beijing Ditan Hospital, Capital Medical University, Beijing 100015, China
Yu Huang, Department of Gastroenterology I, The People’s Hospital of Chuxiong Yi Autonomous Prefecture, Chuxiong Yi Autonomous Prefecture 675000, Yunna Province, China
Lei Sun, Department of Pathology, Beijing Ditan Hospital, Capital Medical University, Peking University Ditan Teaching Hospital, Beijing 100015, China
Ming-Hui Li, Department of Hepatology II, Beijing Ditan Hospital, Capital Medical University, Peking University Ditan Teaching Hospital, Beijing 100015, China
Co-first authors: Meng-Jiao Xu and Xin Wei.
Co-corresponding authors: Yao Xie and Ming-Hui Li.
Author contributions: Xu MJ and Wei X wrote the main manuscript text and prepared table, and they contributed equally to this manuscript as co-first authors; Xu MJ, Wei X, and Sun L prepared figure; Huang Y, Lu Y, Xie Y, and Li MH approved the submitted version after modification; Xie Y and Li MH edited the manuscript, and they contributed equally to this manuscript as co-corresponding authors. All authors made a significant contribution to the work reported and read and approved the final manuscript.
AI contribution statement: No AI tools were used for language polishing, translation, data analysis, or writing assistance of the manuscript. No images in the manuscript were generated by AI.
Supported by Beijing Municipal Health Commission High-Level Public Health Technical Personnel Construction Project, No. Discipline Leader-03-26; Beijing Hospitals Authority Clinical Medicine Development of Special Funding Support, No. ZLRK202301; Beijing Hospitals Authority “Peak” Talent Training Program, No. DFL20241803; and National Key Research and Development Program, No. 2023YFC2306901 and No. 2023YFC2308105.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
Corresponding author: Ming-Hui Li, Department of Hepatology II, Beijing Ditan Hospital, Capital Medical University, Peking University Ditan Teaching Hospital, No. 8 Jingshun East Street, Chaoyang District, Beijing 100015, China. wuhm2000@sina.com
Received: December 1, 2025 Revised: January 11, 2026 Accepted: February 26, 2026 Published online: May 27, 2026 Processing time: 176 Days and 18 Hours
Abstract
There are still debates on the pathogenesis and classification of congenital porto-sinusoidal vascular disorder (PSVD) with portal hypertension, which makes numerous challenges in treatment. This review provides a focused exploration of the disease, highlighting its clinical implications through a detailed case study. We offer a comprehensive on aspects of underlying pathophysiology, epidemiological trends, diagnostic methodologies, clinical therapeutic and management strategies of PSVD, furthermore critically examines ongoing controversies within the field and suggests future research directions as well. We present a special case of congenital PSVD in a 15-year-old child. In the treatment of clinical cases of PSVD, it is essential for comprehensive synthesis of the disease, formulate an individualized treatment plan, and closely monitor the patient’s biochemical indices and clinical symptoms to ensure treatment efficacy and prognosis.
Core Tip: Our review initiated a comprehensive exploration of congenital porto-sinusoidal vascular disorder through a representative clinical case. By incorporating recent and relevant findings, this review aims to deepen the understanding of porto-sinusoidal vascular disorder in etiology, pathology, and treatment approaches, in order to enhance both clinical decision-making and the framework for future investigative efforts in this complex condition.
