Liu XC, Yan HH, Wei W, Du Q. Idiopathic portal hypertension misdiagnosed as hepatitis B cirrhosis: A case report and review of the literature. World J Hepatol 2025; 17(2): 100923 [DOI: 10.4254/wjh.v17.i2.100923]
Corresponding Author of This Article
Qin Du, Chief Physician, Department of Gastroenterology, The Second Affiliated Hospital, Zhejiang University School of Medicine, No. 88 Jiefang Road, Hangzhou 310009, Zhejiang Province, China. duqin@zju.edu.cn
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Hepatol. Feb 27, 2025; 17(2): 100923 Published online Feb 27, 2025. doi: 10.4254/wjh.v17.i2.100923
Idiopathic portal hypertension misdiagnosed as hepatitis B cirrhosis: A case report and review of the literature
Xiao-Chen Liu, Hui-Hui Yan, Wei Wei, Qin Du
Xiao-Chen Liu, Hui-Hui Yan, Wei Wei, Qin Du, Department of Gastroenterology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310009, Zhejiang Province, China
Co-corresponding authors: Wei Wei and Qin Du.
Author contributions: Liu XC and Yan HH contributed to manuscript writing and editing, and data collection; Wei W and Du Q contributed to conceptualization and supervision, both authors contributed equally to this article, and are co-first authors of this manuscript; All authors have read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Qin Du, Chief Physician, Department of Gastroenterology, The Second Affiliated Hospital, Zhejiang University School of Medicine, No. 88 Jiefang Road, Hangzhou 310009, Zhejiang Province, China. duqin@zju.edu.cn
Received: August 30, 2024 Revised: January 10, 2025 Accepted: January 23, 2025 Published online: February 27, 2025 Processing time: 173 Days and 19.6 Hours
Abstract
BACKGROUND
Idiopathic portal hypertension (IPH) is a subtype of portal hypertension that arises in the absence of cirrhosis. IPH frequently manifests with clinical features typical of portal hypertension, including splenomegaly and esophagogastric fundal varices, along with other associated symptoms. Imaging studies may indicate portal hypertension; however, they typically do not provide evidence of cirrhosis. There are no standardized diagnostic criteria for IPH, and diagnosis is often established by excluding other hepatic diseases. Liver biopsy remains the most reliable approach to verify the diagnosis of IPH.
CASE SUMMARY
A patient previously diagnosed with “hepatitis B cirrhosis” at an external hospital presented to our facility with gastrointestinal bleeding. Initial assessment revealed minor liver injury, splenomegaly, esophagogastric varices, and portal hypertension. Imaging studies did not indicate cirrhosis and repeated hepatitis B serology tests yielded negative results. After excluding various causes of cirrhosis and other non-cirrhotic etiologies of portal hypertension, liver biopsy confirmed the diagnosis of IPH. The patient was managed with regular endoscopic therapy and long-term carvedilol administration.
CONCLUSION
Currently, there are no standardized diagnostic criteria for IPH, and its diagnosis is generally established by excluding other conditions. Liver biopsy remains the most reliable method for IPH diagnosis.
Core Tip: Idiopathic portal hypertension (IPH) is challenging to diagnose due to its rarity and non-specific clinical presentation. This report describes the case of a 69-year-old female with recurrent hematemesis, initially misdiagnosed with viral hepatitis B cirrhosis. Subsequent evaluation revealed IPH with esophageal and gastric varices. The diagnostic challenge was compounded by the absence of specific biomarkers and imaging findings, making liver biopsy essential. Treatment primarily involves symptomatic management and endoscopic intervention. The patient demonstrated significant improvement following treatment, highlighting the importance of accurate diagnosis and tailored management. This case underscores the complexities of diagnosing and managing IPH, emphasizing the need for further research to improve diagnostic accuracy and treatment efficacy in this rare disorder.
