Li M, Zhang LK, Qu L, Liu JH, Liu JJ, Sun SZ, Zhang D, Hao NB, Li CZ. Absent inferior vena cava combined with idiopathic noncirrhotic portal hypertension resulting esophagogastric variceal bleeding: A case report. World J Gastrointest Endosc 2026; 18(5): 119573 [DOI: 10.4253/wjge.v18.i5.119573]
Corresponding Author of This Article
Chang-Zheng Li, MD, Department of Gastroenterology, Chinese People’s Liberation Army Rocket Force Characteristic Medical Center, No. 16 Xinjiekouwai Street, Xicheng District, Beijing 100088, China. licz007@aliyun.com
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/
May 16, 2026 (publication date) through May 12, 2026
Times Cited of This Article
Times Cited (0)
Journal Information of This Article
Publication Name
World Journal of Gastrointestinal Endoscopy
ISSN
1948-5190
Publisher of This Article
Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA
Share the Article
Li M, Zhang LK, Qu L, Liu JH, Liu JJ, Sun SZ, Zhang D, Hao NB, Li CZ. Absent inferior vena cava combined with idiopathic noncirrhotic portal hypertension resulting esophagogastric variceal bleeding: A case report. World J Gastrointest Endosc 2026; 18(5): 119573 [DOI: 10.4253/wjge.v18.i5.119573]
World J Gastrointest Endosc. May 16, 2026; 18(5): 119573 Published online May 16, 2026. doi: 10.4253/wjge.v18.i5.119573
Absent inferior vena cava combined with idiopathic noncirrhotic portal hypertension resulting esophagogastric variceal bleeding: A case report
Meng Li, Lei-Kang Zhang, Lei Qu, Jin-Hong Liu, Jia-Jia Liu, Suo-Zhu Sun, Dan Zhang, Ning-Bo Hao, Chang-Zheng Li
Meng Li, Jia-Jia Liu, Dan Zhang, Ning-Bo Hao, Chang-Zheng Li, Department of Gastroenterology, Chinese People’s Liberation Army Rocket Force Characteristic Medical Center, Beijing 100088, China
Lei-Kang Zhang, Lei Qu, Department of Radiation, Chinese People’s Liberation Army Rocket Force Characteristic Medical Center, Beijing 100088, China
Jin-Hong Liu, Suo-Zhu Sun, Department of Pathology, Chinese People’s Liberation Army Rocket Force Characteristic Medical Center, Beijing 100088, China
Co-corresponding authors: Ning-Bo Hao and Chang-Zheng Li.
Author contributions: Li M, Liu JJ, and Zhang D contributed to the conception of the work, data analysis, and drafting of the manuscript; Zhang LK and Qu L were responsible for the collection, interpretation, and 3D reconstruction of the imaging data; Liu JH and Sun SZ were responsible for the acquisition and analysis of the pathological data; Hao NB and Li CZ supervised the entire project, critically revised the manuscript for important intellectual content, and serve as corresponding authors, taking responsibility for all aspects of the work; and all authors reviewed and approved the final version of the manuscript.
Informed consent statement: Written informed consent was obtained from the patient for the publication of this case report, including all clinical details and any accompanying images.
Conflict-of-interest statement: All authors declare that they have no conflict of interest to disclose.
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).
AI contribution statement: DeepSeek was used for language polishing, translation, and writing assistance, but without data analysis.
Corresponding author: Chang-Zheng Li, MD, Department of Gastroenterology, Chinese People’s Liberation Army Rocket Force Characteristic Medical Center, No. 16 Xinjiekouwai Street, Xicheng District, Beijing 100088, China. licz007@aliyun.com
Received: February 2, 2026 Revised: March 11, 2026 Accepted: March 30, 2026 Published online: May 16, 2026 Processing time: 101 Days and 18.2 Hours
Abstract
BACKGROUND
Congenital anomalies of the inferior vena cava (IVC) are rare vascular abnormalities that are often asymptomatic but may lead to long-term alterations in venous hemodynamics. Idiopathic noncirrhotic portal hypertension (INCPH), currently classified within the spectrum of porto-sinusoidal vascular disease, is characterized by portal hypertension in the absence of cirrhosis and with preserved hepatic function. The coexistence of congenital IVC anomalies and INCPH is rarely reported.
CASE SUMMARY
A 71-year-old female presented with recurrent gastrointestinal bleeding for more than one year. Endoscopic examination revealed large esophagogastric varices. Contrast-enhanced computed tomography demonstrated absence of the hepatic segment of the IVC with azygos continuation. Laboratory testing showed preserved liver function and normal platelet counts. Liver biopsy revealed obliterative portal venopathy consistent with INCPH. The patient underwent endoscopic variceal ligation and recovered without recurrent bleeding during follow-up.
CONCLUSION
This case highlights the diagnostic complexity of INCPH in patients with congenital IVC anomalies. Long-standing hemodynamic alterations associated with IVC abnormalities may contribute to intrahepatic vascular remodeling and the development of portal hypertension, although a direct causal relationship remains uncertain.
Core Tip: Inferior vena cava anomalies may induce non-cirrhotic portal hypertension by increasing portal venous inflow via abnormal hemodynamics, leading to variceal bleeding. Diagnosis requires exclusion of cirrhosis, supported by patent hepatic veins and liver biopsy showing obliterative portal venopathy. Splenomegaly/pancytopenia are not obligatory. Endoscopic therapy effectively controls bleeding, but long-term monitoring is essential.
