Combined anticoagulant deficiency causing extrahepatic portal venous obstruction: A case report

doi:10.12998/wjcc.v14.i7.117431

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World Journal of Clinical Cases

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©Author(s) (or their employer(s)) 2026. No commercial re-use. See Permissions. Published by Baishideng Publishing Group Inc.

World J Clin Cases. Mar 6, 2026; 14(7): 117431
Published online Mar 6, 2026. doi: 10.12998/wjcc.v14.i7.117431

Combined anticoagulant deficiency causing extrahepatic portal venous obstruction: A case report

Gopal Krishna Dhali, Jyotirmoy Biswas, Prithviraj Singh, Arijit Sinha, Rick Maity, Arkadeep Dhali

Arkadeep Dhali, Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S5 7AU, United Kingdom

Arkadeep Dhali, School of Medicine and Population Health, University of Sheffield, Sheffield S10 2HQ, United Kingdom

Arkadeep Dhali, Deanery of Clinical Sciences, University of Edinburgh, Edinburgh EH16 4SB, United Kingdom

Arkadeep Dhali, Faculty of Medicine, Imperial College London, London SW7 2AZ, United Kingdom

Rick Maity, School of Medical Science and Technology, Indian Institute of Technology Kharagpur, Kharagpur 721302, India

Arijit Sinha, Department of General Medicine, Medical College and Hospital, Kolkata 700073, India

Prithviraj Singh, Gopal Krishna Dhali, Department of Gastroenterology, Institute of Post Graduate Medical Education and Research, Kolkata 700020, India

Jyotirmoy Biswas, Department of Ophthalmology, Barasat Government Medical College and Hospital, Barasat 700124, India

Co-first authors: Arkadeep Dhali and Rick Maity.

Author contributions: Dhali A and Maity R conceptualized the article, conducted the literature review, and wrote the primary manuscript; Sinha A conducted the literature review and wrote the primary manuscript; Singh P and Biswas J wrote the primary manuscript; Dhali GK supervised the work and wrote the revised manuscript; All authors read and approved the final manuscript. Dhali A took a thorough clinical history, conducted a thorough workup, and prepared the first draft of the manuscript. Maity R was responsible for collection of clinical data, blood specimens, and prepared the first draft of the manuscript. Both authors made crucial and indispensable contributions towards the completion of the project and thus qualified as the co-first authors of the paper.

Informed consent statement: Informed consent was taken from the patient for anonymous publication of the case report.

Conflict-of-interest statement: All 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).

Corresponding author: Rick Maity, MBBS, School of Medical Science and Technology, Indian Institute of Technology Kharagpur, Ardeshir Dalal Avenue, Kharagpur 721302, India. rickmaity98@gmail.com

Received: December 8, 2025
Revised: January 13, 2026
Accepted: February 2, 2026
Published online: March 6, 2026
Processing time: 88 Days and 15.9 Hours

Abstract

BACKGROUND

Extrahepatic portal venous obstruction is a major cause of non-cirrhotic portal hypertension in children and young adults. It most commonly results from portal vein thrombosis. While prothrombotic states are recognized contributors, combined deficiencies of natural anticoagulants, such as protein C, protein S, and antithrombin III, are exceedingly rare and pose diagnostic and therapeutic challenges.

CASE SUMMARY

A 27-year-old male presented with an episode of melena and had a background of portal cavernoma cholangiopathy and recurrent variceal bleeding since infancy. Previously, he underwent endoscopic sclerotherapy and proximal lienorenal shunt surgery and later developed shunt thrombosis and ischemic biliary strictures requiring Roux-en-Y hepaticojejunostomy. Upper gastrointestinal endoscopy (UGIE) revealed large esophageal and gastric varices that were managed by endoscopic variceal ligation and sclerotherapy. Following the UGIE, he suffered a seizure with deterioration of sensorium, followed by melena and spikes in temperature. Repeat UGIE revealed no active bleeding source and confirmed obliteration of the previously detected varices. Evaluation identified deficiencies in protein S, protein C, and antithrombin III, for which anticoagulation with enoxaparin was initiated. Despite recent sclerotherapy, anticoagulation precipitated melena. Sigmoidoscopy and capsule endoscopy revealed portal hypertensive colopathy with rectal varices. This was managed surgically by creating mesocaval shunts after which enoxaparin was restarted. No further recurrence of bleeding occurred; the patient was discharged in a stable condition.

CONCLUSION

This case highlighted the importance of thorough coagulation profiling in extrahepatic portal venous obstruction, particularly in young patients with recurrent thrombotic events. Clinicians must navigate the precarious balance between thrombosis prevention and hemorrhage risk.

Keywords: Extrahepatic portal venous obstruction; Portal vein; Portal vein thrombosis; Protein S deficiency; Protein C deficiency; Antithrombin III deficiency; Case Report

Core Tip: Extrahepatic portal venous obstruction (EHPVO) is a leading cause of non-cirrhotic portal hypertension in young individuals. It is linked to procoagulable states; however, combined anticoagulant deficiencies are rare. This case described a 27-year-old adult with long-standing EHPVO complicated by recurrent thrombotic and hemorrhagic events in the setting of combined deficiencies of protein S, protein C, and antithrombin III. It highlighted the diagnostic uncertainty in distinguishing between acquired and inherited anticoagulant deficiencies in chronic portal vein thrombosis and underscored the clinical challenge of balancing anticoagulation against the risk of bleeding in EHPVO.