Downhill oesophageal variceal bleeding: A rare complication in Behçet's disease-related superior vena cava syndrome

Arab J Gastroenterol. 2015 Mar;16(1):36-8. doi: 10.1016/j.ajg.2015.02.003. Epub 2015 Mar 17.

Abstract

Behçet's disease (BD) is a multisystemic disorder that involves vessels of all sizes. Superior vena cava (SVC) thrombosis is a rare complication that can lead to the development of various collateral pathways. A 31-year-old man presented with SVC syndrome. He had a history of recurrent genital aphthosis. Computed tomography revealed extensive thrombosis of the right internal jugular, axillary, and subclavian veins with collateral circulation. The patient was diagnosed with BD, and he was started on anticoagulation and immunosuppressive therapy. One week later, he presented with haematemesis. Upper gastrointestinal endoscopy disclosed varices in the upper third of the oesophagus with stigmata of recent bleeding. Portal hypertension was ruled out. Anticoagulation therapy was discontinued. He was discharged on immunosuppressive therapy. Bleeding from downhill oesophageal varices should be suspected in any patient presenting with upper gastrointestinal bleeding and a history of SVC syndrome due to BD.

Keywords: Behçet’s disease; Gastrointestinal bleeding; Oesophageal varices; Superior vena cava syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Anticoagulants / therapeutic use
  • Behcet Syndrome / complications*
  • Behcet Syndrome / diagnosis
  • Behcet Syndrome / drug therapy*
  • Esophageal and Gastric Varices / etiology*
  • Hematemesis / etiology*
  • Hematemesis / physiopathology
  • Hematemesis / therapy
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Rare Diseases
  • Risk Assessment
  • Severity of Illness Index
  • Superior Vena Cava Syndrome / complications*
  • Superior Vena Cava Syndrome / diagnosis
  • Treatment Outcome

Substances

  • Anticoagulants
  • Immunosuppressive Agents