Atypical Presentation of Downhill Esophageal Varices: A Diagnostic Enigma

Downhill esophageal varices (DEV) are rare vascular abnormalities of the proximal esophagus, commonly caused by superior vena cava obstruction. We report a 75-year-old man who presented with intermittent dysphagia and globus sensation, in whom endoscopy demonstrated large proximal esophageal varices. Extensive evaluation excluded portal hypertension and cross-sectional imaging, and venography showed a patent superior vena cava and inferior vena cava but demonstrated right internal jugular vein occlusion with possible azygos system compression. These findings were considered remote and insufficient to explain the extensive proximal varices, which were ultimately classified as idiopathic DEV. This atypical presentation underscores the diagnostic challenges of DEV and the need for heightened clinical awareness of this rare entity.