Rare presentation of Behçet's disease in a 22-year-old male with combined retinal artery and vein occlusions.

PURPOSE: To demonstrate how the systemic work-up of a combined retinal and vein occlusion (CRAVO) led to a diagnosis of Behcet's disease.

OBSERVATIONS: A 22-year-old male presented with sudden, profound vision loss in the left eye upon waking up. Presenting visual acuity was no light perception (NLP) in the left eye and fundus examination revealed findings consistent with both central retinal artery and vein occlusion. Laboratory workup for common infectious etiologies was negative; however, inflammatory markers were elevated. Further testing revealed human leukocyte antigen (HLA)∗B51 positivity as well as a patent foramen ovale (PFO) that was found on echocardiogram. The patient was initiated on high dose steroids and immunosuppressants for Behçet's Disease and underwent surgical closure of PFO.

CONCLUSIONS AND

IMPORTANCE

This study highlights the value of a proper diagnostic approach in patients presenting with CRAVO without suspected risk factors. Investigation of autoimmune, genetic, cardiologic, and inflammatory diseases led to the diagnosis of Behçet's Disease, an uncommon condition where timely recognition and early treatment can prevent further vision loss and reduce the risk of future vascular events.