Branch retinal artery occlusion in a young patient with Mayer-Rokitansky-Küster-Hauser type 2 syndrome.

Retinal vascular occlusions are rare in young people, and any occlusion warrants an extensive clinical evaluation to establish the etiology. Cardiac malformations are a source of embolism. We present a case of atrial septal defect (ASD) in a patient with type II Mayer-Rokitansky-Küster-Hauser syndrome, leading to unilateral branch retinal arterial occlusion (BRAO) in a young woman. A 21-year-old woman presented with sudden, painless, blurring of vision, and a superior visual field defect in her right eye. A fundus examination confirmed the BRAO diagnosis. Ocular and systemic evaluation revealed primary amenorrhea, ASD, ectopic kidneys, and pelvic cyst. Any vascular occlusion in a young patient must be thoroughly evaluated, including a detailed systemic history.