This previously healthy 7YO boy presented with asymptomatic retinal findings. Vision was 20/25 in his normal OD and 20/25 OS.
Color photography shows a diffuse network of dilated, tortuous vessels involving the optic disc and posterior pole, extending into the superonasal and temporal midperiphery. Vascular sheathing is noted in the temporal periphery as well. OCT scanning through the disc and nasal macula shows these hyperreflective vessels to variably extend through the full-thickness retina with posterior shadowing. The internal limiting membrane is suspended between two of these lesions.
Learning Points:
A retinal arteriovenous malformation (AVM) is a congenital connection between an artery and vein without an intervening capillary network. These lesions appear along a spectrum ranging from small, isolated lesions to the large ‘bag of worms’ appearance. These more extensive AVMs are usually part of Wyburn-Mason Syndrome, which includes ocular and midbrain vascular lesions. Although retinal AVMs are usually stable lesions, bleeding or secondary venous occlusions may occur.
MRI scanning revealed an intracranial AVM, and the patient was referred for neurosurgical evaluation.
