Bilateral Vasoproliferative Tumors in Usher Syndrome

Vasoproliferative tumors (VPTs) can be associated with retinal dystrophy, but there are few well-documented cases and even fewer linked to Usher syndrome. Most reported cases are unilateral. The purpose of this report was to describe a case of bilateral sequential VPT in the context of MYO7A-related Usher syndrome.

A 13-year-old female presented with pain in her left eye and no light perception in that eye. She was found to have left eye advanced neovascular glaucoma related to VPT in the context of MYO7A-related Usher syndrome. Intraocular pressure and pain were controlled with external diode cyclodestruction. Almost 3 years later, early asymptomatic VPT was noted in the right eye during follow-up and was treated with laser photocoagulation.

Although VPT is typically unilateral, affected patients need careful follow-up for possible bilateral sequential disease. Early treatment avoids blinding complications.