Familial Exudative Vitreoretinopathy-Associated Secondary Ocular Hypertension and Glaucoma: Clinical Features and...

To report clinical characteristics and treatment outcomes of ocular hypertension (OHT) and glaucoma in familial exudative vitreoretinopathy (FEVR).

This retrospective chart review included patients diagnosed as having FEVR and OHT or glaucoma at Siriraj Hospital, Bangkok, Thailand, between June 2001 and May 2021. Demographics, clinical features, treatments, and outcomes were collected.

Among 93 patients (174 eyes) with FEVR, 6 patients (5.2%; 9 eyes, 3 bilateral cases) developed secondary OHT (6 eyes) and glaucoma (3 eyes), and all had bilateral FEVR stage 3A or greater. Median age at FEVR diagnosis was 4 months (interquartile range [IQR]: 3 to 23 months), and median age at OHT and glaucoma diagnosis was 43 months (IQR: 15 to 52 months). All eyes presented with elevated intraocular pressure (IOP). Treatment outcomes showed stable visual acuity in stage 3A; in stage 4A or higher, 3 eyes remained stable and 2 developed phthisis bulbi. OHT and glaucoma were controlled with one to two topical medications in all patients except one with a shallow anterior chamber, who required peripheral iridotomy. Median intraocular pressure decreased from 30 mmHg at presentation (IQR: 25.5 to 37.5 mmHg; n = 9) to 16 mmHg at last visit (IQR: 14 to 17.5 mmHg; n = 5). None of the eyes required glaucoma surgery. No patient experienced ocular pain.

Secondary OHT and glaucoma is a late complication of advanced FEVR and may present as asymptomatic IOP elevation. IOP control is achievable with topical therapy or peripheral iridotomy in a shallow anterior chamber; however, visual prognosis is largely stage dependent.