Sympathetic Ophthalmia in VATER Association Combined with Persisting Hyperplastic Primary Vitreous after Cyclodestructive Procedure

Abstract

Purpose

To report on the occurrence of histology-proven sympathetic ophthalmia in a patient with VATER association and persisting hyperplastic primary vitreous (PHPV) after a cyclodestructive procedure was performed to treat secondary angle-closure glaucoma.

Methods

The left eye of a 13-year-old boy with VATER association was microphthalmic from birth and had been diagnosed with PHPV at age 1 year. It developed iris neovascularization and secondary angle-closure glaucoma, which was treated by combined cyclocryocoagulation and cyclophotocoagulation. Six weeks later, a bilateral fibrinous iritis developed. Despite intensive topical and systemic steroid treatment, the iritis persisted so that the left blind eye was enucleated.

Results

Histology of the enucleated eye showed a marked intraocular inflammation with lymphocytes, epithelioid cells, and multinuclear giant cells grouped around remnants of melanin-bearing cells.

Conclusions

Sympathetic ophthalmia may occur in patients with VATER association and PHPV after a secondary angle-closure glaucoma is treated by a combined cyclocryocoagulation and cyclophotocoagulation.