Retinopathy of prematurity in oculocutaneous albinism

Abstract

We report a case of retinopathy of prematurity (ROP) in an infant with oculocutaneous albinism (OCA), with the challenges faced in diagnosis, and subsequent management. Poor fundus contrast and blanching of retinal vessels on indentation caused significant visualization problems in detection of ridge and extraretinal vessel proliferation. Careful examination revealed zone 2 Stage 3 ROP with preplus disease in both eyes. Laser photocoagulation was attempted, but laser uptake was poor. The disease regressed over 3-week close follow-up. ROP along with OCA is a rare finding. There is a need for high index of suspicion and caution while screening and managing such babies.

Keywords: Hypopigmented fundus; oculocutaneous albinism; retinopathy of prematurity.

Figure 1

(a) Fundus photograph of the right eye showing hypopigmented retina along with easily visible large choroidal blood vessels, zone 2 retinopathy of prematurity with the presence of brushfire like neovascularization extending into the vitreous depicting Stage 3 disease (black arrows); (b) fundus photograph of the left eye showing faint ridge (black arrows) in zone 2 with avascular retina anterior to the ridge

Figure 2

(a) Post-laser fundus photograph of the right eye revealing the disappearance of the ridge along with regressed retinopathy of prematurity; (b) Post-laser fundus photograph of left eye showing faintly visible laser scars in the temporal avascular retina with regressed disease (black arrows)