Primary Congenital Ectropion uveae Associated with Vitreoretinal Degeneration

Abstract

Primary congenital ectropion uveae is an extremely rare ocular malformation frequently associated with unilateral glaucoma. We report on a 15-year-old boy with unilateral congenital ectropion uveae, glaucoma and transvitreal strands in an optically empty vitreous. Dark adaptation was normal, but scotopic ERG showed subnormal b-wave amplitudes in the affected eye, which is a typical finding in hereditary vitreoretinal degenerations. The coincidence of primary congenital ectropion uveae and unilateral vitreoretinal degeneration without a family history seems to be sporadic and very extraordinary but could be due to a common defect of maturation.

Bilateral juvenile glaucoma with iridotrabecular dysgenesis, congenital ectropion uveae, and thickened corneal nerves

“…Ritch et al 2 found glaucoma in seven out of eight cases of CEU, Dowling et al 3 in eight of nine cases, and it was also noted frequently in other reported cases of CEU. 1,4,6,7 The onset of glaucoma is usually in childhood or early adolescence as was seen in cases in this report but cases with late onset also have been reported. 3,5,6 IOP shows an initial decrease when the medication is administered but rises again soon after and filtering surgery is required in most of the cases.…”

“…[1][2][3][4][5][6][7] Not all cases have systemic associations, however. None of the nine patients reported by Dowling et al 3 had any systemic associations.…”

Glaucoma management in congenital ectropion uveae: Surgical outcomes from a large tertiary referral center in South India

“…In CEU associated with juvenile glaucoma, filtering procedures with antifibrotics have been a preferred approach by several investigators. [28][29][30][32][33][34][35][36] We performed trabeculectomy with or without MMC in several children with good long-term IOP control and preservation of visual function. Our results are in accordance with the published reports in the literature.…”

Bilateral, presumed congenital ectropion uveae in a patient with pathologic myopia

“…A similar case of unilateral CEU in a patient with vitreoretinal degeneration (SE, −6.0 diopter) has been described. 3 Although it is tempting to hypothesize how CEU can be associated with pathologic myopia, an underlying unifying abnormality that connects these two findings has not been identified.…”