title: 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.
authors.affiliation: Department of Ophthalmology, University of Cologne, Germany.
authors.forename: T S
authors.initials: TS
authors.lastname: Dietlein
authors.forename: P C
authors.initials: PC
authors.lastname: Jacobi
authors.forename: G K
authors.initials: GK
authors.lastname: Krieglstein
mesh_terms: D000293:Adolescent; D003623:Dark Adaptation; D004483:Ectropion / Q000150:complications / Q000151:congenital* / Q000175:diagnosis; D004585:Electrooculography; D004596:Electroretinography; D015785:Eye Diseases, Hereditary / Q000150:complications / Q000503:physiopathology; D005901:Glaucoma / Q000150:complications / Q000151:congenital / Q000503:physiopathology; D006801:Humans; D007429:Intraocular Pressure; D007498:Iris / Q000002:abnormalities*; D007499:Iris Diseases / Q000150:complications / Q000151:congenital* / Q000175:diagnosis; D008297:Male; D010375:Pedigree; D012162:Retinal Degeneration / Q000150:complications* / Q000151:congenital / Q000503:physiopathology; D014822:Vitreous Body / Q000002:abnormalities