Bilateral spontaneous dislocation of posterior chamber intraocular lens in a patient with gyrate atrophy.

We report a patient with gyrate atrophy, a rare metabolic disease, who had bilateral late spontaneous posterior dislocation of in-the-bag posterior chamber intraocular lens (PCIOL). He underwent pars plana vitrectomy, PCIOL retrieval and anterior chamber intraocular lens implantation in both eyes. This report may imply that patients with gyrate atrophy are at risk for spontaneous dislocation of intraocular lenses.

Retinal detachment and gyrate atrophy of the choroid and retina: case report / Descolamento de retina e atrofia girata da coroide e retina: relato de caso

To report a case of gyrate atrophy of the choroid and retina associated with retinal detachment. Hyperornithinemia confirmed the diagnosis of gyrate atrophy. Pars plana vitrectomy with silicone oil infusion was performed with good anatomical results, despite the persistence of low visual acuity. Retinal detachment is a rare complication of gyrate atrophy and can be managed with pars plana vitrectomy and silicone oil. We discuss the possible mechanisms that led to low visual acuity.

Descrever um caso de atrofia girata da coroide e retina associado com descolamento de retina. Altos níves de ornitina sérica confirmaram o diagnóstico de atrofia girata. Vitrectomia via pars plana com infusão de óleo de silicone foi realizada, com bom resultado anatômico, apesar da baixa acuidade visual persistente. Descolamento de retina é uma rara complicação da atrofia girata e pode ser manejada com vitrectomia via pars plana e óleo de silicone. Discutiremos os possíveis mecanismos que levaram à baixa acuidade visual.

Macular edema associated with gyrate atrophy managed with intravitreal triamcinolone: a case report

PURPOSE: To describe the use of 4 mg intravitreal triamcinolone acetonide (IVTA) for gyrate atrophy-related macular edema (ME) and to report anatomic and functional outcomes, during a nine-month period. CASE REPORT: A 27-year-old female complained of decreased vision since diagnosis of gyrate athrophy (GA), six years before admission. At presentation visual acuity was 20/100 in OD and 20/80 in OS. Ophthalmological examination disclosed significant cataract in OD, pseudophakia in OS and typical GA findings. Fluorescein angiography (FA) disclosed ME that was confirmed by optical coherence tomography (OCT), which also showed subfoveal fluid. OS was treated with a 4-mg IVTA injection. One month later, vision improved to 20/50+1 and foveal thickness decreased, with less leakage in FA. This picture was maintained up to six months, when there was recurrence of ME to a level similar to the baseline. At nine months, visual acuity dropped to 20/80, and ME was maintained, with remodeling in macular profile. CONCLUSION:There is a transient therapeutic effect with 4-mg IVTA injection for GA-related ME. After drug clearance, edema recurs, with return of visual acuity to pretreatment level.

OBJETIVO: Descrever o uso de acetonida de triancinolona intravítrea (TAIV) em caso de edema macular (EM) associado a atrofia girata (AG). RELATO DE CASO: Paciente de 27 anos, do sexo feminino, queixava-se de baixa de visão desde o diagnóstico de AG, há seis anos. À admissão, apresentava acuidade visual corrigida de 20/100 no OD e 20/80 no OE. Exame oftalmológico revelava catarata significativa no OD, pseudofacia no OE e achados típicos de AG. Angiografia fluoresceínica (AFG) mostrou EM, confirmado pela tomografia de coerência óptica (OCT), que também revelou líquido subfoveal. Foi então realizada injeção de 4 mg de TAIV no OE. Após um mês, a visão melhorou para 20/50+1 e a espessura foveal se reduziu, com menos extravasamento à AFG. Esse quadro foi mantido até os seis meses, quando houve recorrência do edema macular em nível semelhante ao inicial. Aos nove meses, a visão retornou a 20/80 e o edema se manteve, com remodelamento no perfil macular. CONCLUSÃO: A injeção de 4 mg de TAIV tem efeito transitório no EM associado a AG. Após a eliminação da droga, há recorrência do EM, com retorno da visão aos níveis pré-tratamento.

Gyrate atrophy of choroid and retina.

Gyrate atrophy of choroid and retina is a rare disorder of autosomal recessive nature. There occurs patchy and progressive atrophy of the choroid and retina at the equatorial region with central area being less affected. Here in this case report, one woman of about 47 years attended at the retina clinic, Tenennt Institute of Ophthalmology, Glasgow University with the history of gradual loss of vision. On fundus examination, sharply defined bizarre shaped atrophic areas of fundus was seen in both the eyes. Velvet like fine granular pigments were present in the macula, the zone of healthy retina and the periphery. The colourless, elongated, glittering crystals were scattered over the dark brown pigments visible through 90 dioptre lens. Bone corpuscles pigments were not found. Fluorescein angiography showed hyperfluorescence in the area of gyrate atrophy. Her plasma ornithine level and plasma tiramine level were 1 90 U mol/l and 357 U mol/l. respectively. A rigid schedule of low protein diet including near total elimination of arginine with supplementation of essential amino acids was advised since the diagnosis was established.