Spontaneous separation of epiretinal membrane in a child with Stargardt macular dystrophy Mehmet Çakır, MD, Osman Çekiç, MD, PhD, S ¸ ükrü Bayraktar, MD, and Ö. Faruk Yılmaz, MD We demonstrate optical coherence tomography findings of sponta- neous separation of an epiretinal membrane associated with Star- gardt macular dystrophy in a child. A period of observation seems appropriate in eyes with an epiretinal membrane associated with Stargardt’s disease, as the membrane may show spontaneous re- lease and the macular area may improve. E piretinal membranes (ERM) grow on the surface of the internal limiting membrane and are a very uncommon condition in children. 1-3 Children usu- ally experience secondary ERM due to combined hamar- tomas, ocular toxoplasmosis, Coats’ disease, ocular trauma, or pars planitis. 4 Spontaneous separation or peeling of the ERM is a rare occurrence in adult eyes, and it has also been reported in a few children. 1,3,5-7 Case Report A 15-year-old boy presented with a bilateral gradual wors- ening of vision for the last 5 years. There was no history of ocular trauma, inflammation, or prior ocular surgery. Vi- sual acuity was reduced to 20/100 in the right and 20/200 in the left eye. Slit-lamp examination of the anterior seg- ment was unremarkable, and intraocular pressures were 12 mmHg bilaterally. Fundus examination disclosed macular atrophy without any flecks at both posterior poles. Optic disks had temporal pallor. The patient had a dense gray- white ERM with slightly distorted retinal vessels and fovea in the left eye. Peripheral retinal examination was normal. The vitreous was clear. A posterior vitreous detachment was not noted. Fluorescein angiography showed early hyperfluores- cence in the macula of the left eye. Electroretinography revealed normal a-wave and reduced b-wave amplitudes and normal a- and b-wave latency in the right eye, while in the left eye there were reduced amplitudes of the a- and b-waves as well as delayed latency of the b-waves compared with normal values (for subjects older than 1 year of age) of our electrophysiology laboratory. Electro-oculography test- ing was not done. Clinical, angiographic, and electrophysi- ologic test findings supported the diagnosis of Stargardt macular dystrophy with some peripheral cone or cone-rod degeneration. Optical coherence tomography (OCT ) revealed a thick macula and an ERM anchored in a parafoveal circle around the fovea with a total retinal and ERM height of 452 m, as well as vitreomacular traction in the left eye (Figure 1A). Central macular thickness was measured at 175 m in the right eye. Four months after initial presen- tation, OCT (Figure 1B) and color fundus photographs of the left eye (Figure 2) demonstrated spontaneous release of the ERM from the retinal interface and reduced central macular thickness of 140 m. Visual acuity in the left eye spontaneously improved to 20/100. Discussion Spontaneous separation or peeling of idiopathic 1,3,6 or secondary 5 ERM is a rare occurrence in children. Al- Author affiliations: Kuledibi Beyoglu Eye Training and Research Hospital, Istanbul, Turkey This case report was submitted for presentation in 25th Annual Meeting of American Society of Retina Specialists, Indian Wells, California. The authors have no conflicts of interest to declose. Submitted June 11, 2007. Revision accepted July 23, 2007. Published online October 26, 2007. Reprint requests: Mehmet Çakır, MD, Kuledibi Beyoglu Eye Training and Research Hospital, Istanbul, Turkey (email: drmcakir@hotmail.com). J AAPOS 2007;11:618-619. Copyright © 2007 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/2007/$35.00 + 0 doi:10.1016/j.jaapos.2007.07.004 FIG 1. Optical coherence tomography (OCT ) images of the left eye of a 15-year-old boy with Stargardt disease. (A) OCT showing thickened central macula attached to dense epiretinal membrane anchored in a parafoveal circle around the fovea and vitreomacular traction. (B) OCT in the same eye demonstrating spontaneous release of epiretinal membrane from the retinal interface and reduced central macular thickness as well as formation of foveal pit over a period of 4 months. Journal of AAPOS 618