Spontan Separation of Epiretinal Mb in Child With Stargardt
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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: [email protected]). 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
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Transcript of Spontan Separation of Epiretinal Mb in Child With Stargardt
Spontaneous separation of epiretinal membrane in achild with Stargardt macular dystrophyMehmet Çakır, MD, Osman Çekiç, MD, PhD, Sükrü 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 seemsappropriate in eyes with an epiretinal membrane associated withStargardt’s disease, as the membrane may show spontaneous re-lease and the macular area may improve.
E piretinal membranes (ERM) grow on the surface ofthe internal limiting membrane and are a veryuncommon 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 theERM is a rare occurrence in adult eyes, and it has also beenreported in a few children.1,3,5-7
Case ReportA 15-year-old boy presented with a bilateral gradual wors-ening of vision for the last 5 years. There was no history ofocular trauma, inflammation, or prior ocular surgery. Vi-sual acuity was reduced to 20/100 in the right and 20/200in the left eye. Slit-lamp examination of the anterior seg-ment was unremarkable, and intraocular pressures were 12mmHg bilaterally. Fundus examination disclosed macularatrophy without any flecks at both posterior poles. Opticdisks had temporal pallor. The patient had a dense gray-white ERM with slightly distorted retinal vessels and foveain the left eye. Peripheral retinal examination was normal.The vitreous was clear. A posterior vitreous detachmentwas not noted.
Fluorescein angiography showed early hyperfluores-cence in the macula of the left eye. Electroretinographyrevealed normal a-wave and reduced b-wave amplitudesand normal a- and b-wave latency in the right eye, while inthe left eye there were reduced amplitudes of the a- andb-waves as well as delayed latency of the b-waves compared
Author affiliations: Kuledibi Beyoglu Eye Training and Research Hospital, Istanbul,Turkey
This case report was submitted for presentation in 25th Annual Meeting of AmericanSociety 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: [email protected]).J AAPOS 2007;11:618-619.
Copyright © 2007 by the American Association for Pediatric Ophthalmology andStrabismus.
1091-8531/2007/$35.00 � 0doi:10.1016/j.jaapos.2007.07.004
618
with normal values (for subjects older than 1 year of age) ofour electrophysiology laboratory. Electro-oculography test-ing was not done. Clinical, angiographic, and electrophysi-ologic test findings supported the diagnosis of Stargardtmacular dystrophy with some peripheral cone or cone-roddegeneration.
Optical coherence tomography (OCT) revealed a thickmacula and an ERM anchored in a parafoveal circlearound the fovea with a total retinal and ERM height of452 �m, as well as vitreomacular traction in the left eye(Figure 1A). Central macular thickness was measured at175 �m in the right eye. Four months after initial presen-tation, OCT (Figure 1B) and color fundus photographs ofthe left eye (Figure 2) demonstrated spontaneous releaseof the ERM from the retinal interface and reduced centralmacular thickness of 140 �m. Visual acuity in the left eyespontaneously improved to 20/100.
DiscussionSpontaneous separation or peeling of idiopathic1,3,6 or
FIG 1. Optical coherence tomography (OCT ) images of the left eye of a15-year-old boy with Stargardt disease. (A) OCT showing thickenedcentral macula attached to dense epiretinal membrane anchored in aparafoveal circle around the fovea and vitreomacular traction. (B) OCT inthe same eye demonstrating spontaneous release of epiretinal membranefrom the retinal interface and reduced central macular thickness as wellas formation of foveal pit over a period of 4 months.
secondary5 ERM is a rare occurrence in children. Al-
Journal of AAPOS
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Volume 11 Number 6 / December 2007 Çakır et al 619
though surgical removal of ERM by vitrectomy and ERMpeeling has resulted in significant visual improvements,8
conservative short-term observation has also been advo-cated because of possible spontaneous release of ERM andexcellent visual recovery in children.1,3
One case report has demonstrated improvement of mac-ular function after surgical removal of an ERM in an adultpatient with Stargardt’s disease.9 We report spontaneousrelease of an ERM without any intervention in a child withStargardt macular dystrophy. OCT clearly showed sepa-ration of a dense ERM from the macular surface andresolution of increased foveal thickness as well as forma-tion of a foveal pit at 4 months after initial presentation.Limited visual improvement was obtained in our casebecause of established Stargardt macular dystrophy.
It should be kept in mind that a long period ofobservation may cause irreversible anatomic damage inchildren with ERM. Early surgical removal of ERMmight offer some advantages to lessen the potential foramblyopia developing in very young patients. Compli-cations of vitrectomy and anesthesia in children shouldalso be considered.
Based on our experience, a period of observation may be
FIG 2. Fundus photographs of right (A) and left (B) eye with the maculaspontaneous epiretinal membrane detachment. A dense epiretinal memb
warranted in patients with ERM associated with Star-
Journal of AAPOS
gardt’s disease, as the ERM may show spontaneous releaseand improvement in vision.
References1. Barr CC, Michels RG. Idiopathic neovascularized epiretinal mem-
branes in young patients. Report of six cases. Ann Ophthalmol 1982;14:335-41.
2. Laatikainen L, Punnonen E. Idiopathic macular fibrosis in youngindividuals. Int Ophthalmol 1987;10:11-14.
3. Meyer CH, Rodrigues EB, Mennel S, Schmidt JC, Kroll P. Sponta-neous separation of epiretinal membrane in young subjects: Personalobservations and review of the literature. Graefes Arch Clin ExpOphthalmol 2004;242:977-85.
4. Trese M, Chandler D, Machemer R. Macular pucker: II. Ultrastruc-ture. Graefes Arch Klin Exp Ophthalmol 1983;221:16-26.
5. Messner KH. Spontaneous separation of preretinal macular fibrosis.Am J Ophthalmol 1977;83:9-11.
6. Desatnik H, Treister G, Moisseiev J. Spontaneous separation of anidiopathic macular pucker in a young girl. Am J Ophthalmol 1999;127:729-31.
7. Greven C, Slusher MM, Weaver RG. Epiretinal membrane re-lease and posterior vitreous detachment. Ophthalmology 1998;95:902-5.
8. Smiddy WE, Michels RG, Gilbert HD, Green W. Clinicopathologicstudy of idiopathic macular pucker in children and adults. Retina1992;12:232-6.
9. Potter MJ, Lee AS, Moshaver A. Improvement in macular functionafter epiretinal membrane removal in a patient with Stargardt disease.
hy without any flecks that suggested Stargardt macular dystrophy afterseen above the retinal surface plane in the left eye.
r atrop
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