Combined intravitreal bevacizumab andtriamcinolone injection in a child with Coats diseaseMehmet Çakır, MD, Osman Çekiç, MD, PhD, and Ö. Faruk Yılmaz, MD

We report a child with Coats disease who demonstrated a rapidresponse to intravitreal bevacizumab and triamcinolone injection. A14-year-old boy presented with stage 3A Coats disease unresponsiveto initial argon laser treatment and was injected with intravitrealbevacizumab and triamcinolone acetonide. Following intravitrealinjection, a superior bullous exudative retinal detachment and sub-foveal serous fluid collection dramatically improved. Additional ar-gon laser photocoagulation resulted in complete regression of tel-angiectatic vessels as well as decrease in macular exudation. Visualacuity improved from 20/400 to 20/125 after treatment, and the boyremained stable 6 months after injection.

C oats disease is an idiopathic condition character-ized by telangiectatic retinal vessels with chronicaccumulation of intraretinal and subretinal exu-

dates. It is usually unilateral, occurs mostly in young males,and can cause severe visual loss resulting from exudativedetachment.1 The primary goal of treatment is to eradicateretinal telangiectasias to achieve resolution of exudation.1,2

Current treatment options are ablation of telangiectaticvessels, either by laser photocoagulation or by cryother-apy, and surgery for retinal detachment.2 Recently a pa-tient with Coats disease was successfully treated with com-bined vitrectomy and intravitreal injection of pegaptanipsodium.3

Case ReportA 14-year-old boy presented with visual acuities of 20/400in the right eye and 20/20 in the left eye. A right exotropiaof 10� was present. Intraocular pressures were 15 mm Hgbilaterally. Biomiscroscopic evaluation disclosed a smallposterior subcapsular opacity in the right eye and normalfindings in the left eye. Dilated fundus examination of theright eye revealed submacular exudation, retinal telangi-ectasias, and intraretinal hemorrhage at the temporal mid-periphery, with a superior bullous exudative retinal de-tachment not involving the macula (Figure 1A), consistentwith stage 3A Coats disease via Shield’s classification.2

Fluorescein angiography demonstrated leakage and early

Author affiliations: Beyoglu Eye Training and Research Hospital, Istanbul, TurkeyThe authors have no conflicts of interest to disclose.Submitted October 15, 2007.Revision accepted January 14, 2008.Reprint requests: Mehmet Çakir, MD, Beyoglu Eye Training and Research Hospital,

Istanbul, Turkey (email: drmcakir@hotmail.com).J AAPOS 2008;12:309-311.

Copyright © 2008 by the American Association for Pediatric Ophthalmology andStrabismus.

1091-8531/2008/$35.00 � 0doi:10.1016/j.jaapos.2008.01.009

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hyperfluoresecence of the telangiectasias in the tempo-ral midperiphery and superior hypofluorescence corre-sponding to the exudative retinal detachment (Figure2A). Posterior segment evaluation of the left eye wasnormal.

Initial treatment consisted of argon laser photocoagula-tion of the telangiectatic vessels and surrounding retina.However, over the next month, the detachment pro-gressed to involve the macula (Figure 1B). After obtainingdetailed, informed consent, he was then treated with anintravitreal injection of 1.25 mg bevacizumab and 2 mg tri-amcinolone acetonide. Resolution of the subfoveal serousdetachment and superior bullous exudative detachment oc-curred within 3 weeks following injection. Elevated in-traocular pressure after injection was controlled by meansof a topical glaucoma medicine. Visual acuity improved to20/125. Additional laser therapy was then applied. Reeval-uation at 3 months postinjection revealed a stationary poste-rior subcapsular cataract, intraocular pressure of 15 mm Hgon topical glaucoma medication, complete regression of tel-angiectatic vessels and subfoveal serous fluid, and a notablysmaller superior retinal detachment (Figures 1C and 2B).The boy remains stable 6 months after injection with a visualacuity of 20/125 and an intraocular pressure of 14 mm Hgwithout any medication. No further subretinal fluid waspresent (Figures 1D and 2C).

DiscussionPhotocoagulation and/or cryopexy are the recom-mended initial treatment modalities in eyes with stage3A Coats disease.2 Usually the combination of cryother-apy and laser works well in combination with the drain-age of subretinal fluid externally if the retina is highlyelevated.4 Our patient responded well to intravitrealinjection of bevacizumab and triamcinolone after noresponse to initial laser treatment. Although the con-tributory effect of the triamcinolone in this patientcannot be definitively assessed, we believe that bothbevacizumab and triamcinolone were responsible forreducing retinal vasopermeability.

A prior report3 found elevated vitreous levels of vas-cular endothelial growth factor in a 2-year-old boy withstage 4 Coats disease. Vitrectomy and injection of in-travitreal pegaptanib sodium resulted in marked reduc-tion of vascular endothelial growth factor level from 908to 167 pg/mL as well as improvement of exudation andhemorrhage and a nearly complete reattachment of ret-ina. We selected combination therapy over mono-

therapy since in our clinical experience we felt the

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photocoagulation at 3 months (C) and 6 months (D) postinjection.

Volume 12 Number 3 / June 2008310 Çakır, Çekiç, and Yılmaz

FIG 2. Fundus fluorescein angiography at presentation shows leakage of dye from retinal telangiectatic vessels, blocked fluorescence due to exudationat macular area, and a superior exudative retinal detachment not involving macula (A). Fluorescein angiography following intravitreal bevacizumab-triamcinolone injection and the second application of laser photocoagulation discloses regression of the macular exudation and superior exudative retinal

FIG 1. Infrared picture at presentation shows macular exudation and superior bullous retinal detachment (A). Following initial laser photoablation, thesuperior retinal detachment progressed to involve the macula as seen in this color photograph (B). Color photographs demonstrate resolution of submacularserous detachment and superior bullous exudative detachment after intravitreal injection of bevacizumab and triamcinolone and second application of laser

detachment at 3 months (B) and 6 months (C) postinjection.

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addition of triamcinolone potentiated the vasoperme-ability reducing effect of bevacizumab. While there wasno progression of preexisting cataract, intraocular pres-sure elevation did occur. Based on this single case re-port, we cannot evaluate potential long-term adverseeffects of such treatment, and further study is needed todetermine the best role of intravitreal bevacizumab andtriamcinolone in the treatment of Coats disease. Thestandard of care for initial intervention remains ablationof the abnormal vessels and avascular areas with laser or

cryotherapy.

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References1. Shields JA, Shields CL, Honavar SG, Demirci H. Clinical variations

and complications of Coats disease in 150 cases: The 2000 SanfordGifford Memorial Lecture. Am J Ophthalmol 2001;131:561-71.

2. Shields JA, Shields CL, Honavar SG, Demirci H, Cater J. Classifica-tion and management of Coats disease: The 2000 Proctor Lecture.Am J Ophthalmol 2001;131:572-83.

3. Sun Y, Jain A, Moshfeghin DM. Elevated vascular endothelial growthfactor levels in Coats disease: Rapid response to pegaptanib sodium.Graefe’s Arch Clin Exp Ophthalmol 2007;245:1387-8.

4. Silodor JJ, Augsburger JA, Shields JA, Tasman W. Natural history andmanagement of advanced Coat’s disease. Ophthalmic Surg 1988;19:

89-93.

First Person

This past June, about a week after school closing here, I was seeing a little boy who had justfinished second grade. “Are you all done with school?” I asked with interest.

“No, Dr. Lewis,” came the quiet reply. “I have to go back next year.”—Richard A. Lewis, MD