Case Reports in Ophthalmological Medicine

Case Reports in Ophthalmological Medicine / 2017 / Article

Case Report | Open Access

Volume 2017 |Article ID 2824874 |

Wen-Shi Shieh, Gaurav K. Shah, Kevin J. Blinder, "Coats’ Disease-Related Macular Edema Treated with Combined Aflibercept and Laser Photocoagulation", Case Reports in Ophthalmological Medicine, vol. 2017, Article ID 2824874, 3 pages, 2017.

Coats’ Disease-Related Macular Edema Treated with Combined Aflibercept and Laser Photocoagulation

Academic Editor: Maurizio Battaglia Parodi
Received12 Sep 2017
Revised16 Nov 2017
Accepted21 Nov 2017
Published12 Dec 2017


Purpose. To describe the clinical response of refractory macular edema associated with Coats’ disease following treatment with aflibercept and laser photocoagulation. Methods. Case report. Results. A 17-year-old female presented with decreased vision of the left eye. Ophthalmic exam demonstrated intraretinal hemorrhages and exudation with associated edema centrally. Angiographic evaluation revealed central leaking microaneurysms and peripheral capillary dropout. These findings and a systemic work-up that yielded an incidental Factor V Leiden mutation lead to a diagnosis of Coats’ disease. Initial treatment consisted of laser photocoagulation and intravitreal bevacizumab but with poor response. Switching to intravitreal aflibercept resulted in resolution of the refractory macular edema and improvement of visual acuity to 20/25 in the left eye. Conclusion. We describe a case of refractory macular edema which responded more favorably to intravitreal aflibercept compared with bevacizumab when combined with laser photocoagulation in a patient with Coats’ disease.

1. Introduction

Coats’ disease (also known as retinal telangiectasis) is characterized by abnormal telangiectasias and aneurysms of retinal vessels leading to retinal exudation and potential serous retinal detachment [1]. This condition generally has a unilateral presentation and affects males more than females with no ethnic or geographic associations [2]. Adult-onset variants of Coats’ disease have also been reported with a spectrum ranging from milder forms limited to macular abnormalities to findings identical to that in children [3]. We present a case report of a female patient with clinical findings consistent with Coats’ disease and its clinical course including treatment of refractory macular edema.

2. Case

A healthy 17-year-old female presented with blurred vision in her left eye. Visual acuity was 20/20 and 20/40 in the right and left eyes, respectively. Fundus examination of the left eye showed intraretinal hemorrhages and exudation with associated edema centrally (Figure 1). Wide-field fluorescein angiography of the left eye revealed central microaneurysms with leakage and capillary nonperfusion peripherally (Figure 2). Spectral domain optical coherence tomography (OCT) initially demonstrated macular edema and subretinal fluid (Figure 3(a)). A work-up yielded normal CBC, PT, aPTT, and INR. Although coagulopathy studies were negative for lupus anticoagulant, anti-cardiolipin antibody, and normal levels of homocysteine, Protein S, and Protein C, a Factor V Leiden mutation was incidentally found.

The patient was diagnosed with Coats’ disease and treated initially with peripheral laser photocoagulation and intravitreal bevacizumab. Despite a series of three monthly intravitreal bevacizumab injections, macular edema persisted at 3 months (Figure 3(b)). A greater response was noted upon switching to intravitreal aflibercept and the patient received 5 total injections at an interval of every 4 to 6 weeks. Additional focal laser to noncenter involving macular edema was also applied at month 10; however, the improvement of macular edema on OCT was most pronounced following two additional monthly injections of intravitreal aflibercept (Figure 3(c)). At 12-month follow-up, visual acuity improved to 20/25 without any further recurrence of edema.

3. Discussion

Early stages of Coats’ disease involve telangiectasis in the temporal macula and mid-periphery. Retinal edema and accumulation of lipid exudates in the macula are common causes for vision loss. In later stages, proliferation of retinal pigment epithelial cells in the subretinal space can lead to fibrosis and retinal detachment. The treatment of choice has been laser photocoagulation and/or cryotherapy in early stages. In advanced stages, retinal detachment repair or enucleation for a blind, painful eye may be necessary [4]. For our patient, the differential diagnoses included a nonischemic central retinal vein occlusion or macular telangiectasia. However, angiographic evidence of peripheral avascular retina, microaneurysms largely localized in the temporal macula, and associated clinical findings of exudation with macular edema are most consistent with Coats’ disease.

Anti-vascular endothelial growth factor (VEGF), namely, intravitreal bevacizumab, has been reported as an effective therapy either alone or combined with other treatment modalities for subretinal fluid and exudation in Coats’ disease [58]. For our patient, focal laser and switching anti-VEGF therapy to intravitreal aflibercept helped to reduce the refractory macular edema. To the best of our knowledge, only one other case report exists of Coats’ disease-associated macular edema responding more favorably to aflibercept injections after initial treatment with ranibizumab and argon laser [9].

In summary, studies have shown elevated levels of VEGF in patients with Coats’ disease as well as correlations between VEGF concentrations and the severity of disease [10, 11]. Although several studies highlight the efficacy of adjunctive bevacizumab injections combined with laser vascular ablation, we present a case of Coats’ disease where treatment with aflibercept was effective in a patient with refractory macular edema unresponsive to bevacizumab and laser photocoagulation.

Conflicts of Interest

The authors declare that there are no conflicts of interest regarding the publication of this article.


This research was sponsored by the Retina Research & Development Foundation.


  1. J. A. Shields, C. L. Shields, S. G. Honavar, H. Demirci, and J. Cater, “Classification and management of Coats disease: the 2000 Proctor Lecture,” American Journal of Ophthalmology, vol. 131, no. 5, pp. 572–583, 2001. View at: Publisher Site | Google Scholar
  2. E. J. Sigler, J. C. Randolph, J. I. Calzada, M. W. Wilson, and B. G. Haik, “Current management of Coats disease,” Survey of Ophthalmology, vol. 59, no. 1, pp. 30–46, 2014. View at: Publisher Site | Google Scholar
  3. D. Beselga, A. Campos, S. Mendes, F. Carvalheira, M. Castro, and D. Castanheira, “Refractory coats' disease of adult onset,” Case Reports in Ophthalmology, vol. 3, no. 1, pp. 118–122, 2012. View at: Publisher Site | Google Scholar
  4. A. Ramasubramanian and C. L. Shields, “Bevacizumab for Coats' disease with exudative retinal detachment and risk of vitreoretinal traction,” British Journal of Ophthalmology, vol. 96, no. 3, pp. 356–359, 2012. View at: Publisher Site | Google Scholar
  5. C.-J. Lin, J.-F. Hwang, Y.-T. Chen, and S.-N. Chen, “The effect of intravitreal bevacizumab in the treatment of coats disease in children,” Retina, vol. 30, no. 4, pp. 617–622, 2010. View at: Publisher Site | Google Scholar
  6. R. Ray, D. E. Barañano, and G. B. Hubbard, “Treatment of Coats' disease with intravitreal bevacizumab,” British Journal of Ophthalmology, vol. 97, no. 3, pp. 272–277, 2013. View at: Publisher Site | Google Scholar
  7. A. Kodama, K. Sugioka, S. Kusaka, C. Matsumoto, and Y. Shimomura, “Combined treatment for coats' disease: retinal laser photocoagulation combined with intravitreal bevacizumab injection was effective in two cases,” BMC Ophthalmology, vol. 14, no. 1, article 36, 2014. View at: Publisher Site | Google Scholar
  8. V. M. Villegas, A. S. Gold, A. M. Berrocal, and T. G. Murray, “Advanced Coats' disease treated with intravitreal bevacizumab combined with laser vascular ablation,” Clinical Ophthalmology, vol. 8, pp. 973–976, 2014. View at: Publisher Site | Google Scholar
  9. M. Guixeres Esteve and A. Pardo Saiz, “Coats’ disease with macular oedema responsive to aflibercept and argon laser,” Archivos de la Sociedad Española de Oftalmología (English Edition), vol. 92, no. 7, pp. 330–333, 2017. View at: Publisher Site | Google Scholar
  10. Y.-G. He, H. Wang, B. Zhao, J. Lee, D. Bahl, and J. McCluskey, “Elevated vascular endothelial growth factor level in Coats' disease and possible therapeutic role of bevacizumab,” Graefe's Archive for Clinical and Experimental Ophthalmology, vol. 248, no. 10, pp. 1519–1521, 2010. View at: Publisher Site | Google Scholar
  11. Q. Zhao, X.-Y. Peng, F.-H. Chen et al., “Vascular endothelial growth factor in Coats' disease,” Acta Ophthalmologica, vol. 92, no. 3, pp. e225–e228, 2014. View at: Publisher Site | Google Scholar

Copyright © 2017 Wen-Shi Shieh et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Related articles

No related content is available yet for this article.
 PDF Download Citation Citation
 Download other formatsMore
 Order printed copiesOrder

Related articles

No related content is available yet for this article.

Article of the Year Award: Outstanding research contributions of 2020, as selected by our Chief Editors. Read the winning articles.