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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 55  |  Issue : 4  |  Page : 304-306

Coats' disease diagnosed in adults: A rare case report


Retina Clinic, Regional Institute of Ophthalmology and Government Ophthalmic Hospital, Chennai, Tamil Nadu, India

Date of Web Publication25-Apr-2018

Correspondence Address:
Dr. Mohan Sivakami
Regional Institute of Ophthalmology and Government Ophthalmic Hospital, Egmore, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_23_17

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  Abstract 


Coats' disease is a form of exudative retinal vasculopathy, commonly diagnosed in the first to second decades. In adults, it usually exhibits a benign course. We report the case of a 33-year-old male who presented with defective vision in the left eye for 2 weeks. He was diagnosed to have Coats' disease by fundus examination and fundus fluorescein angiography. He was treated with intravitreal antivascular endothelial growth factor and sectoral retinal photocoagulation.

Keywords: Adult onset, Coats' disease, retinal exudative vasculopathy


How to cite this article:
Sivakami M, Periyanayagi M, Chandrasekar B. Coats' disease diagnosed in adults: A rare case report. TNOA J Ophthalmic Sci Res 2017;55:304-6

How to cite this URL:
Sivakami M, Periyanayagi M, Chandrasekar B. Coats' disease diagnosed in adults: A rare case report. TNOA J Ophthalmic Sci Res [serial online] 2017 [cited 2019 Oct 14];55:304-6. Available from: http://www.tnoajosr.com/text.asp?2017/55/4/304/231122




  Introduction Top


Coats' disease is a disease of childhood, which is characterized by retinal telangiectasia and massive retinal exudation leading to exudative retinal detachment and may present as leukocoria. In adults, it presents with localized exudation, telangiectasia, and hemorrhages. The visual acuity is usually not much affected unless exudation involves the macula, which requires treatment. Therapeutic options include laser photocoagulation, antivascular endothelial growth factor (anti-VEGF) injections, cryotherapy, surgical treatment of exudative retinal detachment, and enucleation.


  Case Report Top


A 33-year-old male presented with complaints of defective vision in the left eye for the past 2 weeks. There was no history of trauma. There was no similar complaint in the past. The patient did not have a history of systemic diseases such as diabetes, hypertension, tuberculosis, and connective tissue disorders. His general and systemic examinations were within normal limits. Blood pressure was 130/80 mmHg.

Best-corrected visual acuity in the right eye was 6/9 with –0.50DS 6/6 and in the left eye was 6/36 with + 1.5Dcyl at 100 degrees 6/12. Anterior segment examination was normal in both eyes. Intraocular pressure was 12 mmHg in both eyes. Pupillary reaction, color vision, fields, and orthoptic evaluation were normal in both eyes. Fundus examination of the right eye showed a clear media, normal disc and vessels, and the presence of foveal reflex [Figure 1]. Fundus examination of the left eye showed a clear media, normal disc, and localized lipid exudation temporal to fovea. Vessels over the exudation were found to be dilated and telangiectatic. Few hemorrhages were noted over the surface of the exudation [Figure 2] and [Figure 3].
Figure 1: Fundus examination of the right eye showed a clear media, normal disc and vessels, and presence of foveal reflex

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Figure 2: Fundus examination of the left eye showed a clear media, normal disc, and localized lipid exudation temporal to fovea

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Figure 3: Left eye fundus – vessels over the exudation were found to be dilated and telangiectatic. Few hemorrhages were noted over the surface of the exudation

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Fundus fluorescein angiography revealed normal study in the right eye. Abnormal “light bulb dilatations” of large retinal vessels, capillary telangiectasia with extensive leak, and adjacent capillary nonperfusion areas (sparing the macula) were noted at the same area in the left eye [Figure 4] and [Figure 5].
Figure 4: Left eye fundus fluorescein angiography – abnormal “light bulb dilatations” of large retinal vessels and capillary telangiectasia with extensive leak

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Figure 5: Left eye fundus fluorescein angiography – capillary nonperfusion areas sparing the macula

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The patient was treated with intravitreal anti-VEGF injections followed by sectoral retinal photocoagulation [Figure 6] and [Figure 7]. The patient's best-corrected visual acuity after 4 weeks of treatment was 6/9.
Figure 6: Fundus photograph of the left eye after anti vascular endothelial growth factor injection showing reduced exudation

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Figure 7: Fundus photograph left eye postsectoral pan–retinal photocoagulation showing reduced exudation

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  Discussion Top


Coats' disease was first described by George Coats in 1908.[1] It is an idiopathic, unilateral, and progressive exudative retinal vasculopathy characterized by telangiectasia, aneurysms, and abnormal dilatations of retinal vessels. It has wide clinical spectrum ranging from isolated vascular anomalies with no or minimal exudation to extensive intra- and subretinal exudation and generalized lipid deposit. Although Coats' disease can present at any age, the majority of cases are diagnosed in the first two decades of life.

Coats' disease in adults presents unilaterally with male preponderance, similar to childhood-onset Coats' disease.[2] Adult onset Coats' disease differs from that of childhood onset in that many adult patients have no symptoms and may have good visual acuity; it is slowly progressive; the vascular anomalies generally appear in the equatorial and peripheral retina with localized lipid exudation (which appear in a massive but diffuse manner in children); and is associated with more retinal hemorrhages due to bleeding from aneurysmal vessels.[3]

Coats' disease is diagnosed mainly by fundus examination and fundus fluorescein angiography. Fundus examination shows telangiectasia and aneurysmal dilatations of retinal vessels with localized intra- and subretinal exudation. Fundus fluorescein angiography shows telangiectasia, typical “light bulb” aneurysmal dilatation of the vessels, and capillary nonperfusion areas. The most important disease to be differentiated from Coats' disease in children is retinoblastoma. In adult patients, the differential diagnosis includes retinal detachment, toxocariasis, choroidal melanoma, retinitis due to cytomegalovirus, or toxoplasmosis. Adult patients usually maintain a stable visual acuity. Vision loss in adult Coats' disease is rare but can occur through macular edema and exudates, development of an epiretinal membrane, and foveal ischemia.[4]

The goal of treatment in adult-onset Coats' is to obliterate telangiectasia and to facilitate the reabsorption of exudates, thereby maintaining as much visual acuity as possible. Possible therapeutic options described in the literature include observation, laser photocoagulation, cryotherapy, surgical treatment of exudative retinal detachment, and enucleation.[5],[6],[7],[8],[9],[10] Recent studies have shown promising evidence with the use of intravitreal anti-VEGF.[6],[7],[8],[9],[10] Since the disease usually runs a benign clinical course in adults, treatment can be considered if the exudates threaten macula.


  Conclusion Top


Although Coats' disease is primarily a disease of childhood, we must consider its diagnosis in adult patients exhibiting a retinal vasculopathy with prominent localized exudation. Usually adult-onset Coats' disease tends to follow a benign course. But treatment should not be delayed if there is risk of macular involvement. Timely treatment with laser photocoagulation can provide a very good visual outcome, with near-total to total restoration or preservation of vision.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Otani T, Yasuda K, Aizawa N, Sakai F, Nakazawa T, Shimura M, et al. Over 10 years follow-up of Coats' disease in adulthood. Clin Ophthalmol 2011;5:1729-32.  Back to cited text no. 1
    
2.
Dhawan B, Goel A, Singh R, Vig V. Adult onset Coats' disease in a 56-year-old male: An atypical presentation. Med J DY Patil Univ 2015;8:350.  Back to cited text no. 2
  [Full text]  
3.
Smithen LM, Brown GC, Brucker AJ, Yannuzzi LA, Klais CM, Spaide RF, et al. Coats' disease diagnosed in adulthood. Ophthalmology 2005;112:1072-8.  Back to cited text no. 3
    
4.
Koh YT, Sanjay S. Successful outcome of adult-onset Coats' disease following retinal laser photocoagulation. Oman J Ophthalmol 2013;6:206-7.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Beselga D, Campos A, Mendes S, Carvalheira F, Castro M, Castanheira D, et al. Refractory Coats' disease of adult onset. Case Rep Ophthalmol 2012;3:118-22.  Back to cited text no. 5
    
6.
Goel N, Kumar V, Seth A, Raina UK, Ghosh B. Role of intravitreal bevacizumab in adult onset Coats' disease. Int Ophthalmol 2011;31:183-90.  Back to cited text no. 6
[PUBMED]    
7.
Kim J, Park KH, Woo SJ. Combined photodynamic therapy and intravitreal bevacizumab injection for the treatment of adult Coats' disease: A case report. Korean J Ophthalmol 2010;24:374-6.  Back to cited text no. 7
[PUBMED]    
8.
Wang KY, Cheng CK. A combination of intravitreal bevacizumab injection with tunable argon yellow laser photocoagulation as a treatment for adult-onset Coats' disease. J Ocul Pharmacol Ther 2011;27:525-30.  Back to cited text no. 8
[PUBMED]    
9.
Raoof N, Quhill F. Successful use of intravitreal bevacizumab and Pascal laser photocoagulation in the management of adult Coats' disease. Middle East Afr J Ophthalmol 2013;20:256-8.  Back to cited text no. 9
[PUBMED]  [Full text]  
10.
Mino A, Mitamura Y, Katome T, Semba K, Egawa M, Naito T, et al. Case of adult-onset Coats' disease with epiretinal membrane treated with 25-gauge pars plana vitrectomy. J Med Invest 2015;62:85-8.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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