Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 8  |  Issue : 3  |  Page : 350-353  

Adult onset coats disease in a 56-year-old male: An atypical presentation


1 Assistant Professor and vitreoretina consultant, NKP Salve institute of medical sciences, Nagpur, India
2 Vitreoretina Department, SB Dr Sohan Singh Eye Hospital, Amritsar, Punjab, India

Date of Web Publication15-May-2015

Correspondence Address:
Bodhraj Dhawan
SB Dr. Sohan Singh Eye Hospital, Amritsar, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.157084

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  Abstract 

Coats disease is a congenital retinal vascular disease, which is seen commonly in childhood and remits and exacerbates throughout life. However, it is not rare for coats disease to present in adulthood for the first time. This has been referred to as adult onset coats disease. We do hereby present a rare case of adult onset coats disease in a 56-year-old male patient who presented with visual acuity of 20/200 in the left eye. Fundus findings typical of coats disease included telangiectasis, macular exudation and edema. Fluorescein angiography revealed peripheral capillary nonperfusion areas and telangiectasis. Optical coherence tomography showed cystoid macular edema. After the treatment with intravitreal injection of bevacizumab (1.25 mg in 0.05 ml) and sectoral panretinal photocoagulation to the area of capillary nonperfusion the macular edema was reduced by 100 microns, which translated to a visual acuity of 20/80, proving the point that the prognosis in these cases is not as grave as in classical coats disease.

Keywords: Adult onset coats disease, coats disease, fundus fluorescein angiography, optical coherence tomography


How to cite this article:
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;8:350-3

How to cite this URL:
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 [serial online] 2015 [cited 2021 Sep 21];8:350-3. Available from: https://www.mjdrdypu.org/text.asp?2015/8/3/350/157084


  Introduction Top


Coats disease is truly a lifetime disease, recurring many times or presenting late at times though rarely. Coats disease or retinal telangiectasia was first described by Coats in 1908 [1] as a retinal exudative disease associated with congenital retinal telangiectasia and somatic mutations. It typically presents in early childhood with vision loss, strabismus, or leukocoria, and must often be differentiated from retinoblastoma. Several studies have revealed that the earlier the age of presentation, the more severe the disease progression and the greater the likelihood of enucleation. [2],[3],[4],[5] The average age at diagnosis is under 5 years. [6] However, there are case reports of onset of disease at 47 years [7] and case series on these cases reporting the age of presentation between 22 and 62 years. [8] In one recent study on adult onset coats disease there were 13 patients with a mean age at diagnosis of 50 years (range, 36-79). This study arbitrarily defines age of onset of 35 years as the criteria to define a case of adult onset coats disease. [9]


  Case Report Top


A 56-year-old nondiabetic, nonhypertensive Indian male, presented with painless progressive diminution of vision in his left eye of 1-year duration.

Patient gives history of having received intravitreal injection of avatin in this eye, elsewhere, soon after the onset of these symptoms (1-year back), but did not gain much out of it.

Visual acuity in the right eye was 20/20 and 20/200 in the left eye. Anterior segment of both eyes was normal except for early nuclear sclerosis. Goldman applanation tonometry readings in both eyes were 16 mm Hg.

Fundus of the right eye was normal [Figure 1]. Fundus of the left eye showed microaneurysms and telangiectasis with exudation all around fovea in the macular region [Figure 2]. Macula looked edematous on slit lamp biomicroscopy with 90D lens. Temporal periphery showed sclerosed blood vessels.
Figure 1: Color fundus photo of the right eye with normal findings

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Figure 2: Color fundus photograph of the left eye with exudation, microaneurysms and telangiectasis on macula

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Fluoroscein angiography of the left eye revealed multiple microaneurysms, telangiectasis and macular edema temporal to fovea [Figure 3]. In the periphery on temporal side there were capillary nonperfusion areas as well telangiectasis in relation to vessels [Figure 4].
Figure 3: Mid arteriovenous phase angiogram of the left eye showing multiple telangiectasis changes with diffuse leak of edema

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Figure 4: Mid arteriovenous phase angiogram of left eye in temporal periphery showing areas of capillary non perfusion and multiple telangiectasis

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Optical coherence tomography (OCT) of the left eye showed cystoid macular edema [Figure 5].
Figure 5: Spectral domain optical coherence image of the left eye showing macular edema of cystoid pattern

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Based on these finding, the diagnosis of adult onset coats disease was made.

Patient was treated with intravitreal injection of bevacuzimab (1.25 mg in 0.05 ml) in the left eye followed 1-week later with sectoral laser photocoagulation to the temporal periphery [Figure 6]. At 4 weeks follow-up, visual acuity in the left eye improved to 20/80. Fundus showed reduced macular edema [Figure 7], darkening of laser spots in temporal periphery [Figure 8]. OCT confirmed reduced macular edema by 100 microns [Figure 9].
Figure 6: Fundus photograph of the left eye following sector laser photocoagulation. Note fresh greyish white laser marks in the temporal periphery

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Figure 7: Fundus photograph of the left eye on 4 weeks follow-up showing reduction of macular edema

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Figure 8: Fundus photograph of the left eye 4 weeks following sector laser photocoagulation. Note darkening of the laser spots

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Figure 9: Optical coherence tomography picture of the left eye through foveal center 4 weeks after treatment showing reduced macular edema

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At last follow-up on 10 weeks posttreatment, patient was maintaining a visual acuity 20/80 and OCT picture was same as on 4 weeks follow-up. We advised a repeat intravitreal injection of bevacizumab, but the patient refused it for financial reasons. However, he was advised 4 weekly follow-up to document for stability.


  Discussion Top


Coats disease in its classic presentation, has been well-described in the ophthalmic literature. [1],[2],[3] Coats disease can first be diagnosed in adulthood with retinal vascular abnormalities similar to those seen in younger patients. Adult onset form is manifested by many findings typical of coats disease which include the unilateral nature of the disease male predominance, vascular telangiectasis, lipid exudation, macular edema, and areas of capillary nonperfusion with adjacent webs of filigree like capillaries. There are a number of important differences in manifestation in adults, including limited area of involvement, slower apparent progression of disease and hemorrhage near larger vascular dilatations. [9]

The present case has all typical features of coats disease on multimodal imaging and positive response to treatment indicates a better prognosis in contrast to the younger counterpart. However, natural course of adult onset coats disease have to be studied on long-term since there are not many studies to answer that.


  Conclusion Top


Adult onset coats disease is a rare form of coats disease presenting with exudation on posterior pole and features of ischemia (of varying degrees) in periphery with telangiectasis and microaneurysms. It's a relatively benign disease. Recent literature defines age limit of over 35 years to qualify for this diagnosis.

 
  References Top

1.
Gomez Morales A. Coats′ disease. Natural history and results of treatment. Am J Ophthalmol 1965;60:855-65.  Back to cited text no. 1
[PUBMED]    
2.
Cahill M, O′Keefe M, Acheson R, Mulvihill A, Wallace D, Mooney D. Classification of the spectrum of Coats′ disease as subtypes of idiopathic retinal telangiectasis with exudation. Acta Ophthalmol Scand 2001;79:596-602.  Back to cited text no. 2
    
3.
Shields JA, Shields CL, Honavar SG, Demirci H, Cater J. Classification and management of Coats disease: The 2000 Proctor Lecture. Am J Ophthalmol 2001;131:572-83.  Back to cited text no. 3
    
4.
Jones JH, Kroll AJ, Lou PL, Ryan EA. Coats′ disease. Int Ophthalmol Clin 2001;41:189-98.  Back to cited text no. 4
    
5.
Shields JA, Shields CL. Review: Coats disease: The 2001 LuEsther T. Mertz Lecture. Retina 2002;22:80-91.  Back to cited text no. 5
    
6.
Shields JA, Shields CL, Honavar SG, Demirci H. Clinical variations and complications of Coats disease in 150 cases: The 2000 Sanford Gifford Memorial Lecture. Am J Ophthalmol 2001;131:561-71.  Back to cited text no. 6
    
7.
Kapusta MA, Yang MB, Kolin T, Chang LP. 47 year-old man with a peripheral retinal lesion found on routine exam. Grand rounds. Digit J Ophthalmol 1997;3:23. Available at http://www.djo.harvard.edu/print.php?url=/physicians/gr/353&print=1. [Last accessed on 2015 May 07].  Back to cited text no. 7
    
8.
Casswell AG, Chaine G, Rush P, Bird AC. Paramacular telangiectasis. Trans Ophthalmol Soc U K 1986;105:683-92.  Back to cited text no. 8
    
9.
Smithen LM, Brown GC, Brucker AJ, Yannuzzi LA, Klais CM, Spaide RF. Coats′ disease diagnosed in adulthood. Ophthalmology 2005;112:1072-8.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]


This article has been cited by
1 Coatsę Disease Diagnosed During Adulthood
Rahaf A Mandura,Abdullah S Alqahtani
Cureus. 2021;
[Pubmed] | [DOI]



 

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