Delhi Journal of Ophthalmology

A Rare Case Of Unilateral Adult Onset Coats’ Disease

Sonam Mahajan, Ohm Patel, Rahul Sharma, Vivek Kumar
Venu Eye Institute & Research Centre, New Delhi, India 

Corresponding Author:

Sonam Mahajan MBBS 
Resident 
Venu Eye Institute & Research Centre, 
New Delhi, India 
Email id: sonam16388@gmail.com

Received: 29-JUL-2017

Accepted: 27-

Published Online: 05-MAR-2018

DOI:http://dx.doi.org/10.7869/djo.341

Abstract
Coats’ disease is typically seen in childhood and less commonly among adults. The Adult onset form is manifested by various 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. We hereby report a case of a 55 year old male who presented with a visual acuity of 6/6 in both the eyes. Fundus examination revealed localised superotemporal, midperipheral telangiectatic vessles surrounded by hard exudates with exudative retinal detachment in the left eye. Fluorescein angiography showed leakage from the telangiectatic vessels along with capillary non perfusion areas. A provisional diagnosis of Coats’ disease was made. The lesion responded well to focal laser photocoagulation.

Keywords :coats’ disease, retinal telangiectasia, exudative retinal detachment


Case Report
A 55 year old male patient presented to our OPD for routine eye evaluation. The best corrected visual acuity was 6/6 in both the eyes. On examination, he had early immature senile cataract in the right eye and pseudophakia in the left eye. Dilated fundus examination in the right eye was within normal limits, whereas the left eye revealed superotemporal telangiectasias with surrounding ring of lipid exudates and associated localised exudative retinal detachment (Figure 1). Fluorescein angiography of the left eye showed dilated retinal vasculature suggestive of telangiectasis along with leakage and adjacent capillary non perfusion areas (Figure 2 & 3). Spectral domain optical coherence tomography of left eye showed no evidence of macular edema (Figure 4). We advised focal laser photocoagulation of the leaking telangiectatic vessels. Post laser follow up visit at 2 weeks and 2 months revealed regression of the telangiectatic vessels along with resolution of exudative retinal detachment (Figure 5).






Discussion
Retinal telangiectasis, a term proposed by Rees,1 refers to a developmental retinal vascular disorder characterized by ectasias of capillaries of the retina, in which irregular capillary dilation and incompetence occur in the retinal periphery or at the macula. If only the capillaries of the foveal avascular zone are involved, it is known as parafoveal telangiectasia, characterised by the presence of dilated capillaries in the juxtafoveal area. Parafoveal telangiectasias have two basic forms (1) a developmental or congenital vascular anomaly, which may be a part of the larger spectrum of Coats’ disease; and (2) a presumably acquired form found in middle aged and older persons.2 

Coats’ disease is an idiopathic non-hereditary condition characterised by retinal telengectasia with subretinal and intraretinal exudation and exudative retinal detachment without vitreous traction.3 The disease is usually progressive.4 It usually occurs in boys with a peak incidence between 6-8 years of age5 while the adult form presents over 35 years of age.6 In mild cases, one or more localized foci of retinal telangiectasia are noted within the retinal capillary bed, typically in the temporal quadrants between the equator and ora serrata. Coats’ disease in adults seems to advance at a slower rate than it does in children, with majority of patients reaching a final stable visual acuity. 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.7 Our case had superotemporal telangiectasias with surrounding ring of lipid exudates and associated localised exudative retinal detachment which responded well to focal laser. Similar results were obtained in a study by Smithen et al.7

References
  1. Reese AB. Telangiectasis of the retina and Coats’ disease. Am J Ophthalmol 1956; 42:1-8
  2. Ryan, S. (2006). Retina. 4th ed. St. Louis u.a.: Mosby, p.1409.
  3. 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
  4. Silodor S, Augsburger J, Shields J: Natural history and management of advanced Coats’ disease. Ophthalmic Surg 1988; 19:89–93.
  5. Dr. Alessandra Del Longo: Neurogenetics, Instituto ccs mendle, veilaregina margarita 261.00198 Roma, Italy
  6. 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.
  7. Smithen LM, Brown GC, Brucker AJ, Yannuzzi LA, Klais CM, Spaide RF. Coats’ disease diagnosed in adulthood. Ophthalmology 2005; 112:1072-8.


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Sonam Mahajan, Ohm Patel, Rahul Sharma, Vivek KumarA Rare Case Of Unilateral Adult Onset Coats’ Disease.DJO 2018;28:67-68

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Sonam Mahajan, Ohm Patel, Rahul Sharma, Vivek KumarA Rare Case Of Unilateral Adult Onset Coats’ Disease.DJO [serial online] 2018[cited 2019 May 22];28:67-68. Available from: http://www.djo.org.in/articles/28/3/ARareCaseOfUnilateralAdultOnsetCoatsDisease.html