Coats’ disease of adult-onset in 48 eyes
TL;DR: The bilateral presentation emphasizes the need for regular follow-up to detect possible future involvement of the fellow eye and has less extensive involvement, more benign natural course, and a more favorable treatment outcome as against the childhood-onset disease.
Abstract: Background: Coats' disease diagnosed in adulthood is an idiopathic, retinal exudative vascular disease without an inciting factor and has retinal features different from the childhood disease. Aim: To describe clinical features, treatment, and outcomes of eyes with Coats' disease first diagnosed in patients 35 years or older. Materials and Methods: Retrospective chart review of patients first diagnosed with Coats' disease at the age of 35 years or more at a tertiary eye care center between January 1995 and 2012. Eyes with retinal exudation or Coats'-like response from secondary causes were excluded. Results: Forty-five of 646 patients (7%) diagnosed with Coats' disease had adult-onset disease. Mean age at presentation was 47 years. Systemic hypertension was the most common (22%) systemic association and decreased vision the predominant presenting feature (83%). Localized (<6 clock h) presentation (74%) was unique to adults as against diffuse involvement (69%) in children (P < 0.001). Eyes were treated with laser photocoagulation 29 (60%), cryotherapy (4%), or both (2%) with surgical intervention in three (6%) eyes. Following treatment eight (35%) eyes improved, 11 (48%) eyes were stable while four (12%) eyes worsened due to complications. Conclusion: Adult-onset Coats' disease has less extensive involvement, more benign natural course, and a more favorable treatment outcome as against the childhood-onset disease. The bilateral presentation emphasizes the need for regular follow-up to detect possible future involvement of the fellow eye.
Citations
More filters
TL;DR: The youngest patients (3 years or younger) with Coats disease present with worse visual acuity and more advanced disease stage, and are more likely to require ultimate enucleation.
Abstract: PURPOSE To investigate features and outcomes of Coats disease by patient age. METHODS Patients with Coats disease from 1973 to 2018 were evaluated based on age category at presentation (3 years or younger vs older than 3 to 10 years vs older than 10 years). RESULTS There were 351 eyes of 351 patients with Coats disease diagnosed (2 vs 6 vs 27 years, P < .001). The youngest age group had more referral diagnoses of retinoblastoma (29% vs 15% vs 0%, P < .001), worse presenting visual acuity (< 20/200: 80% vs 67% vs 31%, P < .001), more advanced Coats disease stage (stage 3B: 65% vs 38% vs 10%, P < .001), and greater clock-hour extent of telangiectasia (7 vs 5 vs 4, P < .001), light bulb aneurysms (7 vs 4 vs 3, P < .001), exudation (10 vs 7 vs 5, P < .001), and subretinal fluid (10 vs 7 vs 4, P < .001). The oldest patients received a greater total number of treatments (3.3 vs 3.1 vs 4.4, P = .04), with more argon laser photocoagulation (37% vs 52% vs 73%, P < .001) and intravitreal anti-vascular endothelial growth factor (6% vs 9% vs 23%, P < .002) and less cryotherapy (74% vs 84% vs 58%, P < .001). At mean follow-up (70 vs 65 vs 38 months, P = .02), the youngest patients had poorer visual acuity outcome (< 20/200: 83% vs 64% vs 39%, P < .001), had less disease resolution (43% vs 65% vs 62%, P = .01), and were more likely to ultimately require enucleation (22% vs 10% vs 6%, P = .01). CONCLUSIONS Younger patients (3 years or younger) with Coats disease present with worse visual acuity and more advanced disease stage, and are more likely to require ultimate enucleation. [J Pediatr Ophthalmol Strabismus. 2019;56(5):288-296.].
21 citations
TL;DR: Vitreoretinal fibrosis and TRD increase after treatment in Coats' disease and the combination of anti-VEGF agents and cryotherapy may lead to higher risk for TRD.
Abstract: AIM To elucidate the association of treatment modality to vitreoretinal fibrosis and traction retinal detachment (TRD) in Coats' disease. METHODS A PubMed search for Coats' disease with included studies describing eyes with clinical features and treatment course of Coats' disease. Binary logistic regression with fibrosis at presentation and treatment type as independent variables was performed to determine predictors of TRD historically (since 1921) and in the anti-vascular endothelial growth factor (VEGF) era (since 2007). Odds ratios (OR) with 95% confidence intervals (CI) reported. RESULTS Of 175 articles described 1183 eyes. Vitreoretinal fibrosis increased from presentation (5.4%) to follow-up (15.5%) and TRD increased from 0.44% to 3.9% at follow up. Laser was protective against vitreoretinal fibrosis (OR 0.6, 95%CI 0.4-0.9) but TRD was borderline (OR 0.6, 95%CI 0.3-1.1). Cryotherapy showed a higher association with TRD (OR 1.9, 95%CI 1.0-3.7) than with vitreoretinal fibrosis (OR 0.8, 95%CI 0.5-1.2). Similarly, intravitreal anti-VEGF alone was not associated with fibrosis (OR 1.1, 95%CI 0.6-1.8) nor TRD (OR 1.1, 95%CI 0.5-2.6) but the combination of laser and anti-VEGF therapy was protective [Fibrosis: 0.1 (0.03, 0.35); TRD: 0.05 (0.01, 0.23)] compared to anti-VEGF plus cryotherapy (P<0.001). Disease stage ≤2B or ≥3A was not associated with TRD. CONCLUSION Vitreoretinal fibrosis and TRD increase after treatment in Coats' disease. The combination of anti-VEGF agents and cryotherapy may lead to higher risk for TRD. Presence of pre-treatment fibrosis is the highest risk factor for post-treatment worsening of vitreoretinal fibrosis and TRD.
11 citations
TL;DR: Though bilateral combined central retinal artery and vein occlusion is rare in pediatric NS, the treating physician should be aware of this entity as it can be successfully managed.
Abstract: Nephrotic syndrome (NS) is a common disease of childhood but ophthalmic manifestations are seldom reported. We report a rare occurrence of bilateral combined central retinal artery and vein occlusion in a 3-year-old with NS. The child presented with bilateral painless loss of vision, central pallid retinae with cherry red spots, vascular tortuosity, and retinal hemorrhages. There was delayed filling of the arteriolar circulation and a delay in arteriovenous transit time on angiography and increased central retinal thickening on optical coherence tomography. She was treated with oral steroids, subcutaneous low molecular weight heparin, and oral acetylsalicylic acid. The central retinae showed resolution of the hemorrhages, tortuosity, edema, and pallor within 3 weeks. Visual acuity recovered bilaterally to 20/360, 20/190, and 20/40 at 1, 3, and 6 weeks, respectively. We discuss the possible reasons for good recovery in our patient. Though bilateral combined central retinal artery and vein occlusion is rare in pediatric NS, the treating physician should be aware of this entity as it can be successfully managed.
9 citations
TL;DR: A rare case of adult-onset Coats' disease that presented with epiretinal membrane (ERM) is described and utility of ultra-wide-field imaging and rationale of early vitrectomy in such cases are discussed.
Abstract: Coats' disease is characterized by retinal vascular telangiectasia and subretinal and intraretinal exudation. A relatively benign form of the disease that occurs in adults is referred to as adult-onset Coats' disease. Involvement of macula in the form of macular edema and exudation are the common presenting features in both forms of the disease. We describe a rare case of adult-onset Coats' disease that presented with epiretinal membrane (ERM). Laser photocoagulation of retinal vascular telangiectasia resulted in worsening of patient's symptoms and ERM. Early pars plana vitrectomy resulted in resolution of the patient's symptoms. Utility of ultra-wide-field imaging and rationale of early vitrectomy in such cases are discussed.
8 citations
Cites result from "Coats’ disease of adult-onset in 48..."
...[1] The disease is characteristically seen in children, but a variant can be seen in adults and is referred to as adult-onset Coats’ disease.[2] Adult-onset Coats’ disease is characterized by less extensive involvement, more benign natural course, and more favorable treatment outcome as compared to Coats’ disease in the young....
[...]
TL;DR: The patient underwent laser photocoagulation to the vascular telangiectasia followed by pars plana vitrectomy, large internal limiting membrane peeling and gas tamponade resulted in regression of exudation, closure of macular hole and improvement in vision.
Abstract: The occurrence of full thickness macular hole in Coats' disease is extremely rare. The purpose of this case report is to report pars plana vitrectomy for the treatment of full thickness macular hole in a patient with adult onset Coats disease. A young male presented with decreased vision in his right eye because of full thickness macular hole. The macular hole was found to be associated with adult onset Coats' disease that was evident on ultra-wide field imaging. The patient underwent laser photocoagulation to the vascular telangiectasia followed by pars plana vitrectomy, large internal limiting membrane peeling and gas tamponade. This resulted in regression of exudation, closure of macular hole and improvement in vision. Coats disease of adult onset can present with decreased vision because of full thickness macular hole. Vitrectomy with internal limiting membrane peeling can result in excellent visual outcome.
4 citations
Cites background from "Coats’ disease of adult-onset in 48..."
...A variant occurring in adults is known as adult‐onset Coats’ disease and tends to have a limited area of involvement, slower progression, and more posterior involvement.[2,3] The common macular changes include macular exudation, macular edema, epiretinal membrane (ERM), and fibroglial nodule....
[...]
References
More filters
TL;DR: Carefully selected treatment can anatomically stabilize or improve the eye with Coats disease in 76% of eyes, however, poor visual outcome of 20/200 or worse commonly results, and patients with stages 4 and 5 have a poor visual prognosis.
319 citations
"Coats’ disease of adult-onset in 48..." refers methods in this paper
...The few older adults in the series by Shields et al.[3] had findings identical to the children in that series, and they had no other underlying conditions to predispose to exudative retinopathy....
[...]
...Staging of the disease was done based on the extent and the quadrant (s) of retina involved according to classification by Shields et al.[2] Fundus fluorescein angiography confirmed active telangiectatic leakage, areas of capillary nonperfusion, and also helped rule out other differential diagnosis....
[...]
TL;DR: Coats disease is a distinct clinical entity characterized by idiopathic retinal telangiectasia and retinal exudation that is usually unilateral, occurs mostly in young males, and can cause severe visual loss resulting from exudative retinal detachment.
285 citations
TL;DR: Coats disease is an idiopathic condition characterized by retinal vascular telangiectasia and exudative retinopathy that most often affects young males and has been the subject of considerable attention in the ophthalmic literature.
Abstract: Coats disease is a well-known ocular entity that has been the subject of considerable attention in the ophthalmic literature. It is an idiopathic condition characterized by retinal vascular telangiectasia and exudative retinopathy that most often affects young males. The striking ophthalmoscopic fea
140 citations
TL;DR: Coats' disease treated with intravitreal bevacizumab in addition to standard therapy can develop to vitreoretinal fibrosis and potentially traction retinal detachment.
Abstract: Aim To evaluate the effect of supplemental intravitreal bevacizumab for management of Coats9 disease. Methods Retrospective analysis of eight patients with Coats9 disease manifesting total or partial exudative retinal detachment where the retinal telangiectasia was treated with standard laser photocoagulation and/or cryotherapy plus additional intravitreal bevacizumab (1.25 mg/0.05 ml). Results The mean patient age was 88 (range 7–240) months and 63% were male. Coats9 disease was classified as stage 2 (n=1, 12%), 3a (n=3, 38%) and 3b (n=4, 50%). Features included retinal detachment (n=8, 100% with mean detachment extent involving 8 clock hours), telangiectasia (n=8, 100% with mean extent of 8 clock hours), peripheral retinal ischaemia on fluorescein angiography (n=7, 88%) and no evidence of neovascularisation. Treatment consisted of cryotherapy (n=8, 100%), laser photocoagulation (n=4, 50%) and bevacizumab intravitreal injection (n=8) with median number of one injection per eye (mean 1.75, and range 1–4 injections). After a mean follow-up of 8.5 months, resolution of retinopathy (n=8, 100%), Coats9-related subretinal fluid (n=8, 100%) and retinal exudation (n=6, 75%) was noted. However, vitreous fibrosis developed (n=4, 50%) at a mean of 5 months following a mean of 1.75 bevacizumab injections with three (38%) evolving into traction retinal detachment. Conclusion Coats9 disease treated with intravitreal bevacizumab in addition to standard therapy can develop to vitreoretinal fibrosis and potentially traction retinal detachment. These tractional features are not often found in Coats9 disease treated with standard measures without bevacizumab. Caution is advised in the use of bevacizumab for patients with Coats9 disease.
97 citations
"Coats’ disease of adult-onset in 48..." refers methods in this paper
...[10] However, Ramasubramanian and Shields[17] have advised caution in the use of anti‐VEGF as they observed vitreoretinal traction and progressive tractional detachment in their series....
[...]
...The few older adults in the series by Shields et al.[3] had findings identical to the children in that series, and they had no other underlying conditions to predispose to exudative retinopathy....
[...]
...[10] However, Ramasubramanian and Shields[17] have advised caution in the use of anti‑VEGF as they observed vitreoretinal traction and progressive tractional detachment in their series....
[...]
...Staging of the disease was done based on the extent and the quadrant (s) of retina involved according to classification by Shields et al.[2] Fundus fluorescein angiography confirmed active telangiectatic leakage, areas of capillary nonperfusion, and also helped rule out other differential diagnosis....
[...]
TL;DR: The findings of an electron microscopical examination of an early, typical case of Coats's disease add little to existing knowledge of the aetiology of the condition, but are of value in interpreting the morbid histological appearances of conventional microscopy and in understanding its pathogenesis.
Abstract: At the present time there is only one report in the literature on the ultrastructural changes to be found in Coats's disease and this is limited to a description of subretinal and intraretinal \"ghost cells\" (Manschot and de Bruijn, I967). We have recently had an opportunity to carry out an electron microscopical examination of an early, typical case and, although our findings add little to existing knowledge of the aetiology of the condition, they are of value in interpreting the morbid histological appearances of conventional microscopy and in understanding its pathogenesis.
93 citations
"Coats’ disease of adult-onset in 48..." refers background in this paper
...It has been postulated by Tripathi and Ashton[13] that retinal capillary nonperfusion, vessel wall changes, breakdown of the blood‐retinal barrier could be the causes for hemorrhage from microaneurysms or macroaneurysms....
[...]
...It has been postulated by Tripathi and Ashton[13] that retinal capillary nonperfusion, vessel wall changes, breakdown of the blood‑retinal barrier could be the causes for hemorrhage from microaneurysms or macroaneurysms....
[...]