Journal of Pediatric Ophthalmology and Strabismus

Original Article Supplemental Data

Coats Disease: Clinical Features and Outcomes by Age Category in 351 Cases

Lauren A. Dalvin, MD; Sanika Udyaver, BS; Li-Anne S. Lim, MD; Mehdi Mazloumi, MD, MPH; Hatice T. Atalay, MD; Chloe T. L. Khoo, MD; Carol L. Shields, MD

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.]

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.]

Introduction

Coats disease is an idiopathic, unilateral, retinal vascular disorder characterized by retinal telangiectasia, micro and macro “light bulb” aneurysms, and intraretinal and subretinal exudation.1 Common presenting features include vision loss, strabismus, and xanthocoria, the latter of which can mimic leukocoria of retinoblastoma.1 Coats disease is classified into five stages of increasing disease severity, ranging from asymptomatic retinal telangiectasia (stage 1) to telangiectasia with exudation (stage 2), subtotal exudative retinal detachment (stage 3), total retinal detachment with secondary glaucoma (stage 4), and, finally, end-stage disease (stage 5).1,2 Although early stage disease can be managed with a combination of laser photocoagulation, cryotherapy, intravitreal anti-vascular endothelial growth factor (VEGF) therapy, and/or intravitreal or periocular corticosteroids, advanced disease can require surgical drainage of exudative retinal detachment or enucleation for a blind, painful eye.2–6

In a study of 150 consecutive cases of Coats disease by Shields et al.3 in the United States, the median age at diagnosis was 5 years. However, affected patients' ages ranged from 1 month to 63 years.3 A United Kingdom study of 55 cases found an association between younger age and presenting symptoms of leukocoria and strabismus, as well as more advanced stages of Coats disease.7 A series of 98 patients from Switzerland revealed a similar correlation between younger age at presentation and symptoms of leukocoria and strabismus, advanced disease stage, poor visual acuity outcome, and increased risk of enucleation.8 We investigated the clinical features and outcomes of Coats disease by age in a large cohort of 351 cases to further explore the association of disease severity with age at presentation.

Patients and Methods

Medical and imaging records were reviewed to identify all patients diagnosed as having Coats disease on the Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, from November 1, 1973, to July 31, 2018. All patients with Coats disease were included, regardless of disease stage. Patients with bilateral Coats disease–like retinopathy secondary to an underlying systemic syndrome were excluded. This study was in compliance with the Health Insurance Portability and Accountability Act (HIPAA), and institutional review board approval was obtained from Wills Eye Hospital.

All patients underwent a complete ophthalmic examination by an ocular oncologist (CLS). Visual acuity was assessed using Snellen charts for verbal patients and fix and follow for preverbal patients. Color fundus photography, fluorescein angiography, optical coherence tomography (OCT), and B-scan ultrasonography were performed when available and as needed. Digital images in recent years or slides in earlier years were available for review.

Clinical and photographic records were retrospectively reviewed for patient demographics (age, sex, and race). Clinical features on presentation included laterality, involved eye, referral diagnosis, intraocular pressure, visual acuity, associated anterior segment findings (xanthocoria, strabismus, corneal edema, anterior chamber cholesterolosis, iris neovascularization, iris atrophy, and cataract), phthisis bulbi, Coats disease stage, presence and extent of retinal telangiectasia, light bulb aneurysms, intraretinal/subretinal exudation, intraretinal/subretinal fluid, and other associated posterior segment findings (vitreoretinal traction, neovascularization of the disc or elsewhere in the retina, retinal hemorrhage, and vitreous cellularity). Imaging modalities used to assess features in detail included fluorescein angiography (extent and location of retinal telangiectasia, light bulb aneurysms, and retinal non-perfusion, cystoid macular edema [CME], optic disc leakage, and neovascularization of the disc, retina, or iris), OCT (CME, vitreoretinal traction, subretinal fluid, intraretinal/subretinal exudation or fluid, central macular thickness, and subfoveal choroidal thickness), and ultrasonography (retinal detachment, subretinal exudation, and height of subretinal fluid elevation). The number and type of treatment modality were recorded, including observation only, primary enucleation, argon laser photocoagulation, cryotherapy, sub-Tenon's or intravitreal corticosteroid injection, and intravitreal anti-VEGF injection. Outcomes included follow-up time, final visual acuity, use of amblyopia patching, disease resolution or recurrence, development of iris neovascularization, traction retinal detachment, glaucoma, and need for enucleation. Disease resolution was defined as complete resolution of exudation and subretinal fluid with no need for ongoing treatment.

Statistical analysis was performed using SPSS statistical software (version 18; SPSS Inc., Chicago, IL). Patients were divided into three age categories based on age at initial presentation: 3 years and younger included infants and toddlers for whom retinoblastoma could be high in the differential diagnosis, older than 3 to 10 years included children less likely to present as pseudoretinoblastoma, and older than 10 years included adolescents and adults who were more likely to be referred with a known diagnosis of Coats disease. Demographics, clinical features, image features, treatment features, and outcomes were compared by age of presentation (3 years or younger vs older than 3 to 10 years vs older than 10 years). Only patients with at least 3 months of follow-up were included in outcomes analysis.

Categorical variables were compared using the chi-square test (or Fisher's exact test when indicated), and continuous variables were compared using one-way analysis of variance. Post-hoc analysis was performed for between-groups comparison using the Bonferroni test. Statistical significance was defined as a P value of less than .05.

Results

There were 351 eyes of 351 patients with Coats disease at the Ocular Oncology Service, Wills Eye Hospital from November 1, 1973, to July 31, 2018. Patients were categorized by age as infant/toddler (3 years or younger, n = 105 [30%]), child (older than 3 to 10 years, n = 122 [35%]), or adolescent/adult (older than 10 years, n = 124 [35%]). Demographics are listed in Table 1. Comparison of patients by age revealed mean patient age at presentation (2 vs 6 vs 27 years, P < .001), with no difference in percentage of male sex (85% vs 88% vs 89%, P = .31), white race (67% vs 66% vs 82%, P = .05), or study eye (right eye: 46% vs 54% vs 44%, P = .07).

Demographics of 351 Cases of Coats Disease by Age Category

Table 1:

Demographics of 351 Cases of Coats Disease by Age Category

Clinical features (Figure 1) are listed in Table A (available in the online version of this article). A comparison by age category revealed that the youngest age group had more frequent referral diagnosis of retinoblastoma (29% vs 15% vs 0%, P < .001), higher mean intraocular pressure (18 vs 15 vs 16, P = .04), and worse presenting visual acuity (< 20/200: 80% vs 67% vs 31%, P < .001). The youngest age group had more anterior segment findings of xanthocoria (68% vs 44% vs 6%, P < .001), strabismus (50% vs 36% vs 11%, P < .001), iris neovascularization (12% vs 6% vs 3%, P = .02), and advanced Coats disease stage (stage 3B: 65% vs 38% vs 10%, P < .001). The youngest age group presented with 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). There was no difference by age category in presentation with corneal edema, anterior chamber cholesterolosis, iris atrophy, cataract, phthisis bulbi, or neovascularization of the disc or elsewhere in the retina.

Coats disease presenting features and outcomes by age category (infant/toddler [3 years or younger] versus child [older than 3 to 10 years] and adolescent/adult [older than 10 years]). A 10-month-old male infant with xanthocoria in the left eye was referred to rule out retinoblastoma. (A) Examination revealed inability to fix and follow, esotropia, iris neovascularization, 6 clock hours of retinal telangiectasia and light bulb aneurysms, 8 clock hours of exudation, and (B) total retinal detachment, confirmed on ultrasonography, consistent with Coats disease stage 3B. The patient was treated with cryotherapy, laser photocoagulation, and sub-Tenon's triamcinolone. (C) At 3-year follow-up, visual acuity was no light perception, with dense nuclear sclerotic cataract, posterior synechiae, and (D) a phthisical disorganized globe, confirmed on ultrasonography. A 13-year-old boy was referred for evaluation of asymptomatic Coats disease in the left eye. (E) Examination revealed visual acuity of 20/20, 1 clock hour of retinal telangiectasia and light bulb aneurysms near the equator at the 3-o'clock position (arrow), and (F) mild intraretinal edema and exudation localized to the area of retinal telangiectasia on optical coherence tomography (OCT). The patient was treated with a single session of argon laser photocoagulation. (G) At 6.5-year follow-up, visual acuity remained 20/20, and examination revealed temporal laser scars, with no evidence of active retinal telangiectasia (arrow). By OCT, the fovea was intact and (H) the peripheral retina showed resolution of intraretinal edema and exudation.

Figure 1.

Coats disease presenting features and outcomes by age category (infant/toddler [3 years or younger] versus child [older than 3 to 10 years] and adolescent/adult [older than 10 years]). A 10-month-old male infant with xanthocoria in the left eye was referred to rule out retinoblastoma. (A) Examination revealed inability to fix and follow, esotropia, iris neovascularization, 6 clock hours of retinal telangiectasia and light bulb aneurysms, 8 clock hours of exudation, and (B) total retinal detachment, confirmed on ultrasonography, consistent with Coats disease stage 3B. The patient was treated with cryotherapy, laser photocoagulation, and sub-Tenon's triamcinolone. (C) At 3-year follow-up, visual acuity was no light perception, with dense nuclear sclerotic cataract, posterior synechiae, and (D) a phthisical disorganized globe, confirmed on ultrasonography. A 13-year-old boy was referred for evaluation of asymptomatic Coats disease in the left eye. (E) Examination revealed visual acuity of 20/20, 1 clock hour of retinal telangiectasia and light bulb aneurysms near the equator at the 3-o'clock position (arrow), and (F) mild intraretinal edema and exudation localized to the area of retinal telangiectasia on optical coherence tomography (OCT). The patient was treated with a single session of argon laser photocoagulation. (G) At 6.5-year follow-up, visual acuity remained 20/20, and examination revealed temporal laser scars, with no evidence of active retinal telangiectasia (arrow). By OCT, the fovea was intact and (H) the peripheral retina showed resolution of intraretinal edema and exudation.

Clinical Features of 351 Cases of Coats Disease by Age CategoryClinical Features of 351 Cases of Coats Disease by Age CategoryClinical Features of 351 Cases of Coats Disease by Age Category

Table A:

Clinical Features of 351 Cases of Coats Disease by Age Category

Imaging features are listed in Table B (available in the online version of this article). A comparison of fluorescein angiography features by age category (54 vs 66 vs 72 patients) revealed the youngest age group had greater clock-hour extent of telangiectasia (8 vs 6 vs 4, P = .002) and light bulb aneurysms (7 vs 5 vs 3, P = .004) and greater presence of iris neovascularization (15% vs 2% vs 3%, P = .01). There was no difference by age category in extent of retinal non-perfusion, CME, optic disc leakage, or neovascularization of the disc or elsewhere. Comparison of OCT features (14 vs 44 vs 64 patients) showed the youngest age group with greater prevalence of subretinal fluid (79% vs 59% vs 42%, P = .001), but no difference in CME, epiretinal membrane, vitreomacular traction, subretinal or intraretinal exudation, central macular thickness, or subfoveal choroidal thickness. A comparison of ultrasonography features (85 vs 72 vs 59 patients) revealed the youngest age group with greater prevalence of retinal detachment (78% vs 57% vs 37%, P < .001), including open funnel (41% vs 35% vs 17%, P = .01) and closed funnel (16% vs 8% vs 2%, P = .01) types, but no difference in subretinal exudation or degree of subretinal fluid elevation.

Imaging Features of 351 Cases of Coats Disease by Age Category

Table B:

Imaging Features of 351 Cases of Coats Disease by Age Category

Treatment modalities are listed in Table 2. A comparison by age category revealed that the youngest age group were more likely to be treated with primary enucleation (10% vs 2% vs 2%, P = .01). The oldest age group received a greater total number of treatments (3.3 vs 3.1 vs 4.4, P = .04), were more likely to be treated with argon laser photocoagulation (37% vs 52% vs 73%, P < .001) and intravitreal anti-VEGF (6% vs 9% vs 23%, P < .002), and were less likely to be treated with cryotherapy (74% vs 84% vs 58%, P < .001). There was no difference by age category in management with observation alone or with sub-Tenon's or intravitreal corticosteroid treatment.

Treatment Features of 351 Cases of Coats Disease by Age Category

Table 2:

Treatment Features of 351 Cases of Coats Disease by Age Category

Outcomes are listed in Table 3. Follow-up was available by age category (82 vs 89 vs 77 patients). A comparison by age category revealed that the youngest age group had longer duration of follow-up (70 vs 65 vs 38 months, P = .02), had worse final visual acuity (< 20/200: 83% vs 64% vs 39%, P < .001), and were more likely to be treated with amblyopia patching therapy (15% vs 8% vs 0%, P = .001). The youngest age group had less frequent complete disease resolution (43% vs 65% vs 62%, P = .01), more persistent leaking telangiectasia (52% vs 31% vs 35%, P = .01), subretinal fluid (52% vs 38% vs 32%, P = .04), and foveal exudation (57% vs 37% vs 48%, P = .04), and was more likely to require ultimate enucleation (22% vs 10% vs 6%, P = .01). Most enucleation in the youngest age group was performed as primary treatment (n = 11 of 18, 61%) due to severe disease on presentation. There was no difference in disease recurrence after initial regression or development of iris neovascularization, traction retinal detachment, or glaucoma.

OutcomesOutcomes

Table 3:

Outcomes

Discussion

Coats disease is an idiopathic disorder characterized by incompetent retinal vascular endothelial cells, leading to intraretinal and subretinal exudation, exudative retinal detachment, and vision loss.1 Presenting clinical signs include strabismus and xanthocoria, with clinical features of unilaterality, retinal telangiectasia, micro and macro “light bulb” aneurysms, and intraretinal and subretinal exudation.1 A previous large analysis of Coats disease revealed that this disorder typically affects male patients at a median age of 5 years, but patients of all ages can be affected.3 Herein, we specifically investigated features and outcomes of Coats disease by age category (infant/toddler [3 years or younger], child [older than 3 to 10 years], and adolescent/adult [older than 10 years]) in a large consecutive case cohort of 351 patients at a single center.

The youngest age group presenting with Coats disease at age 3 years or younger demonstrated more advanced disease with frequent referral diagnosis of retinoblastoma, higher presenting intraocular pressure, worse presenting visual acuity, more anterior segment findings of xanthocoria, strabismus, and iris neovascularization, and more advanced Coats disease stage, with greater extent of retinal telangiectasia, light bulb aneurysms, exudation, and subretinal fluid. The youngest patients were more likely to be treated with primary enucleation. The oldest patients received a greater total number of treatments, were more likely to be treated with argon laser photocoagulation and intravitreal anti-VEGF, and were less likely to be treated with cryotherapy. Outcomes revealed the youngest patients had worse final visual acuity, had less disease resolution, and were more likely to require enucleation. Despite longer follow-up in the youngest age group, most enucleation was required as primary treatment due to severe disease on presentation rather than disease progression over time.

Prior small reports have found similar associations between Coats disease severity and age at presentation.7–9 In 2010, a United Kingdom study of 55 cases found a mean age at presentation of 146 months (median: 96 months) with presenting symptoms of leukocoria (n = 7) and strabismus (n = 10) more common in younger patients with a median age of 46 and 52 months, respectively.7 Younger patients were more likely to present with advanced disease stage, with more stage 3 disease (n = 8 of 18 cases) in patients presenting from age 0 to 60 months.7 In 2016, a study from India reported 48 cases of adult-onset Coats disease and found 45 of 646 patients (7%) presented with Coats disease at age 35 years or older.9 By comparison (children vs adults), adult patients had less severe disease localized to fewer than 6 clock hours (69% vs 74%, P < .001), were more amenable to treatment with laser photocoagulation (27% vs 60%, P < .001), required less frequent vitreoretinal surgery (26% vs 6%, P < .003), and required no enucleation (10% vs 0%, P = not applicable).9 In 2018, a study of 98 patients in Switzerland found a mean age at diagnosis of 5.4 years, with more advanced disease stage correlating to younger age at diagnosis.8 A comparison by age category (younger than 4 years [n = 47] vs 4 years and older [n = 51]) revealed that younger patients presented more often with leukocoria (26% vs 10%) and strabismus (53% vs 18%) (P < .0001), and had greater clock-hour extent of peripheral retinal nonperfusion (7.9 vs 4.0, P = .0003) and telangiectasia (7.1 vs 5.6, P = .039), more frequent foveal involvement (98% vs 77%, P = .002), more frequent structural complications (total tractional retinal detachment, macular fibrosis, macular atrophy), more need for enucleation (89% vs 52%, P = .001), and poorer final visual acuity (< 20/200: 68% vs 33%, P = .0009).8 We found similar associations of younger age with leukocoria, strabismus, advanced disease stage, enucleation, and poor visual acuity in our comprehensive cohort of 351 patients.

In a previous large series of 150 cases of Coats disease, classification based on disease severity included stage 1 telangiectasia only, stage 2 telangiectasia and exudation (2A extrafoveal exudation, 2B foveal involvement), stage 3 exudative retinal detachment (3A partial, 3B total), stage 4 total retinal detachment with secondary glaucoma, and stage 5 end-stage disease.2 A comparison by disease stage (stage 1 vs 2 vs 3 vs 4 vs 5) revealed poorer final visual acuity (≤ 20/200) (0% vs 53% vs 74% vs 100% vs 100%) and more frequent enucleation (0% vs 0% vs 7% vs 78% vs 0%) with advancing disease stage,2 consistent with our findings of more advanced disease stage, higher risk of enucleation, and poorer visual acuity outcome in younger patients. We recently investigated predictors of enucleation in Coats disease in 351 cases and found factors included greater clock-hour extent of telangiectasia (P < .001), light bulb aneurysms (P < .001), exudation (P < .001), and subretinal fluid (P < .001).10 Predictive factors for enucleation on adjusted analysis included presentation with iris neovascularization (P = .01), clock-hour extent of light bulb aneurysms by fluorescein angiography (P = .02), open funnel (P = .04) and closed funnel (P = .01) retinal detachment by ultrasonography, and millimeter thickness of subretinal fluid by ultrasonography (P = .001).10 Given the increased prevalence of these features in the youngest age group in this study, it is not surprising that more of these patients required enucleation. Earlier detection and improved treatment methods should be explored to improve globe salvage and visual acuity outcomes in patients with Coats disease presenting at age 3 years or younger.

Current globe salvaging treatment modalities for Coats disease include laser photocoagulation, cryotherapy, intravitreal anti-VEGF therapy, intravitreal or periocular corticosteroids, and surgical drainage of exudative retinal detachment.2–6 Although advanced disease was traditionally managed with cryotherapy or enucleation, Levinson and Hubbard11 demonstrated that treatment with yellow wavelength laser photocoagulation could effectively resolve subretinal fluid in 2 of 5 (40%) eyes with total retinal detachment. More recently, treatment with intravitreal corticosteroid or anti-VEGF injections has proven useful alone or in combination with cryotherapy, laser, or surgical drainage of subretinal fluid.12–16 Additional small series have described successful surgical drainage techniques as monotherapy or as part of combination treatment.17,18 These emerging approaches to Coats disease management are currently being evaluated at our institution and could reduce the risk for enucleation of advanced stage eyes in the future.

Study limitations include the retrospective design and non-standardized treatment regimens, with evolution in management of Coats disease over the 45-year time period that could affect disease outcomes. For example, in an analysis of 351 cases of Coats disease over 45 years by decade (1970s vs 1980s vs 1990s vs 2000s vs 2010s), Shields et al.19 found less need for enucleation (7% vs 27% vs 14% vs 13% vs 6%, P = .04) and more frequent resolution of subretinal fluid (64% vs 59% vs 38% vs 58% vs 72%, P = .01) in more recent years. Imaging technology also improved over the study time period, allowing for improved detection of subretinal fluid with increased availability of OCT, and advances in OCT imaging itself could have allowed for detection of more subtle features in more recent years. Finally, Snellen visual acuity could only be assessed in older, verbal patients, which could have biased visual acuity data, especially at the time of presentation in the youngest age group. A strength of the study is the large number of patients treated at a single center with a long duration of follow-up. To our knowledge, this is the largest series to specifically explore features and outcomes of Coats disease based on age at presentation.

In this study of Coats disease, the youngest patients (3 years or younger) had the poorest presenting visual acuity, more advanced disease stage with greater extent of retinal telangiectasia, light bulb aneurysms, exudation, and subretinal fluid, less frequent disease resolution, more frequent need for enucleation, and poorer visual acuity outcome. Future studies should promote early disease detection and investigate better treatment regimens to improve visual acuity and globe salvage for this group of patients.

References

  1. Shields JA, Shields CL. Review: Coats disease: The 2001 LuEsther T. Mertz Lecture. Retina. 2002;22:80–91. doi:10.1097/00006982-200202000-00014 [CrossRef]11884883
  2. Shields JA, Shields CL, Honavar SG, Demirci H, Cater J. Classification and management of Coats disease: The 2000 Proctor Lecture. Am J Ophthalmol. 2001;131:572–583. doi:10.1016/S0002-9394(01)00896-0 [CrossRef]11336931
  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;131:561–571. doi:10.1016/S0002-9394(00)00883-7 [CrossRef]11336930
  4. Ong SS, Buckley EG, McCuen BW 2nd, et al. Comparison of visual outcomes in Coats' disease: a 20-year experience. Ophthalmology. 2017;124:1368–1376. doi:10.1016/j.ophtha.2017.03.051 [CrossRef]28461016
  5. Budning AS, Heon E, Gallie BL. Visual prognosis of Coats' disease. JAAPOS. 1998;2:356–359.
  6. Kusaka S. Surgical management of Coats disease. Asia Pac J Ophthalmol (Phila). 2018;7:156–159.
  7. Morris B, Foot B, Mulvihill A. A population-based study of Coats disease in the United Kingdom I: epidemiology and clinical features at diagnosis. Eye (Lond). 2010;24:1797–1801. doi:10.1038/eye.2010.126 [CrossRef]
  8. Daruich A, Matet A, Munier FL. Younger age at presentation in children with Coats disease is associated with more advanced stage and worse visual prognosis: a retrospective study. Retina. 2018;38:2239–2246. doi:10.1097/IAE.0000000000001866 [CrossRef]
  9. Rishi E, Rishi P, Appukuttan B, Uparkar M, Sharma T, Gopal L. Coats' disease of adult-onset in 48 eyes. Indian J Ophthalmol. 2016;64:518–523. doi:10.4103/0301-4738.190141 [CrossRef]27609165
  10. Udyaver S, Dalvin LA, Lim LS, et al. Predictors of enucleation in Coats disease: analysis of 351 eyes from 351 patients at a single center. In press.
  11. Levinson JD, Hubbard GB 3rd, . 577-nm yellow laser photocoagulation for Coats disease. Retina. 2016;36:1388–1394. doi:10.1097/IAE.0000000000000874 [CrossRef]
  12. Bergstrom CS, Hubbard GB 3rd, . Combination intravitreal triamcinolone injection and cryotherapy for exudative retinal detachments in severe Coats disease. Retina. 2008;28(3 suppl):S33–S37. doi:10.1097/IAE.0b013e318159ecad [CrossRef]
  13. Ghazi NG, Al Shamsi H, Larsson J, Abboud E. Intravitreal triamcinolone in Coats' disease. Ophthalmology. 2012;119:648–649. doi:10.1016/j.ophtha.2011.09.059 [CrossRef]22385486
  14. Lin CJ, Hwang JF, Chen YT, Chen SN. The effect of intravitreal bevacizumab in the treatment of Coats disease in children. Retina. 2010;30:617–622. doi:10.1097/IAE.0b013e3181c2e0b7 [CrossRef]
  15. Gaillard MC, Mataftsi A, Balmer A, Houghton S, Munier FL. Ranibizumab in the management of advanced Coats disease stages 3B and 4: long-term outcomes. Retina. 2014;34:2275–2281. doi:10.1097/IAE.0000000000000248 [CrossRef]25075562
  16. Ramasubramanian A, Shields CL. Bevacizumab for Coats' disease with exudative retinal detachment and risk of vitreoretinal traction. Br J Ophthalmol. 2012;96:356–359. doi:10.1136/bjophthalmol-2011-300141 [CrossRef]
  17. Adam RS, Kertes PJ, Lam WC. Observations on the management of Coats' disease: less is more. Br J Ophthalmol. 2007;91:303–306. doi:10.1136/bjo.2006.103382 [CrossRef]
  18. Stanga PE, Jaberansari H, Bindra MS, Gil-Martinez M, Biswas S. Transcleral drainage of subretinal fluid, anti-vascular endothelial growth factor, and wide-field imaging-guided laser in Coats exudative retinal detachment. Retina. 2016;36:156–162. doi:10.1097/IAE.0000000000000669 [CrossRef]
  19. Shields CL, Udyaver S, Dalvin LA, et al. Coats disease in 351 eyes: analysis of features and outcomes over 45 years (by decade) at a single center. Indian J Ophthalmol. 2019;67:772–783. doi:10.4103/ijo.IJO_449_19 [CrossRef]31124485

Demographics of 351 Cases of Coats Disease by Age Category

CharacteristicAge ≤ 3 Years (105 Eyes in 105 Patients) (%)Age > 3 to 10 Years (122 Eyes in 122 Patients) (%)Age > 10 Years (124 Eyes in 124 Patients) (%)PTotal (351 Eyes in 351 Patients) (%)
Age at presentation (y)
  Mean (median, range)2 (2, 0 to 3)6 (5, 3 to 10)27 (17, 11 to 79)< .001a12 (6, 0 to 79)
Sex
  Male89 (85)107 (88)100 (89).31296 (84)
  Female16 (15)15 (12)24 (21)55 (16)
Race
  White70 (67)81 (66)102 (82).05253 (72)
  African American21 (20)24 (20)9 (7)54 (15)
  Asian1 (1)4 (3)4 (3)9 (3)
  Hispanic12 (11)9 (7)7 (6)28 (8)
  Middle Eastern1 (1)1 (1)1 (1)3 (1)
  Indian0 (0)3 (2)1 (1)4 (1)
Laterality
  Unilateral105 (100)122 (100)124 (100)NA351 (100)
  Bilateral0 (0)0 (0)0 (0)0 (0)
Study eye
  Right eye48 (46)66 (54)49 (44).07163 (46)
  Left eye57 (54)56 (46)75 (67)188 (54)

Treatment Features of 351 Cases of Coats Disease by Age Category

FeatureAge ≤ 3 Years (105 Eyes in 105 Patients) (%)Age > 3 to 10 Years (122 Eyes in 122 Patients) (%)Age > 10 Years (124 Eyes in 124 Patients) (%)PaTotal (351 Eyes in 351 Patients) (%)
Observation19 (18)23 (19)31 (25).3573 (21)
Primary enucleation11 (10)3 (2)3 (2).01b17 (5)
Medical or laser therapy82 (78)96 (79)90 (73).47261 (74)
  No. of treatments, mean (median, range)3.3 (3, 1 to 13)3.1 (3, 1 to 15)4.4 (2, 1 to 26).04b3.6 (3, 1 to 26)
Argon laser photocoagulation30 (37)50 (52)66 (73)< .001b146 (42)
  No. of treatments, mean (median, range)2.3 (2, 1 to 11)1.6 (1, 1 to 4)2.4 (2, 1 to 20).092.1 (1, 1 to 20)
Cryotherapy61 (74)81 (84)52 (58)< .001b194 (55)
  No. of treatments, mean (median, range)2.3 (2, 1 to 9)2.2 (2, 1 to 7)1.7 (1, 1 to 6).03b2.1 (2, 1 to 9)
Sub-Tenon's corticosteroid11 (13)14 (15)16 (18).7141 (12)
  No. of treatments, mean (median, range)1.4 (1, 1 to 3)1.3 (1, 1 to 4)1.2 (1, 1 to 3).801.3 (1, 1 to 4)
Intravitreal corticosteroid4 (5)5 (56)5 (6).9814 (4)
  No. of treatments, mean (median, range)1.3 (1, 1 to 2)1.2 (1, 1 to 2)6.0 (3, 1 to 14).092.9 (1, 1 to 14)
Intravitreal anti-VEGF5 (6)9 (9)21 (23).002b35 (10)
  No. of treatments, mean (median, range)2.4 (2, 1 to 5)2.1 (1, 1 to 7)4.2 (2, 1 to 20).403.4 (2, 1 to 20)
No follow-up23 (22)33 (27)47 (38).14103 (29)

Outcomes

FeatureAge ≤ 3 Years (105 Eyes in 105 Patients) (%)Age > 3 to 10 Years (122 Eyes in 122 Patients) (%)Age > 10 Years (124 Eyes in 124 Patients) (%)PaTotal (351 Eyes in 351 Patients) (%)
Follow-up (months), mean (median, range)70 (30, 3 to 466)65 (37, 3 to 466)38 (22, 3 to 279).02b58 (31, 3 to 466)
Visual acuity
  Verbaln = 46n = 80n = 74N = 200
    > 20/401 (2)10 (13)26 (35)< .001b37 (19)
    20/40 to 20/2007 (15)19 (24)19 (26)45 (23)
    < 20/20038 (83)51 (64)29 (39)118 (59)
  Preverbaln = 36n = 9n = 3N = 48
    Fix and follow2 (6)1 (11)0 (0).413 (6)
    Poor fix and follow1 (3)0 (0)0 (0)1 (2)
    No fix and follow20 (56)2 (22)0 (0)22 (46)
    Uncooperative13 (36)6 (67)3 (100)22 (46)
Patching
  Done12 (15)7 (8)0 (0).001b19 (8)
  Not done70 (85)82 (92)77 (100)229 (92)
  Outcome of patchingn = 12n = 7n = 0N = 19
    Improvement in vision10 (83)3 (43)0 (0).1313 (68)
    Worse vision0 (0)0 (0)0 (0)0 (0)
    No change in vision2 (17)4 (57)0 (0)6 (32)
Disease resolution
  Resolved35 (43)58 (65)48 (62).01b141 (87)
  Not resolved47 (57)31 (35)29 (38)107 (13)
Disease recurrence
  Recurrence4 (5)3 (3)1 (1).498 (3)
  No recurrence78 (5)86 (90)76 (99)240 (97)
Leaking telangiectasia resolution
  Resolved39 (48)61 (69)50 (65).01b150 (60)
  Not resolved43 (52)28 (31)27 (35)98 (40)
  Time to resolution (months), mean (median, range)20 (14, 1 to 110)22 (11, < 1 to 241)9 (13, < 1 to 49).9017 (13, < 1 to 139)
Subretinal fluid resolutionn = 77n = 88n = 74N = 239
  Resolved37 (48)55 (63)50 (68).04b142 (60)
  Not resolved40 (52)33 (38)24 (32)97 (40)
  Time to resolution (months), mean (median, range)19 (13, 1 to 329)18 (10, < 1 to 286)8 (8, < 1 to 45).4414 (14, < 1 to 143)
Foveal exudation resolutionn = 79n = 83n = 58N = 220
  Resolved34 (43)52 (63)30 (52).04b116 (53)
  Not resolved45 (57)31 (37)28 (48)104 (47)
  Time to resolution (months), mean (median, range)22 (14, 1 to 121)5 (13, < 1 to 355)9 (15, < 1 to 60).998 (14, < 1 to 143)
NVI
  Development11 (13)9 (10)7 (9).6927 (11)
  No development71 (87)80 (90)70 (91)221 (89)
  Time to NVI (months), mean (median, range)8 (0, 0 to 91)19 (0, 0 to 130)11 (0, 0 to 38).6511 (0, 0 to 130)
TRD
  Development8 (10)13 (15)4 (5).1325 (10)
  No development74 (90)76 (85)73 (95)223 (90)
  Time to TRD (months), mean (median, range)58 (22, 4 to 255)24 (14, 0 to 98)0 (4, 0 to 22).2426 (12, 0 to 255)
Glaucoma
  Development13 (16)7 (8)9 (12).2729 (12)
  No development69 (84)82 (2)68 (88)219 (88)
  Time to glaucoma (months), mean (median, range)1 (0, 0 to 3)7 (1, 0 to 20)0 (0, 0 to 6).201 (0, 0 to 20)
Enucleation
  Done18 (22)9 (10)5 (6).01b32 (13)
  Not done64 (78)80 (90)72 (94)216 (87)
Time to enucleation (months), mean (median, range)18 (0, 0 to 170)29 (8, 0 to 184)4 (0, 0 to 19).6419 (0, 0 to 184)

Clinical Features of 351 Cases of Coats Disease by Age Category

FeatureAge ≤ 3 Years (105 Eyes in 105 Patients) (%)Age > 3 to 10 Years (122 Eyes in 122 Patients) (%)Age > 10 Years (124 Eyes in 124 Patients) (%)PaTotal (351 Eyes in 351 Patients) (%)
Referral diagnosis
  Coats disease44 (42)84 (82)85 (69)213 (61)
  Retinoblastoma30 (29)15 (15)0 (0)< .001b45 (13)
  Retinal hemangioma0 (0)1 (1)8 (6)9 (3)
  No diagnosis31 (30)22 (18)31 (25)84 (24)
Intraocular pressure, mean (median, range)18 (15, 7 to 60)15 (15, 7 to 48)16 (15, 6 to 57).04b16 (15, 6 to 60)
Visual acuity
  Verbal visual acuityn = 10n = 106n = 124N = 240
    ≥ 20/401 (10)12 (11)39 (31)52 (22)
    20/40 to 20/2002 (20)23 (22)46 (37)< .001b71 (30)
    < 20/2008 (80)71 (67)39 (31)118 (49)
  Preverbal visual acuityn = 94n = 16n = 0N = 110
    Fixes and follows light16 (17)3 (19)0 (0)19 (17)
    Poor fix and follow15 (16)1 (6)0 (0).6516 (15)
    No fix and follow60 (64)10 (63)0 (0)70 (64)
    Uncooperative3 (3)2 (13)0 (0)5 (5)
Related anterior segment findings
  Xanthocoria71 (68)54 (44)7 (6)< .001b132 (36.9)
  Strabismus53 (50)44 (36)14 (11)< .001b111 (31.3)
    Esotropia23 (22)18 (15)4 (3)45 (12.3)
    Exotropia29 (28)25 (21)10 (8).8364 (17.8)
    Hypertropia1 (1)1 (1)0 (0)2 (1.0)
  Corneal edema0 (0)1 (1)3 (2).214 (1.0)
  Anterior chamber cholesterolosis1 (1)0 (0)0 (0).311 (0.2)
  Iris neovascularization13 (12)7 (6)4 (3).02b24 (4.3)
  Iris atrophy2 (2)2 (2)0 (0).334 (1.0)
  Cataract1 (1)7 (6)9 (7).0717 (5.1)
  Pthisis0 (0)1 (1)0 (0).391 (0.2)
Coats disease stage
  10 (0)0 (0)5 (4)5 (1)
  2a0 (0)5 (4)26 (21)31 (9)
  2b8 (8)17 (15)17 (14)42 (12)
  3a16 (6)17 (15)23 (19)46 (13)
  3a211 (10)25 (21)32 (26)< .001b68 (19)
  3b68 (65)45 (38)12 (10)125 (36)
  412 (11)5 (4)3 (2)20 (6)
  50 (0)1 (1)2 (2)3 (1)
  Unknownc0 (0)7 (6)4 (3)11 (3)
Telangiectasia
  Clock hours, mean (median, range)7 (7, 0 to 12)5 (4, 0 to 2)4 (3, 0 to 12)< .001b5 (5, 0 to 12)
  Telangiectasia quadrants
    02 (2)6 (5)8 (6)16 (5)
    119 (18)37 (33)51 (41)107 (30)
    219 (18)39 (34)39 (31)< .001b97 (28)
    329 (28)18 (16)16 (13)63 (18)
    434 (32)14 (12)7 (6)55 (16)
    No viewd2 (2)8 (7)3 (2)13 (4)
  Location of most telangiectasia
    Equator to ora serrata77 (73)79 (68)71 (57)227 (65)
    Macula to equator23 (22)28 (25)42 (34)93 (26)
    Macula1 (1)1 (1)0 (0).142 (1)
    None2 (2)6 (5)8 (6)16 (5)
    No viewd2 (2)8 (7)3 (2)13 (4)
Light bulb aneurysms
  Clock hours, mean (median, range)7 (6, 0 to 12)4 (4, 0 to 12)3 (3, 0 to 12)< .001b5 (4, 0 to 12)
  Light bulb quadrants
    07 (7)12 (11)21 (17)40 (11)
    123 (22)39 (34)50 (40)112 (32)
    222 (21)44 (39)34 (27)< .001b100 (28)
    320 (19)9 (8)13 (10)42 (12)
    431 (30)10 (9)3 (2)44 (13)
    No viewd2 (2)8 (7)3 (2)13 (4)
  Location of most light bulbs
    Equator to ora76 (72)75 (66)65 (53)216 (62)
    Macula to equator20 (19)26 (23)34 (27)80 (23)
    Macula0 (0)1 (1)1 (1)0.072 (1)
    None7 (7)12 (10)21 (17)0 (11)
    No viewd2 (2)8 (7)3 (2)13 (4)
Exudation
  Clock hours, mean (median, range)10 (12, 0 to 12)7 (6, 0 to 12)5 (5, 0 to 12)< .001b7 (7, 0 to 12)
  Exudation quadrants
    01 (1)2 (2)9 (7)12 (3)
    12 (2)19 (16)34 (27)55 (16)
    214 (13)38 (33)44 (35)< .001b96 (27)
    318 (17)17 (15)17 (14)52 (15)
    468 (65)40 (35)17 (14)125 (36)
    No viewd2 (2)6 (5)3 (2)11 (3)
  Location of most exudation
    Equator to ora serrata5 (5)7 (6)27 (22)39 (11)
    Macula to equator48 (46)52 (45)61 (49)161 (46)
    Macula49 (47)55 (47)24 (19)< .001b128 (36)
    None1 (1)2 (2)9 (7)12 (3)
    No viewd2 (2)6 (5)3 (2)11 (3)
Subretinal fluid
  Clock hours, mean (median, range)10 (12, 0 to 12)7 (7, 0 to 12)4 (3, 0 to 12)< .001a7 (6, 0 to 12)
  Subretinal fluid quadrants
    05 (5)12 (10)36 (29)53 (15)
    17 (7)24 (21)32 (26)63 (18)
    24 (4)22 (19)30 (24)< .001b56 (16)
    35 (5)15 (13)8 (6)28 (8)
    482 (78)44 (38)15 (12)141 (40)
    No viewd2 (2)5 (4)3 (2)10 (3)
  Location of most subretinal fluid
    Equator to ora serrata5 (5)21 (18)21 (17)47 (13)
    Macula to equator21 (20)43 (37)43 (35)107 (30)
    Macula0 (0)4 (3)11 (9)< .00115 (4)
    Total serous retinal detachment72 (69)37 (32)10 (8)119 (34)
    None5 (5)12 (10)36 (29)53 (15)
    No viewd2 (2)5 (4)3 (2)10 (3)
Most posterior extent of disease
  Ora0 (0)0 (0)6 (5)6 (2)
  Equator3 (4)7 (6)29 (23)39 (11)
  Arcade11 (10)21 (18)34 (27)66 (19)
  Macula18 (17)28 (24)22 (18)< .001b68 (19)
  Fovea8 (8)24 (21)20 (16)52 (15)
  Total serous retinal detachment63 (60)36 (31)10 (8)109 (31)
  No viewd2 (2)6 (5)3 (2)11 (3)
Related posterior segment findings
  Vitreoretinal traction5 (5)9 (8)14 (11).1928 (8)
  Neovascularization of the disc0 (0)0 (0)2 (2).162 (1)
  Neovascularization of the retina0 (0)2 (2)4 (3).176 (2)
  Retinal hemorrhage12 (11)9 (8)24 (19).02b45 (13)
  Vitritis0 (0)2 (2)3 (2).305 (1)

Imaging Features of 351 Cases of Coats Disease by Age Category

FeatureAge ≤ 3 Years (105 Eyes in 105 Patients) (%)Age > 3 to 10 Years (122 Eyes in 122 Patients) (%)Age > 10 Years (124 Eyes in 124 Patients) (%)PaTotal (351 Eyes in 351 Patients) (%)
Fluorescein angiographybn = 54n = 66n = 72N = 192
  Telangiectasia
    Clock hours, mean (median, range)8 (8, 0 to 12)6 (6, 0 to 12)4 (4, 0 to 12).002c6 (6, 0 to 12)
  Most affected quadrant
    Temporal38 (70)52 (79)55 (76)145 (76)
    Nasal4 (7)3 (5)6 (5)13 (7)
    Inferior8 (15)6 (9)5 (4).9119 (10)
    Superior3 (6)4 (6)4 (3)11 (6)
    No telangiectasia1 (2)1 (2)2 (2)4 (12)
Light bulb aneurysms
  Clock hours, mean (median, range)7 (7, 0 to 12)5 (5, 0 to 12)3 (3, 0 to 12).004c5 (5, 0 to 12)
  Most affected quadrant
    Temporal39 (72)50 (76)48 (39)137 (71)
    Nasal4 (7)2 (3)5 (4)11 (6)
    Inferior8 (15)6 (9)7 (6).5621 (11)
    Superior2 (4)5 (8)5 (4)12 (6)
    No light bulb aneurysms1 (2)3 (5)7 (6)11 (6)
Retinal nonperfusion
  Clock hours, mean (median, range)7 (7, 0 to 12)6 (6, 0 to 12)4 (3, 0 to 12).335 (5, 0 to 12)
  Most affected quadrant
    Temporal32 (61)43 (65)46 (37)121 (63)
    Nasal6 (11)2 (3)4 (3)12 (6)
    Inferior6 (11)6 (9)1 (1).1813 (7)
    Superior2 (4)4 (6)3 (2)9 (5)
    No retinal nonperfusion8 (15)11 (17)18 (15)37 (19)
Other fluorescein features
  Cystoid macular edema4 (7)5 (8)10 (14).3919 (10)
  Optic disc leakage1 (2)0 (0)1 (1).742 (1)
  Neovascularization of the disc1 (2)0 (0)1 (1).742 (1)
  Neovascularization of the retina1 (2)1 (2)3 (4).635 (3)
  Neovascularization of the iris8 (15)1 (2)2 (3).01c11 (6)
Optical coherence tomographybn = 14n = 44n = 64N = 122
  Cystoid macular edema1 (7)13 (30)24 (38).1838 (31)
  Epiretinal membrane2 (14)6 (14)22 (34).0530 (24)
  Vitreomacular traction0 (0)3 (7)7 (11).7010 (8)
  Subretinal fluid11 (79)22 (59)27 (42).001c60 (49)
  Subretinal exudation11 (79)34 (77)50 (78).2395 (78)
  Intraretinal exudation0 (0)11 (25)19 (30).1130 (25)
  Central macular thickness (µm), mean (median, range)370 (362, 308 to 502)335 (328, 176 to 692)367 (336, 191 to 647).20358 (336, 176 to 692)
  Subfoveal choroid thickness (µm), mean (median, range)269 (272, 171 to 384)263 (233, 96 to 473)288 (286, 0 to 630).59279 (282, 0 to 630)
Ultrasoundbn = 85n = 72n = 59N = 216
  Retinal detachment66 (78)41 (57)22 (37)< .001c129 (60)
    Open funnel retinal detachment35 (41)25 (35)10 (17).01c70 (32)
    Closed funnel retinal detachment14 (16)6 (8)1 (2).01c21 (10)
  Subretinal exudate81 (95)64 (89)50 (85).10195 (90)
  Subretinal fluid elevation (mm), mean (median, range)7.6 (7, 0 to 19)5 (2, 0 to 18)2 (0, 0 to 14).055 (2, 0 to 19)
Authors

From the Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania (LAD, SU, L-ASL, MM, HTA, CTLK, CLS); and the Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota (LAD).

Supported in part by the Eye Tumor Research Foundation, Philadelphia, PA (CLS).

The authors have no financial or proprietary interest in the materials presented herein.

Correspondence: Carol L. Shields, MD, Ocular Oncology Service, Wills Eye Hospital, 840 Walnut Street, Suite 1440, Philadelphia, PA 19107. E-mail: carolshields@gmail.com

Received: February 25, 2019
Accepted: July 09, 2019

10.3928/01913913-20190716-01

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