Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture

doi:10.1016/S0002-9394(00)00883-7

American Journal of Ophthalmology

Volume 131, Issue 5, May 2001, Pages 561-571

This study was presented as part of the 2000 Gifford Memorial Lecture, Chicago Ophthalmological Society, Chicago, Illinois, June 1, 2000 (Dr J Shields).

https://doi.org/10.1016/S0002-9394(00)00883-7 Get rights and content

Abstract

PURPOSE: The purpose of this report is to review the clinical variations and natural course of Coats disease, using strict diagnostic guidelines.

METHODS: In a retrospective, consecutive series, Coats disease was defined as idiopathic retinal telangiectasia with intraretinal or subretinal exudation without appreciable signs of retinal or vitreal traction. We reviewed our experience with the clinical features, complications, and diagnostic approaches to Coats disease.

RESULTS: In 150 consecutive patients (158 eyes), Coats disease was diagnosed at a median age of 5 years (range, 1 month to 63 years), occurred in 114 males (76%), and was unilateral in 142 patients (95%). There was no predilection for race or laterality. The most common referral diagnoses were Coats disease in 64 cases (41%) and retinoblastoma in 43 (27%). The first symptom or sign was decreased visual acuity in 68 cases (34%), strabismus in 37 (23%), leukocoria in 31 (20%), and 13 patients (8%) were asymptomatic. Visual acuity at presentation was 20/200 to no light perception in 121 eyes (76%). The anterior segment was normal in 142 eyes (90%). The retinal telangiectasia involved the midperipheral or peripheral fundus in 156 of the 158 eyes (99%) and was restricted to the macular area in two eyes (1%); involved mainly the temporal fundus in 66 eyes (42%), inferior fundus in 41 eyes (26%), and more than one sector in 34 eyes (22%). Retinal exudation was present in all 12 clock hours in 86 eyes (55%) and six or more clock hours in 115 eyes (73%). There was a total retinal detachment in 74 eyes (47%) and neovascular glaucoma in 12 (8%). Retinal macrocysts were present in 18 eyes (11%), a vasoproliferative tumor in nine eyes (6%) and retinal neovascularization in four eyes (3%). Fluorescein angiography in 49 of the 158 eyes (37%) disclosed early hyperfluorescence of the telangiectasias and macular edema in 18 of eyes (37%). Ultrasonography typically showed a retinal detachment but no solid mass.

CONCLUSIONS: Coats disease is a distinct clinical entity characterized by idiopathic retinal telangiectasia and retinal exudation. It is usually unilateral, occurs mostly in young males, and can cause severe visual loss resulting from exudative retinal detachment. The clinician should follow strict criteria in making the diagnosis, to avoid confusing Coats disease with other forms of exudative retinopathy.

Section snippets

Patients and methods

Based on our literature review and personal experience, we defined Coats disease as idiopathic retinal telangiectasia with intraretinal and/or subretinal exudation without appreciable retinal or vitreal traction (Figure 1). We defined retinal telangiectasia as dilated, irregular-caliber, small to medium-sized retinal blood vessels and exudative retinopathy as yellow exudation in the sensory retina or subretinal space. Other evident causes of vasculopathy and exudative retinopathy, such as

Results

During the 24 years of this study, 150 patients (158 eyes) were coded with the diagnosis of with Coats disease, according to the aforementioned criteria (Figure 1). All patients were evaluated personally by the senior authors (Drs Shields) and their colleagues.

Demographic data are presented in Table 1. The mean patient age at the time of diagnosis was 11 years (median, 5 years; range, 1 month to 63 years); 123 (82%) were Caucasians, 17 (11%) African Americans, eight (5%) Hispanics, and two (1%)

Discussion

The terms Coats disease, Coats syndrome, or Coats reaction have been used to define a number of conditions characterized by exudative retinopathy. These include fundus changes seen with muscular dystrophy,12, 13, 14 Turner syndrome,15 epidermal nevus syndrome,16 Cornelia de Lange syndrome,17 Alport syndrome,18 familial renal-retinal dystrophy (Senor-Loken syndrome),19 13-q deletion syndrome,20 post–renal transplantation,21 inversion of chromosome 3,22 Hallermann-Streiff syndrome,23 and aplastic

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^☆: This work was supported by the Eye Tumor Research Foundation, Philadelphia, Pennsylvania, the Award of Merit in Retina Research, Houston, Texas (Dr J Shields) and the Macula Foundation, New York, New York (Dr C Shields), Orbis International, New York, New York (Dr Honavar), and the Hyderabad Eye Research Foundation, Hyderabad, India (Dr Honavar).

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Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture☆

Abstract

Section snippets

Patients and methods

Results

Discussion

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Surv Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Ophthalmology

Ophthalmology

Forms of retinal diseases with massive exudation

Roy Lond Ophthalmol Hosp Rep

Ueber eine durch Vorkommen multipler Miliaraneurysmen charakterisierteForm von Retinaldegeneration

Albrecht von Graefe’s Arch Klin Ophthalmol

Coats’ disease

Coats’ disease

Coats’ disease

Coats’ disease. I. Review of the literature, diagnostic criteria, clinical findings, and plasma lipid studies

Br J Ophthalmol

Coats’ disease and congenital vascular retinopathy

Trans Am Ophthalmol Soc