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. 2017 Aug;37(8):1591-1598.
doi: 10.1097/IAE.0000000000001399.

SUBFOVEAL NODULE IN COATS' DISEASE: Toward an Updated Classification Predicting Visual Prognosis

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Free PMC article

SUBFOVEAL NODULE IN COATS' DISEASE: Toward an Updated Classification Predicting Visual Prognosis

Alejandra L Daruich et al. Retina. .
Free PMC article

Abstract

Purpose: To determine the prevalence, clinical characteristics and nature of subfoveal nodules in Coats' disease and the associated impact on the long-term visual outcome.

Methods: Consecutive cases of Coats' disease with foveal exudation were retrospectively reviewed. The presence of a subfoveal nodule or macular fibrosis was recorded. Clinical characteristics, retinal imaging, and outcome were analyzed by comparative analysis. The histopathological description of an enucleated eye with subfoveal nodule was performed.

Results: Among 40 patients presenting unilateral Stage 2B or 3A1 Coats' disease, a subfoveal nodule was detected in 21 patients (52.5%). The median follow-up was 4.7 years. Nineteen patients (47.5%) did not present a subfoveal nodule. Three patients (15.8%) without subfoveal nodule and 21 patients (100%) with subfoveal nodule progressed to a macular fibrotic scar (P < 0.0001), and the mean time of macular fibrosis onset was 11.0 ± 2.6 months. Final visual acuity was significantly worse in patients who presented a subfoveal nodule at diagnosis (P = 0.01). Of 18 cases with subfoveal nodule who underwent fluorescein angiography, retinal-retinal anastomosis and neovascularization were detected in 13 (72.2%) and 2 eyes (11.1%), respectively. Histopathological analysis of a subfoveal nodule revealed an aggregate of proteinaceous material including fibrin, spindle cells, macrophages, and pigmented cells.

Conclusion: The presence of a subfoveal nodule at presentation is a predictive factor for macular fibrosis development and worse visual outcome in patients with Coats' disease. These observations suggest an updated classification introducing two subcategories within Stage 2B: without subfoveal nodule (Stage 2B1) and with subfoveal nodule (Stage 2B2).

Conflict of interest statement

None of the authors has any financial/conflicting interests to disclose.

Figures

Fig. 1.
Fig. 1.
A and B. Two-year-old boy presenting with Stage-2B Coats' disease. Color fundus photograph (A) showed a yellow lesion suggestive of retinal exudates, and B-scan ultrasonography (B) confirmed the absence of subretinal nodule. C and D. Four-year-old boy presenting with Stage-2B Coats' disease. On color fundus photograph (A), a yellow, exudative, protruding, subfoveal spheroidal lesion was visible and was associated with two second-order arteries and a third-order vein entering the lesion. B-scan ultrasonography (B) confirmed the presence of a subretinal nodule (star).
Fig. 2.
Fig. 2.
A. Color fundus photograph of a two-year-old boy presenting with Stage-2B Coat's disease and a subfoveal nodule. B and D. Fluorescein angiography at presentation showed a hypofluorescent subfoveal lesion during early phase and midphase (B and C), which became hyperfluorescent with minimal leakage during the late phase (D) because of the presence of a retinal–retinal anastomosis (C and D, yellow arrows). Note the peripheral retinal telangiectasias and nonperfusion areas (C) characteristic of Coats' disease. E. Color fundus photograph from the same patient acquired 6 months later after he developed macular fibrosis. F and H. Fluorescein angiography showed an increasing hyperfluorescence with well-delimited borders corresponding to staining. Note the scars resulting from peripheral laser photocoagulation of nonperfused areas (G and H).
Fig. 3.
Fig. 3.
A. Color fundus photograph of a one-year-old boy presenting with Stage-3A1 Coat's disease and a subfoveal nodule. B. Fluorescein angiography showed a retinal–retinal anastomosis at the level of the subfoveal nodule (dotted-line rectangle). C. Magnification of the rectangular area (B) showing the retinal–retinal anastomosis.
Fig. 4.
Fig. 4.
Spectral-domain optical coherence tomography horizontal sections in 3 patients with Coats' disease and a fibrotic subfoveal nodule observed 9 (A), 12 (B), and 26 (C) months after presentation, respectively.
Fig. 5.
Fig. 5.
Histopathological analysis of a subfoveal nodule from an eye with Coats' disease. Specific stainings were performed for hematoxylin eosin (A, ×126), Martius Scarlett Blue (B, ×126), cytokeratins (AE1–AE3) (C, ×63), CD68 (D, ×126). The subfoveal lesion was formed by a round aggregate of proteinaceous material including fibrin (B) admixed with spindle cells, macrophages, and pigmented cells. Some of the cells within the lesion expressed pancytokeratins (C) consistent with a retinal pigment epithelium origin, while other expressed CD68 (D). Specific staining for endothelial cells was negative.

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References

    1. Coats G. Forms of retinal diseases with massive exudation. Roy Lond Ophthalmol Hosp Rep 1908;17:440–525.
    1. Shields JA, Shields CL, Honavar SG, et al. Classification and management of Coats disease: the 2000 proctor lecture. Am J Ophthalmol 2001;131:572–583. - PubMed
    1. Khurana RN, Samuel MA, Murphree AL, et al. Subfoveal nodule in Coats' disease. Clin Exp Ophthalmol 2005;33:301–302. - PubMed
    1. Sigler EJ, Calzada JI. Retinal angiomatous proliferation with chorioretinal anastomosis in childhood Coats disease: a reappraisal of macular fibrosis using multimodal imaging. Retina 2015;35:537–546. - PubMed
    1. Jumper JM, Pomerleau D, McDonald HR, et al. Macular fibrosis in Coats disease. Retina 2010;30:S9–S14. - PubMed
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