Ophthalmic Surgery, Lasers and Imaging Retina

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Case Report 

Bilateral Fluorescein Angiographic Findings in Unilateral Coats’ Disease

Thomas S. Shane, MD; Audina M. Berrocal, MD; Ditte J. Hess, CRA

Abstract

Two patients with clinically unilateral Coats’ disease were imaged with fluorescein angiography during an examination under anesthesia. Both patients were found to have abnormal retinal vasculature in their contralateral eye. These findings may represent a previously unrecognized bilateral component of Coats’ disease.

Abstract

Two patients with clinically unilateral Coats’ disease were imaged with fluorescein angiography during an examination under anesthesia. Both patients were found to have abnormal retinal vasculature in their contralateral eye. These findings may represent a previously unrecognized bilateral component of Coats’ disease.

Bilateral Fluorescein Angiographic Findings in Unilateral Coats’ Disease

From Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida.

Supported by an unrestricted grant from Research to Prevent Blindness, Inc., New York, New York, and NIH Center Grant P30-EY014801.

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

Address correspondence to Thomas S. Shane, MD, 900 NW 17th Street, Miami, FL 33136. E-mail: tshane@med.miami.edu

Received: September 22, 2010
Accepted: January 06, 2011
Posted Online: February 10, 2011

Introduction

Coats’ disease is an idiopathic condition characterized by retinal telangiectasia, exudation, and exudative retinal detachment.1,2 One of the mysteries of this condition is its unilateral nature. Using dilated fundus examinations, previous studies have demonstrated that Coats’ disease affects only one eye in 80% to 95% of cases.3,4 However, no study describes peripheral fluorescein angiography findings in the contralateral retina of patients with unilateral Coats’ disease. It is therefore possible that bilateral peripheral vascular changes exist that are unrecognized by a standard diagnostic work-up. We describe two patients with clinically unilateral Coats’ disease and peripheral vascular abnormalities by fluorescein angiography in their contralateral eye.

Case Reports

Case 1

A 4-year-old boy was referred to Bascom Palmer Eye Institute for evaluation of retinal exudation in his right eye that was noted during a routine optometry visit. The patient denied an ocular history or family history of eye disease. On examination, the best-corrected visual acuity was 20/30 in the right eye and 20/100 in the left eye. Anterior segment examination was normal. Dilated fundus examination was normal in the right eye, but demonstrated exudation in the macula and peripheral retina of the left eye (Fig. 1). Dilated, aneurysmal vessels and telangiectasias were also noted in the peripheral retina of the left eye. The patient was diagnosed as having unilateral Coats’ disease and subsequently underwent an examination under anesthesia with fluorescein angiography by Retcam (Clarity Medical Systems, Pleasanton, CA). In the left eye, the fluorescein angiography demonstrated 360° of peripheral avascular retina, bordered posteriorly by leaking, telangiectatic retinal vascular abnormalities (Fig. 2). In the right eye, the fluorescein angiography was remarkable for peripheral avascular retina demarcated posteriorly by retinal vessels that appeared to run parallel to the ora serrata. These vessels did not leak or demonstrate aneurysmal dilation.

Unilateral Coats’ Disease with Macular Exudate and Superotemporal Retinal Telangiectasias in the Left Eye.

Figure 1. Unilateral Coats’ Disease with Macular Exudate and Superotemporal Retinal Telangiectasias in the Left Eye.

Montage Fluorescein Angiogram Images. (Left) Fluorescein Angiogram of the Right Eye Demonstrating Peripheral Avascular Retina Bordered Posteriorly by Vessels Running Parallel to the Ora Serrata (arrows). (Right) Peripheral Avascular Retina Bordered Posteriorly by Leaking Telangiectasias in the Left Eye.

Figure 2. Montage Fluorescein Angiogram Images. (Left) Fluorescein Angiogram of the Right Eye Demonstrating Peripheral Avascular Retina Bordered Posteriorly by Vessels Running Parallel to the Ora Serrata (arrows). (Right) Peripheral Avascular Retina Bordered Posteriorly by Leaking Telangiectasias in the Left Eye.

Case 2

A 10-year-old boy was referred to Bascom Palmer Eye Institute for evaluation of retinal exudation in his left eye that was noted during a routine optometry visit. The patient denied an ocular history or family history of eye disease. On examination, the best-corrected visual acuity was 20/20 in both eyes. Anterior segment examination was normal. Dilated fundus examination demonstrated exudation in the superotemporal macula and peripheral retina of the right eye (Fig. 3). Retinal examination of the left eye was normal. The patient was diagnosed as having Coats’ disease in his right eye and subsequently underwent an examination under anesthesia with fluorescein angiography by Retcam. In the right eye, the fluorescein angiography highlighted focal areas of avascular retina surrounded by leaking, telangiectatic vessels in the superotemporal periphery (Fig. 4). Both eyes demonstrated areas of avascular retina in the far-periphery demarcated posteriorly by retinal vessels that appeared to run parallel to the ora serrata. These vessels did not leak or demonstrate aneurysmal dilation in the left eye.

Unilateral Coats’ Disease with Macular Exudate and Superotemporal Retinal Telangiectasias in the Right Eye.

Figure 3. Unilateral Coats’ Disease with Macular Exudate and Superotemporal Retinal Telangiectasias in the Right Eye.

(Top) Fluorescein Angiogram of the Right Eye Demonstrating Focal Areas of Avascular Retina Bordered by Leaking Telangiectasias. Peripheral Retina Is Avascular and Bordered Posteriorly with Loops of Vessels Running Parallel to the Ora Serrata. (Bottom) The Left Eye also Demonstrates Peripheral Avascular Retina Bordered Posteriorly by Vessels Running Parallel to the Ora Serrata (arrows).

Figure 4. (Top) Fluorescein Angiogram of the Right Eye Demonstrating Focal Areas of Avascular Retina Bordered by Leaking Telangiectasias. Peripheral Retina Is Avascular and Bordered Posteriorly with Loops of Vessels Running Parallel to the Ora Serrata. (Bottom) The Left Eye also Demonstrates Peripheral Avascular Retina Bordered Posteriorly by Vessels Running Parallel to the Ora Serrata (arrows).

Discussion

We present two patients with unilateral Coats’ disease diagnosed by clinical examination who demonstrate abnormal peripheral retinal vasculature in their contralateral eye on fluorescein angiography. In both patients, peripheral retinal vasculature in the contralateral eye is noted to turn parallel to the ora serrata, with avascular retina located anteriorly. This phenomenon may represent a previously unrecognized bilateral component of Coats’ disease. Furthermore, its phenotypic similarity to forms of familial exudative vitreoretinopathy may support arguments that these conditions exist on a continuous spectrum of congenital retinal vascular disorders.5,6 Further study is required to determine the frequency of these vascular changes in patients with clinically unilateral disease. The results of this research could change our understanding of Coats’ disease and lead to novel genetic explanations for its occurrence.

References

  1. Mandava N, Yannuzzi LA. Coats’ disease. In: Guyer DR, Yannuzzi LA, Chang S, Shields JA, Green WR, eds. Retina-Vitreous-Macula. Philadelphia: W. B. Saunders; 1999:390–397.
  2. Haller JA. Coats’ disease. In: Ryan SJ, ed. Retina, Vol. 2, 2nd ed. St. Louis: C. V. Mosby; 1994:1453–1460.
  3. Egerer I, Tasman W, Tomer TL. Coats’ disease. Arch Ophthalmol. 1974;92:109–112.
  4. Shields JA, Shields CL, Honavar S, 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]
  5. Sims KB. GeneReviews: NDP-related retinopathies. Bethesda, MD: National Center for Biotechnology Information; 2009. Available at: http://www.ncbi.nlm.nih.gov/books/NBK1331.
  6. Dickinson JL, Sale MM, Passmore A, et al. Mutations in the NDP gene: contribution to Norrie disease, familial exudative vitreoretinopathy and retinopathy of prematurity. Clin Exp Ophthalmol. 2006;34:682–688. doi:10.1111/j.1442-9071.2006.01314.x [CrossRef]
Authors

From Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida.

Supported by an unrestricted grant from Research to Prevent Blindness, Inc., New York, New York, and NIH Center Grant P30-EY014801.

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

Address correspondence to Thomas S. Shane, MD, 900 NW 17th Street, Miami, FL 33136. E-mail: tshane@med.miami.edu

10.3928/15428877-20110203-03

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