Ophthalmic Surgery, Lasers and Imaging Retina

Case Report 

Intraoperative Spectral-Domain Optical Coherence Tomography in Coats’ Disease

Christopher R. Henry, MD; Audina M. Berrocal, MD; Ditte J. Hess, CRA; Timothy G. Murray, MD, MBA

Abstract

The authors describe the spectral-domain optical coherence tomography findings in three patients undergoing examinations under anesthesia for treatment of Coats’ disease. Patients were treated using a multifaceted approach of intravitreal bevacizumab and indirect diode laser vascular ablation. A handheld, portable spectral-domain optical coherence tomography system was used as a novel and critical intraoperative tool in differentiating Coats’ disease from diffuse retinoblastoma and other simulating conditions and to monitor reductions in subretinal and intraretinal exudation during the course of treatment.

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

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

Address correspondence to Timothy G. Murray, MD, MBA, Bascom Palmer Eye Institute, 900 N.W. 17th Street, Miami, FL 33136. E-mail: TMurray@med.miami.edu

Received: December 19, 2011
Accepted: June 07, 2012
Posted Online: July 26, 2012

Abstract

The authors describe the spectral-domain optical coherence tomography findings in three patients undergoing examinations under anesthesia for treatment of Coats’ disease. Patients were treated using a multifaceted approach of intravitreal bevacizumab and indirect diode laser vascular ablation. A handheld, portable spectral-domain optical coherence tomography system was used as a novel and critical intraoperative tool in differentiating Coats’ disease from diffuse retinoblastoma and other simulating conditions and to monitor reductions in subretinal and intraretinal exudation during the course of treatment.

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

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

Address correspondence to Timothy G. Murray, MD, MBA, Bascom Palmer Eye Institute, 900 N.W. 17th Street, Miami, FL 33136. E-mail: TMurray@med.miami.edu

Received: December 19, 2011
Accepted: June 07, 2012
Posted Online: July 26, 2012

Intraoperative Spectral-Domain Optical Coherence Tomography in Coats’ Disease

Introduction

First described in 1908, Coats’ disease is an idiopathic retinal vascular condition characterized by telangiectasias, exudation, and exudative retinal detachments without signs of retinal or vitreous traction.1,2 It occurs most commonly in young males and previous studies have demonstrated unilateral involvement in 80% to 95% of cases, although more recent studies have demonstrated bilateral findings on fluorescein angiography.2–4

We describe three patients in whom intraoperative spectral-domain optical coherence tomography (SD-OCT), using a portable device equipped with a handheld probe, was used as a novel and critical tool in differentiating Coats’ disease from diffuse retinoblastoma and other simulating conditions. Additionally, SD-OCT was used to demonstrate significant reductions of subretinal exudation during the course of treatment with bevacizumab and indirect diode laser vascular ablation. Intraoperative, portable SD-OCT systems are a new and important imaging modality that can be used in the diagnosis and treatment of patients with Coats’ disease.

Case Reports

Case 1

A 5-year-old boy was referred to the Bascom Palmer Eye Institute after failing a school vision screening examination. Examination by a previous retina specialist revealed a subretinal white lesion in the left eye that was concerning for diffuse retinoblastoma. The patient had no significant medical, ocular, or family history of eye disease.

On examination, the patient had a visual acuity of 20/20 in the right eye and 5/200 in the left eye. A left afferent pupillary defect was present. Anterior segment examination was unremarkable in both eyes. Dilated fundus examination of the left eye revealed subretinal exudation, vascular telangiectasias, secondary angioma, and complex retinal detachment consistent with Coats’ disease. The patient underwent a subsequent examination under anesthesia in which fundus photography and fluorescein angiography by Retcam (Clarity Medical Systems, Pleasanton, CA) and B-scan ultrasonography were performed (Figs. 1A and 1B). Additionally, intraoperative SD-OCT was performed using a portable system with a handheld probe (Bioptigen, Research Triangle Park, NC). SD-OCT revealed intraretinal and subretinal exudation with exudative retinal detachment (Fig. 1C).

(A) Retcam (Clarity Medical Systems, Pleasonton, CA) photograph of the left eye demonstrating exudative retinal detachment at baseline examination. (B) Retcam fluorescein angiography demonstrating leakage from telangiectatic vessels and areas of adjacent capillary dropout. (C) Intraoperative spectral-domain optical coherence tomography of the left eye demonstrating the extent of intraretinal and subretinal exudation, with exudative retinal detachment at baseline examination. (D) Intraoperative spectral-domain optical coherence tomography of the left eye demonstrating marked reduction in subretinal exudation and resolution of exudative retinal detachment following three monthly treatments with intravitreal bevacizumab and indirect diode laser vascular ablation.

Figure 1. (A) Retcam (Clarity Medical Systems, Pleasonton, CA) photograph of the left eye demonstrating exudative retinal detachment at baseline examination. (B) Retcam fluorescein angiography demonstrating leakage from telangiectatic vessels and areas of adjacent capillary dropout. (C) Intraoperative spectral-domain optical coherence tomography of the left eye demonstrating the extent of intraretinal and subretinal exudation, with exudative retinal detachment at baseline examination. (D) Intraoperative spectral-domain optical coherence tomography of the left eye demonstrating marked reduction in subretinal exudation and resolution of exudative retinal detachment following three monthly treatments with intravitreal bevacizumab and indirect diode laser vascular ablation.

A diagnosis of Coats’ disease was made. After three monthly treatments with intravitreal bevacizumab and diode laser treatment, a marked reduction in subretinal exudation and resolution of exudative retinal detachment was demonstrated on intraoperative SD-OCT (Fig. 1D). Central macular thickness improved to 497 microns and visual acuity improved to 20/200 in the left eye.

Case 2

A 3-year-old boy was referred to the Bascom Palmer Eye Institute by an ophthalmologist for evaluation of retinal exudation consistent with Coats’ disease and concern for possible underlying retinoblastoma. The patient had no significant medical, ocular, or family history of eye disease.

On examination, the patient had a visual acuity of 20/30 in the right eye and light perception in the left eye. A left afferent pupillary defect and 15 diopters exotropia were noted. Anterior segment examination was normal in both eyes. Dilated fundus examination of the left eye revealed subretinal exudation encompassing most of the posterior pole with admixed hemorrhage and extensive peripheral vascular telangiectasias. The patient underwent a subsequent examination under anesthesia in which fundus photography by Retcam and B-scan ultrasonography were performed. SD-OCT revealed the presence of subretinal and intraretinal exudation but no mass.

The patient was treated using a multifaceted approach of intravitreal bevacizumab and large spot diode laser treatment. Six weeks after treatment, a reduction in subretinal and intraretinal exudation was demonstrated on SD-OCT with central macular thickness decreasing from 476 to 313 microns.

Case 3

A 4-year-old boy was referred to the Bascom Palmer Eye Institute for the evaluation of retinal exudation concerning for Coats’ disease or familial exudative vitreoretinopathy. The patient had no significant medical, ocular, or family history of eye disease.

On examination, the patient had a visual acuity of 20/30 in the right eye and 20/100 in the left eye. A trace left afferent pupillary defect was seen. Anterior segment examination was normal in both eyes. A dilated fundus examination of the right eye revealed avascularity in the peripheral retina, but was otherwise unremarkable. A dilated fundus examination of the left eye revealed exudation in the macula, vascular telangiectasias, a focal shallow retinal detachment, and vascular engorgement in the peripheral retina. The patient underwent a subsequent examination under anesthesia in which fundus photography by Retcam and B-scan ultrasonography were performed (Fig. 2A). Additionally, intraoperative SD-OCT was performed using a portable system with a handheld probe. Initial SD-OCT revealed extensive intraretinal exudation, primarily at the level of the outer plexiform layer, in addition to subretinal exudation. Central macular thickness was 397 microns (Fig. 2B).

(A) Retcam (Clarity Medical Systems, Pleasonton, CA) photograph of the left eye demonstrating subretinal exudation at baseline examination. (B) Baseline intraoperative spectral-domain optical coherence tomography revealed subretinal exudation and extensive intraretinal exudates at the level of the outer plexiform layer that creates a shadowing effect. Central macular thickness was measured to be 397 microns. (C) Retcam photograph of the left eye demonstrating reduced exudation following 1 year of treatment. (D) Spectral-domain optical coherence tomography of the left eye demonstrating reduction in subretinal and intraretinal exudation following treatment with intravitreal bevacizumab and indirect diode laser vascular ablation. Central macular thickness improved to 348 microns, but a prominent area of consolidated exudate, encompassing nearly all retinal layers, did not resolve.

Figure 2. (A) Retcam (Clarity Medical Systems, Pleasonton, CA) photograph of the left eye demonstrating subretinal exudation at baseline examination. (B) Baseline intraoperative spectral-domain optical coherence tomography revealed subretinal exudation and extensive intraretinal exudates at the level of the outer plexiform layer that creates a shadowing effect. Central macular thickness was measured to be 397 microns. (C) Retcam photograph of the left eye demonstrating reduced exudation following 1 year of treatment. (D) Spectral-domain optical coherence tomography of the left eye demonstrating reduction in subretinal and intraretinal exudation following treatment with intravitreal bevacizumab and indirect diode laser vascular ablation. Central macular thickness improved to 348 microns, but a prominent area of consolidated exudate, encompassing nearly all retinal layers, did not resolve.

The patient was initially treated with diode laser photocoagulation. When the exudation did not improve, the patient was later treated with intravitreal bevacizumab coupled with large spot diode laser photocoagulation. Over the course of 1 year, the patient received six treatments with large spot diode laser concomitant with six treatments with intravitreal bevacizumab. Retcam photographs demonstrated a reduction in exudation (Fig. 2C). SD-OCT images demonstrated a reduction in subretinal and intraretinal exudation with an improvement in central macular thickness to 348 microns. A prominent area of consolidated exudate near the fovea, encompassing nearly all retinal layers, did not resolve despite aggressive treatment (Fig. 2D). Visual acuity at last follow-up visit was 20/80.

Discussion

Coats’-like vascular features have been described in patients with underlying retinoblastoma, and in some cases Coats’ disease may be difficult to differentiate clinically from diffuse retinoblastoma or other simulating conditions.5,6 In one series of 158 eyes with Coats’ disease, only 41% of patients were referred with the correct diagnosis, whereas 27% were referred with suspected retinoblastoma.2 Additionally, several published reports document children with Coats’ disease undergoing enucleation because clinical examinations, magnetic resonance imaging, and ultrasound could not exclude retinoblastoma.7,8 Previous studies have highlighted key features in differentiating these two conditions, including ultrasonographic features, the color of subretinal fluid, the presence of a mass, and the configuration of dilated retinal and tumor vessels.9

We present three patients in whom intraoperative SD-OCT was used as a novel and critical tool in confirming a diagnosis of Coats’ disease and monitoring response to treatment. The use of this system has now become integrated into the imaging armamentarium at the Bascom Palmer Eye Institute in children undergoing examinations under anesthesia for retinoblastoma and Coats’ disease and other simulating conditions such as vascular tumors or familial exudative vitreoretinopathy. Stationary OCT units have several limitations, including the lack of portability, design for adult patient populations, and the required cooperation of a patient. They can be particularly limiting for young children. Thus, the use of a portable system while children are undergoing examinations under anesthesia can serve as an outstanding alternative.

The use of a portable, intraoperative SD-OCT system has been recently described as a useful tool for macular surgery. Dayani et al. described four patients with macular hole, three patients with epiretinal membrane, and one patient with vitreomacular traction in whom preoperative and intraoperative SD-OCT images were obtained intraoperatively.10 Additionally, Wykoff et al. described the use of a portable SD-OCT system to demonstrate successful closure of a full-thickness macular hole.11 Our cases demonstrate additional applications for this unique technology.

The literature is surprisingly sparse regarding OCT findings in Coats’ disease. An electronic literature search (PubMed) revealed no previous articles dedicated to this subject. One previous study by Shields et al. described OCT findings in 44 patients with ocular tumors and simulating conditions.12 Four of the patients in this series had Coats’ disease, although specific OCT findings were not described. Other reports in the literature have demonstrated nonspecific resolution of intraretinal and subretinal fluid in Coats’ disease following the use of bevacizumab.13,14

In the three patients in our series, SD-OCT demonstrated intraretinal and subretinal exudation but no mass. In one patient in our series, intraretinal exudate was seen most prominently within the outer plexiform layer, whereas in the other two patients exudates were visualized within several retinal layers. In contrast, SD-OCT of retinoblastoma (Fig. 3) will typically demonstrate an absence of exudation, a hyperreflective band representing the inner aspect of the tumor, disruption of the surrounding retinal architecture, and a shadowing effect beneath the leading edge of the tumor.

Spectral-domain optical coherence tomography of a patient with retinoblastoma demonstrating an absence of exudation, a corrugated hyperreflective band representing the inner aspect of the tumor, disruption of the surrounding retinal architecture, and a shadowing effect beneath the inner edge of the tumor.

Figure 3. Spectral-domain optical coherence tomography of a patient with retinoblastoma demonstrating an absence of exudation, a corrugated hyperreflective band representing the inner aspect of the tumor, disruption of the surrounding retinal architecture, and a shadowing effect beneath the inner edge of the tumor.

Failure to differentiate Coats’ disease from retinoblastoma can result in worse visual outcomes, unnecessary enucleation, or potentially death. In all three of our cases, the referring physician was uncertain whether the disease represented Coats’ disease, diffuse retinoblastoma, or another simulating condition. SD-OCT imaging served as an adjunctive tool in distinguishing between these clinical entities. Additionally, in all of our cases, SD-OCT was used to demonstrate resolution of exudation following therapy both qualitatively and through quantitative measurement of central macular thickness. Our cases demonstrate a new and exciting adaptation of intraoperative, portable SD-OCT technology.

References

  1. Coats G. Forms of retinal diseases with massive exudation. Roy Lond Ophthalmol Hosp Rep. 1908;17:440–525.
  2. 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]
  3. Egerer I, Tasman W, Tomer TL. Coats’ disease. Arch Ophthalmol. 1974;92:109–112. doi:10.1001/archopht.1974.01010010115006 [CrossRef]
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  5. Shields CL, Uysal Y, Benevides R, Eagle RC, Malloy B, Shields JA. Retinoblastoma in an eye with features of Coats’ disease. J Pediatr Ophthalmol Strabismus. 2006;43:313–315.
  6. Jaffe MS, Shields JA, Canny CL, Eagle RC, Fry RL. Retinoblastoma simulating Coats’ disease: a clinicopathologic report. Ann Ophthalmol. 1977;9:863–868.
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  8. Chuah CT, Lim MC, Seah LL, Ling Y, Chee SP. Pseudoretinoblastoma in enucleated eyes of Asian patients. Singapore Medical Journal. 2006;47:617–620.
  9. Shields JA, Shields CL. Differentiation of Coats’ disease and retinoblastoma. J Pediatr Ophthalmol Strabismus. 2001;38:262–266.
  10. Dayani PN, Maldonado R, Farsiu S, Toth CA. Intraoperative use of handheld spectral domain optical coherence tomography imaging in macular surgery. Retina. 2009;29:1457–1468. doi:10.1097/IAE.0b013e3181b266bc [CrossRef]
  11. Wykoff CC, Berrocal AM, Schefler AC, Uhlhorn SR, Ruggeri M, Hess D. Intraoperative OCT of a full-thickness macular hole before and after internal limiting membrane peeling. Ophthalmic Surg Lasers Imaging. 2010;41:7–11. doi:10.3928/15428877-20091230-01 [CrossRef]
  12. Shields CL, Mashayekhi A, Luo CK, Materin MA, Shields JA. Optical coherence tomography in children: analysis of 44 eyes with ocular tumors and simulating conditions. J Pediatr Ophthalmol Strabismus. 2004;41:338–344.
  13. Sisk RA, Berrocal AM, Albini TA, Murray TG. Bevacizumab for the treatment of pediatric retinal and choroidal diseases. Ophthalmic Surg Lasers Imaging. 2010;41:582–592. doi:10.3928/15428877-20100830-03 [CrossRef]
  14. Lin CJ, Hwang JF, Chen YT, Chen SN. The effect of intravitreal bevacizumab in the treatment of Coats’ disease in children. Retina. 2010;617–622. doi:10.1097/IAE.0b013e3181c2e0b7 [CrossRef]

10.3928/15428877-20120719-01

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