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Coats Disease Masquerading as Panuveitis

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Title: Coats Disease Masquerading as Panuveitis

Authors: Christopher Le, MSIV University of Colorado School of Medicine; Eric Hansen, MD.

Date: 7/8/2023

Keywords/Main Subjects: coats disease, uveitis masquerade syndromes

Diagnosis: Coats Disease

Description of Case:

Introduction:

Coats disease is a rare, congenital, nonhereditary, idiopathic disorder of the retinal vasculature leading to massive subretinal and intraretinal exudation. The disease typically affects young males in the first or second decades of life and is unilateral.¹ The pathophysiology of Coats disease remains unclear – however, it has been proposed that defects in endothelial cells of the retinal vasculature along with abnormal pericytes contribute to the breakdown of the blood-retinal barrier leading to the development of telangiectatic vessels, retinal ischemia, and exudation of lipid-rich fluid.² Patients with Coats disease have varying symptoms on initial presentation including: decreased visual acuity, eye pain, strabismus, and leukocoria.³ Rarely, Coats disease may present with symptoms and exam findings suggestive of intraocular inflammatory conditions such as uveitis or endophthalmitis. Diseases that present with an intraocular inflammatory picture but are not caused by immune-mediated or infectious processes are referred to as uveitis masquerade syndromes (UMS).⁴

Case Presentation:

We present a case of Coats disease in a 14-year-old male masquerading as a panuveitis. The patient presented to the emergency department at an outside institution with a three-day history of blurred vision, pain, and redness in his left eye, as well as fever, body aches, and a facial rash. On initial examination, visual acuity in his left eye was markedly decreased at light perception only. There was conjunctival injection and diffuse cell in the anterior chamber and vitreous. On dilated funduscopic examination, a multifocal exudative retinal detachment without macular involvement was appreciated with diffuse subretinal and intraretinal yellow-white exudation and peripheral retinal hemorrhages. Interestingly, the patient reported having a normal dilated eye exam just one year prior.

Shortly after presentation to the outside facility, the patient was transferred to the Moran Eye Center at the University of Utah. Due to concern for possible endophthalmitis or infectious panuveitis, the patient underwent a vitreous and anterior chamber tap as well as injection of broad-spectrum antimicrobials. The patient was evaluated by uveitis specialist who recommended a broad uveitis workup and vitreous biopsy given a dry vitreous tap. The patient’s respiratory viral panel resulted positive for rhinovirus. Broad uveitis workup – including evaluation for HSV, VZV, CMV, toxoplasma, vitreous PCR, quantiferon, RPR, syphilis antibody, bartonella, lysozyme, ACE, and ANCA – returned unremarkable. The patient underwent an exam under anesthesia and a 27-gauge pars plana vitrectomy with vitreous biopsy and intravitreal injections of antimicrobial agents including vancomycin, ceftazidime, clindamycin, and foscarnet. Interestingly, the culture from patient’s vitreous biopsy grew pan-sensitive staphylococcus Capitis. However, the patient’s Karius panel was negative for bacteria and positive for candida. Both positive results were determined to be likely contaminants in consultation with the infectious disease department.

The patient followed-up in clinic and underwent fundus photography and fluorescein angiography following vitrectomy. Fundus examination demonstrated telangiectatic vessels and bulbs with associated diffuse subretinal exudative material and exudative retinal detachment. Fluorescein angiography highlighted the telangiectatic vessels and demonstrated terminal light bulb aneurysms, capillary nonperfusion, and perivascular leakage. The patient was diagnosed with Coats disease, stage 3A given this constellation of findings. The patient was scheduled to undergo laser photocoagulation therapy with adjuvant intravitreal therapy.
At the time of treatment, he was found to have diffuse vitreous hemorrhage obscuring a clear view of the retina with worsening exudative retinal detachment. Subsequently, he was taken to the operating room for a pars plana vitrectomy with scleral buckling, external drainage of exudative material, application of endolaser, silicone oil endotamponade, and intravitreal Avastin injection. At the time of this vitrectomy, the patient’s exudative retinal detachment had worsened to total detachment –classifying the patient’s disease as stage 3B. The patient subsequently underwent multiple exams under anesthesia with laser photocoagulation, sub-tenons Kenalog and intravitreal Avastin injections resulting in resolution of the exudative retinal detachment, significantly improved exudation, and residual fibrosis and vitreoretinal traction sparing the macula. The patient’s visual acuity has improved to counting fingers, and he remains without any signs of neovascular glaucoma.

Discussion:

This case highlights a rare and atypical presentation of Coats disease masquerading as a panuveitis. The critical step in obtaining this patient’s diagnosis, and in diagnosis of Coats disease in general, was obtaining fluorescein angiography with clear view which demonstrated pathognomonic features of the disease: telangiectatic retinal vasculature with terminal light bulb aneurysms, capillary nonperfusion, and perivascular leakage. Coats disease should be included in the differential diagnosis for young males in the first or second decades of life presenting with panuveitis and exudative retinal detachments.

Images or video:

Image 1

Image 1: Fundus photograph taken after initial vitrectomy demonstrating telangiectatic retinal vasculature, yellow subretinal exudative material, and multifocal exudative retinal detachment.

Image 2

Image 2: Widefield fluorescein angiography taken after initial vitrectomy demonstrating telangiectatic vessels with terminal light bulb aneurysms, capillary nonperfusion, and perivascular leakage.

Image 3

Image 3: Fundus photograph taken during most recent exam under anesthesia in June 2023, following several laser photocoagulation and intravitreal Avastin treatments, demonstrating improved exudative retinal detachment, persistent but improved exudation, and residual fibrosis and traction involving the macula.

Summary of the Case:

A 14-year-old male presented with a marked decrease in visual acuity to light perception only, eye pain, and eye redness of the left eye in the setting of fever, chills, and a facial rash. Initial examination was remarkable for a multifocal exudative retinal detachment and significant anterior chamber and vitreous cell suggestive of a panuveitis or intraocular inflammatory condition. Vitreous culture from intraoperative biopsy grew staphylococcus Capitis, but was ultimately determined to be a contaminant. All other elements of the patient’s broad uveitis workup were unremarkable. Fluorescein angiography was performed following the patient’s initial vitrectomy and revealed telangiectatic vessels, capillary nonperfusion, perivascular leakage – findings pathognomonic for Coats disease. The patient underwent a series of interventions including vitrectomy/buckle with external drainage of exudates and several treatments with laser photocoagulation and intravitreal Avastin injections. His most recent examination and fundus photos demonstrated improvement in the exudative retinal detachment and persistent, but improved, exudation. His visual acuity has improved to counting fingers and he remains without any signs of development of neovascular glaucoma.

Format: Case Report

References

1. 1. Yousef YA, ElRimawi AH, Nazzal RM, et al. Coats’ disease: characteristics, management, outcome, and scleral external drainage with anterior chamber maintainer for stage 3b disease. Medicine (United States). 2020;99(16):E19623. doi:10.1097/MD.0000000000019623
   2. Sen M, Shields CL, Honavar SG, Shields JA. Coats disease: An overview of classification, management and outcomes. BMC Ophthalmol. 2017;17(1):1. doi:10.4103/ijo.IJO
   3. Yang X, Wang C, Su G. Recent advances in the diagnosis and treatment of Coats’ disease. Int Ophthalmol. 2019;39(4):957-970. doi:10.1007/s10792-019-01095-8
   4. Hsu YR, Wang LU, Chen FT, et al. Clinical Manifestations and Implications of Nonneoplastic Uveitis Masquerade Syndrome. Am J Ophthalmol. 2022;238(August 2020):75-85. doi:10.1016/j.ajo.2021.12.018

Faculty Approval by: Eric Hansen, MD; Griffin Jardine, MD.

Copyright: Christopher Le, © 2023. For further information regarding the rights to this collection, please visit: http://morancore.utah.edu/terms-of-use/

Identifier: Moran_CORE_126934