Journal of Pediatric Ophthalmology and Strabismus

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Short Subjects 

Cholesterolosis Bulbi of the Anterior Chamber in Coats Disease

Noopur Gupta, MS, DNB; Sarita Beri, MD

Abstract

Coats disease is characterized by the presence of cholesterol crystals and lipid-laden macrophages in the subretinal space. These cholesterol crystals may rarely be found in the anterior chamber in Coats disease, a phenomenon known as cholesterolosis bulbi. This report describes a patient with advanced Coats disease who presented with anterior chamber cholesterolosis.

Abstract

Coats disease is characterized by the presence of cholesterol crystals and lipid-laden macrophages in the subretinal space. These cholesterol crystals may rarely be found in the anterior chamber in Coats disease, a phenomenon known as cholesterolosis bulbi. This report describes a patient with advanced Coats disease who presented with anterior chamber cholesterolosis.

From the Department of Ophthalmology, Lady Hardinge Medical College and Associated Smt. Sucheta Kripalani Hospital & Kalawati Saran Children Hospital, New Delhi, India.

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

Address correspondence to Noopur Gupta, MS, DNB, R-6, First Floor, Green Park Extension, New Delhi 110001, India.

Received: February 27, 2008
Accepted: October 29, 2008
Posted Online: May 21, 2010

Introduction

Cholesterolosis bulbi, or the presence of cholesterol crystals in the anterior chamber of the eye, is an uncommon condition.1 It has been reported to occur after intraocular hemorrhage,2 intraocular inflammation, phacolysis,3 penetrating trauma,4,5 and long-standing retinal detachment.6

Anterior chamber cholesterolosis rarely occurs in Coats disease, and to the best of the authors’ knowledge, it has been reported only once.7 This report describes a rare association of anterior chamber cholesterolosis with an organized pseudohypopyon in an atrophic eye with Coats disease.

Case Report

A 13-year-old boy presented with a history of “shiny particles” in the left eye of 7 months’ duration. The patient’s hospital records indicated that, at 8 months of age, he had left esotropia, leukocoria, normal intraocular pressure, and inferior retinal telangiectasia with exudative retinal detachment, consistent with the diagnosis of Coats disease (stage 2A: telangiectasia with extrafoveal retinal exudation8). B-scan ultrasound and computed tomography scan at that time confirmed these findings, with no evidence of vitritis, vitreous bands, vascular stalk remnants, or solid tumor. The family history was unremarkable.

At the current visit, findings on systemic examination were normal. Findings of ocular examination of the right eye were within normal limits, with visual acuity of 6/6. There was no perception of light in the left eye, and the left eye had a narrow palpebral aperture with exotropia of 15°. Numerous shiny, rectangular, hyper-refringent, crystalline cholesterol flakes of varying sizes and geometric forms were seen floating in the anterior chamber. These sparkling particles moved with eye movements and formed a mobile pseudohypopyon in the dependent area (Fig. 1A). The cornea was clear and transparent, except for the lower one-third, corresponding to an inferior 3-mm area of fixed, vascularized “pseudohypopyon” extending from the 5-o’clock position to the 7-o’clock position (Fig. 1B). The pupil was irregular in shape (measuring 2.5 mm vertically and 1.5 mm horizontally), fixed, and nonreacting, with the presence of total posterior synechiae. The iris pattern was lost, with no evidence of neovascularization. The lens was cataractous, subluxated, and covered with the “shiny particles.” Fundus evaluation was not possible. Intraocular pressure recorded with a Goldmann applanation tonometer was 5 mm Hg. Ultrasound B-scan and computed tomography scan (Fig. 2) showed features of atrophic bulbi (axial length 16 mm, with a thickened retina–choroid complex), foci of dense calcification near the optic disc, and total retinal detachment.

(A) Clinical Photograph of a 13-Year-Old Boy Showing Anterior Chamber Cholesterolosis of the Left Eye. A Starry-Studded Appearance Formed by Swirling, Shiny Cholesterol Crystals in the Atrophic Eye Is Evident. (B) Slit-Lamp Photograph of the Left Eye Showing Anterior Chamber Cholesterolosis, Vascularized “pseudohypopyon,” and a Subluxated, Cataractous Lens.

Figure 1. (A) Clinical Photograph of a 13-Year-Old Boy Showing Anterior Chamber Cholesterolosis of the Left Eye. A Starry-Studded Appearance Formed by Swirling, Shiny Cholesterol Crystals in the Atrophic Eye Is Evident. (B) Slit-Lamp Photograph of the Left Eye Showing Anterior Chamber Cholesterolosis, Vascularized “pseudohypopyon,” and a Subluxated, Cataractous Lens.

Computed Tomography Scan of a 13-Year-Old Boy. (A) Coronal View Showing a Shrunken Left Eyeball with a Focus of Dense Calcification and a Thickened Retina–Choroid Complex, Consistent with the Diagnosis of Atrophic Bulbi. (B) Axial View Showing Calcification near the Optic Disc and Total Retinal Detachment in the Left Eye. The Axial Length of the Left Eye (16 mm) Was Shorter than the Right Eye (21 mm).

Figure 2. Computed Tomography Scan of a 13-Year-Old Boy. (A) Coronal View Showing a Shrunken Left Eyeball with a Focus of Dense Calcification and a Thickened Retina–Choroid Complex, Consistent with the Diagnosis of Atrophic Bulbi. (B) Axial View Showing Calcification near the Optic Disc and Total Retinal Detachment in the Left Eye. The Axial Length of the Left Eye (16 mm) Was Shorter than the Right Eye (21 mm).

Discussion

Coats disease is characterized by idiopathic retinal telangiectasia with intraretinal or subretinal exudation without any signs of vitreoretinal traction.8 The natural progression of Coats disease is the development of a blind, painful eye. Spontaneous regression may occur and, rarely, some eyes may progress to phthisis bulbi,9 as was the case in the current patient. According to a classification of Coats disease that was recently proposed by Shields et al.,10 the current patient has stage 5 Coats disease. It is one of the rarer stages of Coats disease (2% of cases in the series by Shields et al.8) characterized by a blind, nonpainful eye, with total retinal detachment, cataract, and phthisis bulbi.

Anterior chamber cholesterolosis is an unusual complication of Coats disease that occurs in approximately 3% of cases.8,11 In Coats disease, cholesterol crystals and lipid-laden macrophages are usually confined to the subretinal space12 and diffusion into the anterior chamber is facilitated by aphakia or subluxation of the crystalline lens. In the current patient, cholesterol crystals escaped the subretinal space and entered the vitreous cavity, possibly through peripheral retinal dialysis secondary to pressure from the subretinal fluid near the ora serrata and long-standing retinal detachment. The crystals reached the anterior chamber after subluxation of the cataractous, crystalline lens.

Neovascular glaucoma or hyphema usually develops in eyes with anterior chamber cholesterolosis, and these eyes become painful and blind.5 There have been no reported cases of prolonged presence of anterior chamber crystals without the development of complications. In the current patient, the eye was quiet; however, secondary glaucoma commonly occurs in eyes with anterior chamber cholesterolosis.

The most striking feature in the current case was a “fixed pseudohypopyon,” which probably developed because of prolonged contact of the cholesterol crystals with the endothelium. To the best of the authors’ knowledge, this case is the first report in the literature in which anterior chamber cholesterolosis and fixed pseudohypopyon developed in an atrophic, painless eye with Coats disease.

References

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  10. Shields JA, Shields CL, Honavar SG, Demirci H, Cater J. Classification and management of Coats’ disease: the 2000 Proctor Lecture. Am J Ophthalmol. 2001;131:572–583. doi:10.1016/S0002-9394(01)00896-0 [CrossRef]
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Authors

From the Department of Ophthalmology, Lady Hardinge Medical College and Associated Smt. Sucheta Kripalani Hospital & Kalawati Saran Children Hospital, New Delhi, India.

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

Address correspondence to Noopur Gupta, MS, DNB, R-6, First Floor, Green Park Extension, New Delhi 110001, India.

Received: February 27, 2008
Accepted: October 29, 2008
Posted Online: May 21, 2010

10.3928/01913913-20090616-04

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