Ophthalmic Surgery, Lasers and Imaging Retina

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Case Report 

Optical Coherence Tomography Findings of the Acute Exudative Polymorphous Vitelliform Maculopathy

Oswaldo Ferreira Moura Brasil, MD; Raul Nunes Galvarro Vianna, MD, PhD; Remo Turchetti Moraes, MD

Abstract

The optical coherence tomography (OCT) findings of a 26-year-old HIV-positive male with acute exudative polymorphous vitelliform maculopathy were described. His with best-corrected visual acuity at the presentation of the disease was 20/25 in the right eye and 20/30 in the left eye. Fundus examination showed bilateral yellowish subretinal lesions arranged in a honeycombed pattern throughout the posterior pole in both eyes. OCT scans on the topography of these lesions showed well-defined hyporeflective spaces that occasionally merged, limited superiorly by the retinal pigment epithelium and inferiorly by a hypereflective tissue that could correspond to Bruch’s membrane. Sub-sensory fluid was found in both maculas.

Abstract

The optical coherence tomography (OCT) findings of a 26-year-old HIV-positive male with acute exudative polymorphous vitelliform maculopathy were described. His with best-corrected visual acuity at the presentation of the disease was 20/25 in the right eye and 20/30 in the left eye. Fundus examination showed bilateral yellowish subretinal lesions arranged in a honeycombed pattern throughout the posterior pole in both eyes. OCT scans on the topography of these lesions showed well-defined hyporeflective spaces that occasionally merged, limited superiorly by the retinal pigment epithelium and inferiorly by a hypereflective tissue that could correspond to Bruch’s membrane. Sub-sensory fluid was found in both maculas.

Optical Coherence Tomography Findings of the Acute Exudative Polymorphous Vitelliform Maculopathy

From the Instituto Brasileiro de Oftalmologia (OFMB, RNGV, RTM), Rio de Janeiro; Vision Institute (OFMB), Federal University of São Paulo, São Paulo; and Antônio Pedro University Hospital (RNGV), Fluminense Federal University, Rio de Janeiro, Brazil.

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

Address correspondence to Oswaldo Ferreira Moura Brasil, MD, Praia de Botafogo 206, Rio de Janeiro, RJ, CEP:22250-040, Brazil.

Accepted: May 21, 2009
Posted Online: March 09, 2010

Introduction

The acute exudative polymorphous vitelliform maculopathy is a rare retinal disease that was initially described by Gass in two patients complaining of headaches and bilateral visual loss associated with multiple yellow–white subretinal lesions and exudative macular detachment.1 Only 11 cases have been reported to date.1–7

Optical coherence tomography (OCT) has been performed in some of these reported cases. Macular scans have shown both subretinal hyporeflective spaces that correspond to serous fluid and subretinal hypereflective deposits that displace anteriorly the neurosensory retina.3,5–7

We report a case of acute exudative polymorphous vitelliform maculopathy where we observed OCT findings besides the described macular ones.

Case Report

A 26-year-old male complaining of headaches and pain when moving the eyes for the last 3 months, referred blurred vision in both eyes. He was HIV positive with CD4 count of 350/μL, and was not taking any medications. He had no history of systemic manifestations related to HIV and his past ocular history was unremarkable. Best-corrected visual acuity was 20/25 in the right eye and 20/30 in the left eye. In prior ophthalmic evaluations, the patient had 20/20 uncorrected vision in both eyes. No anterior segment abnormalities were detected. Fundus examination disclosed in both eyes yellowish subretinal lesions arranged in a honeycombed pattern throughout the posterior pole, but sparing the macula. OCT (Figs. 1 and 2) showed submacular fluid in both eyes. In the left eye, intra-retinal cystoid spaces were also observed in the macula. The scans of the yellowish lesions, concentrated mostly under the vascular arcades, were also documented. Several separated hyporeflective areas under the retinal pigment epithelium are observed on the topography of the yellowish lesions. Some of these areas merged (Fig. 2), whereas others remained very well-defined (Fig. 1). Interestingly, in all acquired OCT scans, we can observe a hypereflective tissue under the hyporeflective spaces that could correspond to Bruch’s membrane.

Color Photograph and OCT Scans of the Right Eye. The Scan that Crosses the Center of the Fovea Discloses a Submacular Hyporeflective Area that Corresponds to Fluid Under the Retinal Pigment Epithelium. The Scan on the Topography of the Yellowish Subretinal Lesions Shows Well-Defined Hyporeflective Spaces Limited Superiorly by the Retinal Pigment Epithelium and Inferiorly by a Hypereflective Tissue that Could Correspond to Bruch’s Membrane.

Figure 1. Color Photograph and OCT Scans of the Right Eye. The Scan that Crosses the Center of the Fovea Discloses a Submacular Hyporeflective Area that Corresponds to Fluid Under the Retinal Pigment Epithelium. The Scan on the Topography of the Yellowish Subretinal Lesions Shows Well-Defined Hyporeflective Spaces Limited Superiorly by the Retinal Pigment Epithelium and Inferiorly by a Hypereflective Tissue that Could Correspond to Bruch’s Membrane.

Color Photograph and OCT Scans of the Left Eye. The Scan that Crosses the Center of the Fovea Shows a Submacular Hyporeflective Area that Corresponds to Fluid Under the Retinal Pigment Epithelium and also Hyporeflective Cystoid Spaces Within the Retinal Layers. The Scan on the Topography of the Yellowish Subretinal Lesions Discloses Both Well-Defined and Merged Hyporeflective Spaces Limited Superiorly by the Retinal Pigment Epithelium and Inferiorly by a Hypereflective Tissue that Could Correspond to Bruch’s Membrane.

Figure 2. Color Photograph and OCT Scans of the Left Eye. The Scan that Crosses the Center of the Fovea Shows a Submacular Hyporeflective Area that Corresponds to Fluid Under the Retinal Pigment Epithelium and also Hyporeflective Cystoid Spaces Within the Retinal Layers. The Scan on the Topography of the Yellowish Subretinal Lesions Discloses Both Well-Defined and Merged Hyporeflective Spaces Limited Superiorly by the Retinal Pigment Epithelium and Inferiorly by a Hypereflective Tissue that Could Correspond to Bruch’s Membrane.

Discussion

We described a typical case of acute exudative polymorphous vitelliform maculopathy in a HIV-positive young adult male. HIV-related disorders include several opportunistic infections, most commonly CMV retinitis, microvascular abnormalities, neoplasia, such as Kaposi sarcoma, intraocular lymphoma and squamous cell carcinoma of the conjunctiva, and neuroophthalmic disorders associated with orbital and intracranial disease.8 HIV is also related to chronic intraocular inflammation of uncertain pathogenesis.8 However, to our knowledge, there is no known relationship between HIV and this very rare retinal disease.

Previous publications have raised awareness to how OCT can contribute to evaluate the subretinal findings of the disorder, even though the assessment of choroidal anatomy is a limitation of this diagnostic tool.

In our case, we also described the anatomic arrangement of the subretinal lesions in the OCT scans. We noticed well-defined hyporeflective spaces that occasionally merged, limited superiorly by the retinal pigment epithelium and inferiorly by a hypereflective tissue that could correspond to Bruch’s membrane.

References

  1. Gass JD, Chuang EL, Granek H. Acute exudative polymorphous vitelliform maculopathy. Trans Am Ophthalmol Soc. 1988;86:354–366.
  2. Gass JD. Stereoscopic Atlas of Macular Disease. St. Louis: CV Mosby, 1997:168–169.
  3. Chan CK, Gass JD, Lin SG. Acute exudative polymorphous vitelliform maculopathy syndrome. Retina. 2003;23:453–462. doi:10.1097/00006982-200308000-00002 [CrossRef]
  4. Vianna RN, Muralha A, Muralha L. Indocyanine-green angiography in acute idiopathic exudative polymorphous vitelliform maculopathy. Retina. 2003;23:538–541. doi:10.1097/00006982-200308000-00016 [CrossRef]
  5. Cruz-Villegas V, Villate N, Knighton RW, Rubsamen P, Davis JL. Optical coherence tomography findings in acute exudative polymorphous vitelliform maculopathy. Am J Ophthalmol. 2003;136:760–763. doi:10.1016/S0002-9394(03)00426-4 [CrossRef]
  6. Kozma P, Locke KG, Wang YZ, Birch DG, Edwards AO. Persistent cone dysfunction in acute exudative polymorphous vitelliform maculopathy. Retina. 2007;27:109–113. doi:10.1097/01.iae.0000226537.95346.ca [CrossRef]
  7. Vaclavik V, Ooi KG, Bird AC, Robson AG, Holder GE, Webster AR. Autofluorescence findings in acute exudative polymorphous vitelliform maculopathy. Arch Ophthalmol. 2007;125:274–277. doi:10.1001/archopht.125.2.274 [CrossRef]
  8. Holland GN. AIDS and ophthalmology: the first quarter century. Am J Ophthalmol. 2008;145:397–408. doi:10.1016/j.ajo.2007.12.001 [CrossRef]
Authors

From the Instituto Brasileiro de Oftalmologia (OFMB, RNGV, RTM), Rio de Janeiro; Vision Institute (OFMB), Federal University of São Paulo, São Paulo; and Antônio Pedro University Hospital (RNGV), Fluminense Federal University, Rio de Janeiro, Brazil.

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

Address correspondence to Oswaldo Ferreira Moura Brasil, MD, Praia de Botafogo 206, Rio de Janeiro, RJ, CEP:22250-040, Brazil.

10.3928/15428877-20100215-52

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