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Ocular System

Bull’s-eye maculopathy (BEM)

Ophthalmoscopic appearance of a ring of paracentral atrophy of the retinal pigment epithelium (RPE) surrounding the fovea.

Contents

Ophthalmoscopic appearance of a ring of paracentral atrophy of the retinal pigment epithelium (RPE) surrounding the fovea.

History:

Kearns and Hollenhorst introduced the term bull’s-eye maculopathy (BEM) in 1966 to describe the characteristic clinical appearance of chloroquine retinopathy. In 1971, Deutman used the term to describe similar lesions in patients with inherited retinal dystrophies initially characterized by a central red spot surrounded by a ring of atrophic pigment epithelium or pigment epithelial mottling.


Aetiology

  • Long-term chloroquine/hydroxychroloquine usage (drug binds to melanin in the retinal pigment epithelium (RPE), causing cytotoxic effects, particularly affecting the macula)
  • Congenital cone deficiency
  • X-linked retinoschisis

Diagnosis

Fundus examination:

Colored fundus photos of right (A) and left (B) eye of the patient with 20 years of hydroxychloroquine (HCQ) treatment of mean dosage 2.95 mg/kg/day. White dots circled area outlined the Bull’s eye macular configuration in HCQ retinal toxicity. | Au S. (2020). Hydroxychloroquine retinal toxicity: The bull’s eye in the human eye. Visual journal of emergency medicine, 21, 100818. https://doi.org/10.1016/j.visj.2020.100818

Fundus autofluorescence (FAF):

Fundus autofluorescence (FAF) photos of right (A) and left (B) eye of the patient few months after Fig. 1 was taken. Progression of Bull’s eye maculopathy was evidenced by the concentric rings over the fovea. Normal retinal pigment epithelium (RPE) is autofluorescence, appearing as white color on FAF, as in the peripheral part of (A) & (B). Hypo-autofluorescence appears as black color on FAF, and the areas signified the atrophy of the melanin containing RPE (other than the optic disk and the retinal vasculature). | Au S. (2020). Hydroxychloroquine retinal toxicity: The bull’s eye in the human eye. Visual journal of emergency medicine, 21, 100818. https://doi.org/10.1016/j.visj.2020.100818

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