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Case Reports and Small Case Series
August 1998

Bull's-eye Maculopathy Associated With Chronic Macular Hole

Arch Ophthalmol. 1998;116(8):1116-1117. doi:

Bull's-eye maculopathy has been associated with various macular diseases, most notably chloroquine and hydroxychloroquine maculopathy, cone dystrophy, and Stargardt disease (Table 1).14 To our knowledge, bull's-eye maculopathy associated with chronic macular holes has not been previously reported.

Differential Diagnosis of Bull's-eye Maculopathy*
Differential Diagnosis of Bull's-eye Maculopathy*
Report of Cases
Case 1

A 71-year-old woman developed progressive visual loss in her left eye in 1990. Examination of the left eye disclosed a macular hole and a visual acuity of 20/200. Seven years later, best-corrected visual acuity was 20/200 OS (Figure 1, left). Fundus photography revealed a bull's-eye maculopathy, and fluorescein angiography showed a retinal pigment epithelial window defect in the macula surrounding a central area of normal fluorescence (Figure 1, right).

Figure 1.
Case 1. Left, Macular hole in the left eye associated with bull's-eye maculopathy. Right, Fluorescein angiogram demonstrates retinal pigment epithelial window defect surrounding a circular area of intact retinal pigment epithelium in the central macula.

Case 1. Left, Macular hole in the left eye associated with bull's-eye maculopathy. Right, Fluorescein angiogram demonstrates retinal pigment epithelial window defect surrounding a circular area of intact retinal pigment epithelium in the central macula.

Case 2

A 60-year-old man noted central distortion of vision in his left eye after he covered his right eye in 1978. Examination of the left eye disclosed a stage 2 macular hole (Figure 2, left) and a visual acuity of 20/50. Twenty years later, best-corrected visual acuity was 20/200 OS. Fundus photography revealed a characteristic bull's-eye maculopathy (Figure 2, right).

Figure 2.
Case 2. Left, Macular hole in the left eye (in 1978). Right, Macular hole (in 1998) associated with bull's-eye maculopathy.

Case 2. Left, Macular hole in the left eye (in 1978). Right, Macular hole (in 1998) associated with bull's-eye maculopathy.

Comment

The term bull's-eye maculopathy refers to the ophthalmoscopic appearance of a central area of retinal pigment epithelial depigmentation surrounded by relatively normal retinal pigment epithelium giving a "bull's-eye" appearance to the macula. This appearance is shared by a relatively large group of unrelated conditions.1 The 2 patients described herein demonstrate that chronic macular holes should be included in the differential diagnosis of bull's-eye maculopathy. Selective depigmentation of the retinal pigment epithelium may occur under the cuff of subretinal fluid that surrounds a chronic macular hole. The retinal pigment epithelial pigmentation underlying the macular hole is usually preserved and corresponds to the center of the bull's-eye pattern.

Several historical and clinical features aid in the specific diagnosis of a patient with bull's-eye maculopathy. Certainly, a history of chloroquine or hydroxychloroquine use would lead the clinician to suspect toxicity from systemic medications. Patients with cone dystrophy or Stargardt disease generally have symptoms of visual loss within the first 2 decades of life and may report a family history of ocular disease.2 In contrast, patients with idiopathic macular holes generally have normal vision until the sixth through eighth decades of life and have no family history of macular disease. The bull's-eye maculopathy associated with chronic macular holes generally has very sharp borders between the depigmented macular area and the surrounding normal retinal pigment epithelium, in contrast to the rather indistinct borders of the bull's-eye maculopathy associated with toxic maculopathies, cone dystrophy, or Stargardt disease. The configuration of the bull's-eye associated with chronic macular hole appears more rounded in comparison to the more oval configuration in most other bull's-eye maculopathies. On contact lens biomicroscopy, visualization of a cuff of subretinal fluid usually allows the clinician to confirm the diagnosis of bull's-eye maculopathy due to chronic macular holes. The retina is generally flat in the other conditions associated with bull's-eye maculopathy, and the elevated cuff of subretinal fluid around a macular hole is a key finding in distinguishing a macular hole from other entities in the differential diagnosis of bull's-eye maculopathy. Chronic macular hole should be included in the differential diagnosis of bull's-eye maculopathy.

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Article Information

Supported in part by a Heed Ophthalmic Foundation Fellowship, Cleveland, Ohio (Dr Scott), and a Ronald G. Michels Fellowship, Philadelphia, Pa (Dr Scott).

References
1.
Gass  JDM Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment. 4th ed.Vol 1. St Louis, Mo Mosby–Year Book Inc1997;I- 9
2.
Reichel  E Hereditary cone dysfunction syndromes. Albert  DMJakobiec  FAeds.Principles and Practice of Ophthalmology. Vol 2. Philadelphia, Pa WB Saunders Co1994;1238- 1248
3.
Ryan  SJedSchachat  APedMurphy  RBed Retina. 2nd ed. St Louis, Mo Mosby–Year Book Inc1994;xxxiv
4.
Berkow  JWFlower  RWOrth  DHKelley  JS Fluorescein and Indocyanine Green Angiography: Technique and Interpretation. 2nd ed. San Francisco, Calif Palace Press1997;108- 112American Academy of Ophthalmology monograph.
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