31. Benign Concentric Annular Macular Dystrophy

Medical History

A 30-year-old male patient with no visual complaint was examined in our clinic. 

Diabetes mellitus (-)
Systemic hypertension (ı)
Family history (-)
Smoking (-)
Trauma (-)

Examination Findings

Best corrected visual acuity was 10/10 in the right eye and 10/10 in the left eye. Intraocular pressure was 16 mmHg in the right eye and 16 mmHg in the left eye. Anterior segment examination revealed no abnormality in both eyes. Fundus examination revealed hyperpigmentation at the fovea and hypopigmented ring around the hyperpigmentation in both eyes (Figure 1).

Figure 1A

Figure 1B

Multicolor imaging shows hyporeflective ring at the fovea and hypo- and hyper reflectance around the ring at the right eye, hypo- and hyper reflectance at the fovea in the left eye (Figure 3).

Figure 2A

Figure 2B

Fundus autofluorescence imaging shows hypoautofluorescence at the fovea and hypo and hyperautofluorescence around the fovea in both eyes (Figure 3) 

Figure 3A

Figure 3B

SD-OCT imaging shows disruption of ellipsoid zone and external limiting membrane at the parafoveal area in both eyes (Figure 4)

Figure 4A

Figure 4B

Electrophysiological tests were performed. In ERG test, scotopic and photopic responses were normal in both eyes.  In EOG test, Arden ratio was 2.6 in both eyes

Differential Diagnosis

Cone dystrophy, Stargardt Disease, Central areolar choroidal dystrophy


Benign concentric annular macular dystrophy 

Benign concentric annular macular dystrophy is a rare hereditary pathology with autosomal dominant inheritance.  It was described by Deutman in 1974, firstly. The mutated gene is located on the 6th chromosome.  The disease has a good prognosis. In some cases, central and peripheral vision loss may occur.


There is no treatment for benign concentric annular macular dystrophy.


Luísa Salles de Moura Mendonça et al. Benign concentric annular macular
dystrophy. Rev Bras Oftalmol. 2015; 74 (3): 183-5.



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