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).
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).
Fundus autofluorescence imaging shows hypoautofluorescence at the fovea and hypo and hyperautofluorescence around the fovea in both eyes (Figure 3)
SD-OCT imaging shows disruption of ellipsoid zone and external limiting membrane at the parafoveal area in both eyes (Figure 4)
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
Diagnosis
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.
Treatment
There is no treatment for benign concentric annular macular dystrophy.
