Best disease associated with macular hole.
OBJECTIVE
To report a case of Best vitelliform macular dystrophy complicated by macular hole.


METHODS
History and clinical examination, electroretinogram, and optical coherence tomography.


RESULTS
We report a case of a 20-year-old boy with progressive visual loss in his left eye. In fundus examination, there was a typical vitelliform lesion in the right eye and a macular hole in the left eye. The electroretinogram Arden ratio was <1.1 in both eyes. Optical coherence tomography revealed deposition of hyperreflective material and hyporeflective area between the junction of the inner segment and outer segment of the photoreceptors and the retinal pigment epithelium in the right eye, and large full-thickness macular hole with cystoid changes in the retinal layers in the left eye. There was no associated retinal detachment.


CONCLUSIONS
Although extremely rare, macular hole should be considered as a cause of the significant visual loss in patients with Best vitelliform macular dystrophy.
Ophthalmic Research Center, Labbafinejad Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Ramin
R
Nourinia
Danial
D
Roshandel
Behzad S
BS
Lima
Shima
S
Sayanjali
D004596:Electroretinography; D006801:Humans; D008297:Male; D012167:Retinal Perforations / Q000473:pathology*; D041623:Tomography, Optical Coherence; D057826:Vitelliform Macular Dystrophy / Q000473:pathology*; D055815:Young Adult