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David Sarraf, MD; Olga Ceron, MD; Karim Rasheed, MD, MSc, FRCOphth; Kimberly A. Drenser, MD, PhD; Richard Casey, MD

Arch Ophthalmol. 2003;121:338-342.

Objective  To describe the findings of a new crystalline maculopathy exclusively affecting elderly members of the Igbo tribe of southeast Nigeria.

Design  Retrospective, observational noncomparative case series.

Methods  Six patients referred over a 2-year period to the medical retina consultation service of the King/Drew Medical Center (Los Angeles, Calif) were identified as having a characteristic crystalline maculopathy. Each underwent detailed historical questioning and comprehensive ocular evaluation, including formal retinal examination. Color vision testing, fluorescein angiography, Humphrey visual field analysis, and electrophysiologic assessment were also performed.

Results  Each of the 6 patients was an elderly member of the Igbo tribe of southeast Nigeria and demonstrated a unique crystalline maculopathy. A central, superficial cluster of green or yellow, refractile, foveal crystals that were bilateral and asymmetric in distribution was noted in each case. The crystals were benign and unassociated with visual deficit. Retinal sequelae were notably absent and fluorescein angiography results were unremarkable. Additional ancillary testing was generally normal, although 1 patient demonstrated unexplained mild to moderate depression of the scotopic and photopic responses on electrophysiologic analysis.

Conclusions  Elderly members of the Igbo tribe of southeast Nigeria may harbor characteristic bilateral but asymmetric foveal crystals, comprising a novel syndrome of crystalline maculopathy unassociated with obvious visual deficits or retinal sequelae. The etiology of this crystalline maculopathy remains unclear, although genetic, degenerative, and toxic causes are postulated.

From the Jules Stein Eye Institute, Department of Ophthalmology, University of California–Los Angeles School of Medicine (Drs Sarraf, Drenser, and Casey); Department of Ophthalmology, Charles R. Drew School of Medicine and Science, Martin Luther King Medical Center (Drs Sarraf, Ceron, Rasheed, and Casey); and the Greater Los Angeles Veterans Affairs Healthcare Center (Dr Sarraf), Los Angeles, Calif.

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