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Characterization of kjellin syndrome using spectral-domain optical coherence tomography and fundus autofluorescence
Ryan M Tarantola1, Victoria Nguyen, Anita Agarwal
1From the *Vanderbilt Eye Institute, Vanderbilt University Medical Center, Nashville, Tennessee; and †University of Oklahoma College of Medicine, Oklahoma City, Oklahoma.
Purpose:
The purpose of this study was to describe the characteristic funduscopic appearance of Kjellin syndrome, to report unique spectral-domain optical coherence tomography (Carl Zeiss Meditec, Dublin, CA) and fundus autofluorescence findings, and to provide long-term follow-up observations about this patient.
Methods:
A 43-year-old man presented for an ophthalmologic examination with a known diagnosis of hereditary spastic paraplegia and no visual complaints.
Results:
Retinal flecks were present throughout the macula in both eyes of the patient. Fluorescein angiography showed flecks with central hypofluorescence and peripheral hyperfluorescence. Fundus autofluorescence showed hyperautofluorescence in the center of the visible flecks and a surrounding halo of hypoautofluorescence. An abnormal latticelike network of hyperautofluorescence was present in the nasal retina of both eyes. Spectral domain-optical coherence tomography showed hyperreflective material, accounting for the flecks located at the level of the retinal pigment epithelium. At the 6-year follow-up, this patient had not developed any subjective visual changes, and the appearance of his fundus remained relatively unchanged.
Conclusion:
The presence of hyperautofluorescent material outside the posterior pole in patients with Kjellin syndrome has not been previously reported and suggests that retinal pigment epithelium involvement in this condition is not limited to the macula. Spectral domain-optical coherence tomography of this patient showed that the accumulation of hyperreflective material accounting for the flecks was located at the level of the retinal pigment epithelium. The 6-year follow-up findings suggest that this condition is either stationary or slowly progressive.
