Background: Stargardt disease is an early-onset, autosomal recessive, inherited retinal degenerative disease. Here, we report a case of late-onset Stargardt disease with bilateral macular holes.
Case: A 57-year-old woman was first examined at Fukushima Medical University Hospital in 2004. Visual acuity was 1.0 in both eyes; fundus examination indicated retinal pigment epithelium (RPE) degeneration in the fovea of both eyes and yellow-white deposits in the macula and surrounding areas of both eyes. Fluorescein fundus angiography indicated enhanced fluorescence and decreased luminance in choroidal background fluorescence. These findings were consistent with those of macular atrophy. Based on the aforementioned findings, the patient was diagnosed with Stargardt disease. Spectral domain optical coherence tomography performed in 2007 indicated a loss of the ellipsoid zone in the fovea of both eyes and posterior vitreous detachment (PVD) in the parafoveal region. During the seven years since the initial examination, macular holes were formed following the damage to the outer layers of the retina at the fovea of both eyes. During the follow-up period, cyst formation was not observed in the region surrounding the macular holes.
Conclusion: Macular holes may result from traction following parafoveal PVD on the foveal tissue that is weakened by RPE degeneration.
Nippon Ganka Gakkai Zasshi (J Jpn Ophthalmol Soc) 123: 608-613, 2019.