Dr.Nivedita Murali, N21587
BACKGROUND: Gyrate atrophy (GA) is a rare autosomal recessive choroidal disease causing poor night vision and peripheral vision. Patients frequently present with high myopia and posterior subcapsular cataracts.
CASE: We report 2 Indian siblings, a girl of 11 and a boy of 9 years old, products of third degree consanguineous marriage, presenting with diminution of vision and poor night vision. Girl had a best corrected visual acuity of 6/18 in both eyes (BE) with -8.5DS/-1.5 x 15 in right eye (RE) and -8.5DS/-2.00 x 165 in left eye (LE) and the boy had 6/9 in BE with -6.00DS/-2.00 x 15 in RE and -5.5DS/-2.00 x 165 in LE. Fundoscopy showed bilateral confluent areas of chorioretinal atrophy suggesting GA. Spectral domain optical coherence tomography (OCT) revealed hyporeflective cystic spaces suggesting cystoid macular edema (CME). Electroretinography responses were undetectable.
CONCLUSION: Rule out CME by doing OCT in patients with GA complaining sudden diminution of central vision.
Leave a Comment