PARACENTRAL ACUTE MIDDLE MACULOPATHY in PRIMARY CONGENITAL GLAUCOMA

Purpose:To report a case of primary congenital glaucoma associated with paracentral acute middle maculopathy.Methods:A case report.Results:A 14-year-old girl with undiagnosed primary congenital glaucoma was referred for the evaluation of sudden vision loss a week after the initial symptoms. Visual acuity was counting fingers at 2 m in the right eye. Examination of her right eye revealed corneal stromal scar like Haab striae, splinter hemorrhage at the temporal border of the optic disk, perivenular hemorrhage, and fern-like white area at the macula. On optical coherence tomography of the right eye, hyperreflective plaques at the level of the inner nuclear layer/outer plexiform layer were present, corresponding to the opaque areas in fundoscopy. On optical coherence tomography angiography of the right eye, ischemic areas in deep capillary plexuses were present. These findings in optical coherence tomography angiography and optical coherence tomography were consistent with paracentral acute middle maculopathy.Conclusion:This is the first case of primary congenital glaucoma associated with paracentral acute middle maculopathy in the literature, and primary congenital glaucoma might be a predisposing factor.