Optic Disc Drusen with Structural and Functional Changes

A young child presented with crossing of the eyes without headaches or associated neurologic symptoms. Visual acuity was OD 20/30 and OS 20/40. Motility examination demonstrated moderate angle esotropia with full versions. Intraocular pressure (IOP) was normal. Dilated fundus exam revealed bilateral elevated optic discs, more prominent on the right, without obscuration of blood vessels. Cycloplegic refraction showed moderate hyperopia bilaterally. OCT retinal nerve fiber layer (RNFL) demonstrated diffuse thickening. Ultrasound showed bilateral optic disc drusen (ODD). She was diagnosed with pseudopapilledema and accommodative esotropia and treated with glasses. The optic nerve appearance remained stable and OCT RNFL and GCC imaging studies were serially repeated. Eight years later her visual acuity was stable at 20/25 in the right eye and 20/20 in the left eye; however, OCT RNFL and GCC demonstrated structural deterioration. From 2013 to 2021, the average RNFL thickness decreased by -23.32 µm +/- 7.92 per year in the right eye and - 5.69 µm +/- 3.12 um per year in the left eye. From 2014 to 2021, the average GCC decreased from 76 µm to 62 µm in the right eye. IOP remained normal. The patient was started on brimonidine tartrate ophthalmic solution for its neuroprotective effect. The latest visual field performed showed a supero-temporal scotoma in the right eye. The structural changes preceded the functional findings. There were no environmental or nutritional habits that could explain a neurotoxicity. We concluded that the anatomical and functional degradation of the optic nerve is related to the ODD.

Presentation Date: 09/09/2021

Issue Date: 09/24/2021