Congenital Herniation of the Gyrus Rectus Resulting in Compressive Optic Neuropathy

Abstract

We report a 34-year-old male with a previously uninvestigated lifelong blindness of the right eye from compressive optic neuropathy secondary to congenital herniation of the gyrus rectus (HGR). His past medical history was otherwise unremarkable, with no history of prior head or ocular trauma. On examination, he had no light perception in the right eye, right relative afferent pupillary defect (RAPD), and primary optic atrophy. His left eye had normal visual acuity, color vision, and a healthy optic disc. There was a sensory exotropia in the right eye; however, extraocular movements were intact and the remainder of his neurological exam was normal. MRI revealed compression of the prechiasmatic right optic nerve from HGR and atrophy of the right optic nerve and optic chiasm (Figures 1 and 2), without any parenchymal mass lesions. There were no signal abnormalities in the optic nerves or the chiasm.

Compression From Above: A Case Series of Compressive Optic Neuropathy Secondary to Idiopathic Gyrus Rectus Herniation

“…In contrast, our patients reported subacute vision loss with optic nerve head pallor, typical of compressive optic neuropathy. More recently, Gilani et al (2) reported a man with previously unexplained unilateral blindness since birth. Neuroimaging revealed inferior herniation of the ipsilateral gyrus rectus compressing the optic nerve, which was inferred to be the cause of visual loss.…”