OCT in a Myelinated Retinal Nerve Fiber Syndrome with Reduced Vision.

PURPOSE: The prognosis of success with vision therapy in refractive "amblyopia" associated with the syndrome of myelinated nerve fibers (MRNF), optic disc hypoplasia, and myopia is reported to be poorer than that of anisomyopic amblyopia without these features. The reason for the poorer prognosis has not been well understood. The purpose of this study was to perform spectral domain (SD) ocular coherence tomography (OCT) to determine if there is a structural etiology that may explain the poorer prognosis.

CASE REPORTS: Case 1 was a 12-year-old male patient with anisometropic "amblyopia" in the right eye, MRNF denser superiorly, a hypoplastic disc, and a myopic fundus with a flat intact macula. The OCT demonstrated an attenuated photoreceptor integrity line (PIL) in the macula. Case 2 was a 10-year-old male patient with a constant left esotropia, MRNF denser superiorly, a hypoplastic disc, and a myopic fundus with a flat intact macula. The OCT demonstrated an absent PIL. Case 3 was a 58-year-old female patient with a history of diabetic retinopathy OU, long-standing reduced vision in the right eye, MRNF denser superiorly, optic nerve hypoplasia, and a myopic fundus with an intact macula. The OCT demonstrated an absent PIL in the macula.

CONCLUSIONS

This case series identifies three patients with the syndrome of MRNF, optic nerve hypoplasia, and anisomyopia in one eye with reduced vision and reports OCT findings using SD-OCT systems. All three patients demonstrated an absence or attenuation of the photoreceptor integrity line (PIL) in the macula in the affected eye. To our knowledge, there is no known association between this syndrome and abnormality of the PIL reported in the literature. Patients with this syndrome may have a guarded prognosis in the success of vision therapy.