Paravascular inner retinal defect associated with high myopia or epiretinal membrane

Paravascular retinal abnormalities are common in highly myopic eyes. However, affected areas may be underestimated, and the pathogenesis and effects on retinal function remain unclear.

To prospectively investigate the characteristics and pathogenesis of paravascular inner retinal defects (PIRDs).

Prospective and observational case series (between April 2013 and April 2014) at a referral retinal practice among 28 patients (41 eyes) with PIRDs. The entire affected retinal area was examined in 4 quadrants in sequential thin sections using optical coherence tomography. The effect of PIRDs on retinal function was examined using Goldmann perimetry.

Morphological changes on optical coherence tomography sections and visual field test by Goldmann perimetry.

On fundus photography, PIRDs appeared as spindle-shaped or caterpillar-shaped dark areas along the major retinal vessels disconnected from the optic disc. On optical coherence tomography cross-sections of retinal vessels, PIRDs often appeared as cystoid or fissure-like spaces; however, longitudinal optical coherence tomography sections along retinal vessels revealed that most PIRDs were actually wide defects in the inner retina or located beneath the major retinal vessels, often deviating into the vitreous cavity. Of 41 eyes with PIRDs, 37 (90%) were myopic; 21 eyes (51%) had high myopia. The mean refractive error of the eyes with PIRDs was -7.94 (95% CI, -9.48 to -6.40) diopters. The mean axial length of the eyes with PIRDs was 26.96 (95% CI, 25.42-28.49) mm. Twenty-one eyes (51%) showed epiretinal membrane in the macular area. In these eyes, PIRDs had formed along the temporal arcade vessels, which increasingly deviated toward the fovea by epiretinal membrane traction. Of 41 eyes with PIRDs, 35 showed visual field defects corresponding to the PIRD locations. The most common visual field defects were relative Bjerrum scotoma (in 75% [60 of 80]; 95% CI, 66%-85%) and nasal steps (in 59% [47 of 80]; 95% CI, 48%-70%) corresponding to the PIRD predilection locations.

Paravascular inner retinal defects primarily occur in eyes with high myopia or epiretinal membrane. Deviated retinal vessels due to axial elongation or epiretinal membrane traction may be involved in the pathogenesis. Paravascular inner retinal defects often cause retinal dysfunction corresponding to the location. A PIRD may partially overlap with retinal lesions previously reported as cleavage of the retinal nerve fiber layer, inner retinal cleavage, paravascular retinal cysts, or lamellar holes. However, the term PIRD more precisely describes the characteristic features of the lesion.