Periventricular Leukomalacia and Pseudoglaucomatous Cupping

Journal Highlights

American Journal of Ophthalmology, March 2020

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Groth et al. set out to describe two young patients with a history of pre­maturity who presented with enlarged cup-to-disc ratio, normal intraocular pressure, retinal nerve fiber layer thin­ning superiorly, and visual field (VF) defects inferiorly and homonymously. The authors explained that, as advanc­es in perinatal care have improved survival for premature babies, many will later present to clinicians who may be unaware of the relationship of cupping, field defects, and prematurity—and who may diagnose manifest or suspected normal tension glaucoma (NTG). However, the correct diagnosis may be periventricular leukomalacia (PVL), a structural loss of white matter pathways that carry visual information from the lateral geniculate bodies to the visual cortex.

For this case series, the authors illustrated the pseudoglaucomatous cupping related to PVL with optic disc photographs. Coronal and sagittal magnetic resonance images of the PVL patients’ brains, along with those from normal controls, were used to illustrate the loss of white matter. These periven­tricular white matter pathways are part of the optic radiations that are adjacent to the lateral ventricle and carry visual impulses to the visual cortex; a second­ary trans-synaptic degeneration of the retinogeniculate axons produces cup­ping similar to that seen in glaucoma.

The authors emphasize that PVL should be added to the differential di­agnosis of NTG when there is a history of prematurity. Similarly, when NTG is suspected, the clinician should be careful to obtain a birth history. Careful examination of the optic nerve will assist in differentiating the two condi­tions. Specifically, horizontal cupping with minimal or no nasal displacement of vessels and superior optic nerve thinning with inferior VF defects should raise suspicion of PVL.

The original article can be found here.