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Postnatal optic disc cupping in a non-glaucomatous infant with periventricular leukomalacia: nosologic implications
  1. Susannah Q Longmuir1,
  2. Michael C Brodsky2,3
  1. 1From the Department of Ophthalmology and Visual Sciences, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA
  2. 2Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA
  3. 3Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Susannah Longmuir, From the Department of Ophthalmology and Visual Sciences, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, Iowa City, IA 55242-1091; susannah-longmuir{at}

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Case report

This male infant was born at 24 5/7 weeks gestation with birth weight of 693 g and history of intrauterine alcohol and marijuana exposure. At 31 4/7 weeks postmenstrual adjusted age, he was found to have stage 1 zone 2 retinopathy of prematurity (ROP) without plus disease. He was followed closely for ROP which peaked at stage 2 zone 2 ROP from weeks 32 to 36 and regressed at postmenstrual adjusted age of 38 weeks. At 10 weeks of age, retinal examination showed a vertically oblique configuration to the optic discs, and a cup to disc ratio of 0.4 in both eyes, with no hypoplasia or pallor of the optic discs (figure 1A,B).

Figure 1

Postnatal optic disc cupping in periventricular leukomalacia. Top: Retinal photographs at 2½ months postgestational age show the right eye (A) and the left eye (B). The cup to disc ratio was 0.4 in both eyes. Middle: Retinal photographs taken at 15 months of age show apparent rounding out and enlargement of the optic disc areas with round cups and a cup to disc ratio of 0.6 in the right eye (C) and 0.7 in the left eye (D). Bottom: MRI. Left: Coronal T2-weighted FLAIR MRI of frontal horns shows focal T2 hyperintensities in periventricular white matter (arrows) (E). Right: Axial T2-weighted FLAIR MRI shows focal T2 hyperintensities in periventricular white matter (arrows), ventricular dilatation and irregular borders of the lateral ventricles (F).

At 15 months of age, he followed optokinetic stimuli and had no strabismus or nystagmus. Intraocular pressures and pachymetry results were normal at 12 months of age and intraocular pressures were again determined to be normal at 17 months of age. Cycloplegic refraction was +1.50 sphere of hyperopia in both eyes. Retinal examination showed apparent enlargement of the optic discs, a rounder optic disc configuration, and an increased cup to disc ratio of 0.6 in the right eye and 0.7 in the left eye (figure 1C,D). No buphthalmos or Haab's striae were present, and both corneas were clear. MRI of brain disclosed bilateral periventricular leukomalacia (PVL) (figure 1E,F).


PVL is caused by gestational injury to periventicular white matter that occurs between 24 and 34 weeks gestation.1 2 PVL preferentially involves the optic radiations adjacent to the trigone of the lateral ventricles posteriorly, and the descending anterior corticospinal fibres adjacent to the intraventricular foramen anteriorly.1 2 In 1993, Brodsky and Glasier documented optic nerve hypoplasia in 10 children with PVL.3 In 1997, Jacobson et al4 reported that PVL produces a unique form of bilateral optic nerve hypoplasia associated with large optic cups and a thin neuroretinal rim. The larger cup size was attributed to bilateral injury to the optic radiations that resulted in retrograde transsynaptic degeneration of retinogeniculate axons after the scleral canals had developed normal diameters. Park et al recently identified a mild increase in optic disc cupping which is greater in preterm and low birth weight children.5 An increase in optic disc diameter of over 50% has also been shown to occur in preterm infants.6

The time period between examinations in our patient precludes precise identification of the time period of postnatal cupping. Further prospective studies using serial fundus photography in the first few months of life should cast light on the natural history of pseudoglaucomatous cupping and other configurational changes of the optic discs in premature infants with PVL. However, this case demonstrates that the pseudoglaucomatous cupping of PVL can present as an acquired, postnatal enlargement of the optic cups. This evolution indicates that this pseudoglaucomatous cupping may be more properly classified as a form of optic atrophy.

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  • Funding Supported in part by a grant to the Department of Ophthalmology, Mayo Clinic from Research to Prevent Blindness, New York, New York and Mayo Foundation.

  • Competing interests None.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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