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A Variant of Optic Nerve Hypoplasia in Children With Periventricular Leukomalacia

Lena Jacobson, MD; Ann Hellström, MD; Olof Flodmark, MD, PhD

Arch Ophthalmol. 1997;115(10):1263-1269.

Abstract
Objective
To evaluate optic disc morphologic features in children with periventricular leukomalacia (PVL).

Patients and Methods
Seventeen children with PVL (patient group) were compared with 17 sex- and age-matched, full-term healthy control children (control group). Clinical ophthalmological examination and digital image analysis of fundus photographs were performed in all children. In children with PVL, cerebral imaging was performed.

Results
Children with PVL had larger optic disc cupping (P<.02) than did control children. A large proportion of the children with PVL had strabismus, nystagmus, and restricted visual fields.

Conclusion
Our study indicates that optic nerve hypoplasia in children with PVL is often associated with a normal-sized optic disc with a large cup. This unusual form of optic nerve hypoplasia most likely results from transsynaptic degeneration of optic axons caused by the primary bilateral lesion in the optic radiation.

Author Affiliations
From the Department of Ophthalmology, Karolinska Institute, Huddinge University Hospital, Huddinge (Dr Jacobson); Department of Clinical Neurosciences, Section of Ophthalmology, Department of Pediatrics, International Pediatric Growth Research Centre, Gothenburg University, Gothenburg (Dr Hellström); and Department of Neuroradiology, Karolinska Institute, Karolinska University Hospital, Stockholm (Dr Flodmark), Sweden.

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