Abstract
Purpose: The authors document the association of peripapillary staphyloma and an atypical variant of hemimegalencephaly with the linear nevus sebaceous syndrome. Background: Linear nevus sebaceous syndrome is an uncommon neurocutaneous disorder that has a propensity to involve the eyes. Methods: Clinical, histopathologic, and neuroimaging findings are examined in a child with linear nevus sebaceous syndrome. Results: In addition to bilateral peripapillary staphylomas, ophthalmologic abnormalities included a corneal dermoid, a complex conjunctival choristoma, macular hypoplasia, and optic nerve hypoplasia with contralateral optic atrophy. Magnetic resonance imaging disclosed a rare form of hemimegalencephaly characterized by hypoplasia of an optic radiation within the enlarged, dysgenetic cerebral hemisphere. Conclusion: The spectrum of oculocerebral dysgenesis in the linear nevus sebaceous syndrome can be expanded to include peripapillary staphyloma and atypical hemimegalencephaly with hypoplasia of an optic radiation.
Original language | English (US) |
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Pages (from-to) | 497-503 |
Number of pages | 7 |
Journal | Ophthalmology |
Volume | 104 |
Issue number | 3 |
DOIs | |
State | Published - 1997 |
ASJC Scopus subject areas
- Ophthalmology