Oculocerebral dysgenesis in the linear nevus sebaceous syndrome

Michael C Brodsky, J. M. Kincannon, P. Nelson-Adesokan, H. H. Brown

Research output: Contribution to journalArticle

26 Citations (Scopus)

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 languageEnglish (US)
Pages (from-to)497-503
Number of pages7
JournalOphthalmology
Volume104
Issue number3
StatePublished - 1997
Externally publishedYes

Fingerprint

Sebaceous of Jadassohn Nevus
Neurocutaneous Syndromes
Radiation
Optic Atrophy
Choristoma
Dermoid Cyst
Cerebrum
Optic Nerve
Neuroimaging
Magnetic Resonance Imaging
Hemimegalencephaly

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Brodsky, M. C., Kincannon, J. M., Nelson-Adesokan, P., & Brown, H. H. (1997). Oculocerebral dysgenesis in the linear nevus sebaceous syndrome. Ophthalmology, 104(3), 497-503.

Oculocerebral dysgenesis in the linear nevus sebaceous syndrome. / Brodsky, Michael C; Kincannon, J. M.; Nelson-Adesokan, P.; Brown, H. H.

In: Ophthalmology, Vol. 104, No. 3, 1997, p. 497-503.

Research output: Contribution to journalArticle

Brodsky, MC, Kincannon, JM, Nelson-Adesokan, P & Brown, HH 1997, 'Oculocerebral dysgenesis in the linear nevus sebaceous syndrome', Ophthalmology, vol. 104, no. 3, pp. 497-503.
Brodsky MC, Kincannon JM, Nelson-Adesokan P, Brown HH. Oculocerebral dysgenesis in the linear nevus sebaceous syndrome. Ophthalmology. 1997;104(3):497-503.
Brodsky, Michael C ; Kincannon, J. M. ; Nelson-Adesokan, P. ; Brown, H. H. / Oculocerebral dysgenesis in the linear nevus sebaceous syndrome. In: Ophthalmology. 1997 ; Vol. 104, No. 3. pp. 497-503.
@article{cbc7834f0c464bbdbec35668b55e0a0d,
title = "Oculocerebral dysgenesis in the linear nevus sebaceous syndrome",
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.",
author = "Brodsky, {Michael C} and Kincannon, {J. M.} and P. Nelson-Adesokan and Brown, {H. H.}",
year = "1997",
language = "English (US)",
volume = "104",
pages = "497--503",
journal = "Ophthalmology",
issn = "0161-6420",
publisher = "Elsevier Inc.",
number = "3",

}

TY - JOUR

T1 - Oculocerebral dysgenesis in the linear nevus sebaceous syndrome

AU - Brodsky, Michael C

AU - Kincannon, J. M.

AU - Nelson-Adesokan, P.

AU - Brown, H. H.

PY - 1997

Y1 - 1997

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=0030935595&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0030935595&partnerID=8YFLogxK

M3 - Article

C2 - 9082279

AN - SCOPUS:0030935595

VL - 104

SP - 497

EP - 503

JO - Ophthalmology

JF - Ophthalmology

SN - 0161-6420

IS - 3

ER -