Neuro-ophthalmologic findings in patients with rhombencephalosynapsis

Paul H. Phillips; Charles M. Glasier; Michael C. Brodsky

doi:10.1016/j.jaapos.2007.08.010

Neuro-ophthalmologic findings in patients with rhombencephalosynapsis

Paul H. Phillips, Charles M. Glasier, Michael C. Brodsky

Ophthalmology

Research output: Contribution to journal › Article › peer-review

10 Scopus citations

Abstract

Rhombencephalosynapsis is a rare, sporadic, posterior fossa malformation characterized by agenesis of the cerebellar vermis, dorsal fusion of the cerebellar hemispheres, and fusion of the dentate nuclei and superior cerebellar peduncles.¹ Ophthalmologic findings in patients with rhombencephalosynapsis have not been described in detail. Optic nerve and chiasmal hypoplasia were reported in two patients.^2,3 Strabismus has been noted in several patients; however, specific details were not provided.¹ We describe the association of A-pattern strabismus and superior oblique overaction with rhombencephalosynapsis.

Original language	English (US)
Pages (from-to)	97-99
Number of pages	3
Journal	Journal of AAPOS
Volume	12
Issue number	1
DOIs	https://doi.org/10.1016/j.jaapos.2007.08.010
State	Published - Feb 2008

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Ophthalmology

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title = "Neuro-ophthalmologic findings in patients with rhombencephalosynapsis",

abstract = "Rhombencephalosynapsis is a rare, sporadic, posterior fossa malformation characterized by agenesis of the cerebellar vermis, dorsal fusion of the cerebellar hemispheres, and fusion of the dentate nuclei and superior cerebellar peduncles.1 Ophthalmologic findings in patients with rhombencephalosynapsis have not been described in detail. Optic nerve and chiasmal hypoplasia were reported in two patients.2,3 Strabismus has been noted in several patients; however, specific details were not provided.1 We describe the association of A-pattern strabismus and superior oblique overaction with rhombencephalosynapsis.",

author = "Phillips, {Paul H.} and Glasier, {Charles M.} and Brodsky, {Michael C.}",

note = "Funding Information: This work was supported in part by an unrestricted grant from Research to Prevent Blindness (New York, NY) and the Pat and Willard Walker Eye Research Center, Jones Eye Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas. Copyright: Copyright 2008 Elsevier B.V., All rights reserved.",

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AU - Phillips, Paul H.

AU - Glasier, Charles M.

AU - Brodsky, Michael C.

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PY - 2008/2

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N2 - Rhombencephalosynapsis is a rare, sporadic, posterior fossa malformation characterized by agenesis of the cerebellar vermis, dorsal fusion of the cerebellar hemispheres, and fusion of the dentate nuclei and superior cerebellar peduncles.1 Ophthalmologic findings in patients with rhombencephalosynapsis have not been described in detail. Optic nerve and chiasmal hypoplasia were reported in two patients.2,3 Strabismus has been noted in several patients; however, specific details were not provided.1 We describe the association of A-pattern strabismus and superior oblique overaction with rhombencephalosynapsis.

AB - Rhombencephalosynapsis is a rare, sporadic, posterior fossa malformation characterized by agenesis of the cerebellar vermis, dorsal fusion of the cerebellar hemispheres, and fusion of the dentate nuclei and superior cerebellar peduncles.1 Ophthalmologic findings in patients with rhombencephalosynapsis have not been described in detail. Optic nerve and chiasmal hypoplasia were reported in two patients.2,3 Strabismus has been noted in several patients; however, specific details were not provided.1 We describe the association of A-pattern strabismus and superior oblique overaction with rhombencephalosynapsis.

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Neuro-ophthalmologic findings in patients with rhombencephalosynapsis

Abstract

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