Ocular motility abnormalities in orbitofacial neurofibromatosis type 1

Darren T. Oystreck, Ibrahim A. Alorainy, Jose Morales, Imtiaz A. Chaudhry, Sahar M. Elkhamary, Thomas Bosley

Research output: Contribution to journalArticle

Abstract

Purpose To evaluate the causes of ocular motility disturbances in a group of patients with orbitofacial neurofibromatosis (OFNF) with neurofibromas on the lid, brow, face, or in the orbit from infancy or early childhood. Methods The medical records of patients with OFNF from one institution were retrospectively reviewed; selected patients were reexamined. Results A total of 45 patients with unilateral OFNF and 4 with bilateral OFNF were included. Of these, 14 had no strabismus and relatively good vision, with no ductional abnormalities on either side despite large globes, sphenoid dysplasia, and neurofibromas in the orbit and/or cavernous sinus in many. The 8 patients with comitant strabismus also had no ductional abnormalities with a similar constellation of anatomic abnormalities, but these patients all had poor vision in at least one eye. The 27 patients with incomitant strabismus all had downward displacement of the globe and limited ductions. Conclusions The pathologic anatomic changes associated with OFNF do not always cause ocular motility abnormalities: strabismus generally was not present when ocular motility was full and visual acuity was good. Comitant strabismus occurred in the setting of full ocular motility with reduced vision in at least one eye. Incomitant strabismus was always accompanied by reduced vision and a ductional abnormality in one or both eyes due to anatomic abnormalities of the orbit and skull.

Original languageEnglish (US)
Pages (from-to)338-343
Number of pages6
JournalJournal of AAPOS
Volume18
Issue number4
DOIs
StatePublished - 2014
Externally publishedYes

Fingerprint

Eye Abnormalities
Neurofibromatosis 1
Strabismus
Neurofibromatoses
Orbit
Low Vision
Neurofibroma
Cavernous Sinus
Skull
Visual Acuity
Medical Records

ASJC Scopus subject areas

  • Ophthalmology
  • Pediatrics, Perinatology, and Child Health
  • Medicine(all)

Cite this

Oystreck, D. T., Alorainy, I. A., Morales, J., Chaudhry, I. A., Elkhamary, S. M., & Bosley, T. (2014). Ocular motility abnormalities in orbitofacial neurofibromatosis type 1. Journal of AAPOS, 18(4), 338-343. https://doi.org/10.1016/j.jaapos.2014.02.018

Ocular motility abnormalities in orbitofacial neurofibromatosis type 1. / Oystreck, Darren T.; Alorainy, Ibrahim A.; Morales, Jose; Chaudhry, Imtiaz A.; Elkhamary, Sahar M.; Bosley, Thomas.

In: Journal of AAPOS, Vol. 18, No. 4, 2014, p. 338-343.

Research output: Contribution to journalArticle

Oystreck, DT, Alorainy, IA, Morales, J, Chaudhry, IA, Elkhamary, SM & Bosley, T 2014, 'Ocular motility abnormalities in orbitofacial neurofibromatosis type 1', Journal of AAPOS, vol. 18, no. 4, pp. 338-343. https://doi.org/10.1016/j.jaapos.2014.02.018
Oystreck, Darren T. ; Alorainy, Ibrahim A. ; Morales, Jose ; Chaudhry, Imtiaz A. ; Elkhamary, Sahar M. ; Bosley, Thomas. / Ocular motility abnormalities in orbitofacial neurofibromatosis type 1. In: Journal of AAPOS. 2014 ; Vol. 18, No. 4. pp. 338-343.
@article{4e63893b40504858a62560f8b6c75f49,
title = "Ocular motility abnormalities in orbitofacial neurofibromatosis type 1",
abstract = "Purpose To evaluate the causes of ocular motility disturbances in a group of patients with orbitofacial neurofibromatosis (OFNF) with neurofibromas on the lid, brow, face, or in the orbit from infancy or early childhood. Methods The medical records of patients with OFNF from one institution were retrospectively reviewed; selected patients were reexamined. Results A total of 45 patients with unilateral OFNF and 4 with bilateral OFNF were included. Of these, 14 had no strabismus and relatively good vision, with no ductional abnormalities on either side despite large globes, sphenoid dysplasia, and neurofibromas in the orbit and/or cavernous sinus in many. The 8 patients with comitant strabismus also had no ductional abnormalities with a similar constellation of anatomic abnormalities, but these patients all had poor vision in at least one eye. The 27 patients with incomitant strabismus all had downward displacement of the globe and limited ductions. Conclusions The pathologic anatomic changes associated with OFNF do not always cause ocular motility abnormalities: strabismus generally was not present when ocular motility was full and visual acuity was good. Comitant strabismus occurred in the setting of full ocular motility with reduced vision in at least one eye. Incomitant strabismus was always accompanied by reduced vision and a ductional abnormality in one or both eyes due to anatomic abnormalities of the orbit and skull.",
author = "Oystreck, {Darren T.} and Alorainy, {Ibrahim A.} and Jose Morales and Chaudhry, {Imtiaz A.} and Elkhamary, {Sahar M.} and Thomas Bosley",
year = "2014",
doi = "10.1016/j.jaapos.2014.02.018",
language = "English (US)",
volume = "18",
pages = "338--343",
journal = "Journal of AAPOS",
issn = "1091-8531",
publisher = "Mosby Inc.",
number = "4",

}

TY - JOUR

T1 - Ocular motility abnormalities in orbitofacial neurofibromatosis type 1

AU - Oystreck, Darren T.

AU - Alorainy, Ibrahim A.

AU - Morales, Jose

AU - Chaudhry, Imtiaz A.

AU - Elkhamary, Sahar M.

AU - Bosley, Thomas

PY - 2014

Y1 - 2014

N2 - Purpose To evaluate the causes of ocular motility disturbances in a group of patients with orbitofacial neurofibromatosis (OFNF) with neurofibromas on the lid, brow, face, or in the orbit from infancy or early childhood. Methods The medical records of patients with OFNF from one institution were retrospectively reviewed; selected patients were reexamined. Results A total of 45 patients with unilateral OFNF and 4 with bilateral OFNF were included. Of these, 14 had no strabismus and relatively good vision, with no ductional abnormalities on either side despite large globes, sphenoid dysplasia, and neurofibromas in the orbit and/or cavernous sinus in many. The 8 patients with comitant strabismus also had no ductional abnormalities with a similar constellation of anatomic abnormalities, but these patients all had poor vision in at least one eye. The 27 patients with incomitant strabismus all had downward displacement of the globe and limited ductions. Conclusions The pathologic anatomic changes associated with OFNF do not always cause ocular motility abnormalities: strabismus generally was not present when ocular motility was full and visual acuity was good. Comitant strabismus occurred in the setting of full ocular motility with reduced vision in at least one eye. Incomitant strabismus was always accompanied by reduced vision and a ductional abnormality in one or both eyes due to anatomic abnormalities of the orbit and skull.

AB - Purpose To evaluate the causes of ocular motility disturbances in a group of patients with orbitofacial neurofibromatosis (OFNF) with neurofibromas on the lid, brow, face, or in the orbit from infancy or early childhood. Methods The medical records of patients with OFNF from one institution were retrospectively reviewed; selected patients were reexamined. Results A total of 45 patients with unilateral OFNF and 4 with bilateral OFNF were included. Of these, 14 had no strabismus and relatively good vision, with no ductional abnormalities on either side despite large globes, sphenoid dysplasia, and neurofibromas in the orbit and/or cavernous sinus in many. The 8 patients with comitant strabismus also had no ductional abnormalities with a similar constellation of anatomic abnormalities, but these patients all had poor vision in at least one eye. The 27 patients with incomitant strabismus all had downward displacement of the globe and limited ductions. Conclusions The pathologic anatomic changes associated with OFNF do not always cause ocular motility abnormalities: strabismus generally was not present when ocular motility was full and visual acuity was good. Comitant strabismus occurred in the setting of full ocular motility with reduced vision in at least one eye. Incomitant strabismus was always accompanied by reduced vision and a ductional abnormality in one or both eyes due to anatomic abnormalities of the orbit and skull.

UR - http://www.scopus.com/inward/record.url?scp=84906836444&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84906836444&partnerID=8YFLogxK

U2 - 10.1016/j.jaapos.2014.02.018

DO - 10.1016/j.jaapos.2014.02.018

M3 - Article

C2 - 25173895

AN - SCOPUS:84906836444

VL - 18

SP - 338

EP - 343

JO - Journal of AAPOS

JF - Journal of AAPOS

SN - 1091-8531

IS - 4

ER -