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Ophthalmologic Findings in the Cornelia de Lange Syndrome
Journal article

Ophthalmologic Findings in the Cornelia de Lange Syndrome

Tamara Wygnanski-Jaffe, John Shin, Enza Perruzza, Mohamed Abdolell, Laird G. Jackson, Alex V. Levin and Jennifer Y Shin
Journal of AAPOS, v 9(5), pp 407-415
2005
PMID: 16213388

Abstract

Background: Cornelia de Lange Syndrome (CdLS) is a disorder caused in many patients by a mutation in the NIPBL gene with a dominant pattern of inheritance characterized by mental retardation, prenatal and postnatal growth retardation, upper-limb abnormalities, and characteristic facies. Few data exist concerning the ophthalmic findings in this syndrome. Methods: One hundred twenty individuals with CdLS underwent ophthalmic examination to ascertain the relative frequencies of oculofacial and ophthalmic abnormalities. Results: We confirmed the frequent findings of synophrys (99%), long lashes (99%), hypertrichosis of the brows (96%), ptosis (44%), epiphora (22%), nasolacrimal duct obstruction (16%), blepharitis (25%), and myopia (58%). In addition, we found peripapillary pigment (83%), and microcornea (21%), which have infrequently been mentioned in the literature. Conclusion: Patients with CdLS can have mutiple eye problems. Many of these problems can be readily treated, including myopia, blepharitis, nasolacrimal duct obstruction, and ptosis. Early examination is recommended for all children known or suspected to have CdLS.

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Collaboration types
Domestic collaboration
International collaboration
Web of Science research areas
Ophthalmology
Pediatrics
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