Document Type

Article

Original Publication Date

2020

Journal/Book/Conference Title

Case Reports in Pediatrics

Volume

2020:2031701

First Page

1

Last Page

5

DOI of Original Publication

10.1155/2020/2031701

Comments

Originally published at https://doi.org/10.1155/2020/2031701.

Funded in part by the VCU Libraries Open Access Publishing Fund.

Date of Submission

August 2020

Abstract

The recurrent 16p11.2 microdeletion is characterized by developmental delays and a wide spectrum of congenital anomalies. It has been well reported that individuals with this ∼593-kb interstitial deletion have an increased susceptibility toward the autism spectrum disorder (ASD). Abnormalities of the eye and ocular adnexa are also commonly associated findings seen in individuals with the 16p11.2 microdeletion syndrome, although these ophthalmic manifestations have not been well characterized. We conducted an extensive literature review to highlight the eye features in patients with the 16p11.2 microdeletion syndrome and describe a 5-year-old boy with the syndrome. The boy initially presented with intellectual disability, speech delay, and defiant behavior; diagnoses of attention deficit hyperactivity disorder (ADHD) and oppositional defiant disorder (ODD) were established. He had a Chiari malformation type 1. His ophthalmic features included strabismus, hyperopia, and ptosis, and a posterior embryotoxon was present bilaterally. From a systematic review of prior reported cases, the most common eye and ocular adnexa findings observed were downslanting palpebral fissures, deep-set eyes, ptosis, and hypertelorism.

Rights

© 2020 Cybil S. Stingl et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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VCU Ophthalmology Publications

Included in

Ophthalmology Commons

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