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Published in: Child's Nervous System 12/2020

01-12-2020 | Case-Based Review

Cleidocranial dysplasia, a rare skeletal disorder with failure of the cranial closure: case-based update

Authors: Laryssa C. Azevedo Almeida, Franklin B. Faraj de Lima, Hamilton Matushita, Marcelo M. Valença, Tamireis L. Ferreira Castro, Rafael N. de Mendonça

Published in: Child's Nervous System | Issue 12/2020

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Abstract

Background and aim

Cleidocranial dysplasia is a rare disorder of skeletal development that mainly promotes, among other malformations, inadequate development of clavicles and failure in cranial closure. In this affection, the role of neurosurgery in addressing cranial defects is rarely discussed.

Material and methods

We conducted an extensive review of the literature using the PubMed database, giving a greater focus to publications in the field of neurosurgery. Additionally, we report a case of a 2-year-old female child with cleidocranial dysplasia.

Results

In our review, we encountered several cases of orthodontic implications but a few cases on cranial defect approach.

Conclusion

The articles present literature that is unanimous on the recommendation of expectant conduct in children since the cranial block can occur spontaneously, even if the delayed form. In our approach, we opted for an expected strategy concerning the cranial defect, using a helmet made for brain protection. We also made the referral for multidisciplinary monitoring of pediatrics, neuropediatrics, ophthalmology, dentistry, and orthopedics.
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Metadata
Title
Cleidocranial dysplasia, a rare skeletal disorder with failure of the cranial closure: case-based update
Authors
Laryssa C. Azevedo Almeida
Franklin B. Faraj de Lima
Hamilton Matushita
Marcelo M. Valença
Tamireis L. Ferreira Castro
Rafael N. de Mendonça
Publication date
01-12-2020
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 12/2020
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-020-04831-z

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