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

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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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Authors and Affiliations

  1. Centre for Neurology and Neurosurgery at Santa Casa de Misericórdia de Belo Horizonte, Belo Horizonte, Minas Gerais, Brazil

    Laryssa C. Azevedo Almeida & Franklin B. Faraj de Lima

  2. Division of Neurological Surgery, University of São Paulo, Sao Paulo, Brazil

    Hamilton Matushita

  3. Neurology and Neurosurgery Unit, Department of Neuropsychiatry, Federal University of Pernambuco, Recife, Pernambuco, Brazil

    Marcelo M. Valença

  4. University Center of Belo Horizonte, Belo Horizonte, Minas Gerais, Brazil

    Tamireis L. Ferreira Castro & Rafael N. de Mendonça

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  1. Laryssa C. Azevedo Almeida
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  2. Franklin B. Faraj de Lima
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Correspondence to Laryssa C. Azevedo Almeida.

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Azevedo Almeida, L.C., Faraj de Lima, F.B., Matushita, H. et al. Cleidocranial dysplasia, a rare skeletal disorder with failure of the cranial closure: case-based update. Childs Nerv Syst 36, 2913–2918 (2020). https://doi.org/10.1007/s00381-020-04831-z

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