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

Autor: Marcelo Moraes Valença, Laryssa Crystinne Azevedo Almeida, Rafael N. de Mendonça, Tamireis L. Ferreira Castro, Franklin B. Faraj de Lima, Hamilton Matushita
Rok vydání: 2020
Předmět:
Zdroj: Child's Nervous System. 36:2913-2918
ISSN: 1433-0350
0256-7040
DOI: 10.1007/s00381-020-04831-z
Popis: 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. 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. In our review, we encountered several cases of orthodontic implications but a few cases on cranial defect approach. 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.
Databáze: OpenAIRE