Resumen

HOYOS SERRANO, Maddelainne. SINDROME DE CROUZON. Rev. Act. Clin. Med [online]. 2014, vol.46, pp. 2457-2462. ISSN 2304-3768.

The Crouzon Syndrome, is an congenital craniofacial dysostosis characterized intrauterine for early closure of the coronal, sagittal and lambdoid sutures, so that causes abnormal growth of skull and facial asymmetry maxillary hypoplasia. Their origin lies in the mutation of the gene responsible for encoding receptor type fibroblast growth factor 3, so that the healing and collagen production are altered. The most important clinical features include craniofacial and dental level, they range from: premature fusion of all sutures, maxillary hypoplasia, proptosis, brachycephaly, hydrocephalus, degrees of mental retardation, malocclusion, palate inverted V-shaped, strabismus, hearing loss, etc.. With respect to radiological diagnosis, extraoral radiographs and CT scans fingerprints angular defects or radiopaque lines belonging to the fused suture is observed. The treatment for this syndrome is interdisciplinary, which means that many health professionals to be part of it, such as: pediatric neurosurgeon, maxillofacial surgeon, pediatric dentist or oral surgeon, so that each professional will affect some stage the patient, so that treatments are only to address structural dysfunctions that exist.

Palabras llave : Crouzon syndrome; Syndromic craniosynostosis; Congenital craniofacial dysostosis.