Disostosis cleidocraneal: Revisión bibliográfica a propósito de un caso clínico.
Abstract
La Disostosis Cleidocraneal (CCD) es un raro trastorno congénito del esqueleto, asociado a hipoplasia o aplasia clavicular, retardo en el cierre de las fontanelas craneales, con tipo braquicéfalos, retraso de la exfoliación de la dentición temporal, erupción retardada de dientes permanentes, presencia de varios supernumerarios y alteraciones morfológicas del maxilar y mandíbula. El trastorno se hereda como una condición autosómica dominante y en el 40% de los casos de la CCD aparece espontáneamente sin una causa genética aparente. El diagnóstico precoz es muy importante para dar a los pacientes las mejores opciones de tratamiento. En este trabajo, se revisó la literatura sobre esta enfermedad, su etiología, diagnóstico precoz, características generales y dentales. Se presenta un paciente con esta condición.
Palabras claves: disostosis cleidocraneal, hipoplasia, dientes supernumerarios, maloclusión.
Cleidocranial dysostosis (CCD) is a rare congenital skeletal disorder associated to clavicular hypoplasia or aplasia, delayed closure of fontanelles head with brachycephalic type, delayed exfoliation of primary teeth, delayed eruption of permanent teeth, presence of several supernumeraries and morphological changes on the maxilla and mandible. This disorder is inherited as an autosomal dominant condition and 40% of the cases appear spontaneously without apparent genetic cause. Early diagnosis is very important to give patients the best treatment options. In this article, literature regarding CCD, its etiology, early diagnosis, and general and dental features. A patient with this condition is presented.
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