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craniofacial dysostosis

   Also found in: Acronyms, Wikipedia 0.04 sec.
dysostosis /dys·os·to·sis/ (dis″os-to´sis) defective ossification; defect in the normal ossification of fetal cartilages.
cleidocranial dysostosis  a hereditary condition marked by defective ossification of the cranial bones, absence of the clavicles, and dental and vertebral anomalies.
craniofacial dysostosis  a hereditary condition marked by acrocephaly, exophthalmos, hypertelorism, strabismus, parrot-beaked nose, and hypoplastic maxilla.
mandibulofacial dysostosis  a hereditary disorder occurring in a complete form (Franceschetti syndrome) and a less severe form (Treacher Collins syndrome), with antimongoloid slant of the palpebral fissures, coloboma of the lower lid, micrognathia and hypoplasia of the zygomatic arches, and microtia.
metaphyseal dysostosis  a skeletal abnormality in which the epiphyses are normal and the metaphyseal tissues are replaced by masses of cartilage, producing interference with enchondral bone formation.
dysostosis mul´tiplex  Hurler's syndrome.
orodigitofacial dysostosis  orofaciodigital syndrome.

craniofacial dysostosis
n.
An inherited cranial deformity characterized by widening of the skull and high forehead, abnormal width between and protrusion of the eyes, a beaked nose, and hypoplasia of the maxilla. Also called Crouzon's disease.

craniofacial dysostosis
Etymology: Gk, kranion + L, facies, face; Gk, dys, bad, osteon, bone
an abnormal hereditary condition characterized by acrocephaly, exophthalmos, hypertelorism, strabismus, parrot-beaked nose, and hypoplastic maxilla with relative mandibular prognathism. This condition is transmitted as an autosomal-dominant trait. See also dysostosis.

craniofacial anomalies (nom´lēs),
n.pl congenital malformations of the skull and face, frequently associated with genetically transmitted syndromes.
craniofacial dysostosis
craniofacial templates,
n.pl a series of cephalometric tracings of normal faces by age, sex, and race by which variations in the facial form of a patient can be determined and a treatment objective arranged.

dysostosis (disostō´sis),
n defective ossification.
dysostosis, cleidocranial
(klī´dōkrā´nēl),
n (Sainton's disease), See dysplasia, cleidocranial.
dysostosis, craniofacial,
dysostosis, mandibulofacial (TreacherCollins syndrome),
n a developmental disturbance of the cranial bones and hypoplasias of the upper part of the face. The mandibular body is underdeveloped, but the ramus is hyperplastic. The teeth are crowded and malposed.
dysostosis multiplex,

craniofacial dysostosis
Crouzon's disease Pediatrics An AD condition characterized by cranial suture defects, widened skull, a high forehead, ocular hypertelorism, exophthalmos, beaked nose and maxillary hypoplasia


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Crouzon's syndrome is a hereditary autosomal-dominant form of the craniofacial dysostosis disorders.
 
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