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craniofacial dysostosis |
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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 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 ( 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 dysostosis, craniofacial, n See syndrome, Crouzon. 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, n See syndrome, Hurler's. 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 How to thank TFD for its existence? Tell a friend about us, add a link to this page, add the site to iGoogle, or visit webmaster's page for free fun content. |
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Crouzon's syndrome is a hereditary autosomal-dominant form of the craniofacial dysostosis disorders. |
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