dysostosis /dys·os·to·sis/ (dis″os-to´sis) defective ossification; defect in the normal ossification of fetal cartilages.
cleidocranial dysostosis
dysostosis [dis″os-to´sis]
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.
| cleidocranial dysostosis or clidocranial dysostosis n. A congenital complex that may be hereditary and is characterized by absent or rudimentary development of the clavicles, an enlarged incompletely ossified skull with frontal protuberances, and poor tooth formation. Also called cleidocranial dysplasia, craniocleidodysostosis. |
cleidocranial dysostosis
[klī′dōkrā′nē·əl]
Etymology: Gk, kleis, key, kranion, skull, dys, bad, osteon, bone
a rare abnormal hereditary condition characterized by defective ossification of the cranial bones and by the complete or partial absence of the clavicles. It is transmitted as an autosomal-dominant trait. The defective ossification of the cranial bones delays the closing of the cranial sutures and produces large fontanels. The complete or partial absence of the clavicles allows the shoulders to be drawn together. This condition also involves dental and vertebral anomalies. Also called cleidocranial dysplasia, dystrophia. See also dysostosis.
dysostosis [dis″os-to´sis]
defective ossification; a defect in the normal ossification of fetal cartilages.
cleidocranial dysostosis an autosomal dominant condition in which there is defective ossification of the cranial bones, complete or partial absence of the clavicles, so that the shoulders may be brought together, or nearly together, in front, and dental and vertebral anomalies. See illustration.
Cleidocranial dysostosis. From Dorland's, 2000.
craniofacial dysostosis an autosomal dominant condition marked by a pointed or conical skull, protruding wide-set eyes, strabismus, parrot-beaked nose, and hypoplastic maxilla with relative mandibular prognathism. Called also Crouzon's disease.
mandibulofacial dysostosis a hereditary disorder occurring in two different forms: the complete form is Franceschetti syndrome and the incomplete form is Treacher Collins syndrome. Persons with the condition have downslanting eyes (antimongoloid palpebral fissures); absence of all or part of the lower lid; underdeveloped cheekbones that appear depressed; a prominent nose, wide mouth, and small receding chin; underdeveloped, malformed, or prominent ears; and small tufts of hair in front of the ears. There is often, but not always, some degree of hearing loss, usually conductive.
metaphyseal dysostosis a skeletal abnormality in which the epiphyses are normal or nearly so, and the metaphyseal tissues are replaced by masses of cartilage, producing interference with endochondral bone formation and expansion and thinning of the metaphyseal cortices.
orodigitofacial dysostosis orofaciodigital syndrome.
dysostosis (disostō´sis),
n defective ossification.
dysostosis, cleidocranial
l), 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.
cleidocranial dysostosis
Cleidocranial dysplasia Pediatrics An AD condition characterized by partial or complete absence of clavicle, dental defects, joint laxity and a facies–heavy brow, protruding jaw, wide nasal nasal bridge,
malaligned teeth, characteristic facies–frontal bossing
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