mandibulofacial dysostosis

dysostosis /dys·os·to·sis/ (dis″os-to´sis) defective ossification; defect in the normal ossification of fetal cartilages.

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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.

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mandibulofacial dysostosis

n.

A syndrome that includes various malformations of specialized structures in the head and neck, including notched eyelid fissures, hypoplasia of the mandible, enlarged mouth with high or cleft palate and incorrectly positioned teeth, and atypical hair growth. Also called Franceschetti's syndrome.

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mandibulofacial dysostosis

[mandib′yəlofā′shəl]

Etymology: L, mandere + facies, face; Gk, dys, bad, osteon, bone

an abnormal hereditary condition characterized by an antimongoloid slant of the palpebral fissures, coloboma of the lower lid, micrognathia and hypoplasia of the zygomatic arches, and microtia. Evidence indicates that this disorder is transmitted as an autosomal-dominant trait. The condition occurs in the complete form as Franceschetti's syndrome and in the incomplete form as Treacher Collins' syndrome. See also dysostosis.

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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.

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dysostosis (disostō´sis),

n defective ossification.

dysostosis, cleidocranial

(klī´dōkrā´nēl),
n (Sainton's disease), See dysplasia, 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.

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mandibular

(mandib´ylr),
adj pertaining to the lower jaw.

mandibular angle,

n See angle of the mandible.

mandibular axis,

n See axis, mandibular.

mandibular border,

n See border, mandibular.

mandibular canal,

n See canal, mandibular.

mandibular centric relation,

n the closing relation of the mandible with the fixed craniofacial complex as determined clinically by instruments that record jaw motion.

mandibular condyle,

n See condyle, mandibular.

mandibular flexure,

n See flexure, mandibular.

mandibular foramen,

n See foramen, mandibular.

mandibular fracture,

n a break in the continuity of the bone of the mandible. See also fracture.

mandibular glide,

n See glide, mandibular.

mandibular guide prosthesis,

n a prosthesis with an extension designed to direct a resected mandible into a functional relation with the maxillae.

mandibular hinge position,

n See position, hinge, mandibular.

mandibular labial frenum

(mandib´ylr lā´bēl frē´nm),
n a vertical band of oral mucosa located between the midlines of the mandibular central incisors, which connects the attached gingiva to the lower lip and safeguards against any excessive motions.

mandibular movement,

n See movement, mandibular.

mandibular nerve,

n See nerve, mandibular.

mandibular notch,

n See notch, mandibular.

mandibular pain-dysfunction syndrome,

n See temporomandibular joint pain-dysfunction syndrome.

mandibular prognathism

(mandib´ylr prog´nthiz´m),
n a condition in which the mandible projects outward farther than the maxilla.

mandibular rest position,

n See position, rest, mandibular.

mandibular retraction,

n See retraction, mandibular.

mandibulofacial dysostosis

(mandib´ūlōfā´shl),
n See syndrome, Treacher Collins.