orodigitofacial 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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orodigitofacial dysostosis

n.

An inherited syndrome that is lethal in males and is characterized by various defects of the oral cavity, face, and hands. Also called orofaciodigital syndrome.

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orodigitofacial dysostosis.

See oral-facial-digital syndrome, types I, III.

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orodigitofacial dysostosis (OFD syndrome),

n a syndrome characterized by abnormal development of the jaws and tongue, cleft lip and palate, hypoplasia of bones of the skull with ocular hypertelorism, nasal alar deformity, malformation of digits (frequently manifested as brachydactyly and syndactyly), mental retardation, granular skin, and alopecia of the scalp.