Oral manifestations of severe short-limb dwarfism resembling Grebe chondrodysplasia: report of a case.

The oral and dental abnormalities associated with a distinct variety of severe short-limb dwarfism are described. The patient, a 9-year-old Arab boy, had delayed development and eruption of teeth, severe oligodontia of permanent dentition, hypodontia, microdontia, supplemental incisor, enamel hypoplasia of primary teeth, doubled and abnormal frenal attachments, bifid uvula, hypoplastic maxilla, and malocclusion. Clinical and radiographic examinations revealed asymmetric dysplasia and anaplasia of long bones, craniofacial dysmorphia, prominent forehead, budlike fingers and bulbous toes, dysplastic nails, severe hearing loss, and reduced joint mobility. These features resemble, in general, those characteristic of Grebe chondrodysplasia, an extremely rare ill-defined syndrome that is inherited as an autosomal-recessive disorder.