| Title | Gerodermia osteodysplastica: report on two patients and surgical correction of facial deformity. |
| Author(s) | Lustmann J, Nahlieli O, Harary D, Casap N, Neder A, Zlotogora J |
| Institution | Department of Oral and Maxillofacial Surgery, Hadassah School of Dental Medicine, Jerusalem, Israel. |
| Source | Am J Med Genet 1993 Aug 15; 47(2):261-7. |
| MeSH | Adult
Cephalometry
Cutis Laxa
Dental Cementum
Female
Genes, Recessive
Humans
Jaw Abnormalities
Joint Instability
Male
Malocclusion, Angle Class III
Osteoporosis
Prognathism
Skin Abnormalities
Syndrome
Tooth Abnormalities
|
| Abstract | We present four individuals with Gerodermia Osteodysplastica in a Jewish family from Morocco confirming the autosomal recessive inheritance of the disorder. Three previously unreported findings are described: a) enlarged funnel-shaped mandibular lingula; b) extension of the mandibular premolar and molar roots below the inferior dental canal, and of the second molars into the lower border of mandibular cortical bone; and c) hypercementosis of the maxillary incisors and mandibular molars surrounded by a radiolucent halo in several teeth. The facial deformity resulting from maxillary hypoplasia and mandibular prognathism was corrected by orthognathic surgery: Le Fort I maxillary osteotomy and vertical mandibular osteotomy. |
| Language | eng |
| Pub Type(s) | Case Reports
Journal Article
Review
|
| PubMed ID | 8213917 |
