Gerodermia osteodysplastica: report on two patients and surgical correction of facial deformity.
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.
Lustmann
J
J
Department of Oral and Maxillofacial Surgery, Hadassah School of Dental Medicine, Jerusalem, Israel.
Nahlieli
O
O
Harary
D
D
Casap
N
N
Neder
A
A
Zlotogora
J
J
D000328:Adult; D002508:Cephalometry; D003483:Cutis Laxa / Q000235:genetics; D003739:Dental Cementum / Q000002:abnormalities; D005260:Female; D005808:Genes, Recessive; D006801:Humans; D007569:Jaw Abnormalities / Q000235:genetics* / Q000601:surgery*; D007593:Joint Instability / Q000235:genetics; D008297:Male; D008313:Malocclusion, Angle Class III / Q000601:surgery; D010024:Osteoporosis / Q000235:genetics*; D011378:Prognathism / Q000601:surgery; D012868:Skin Abnormalities; D013577:Syndrome; D014071:Tooth Abnormalities / Q000235:genetics