TY - JOUR
T1 - Gerodermia Osteodysplastica
T2 - Report on two patients and surgical correction of facial deformity
AU - Lustmann, J.
AU - Nahlieli, O.
AU - Harary, D.
AU - Casap, N.
AU - Neder, A.
AU - Zlotogora, J.
PY - 1993/1/1
Y1 - 1993/1/1
N2 - 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.
AB - 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.
KW - gerodermia osteodysplastica
KW - jaw deformity
KW - orthognathic surgery
UR - http://www.scopus.com/inward/record.url?scp=0027182947&partnerID=8YFLogxK
U2 - 10.1002/ajmg.1320470224
DO - 10.1002/ajmg.1320470224
M3 - Article
AN - SCOPUS:0027182947
VL - 47
SP - 261
EP - 267
JO - American Journal of Medical Genetics, Part A
JF - American Journal of Medical Genetics, Part A
SN - 1552-4825
IS - 2
ER -