E. Karlstedt et al., TRANSVERSE FACIAL MORPHOLOGY IN PATIENTS WITH DIASTROPHIC DYSPLASIA, Journal of craniofacial genetics and developmental biology, 17(4), 1997, pp. 178-183
Diastrophic dysplasia (DTD) is a well-characterized, recessively inher
ited osteochondrodysplasia. Thirty-seven patients were studied for tra
nsverse craniofacial characteristics. Of these patients, 10 had cleft
palate and 11 had submucous cleft palate. A cephalometric analysis bas
ed on posteroanterior (PA) cephalograms was performed: 16 landmarks we
re identified and digitized into a computer. Seven linear and four ang
ular variables were calculated and the values compared with those of a
matched control population. DTD patients differed from controls only
in cases with cleft palate where the mesio-orbital, bigonial, and ante
gonial widths were large compared with controls. The present findings
indicate that although the development and growth of cartilaginous str
uctures are disturbed in DTD, the intramembranously developing bones a
nd the appositional growth pattern do not seem to be primarily affecte
d.