Transverse facial morphology in patients with diastrophic dysplasia.

Abstract

Diastrophic dysplasia (DTD) is a well-characterized, recessively inherited osteochondrodysplasia. Thirty-seven patients were studied for transverse craniofacial characteristics. Of these patients, 10 had cleft palate and 11 had submucous cleft palate. A cephalometric analysis based on posteroanterior (PA) cephalograms was performed: 16 landmarks were identified and digitized into a computer. Seven linear and four angular 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 antegonial widths were large compared with controls. The present findings indicate that although the development and growth of cartilaginous structures are disturbed in DTD, the intramembranously developing bones and the appositional growth pattern do not seem to be primarily affected.