Abstract
One case of van der Woude syndrome and three cases of familial occured conical elevation (C. E. ) were presented. Furthermore, in our clinic we investigated the incidence of C. E. in 40 patints with a postoperative isolated cleft palate (ICP) and examined the incidence of hypodontia regarding the C. E. cases. Four hundreds and six non cleft children were examined as control.
1. A case of van der Woude syndrome: A six-year old girl had a single C. E., unilateral congenital lower lip sinus and complete unilateral cleft lip and palate.
2. The familial occured C. E. cases: case 2 (propositus), one-year old i nfant male, C. E. with ICP. case 3 (elder sister), four-year old girl, C. E. with submuc o us cleft palate (SMCP). case 4 (cousin), three-year old boy, C. E. with SMCP. Mothers of case 2,3 and case 4 had C. E. respec tively. In case 3, SMCP was discovered while being examined for C. E..
3. Family history of the three C. E. patients indicated the autosomal dominant inheritance.
4. Fifteen out of fourty patients (37.0%) had a clinically distinct C. E.. In these 15 patints, hypodontia was recognized in 72.7& of the cases.
5. In 406 non cleft children, only 2 children (0.49%) had a distinct C. E..
6. It was suggested that C. E. was helpful for an earlier diagnosis of SMCP that tended to be difficult to establish.
