[Syndromes 21: Ellis-Van Creveld syndrome].
Patients with the Ellis-Van Creveld syndrome have a short stature. The extremities are often plump and markedly shortened, progressively from the trunk to the fingers and the toes. A bilateral postaxial sixth finger is frequent. The most striking and consistent finding in the mouth is fusion of the middle part of the upper lip to the labial sulcus, resulting in a so-called 'whistling deformity'. Congenitally missing teeth, particularly in the frontal region, are a constant finding too. Teeth are usually small and have conical crowns. Supernummer teeth have also been noted. The oral and maxillofacial surgeon will treat the hypertrophic upper frenulum; the dentist will treat the oligodontia of the frontal region and the conical crowns by means of laminated veneers and etch composite bridgework.
Baart
J A
JA
Afdeling Mondziekten en Kaakchirurgie/Orale Pathologie van de Vrije Universiteit/Academisch Centrum Tandheelkunde (ACTA) in Amsterdam.
van Hagen
J M
JM
D019465:Craniofacial Abnormalities / Q000601:surgery; D003937:Diagnosis, Differential; D004613:Ellis-Van Creveld Syndrome / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D006801:Humans; D019647:Oral Surgical Procedures; D014071:Tooth Abnormalities / Q000235:genetics / Q000601:surgery*