BIFID UVULA AND SUBMUCOUS CLEFT PALATE IN CORNELIA DE LANGE SYNDROM

Cornelia de Lange syndrome is a rare congenital disease characterized by growth and psychomotor retardation, peculiar facial feature as skeletal and craniofacial deformities, gastrointestinal and cardiac problems and malformation of the upper limb. The prevalence is estimated around 0.6/100000 in the population. The diagnosis is based on clinical findings and the etiology is still unclear. We present a case of a 17-year-old patient, who came to our attention for dental pain. After an oral examination carried out under general anesthesia the patients presented most of the charactericts described in the literature as micrognathia , high arched palte, delayed aruption, missing of some teeth. The most peculiar findings were the bifid uvula and the submucous cleft palate. The entity of clefting can be determined only with a Magnetic Resonance Imaging which should be carried out under general anesthesia . Caries and periodontal disease were present and the entire dental treatment has been carried out in one sitting without any anestesiologic problems.