Abstract
With the advance of gene analysis, it has been elucidated that mutations in the paired-domain transcription factor PAX9 are associated with oligodontia. We are treating sibling cases with athetoid cerebral palsy accompanying the deletion including PAX9.
Patient 1 is a 16-year-old girl who shows persistent pulmonary hypertension of the newborn （PPHN）. During the neonatal period, she was diagnosed as hypothyroidism and was treated with thyroid hormone. She had intellectual disability and athetoid cerebral palsy. She showed agenesis of 4 primary teeth and 21 permanent teeth. Patient 2 is an 8-year-old girl, and is the younger sister of Patient 1. She also shows PPHN, hypothyroidism, intellectual disability and athetoid cerebral palsy. She also showed agenesis of 4 primary teeth and 15 permanent teeth.
The patients have been provided with regular dental examination and prophylaxis. Once, we tried to apply removable dentures to patient 1 who had difficulty in chewing hard food, but she could not get used to wearing the dentures.
Several approaches such as removable dentures can be applied to patients with oligodontia. Therefore, we need to find the most suitable prosthesis for such patients to improve their oral condition functionally and esthetically.
