Oligodontia of the permanent dentition in two sisters with polycystic ovarian syndrome: case reports.

Oligodontia or severe hypodontia is a rare developmental dental anomaly commonly associated with syndromes and systemic abnormalities. This report presents two sister, aged 18 and 21, who collectively had 56 congenitally missing permanent teeth. Both patients exhibited pubertal hirsutism, menstrual disturbances, and enlarged ovaries with multicystic lesions defined ultrasonically. These features are consistent with the diagnosis of polycystic ovarian syndrome, a disease not previously linked to hypodontia. A genetic component of this condition is proposed. The significance of this entity is discussed and the importance of early diagnosis and treatment regimens are emphasized.