Abstract
The 48, XXYY syndrome is a rare sex chromosome aneuploidy condition characterized by a presence of two extra X and Y chromosomes. This syndrome is clinically manifested later in life with developmental delays, learning disabilities, behavioral problems, delayed or incomplete puberty and dental anomalies.
A 10 year-old boy was reported to the Dentistry Department of Faculty Hospital in Pilsen, Czech Republic for management of multiple carious teeth and for dental anxiety. Delayed development, learning difficulties and behavioral problems were present in the boy.
A clinical examination showed minor dysmorphic facial features, incomplete dentition, multiple carious lesions and gingivitis. The panoramic radiograph revealed taurodontism of all first permanent molars, agenesis of the second lower premolars, the conical clinical crowns of permanent upper lateral incisors and impaction of the upper left permanent canine.
Due to these findings the genetic examination was made and the caryotype of the boy revealed the aneuploidy. Patients with 48, XXYY syndrome require multidisciplinary treatment.
Dental management can be complicated by behavioral difficulties.