Oral features in Apert syndrome: a histological investigation

Abstract

The number of publications on the oral features in Apert syndrome is limited. The present study investigated dental tissues in Apert syndrome histologically, to determine the nature and extent of anomalies, to provide some insight into the nature of the condition, and to explain how observed anomalies may affect the dental management of individuals with Apert syndrome. Extracted primary and secondary teeth were collected from patients with Apert who had attended the Australian Craniofacial Unit, Adelaide, South Australia. The total study sample comprised 13 individuals, aged from 14 to 21 , with nine men and four women. A total of 40 teeth were available for histological examination (the number belonging to each individual varied from 2 to 5 per patient). The teeth were sectioned longitudinally, and one-half of each tooth underwent decalcification. Sections were stained with H&E for routine histological examination. Ground sections were prepared from undecalcified tooth halves. Histological assessment of the dental hard tissues revealed an intact enamel and dentinal structure but some irregularities were noted in the region of the dentino-enamel junction (DEJ), which could affect caries progression and also make dental management more difficult. This study identified histological anomalies of the DEJ of Apert syndrome teeth. An improved appreciation of the nature and extent of dental anomalies in Apert syndrome should assist clinicians when undertaking management of affected individuals.