Dental care approach in patients with osteopetrosis

To describe dental and dentofacial characteristics observed in patients diagnosed with osteopetrosis and to advise a dental care approach in these patients.

Four patients were clinically diagnosed with osteopetrosis, characterised by increased bone density, bone marrow failure, blindness and deafness due to compression of cranial nerves. All patients were dentally screened at different ages (2.5-31 years) and three of them were treated with a haematopoietic stem cell transplantation (HSCT) at the age of 6 months, 1 and 3.1 years.

All patients showed similar dental characteristics but varying severity and extent. Dental pits, abnormalities in form, agenesis and enamel deformations are seen. The eruption of the permanent dentition occurs at a slow rate, primary teeth can persist, have no successor, and aberrant form of the primary/permanent teeth can delay eruption. Uneven surfaces and atypical dental crowns combined with visual impairment make brushing of the teeth and plaque removal more difficult to manage.

Dental problems such as delay in tooth eruption, crown anomalies and agenesis are seen in the patients diagnosed with osteopetrosis, although the severity and extensiveness of the symptoms differ and possibly depend on the age of the patient at HSCT. Treatment management: Frequent dental follow-up examinations are necessary for guiding the eruption and professional dental cleanings. Aid in the eruption can be helpful. In the case of surgical interventions, an antibiotic prophylaxis is advised. A fluoride treatment can be added to prevent caries. The role of HSCT in dental findings needs further research.