Pediatric Dental Management of a Patient With Infantile Osteopetrosis in Remission: A Clinical Case Report.

BACKGROUND

Osteopetrosis is a rare genetic disorder characterized by increased bone density due to defective osteoclast function. Its clinical presentation varies according to subtype, and oral manifestations are frequent, potentially serving as early indicators of the disease. These findings place the pediatric dentist in a key role for early recognition and safe dental management.

CASE: This case report describes the comprehensive dental management of a 7-year-old girl with a history of infantile osteopetrosis treated with hematopoietic stem cell transplantation during early infancy. The patient presented with oligodontia, generalized enamel hypoplasia and hypomineralization, dental hypersensitivity, and altered dental morphology. Management focused on prevention-centered care, including professional prophylaxis, topical fluoride therapy (5% sodium fluoride varnish), home fluoridated toothpaste and mouth rinse use, pit and fissure sealants, selective minimally traumatic extractions with antibiotic prophylaxis, and interceptive orthodontic treatment using slow maxillary expansion with a Bertoni appliance. Monthly follow-up visits were conducted over a 12-month period.

RESULTS

Enamel defects remained stable, dental hypersensitivity decreased, no new carious lesions developed, and no infectious or osteonecrotic complications were observed. Slow maxillary expansion improved arch coordination without adverse effects.

CONCLUSION

A conservative, prevention-oriented, and multidisciplinary dental approach can be safely implemented in pediatric patients with osteopetrosis. Early identification, minimally invasive procedures, and close follow-up are essential to reduce complications and improve oral health outcomes and quality of life.