Challenges in the Orthodontic Treatment of a Patient with Pycnodysostosis

Abstract

Pycnodysostosis is a rare, autosomal recessive syndrome characterized by osteosclerosis, brittle bones, stunting, and significant craniofacial changes. The objective of this study was to report a case of a 6-year-old patient with pycnodysostosis orthodontically treated and followed up until age 10 years and to discuss the risk factors, options for orthodontic treatment, and limitations involving this type of treatment, which has not yet been performed. Prevention through counseling and periodic follow-up visits is essential in eliminating factors that predispose patients to infections and fractures. New studies are necessary to establish safe and efficient orthodontic treatment plans.

Clinical and radiographic features of pycnodysostosis: A case report

“…Only few reports concerning PYCD describing the oral and maxillofacial features are available. So far, there is no specific protocol for PYCD bearers in dental clinics and its treatment is basically supportive, such as orthodontic therapy ( 6 , 7 , 13 , 14 ). According to Girbal et al ( 15 ), osteomyelitis is a major risk, and can be faced as a serious complication of PYCD in patients who performed prior dental treatments.…”

Pycnodysostosis with Special Emphasis on Dentofacial Characteristics

“…Therefore, early treatment with multibanded fixed appliances will prolong the overall treatment duration as well as increasing the chances of developing white spot lesions. Others suggest planned and sequential extractions in early age to guide the eruption of permanent teeth in better position [ 4 , 24 ]. In addition, early extractions of retained deciduous teeth may reduce the chances of impaction of the permanent teeth.…”

“…In addition, early extractions of retained deciduous teeth may reduce the chances of impaction of the permanent teeth. Ortegosa et al [ 24 ] in their study planned serial extractions in a patient with pycnodysostosis, aged 6 years and 9 months to reduce crowding and for correction of abnormal eruption sequence. In addition, preventive dental education programs and strict oral hygiene instructions should be instituted to them right from an early age [ 18 ].…”

“…In the present case, maxillary orthopedic expansion with bonded expander was performed with successful results. According to Ortegosa et al [ 24 ], activation of midline screw exerts force upon the teeth and the palate resulting in the separation of the maxillary and palatine process in the median palatal suture region. However, the osteosclerosis of bone in these patients may increase the risk of nonrupture of the median palatine suture, fracture of the alveolar rims, mobility of the teeth, and maxillary osteomyelitis.…”

Clinical and Cephalometric Analysis of Three Cases With Pycnodysostosis: Case Reports

“…However, no published article in the current literature could be found about orthodontic or orthopedic treatment of PKND. Ortegosa et al ( 11 ) reported that in such cases, maxillary removable appliance with springs associated with serial extractions could be safe for teeth movement.…”