Dentofacial Malocclusion in Neurofibromatosis 1 in Finland

Neurofibromatosis 1 (NF1) is an inherited disease that can be accompanied by oral health problems such as caries, periodontitis, and tumors affecting the oral cavity. Also, different maxillary and mandibular malformations are associated with NF1. In this retrospective register-based study, we evaluated hospital visits related to dentofacial malocclusion in 1349 individuals with NF1, 13,870 matched controls and 1894 non-NF1 siblings followed up over 1998-2014 using the Finnish Care Register for Health Care that covers information on inpatient care and specialist outpatient care. Hazard ratios (HRs) and their 95% confidence intervals (CI) were estimated with the Cox proportional hazards model. Individuals with NF1 had a higher hazard for hospital visits related to embedded and impacted teeth (HR 2.1, 95% CI 1.2-3.5), disorders of tooth development and eruption (HR 3.7, 95% CI 1.9-7.1), and dentofacial anomalies (HR 2.7, 95% CI 1.9-3.8) such as anomalies in dental arch relationship (HR 4.8, 95% CI 2.9-7.9) and anomalies of jaw-cranial base relationship (HR 2.2, 95% CI 1.1-4.3) compared with controls. Plexiform neurofibromas did not markedly affect the estimates. Early detection of jaw and dental alterations, which may be linked to previously identified cephalometric features of NF1, is important for preventing occlusal defects, maintaining oral hygiene, and preserving quality of life.