Occlusion is the way in which the dental arches are related to each other and depends on craniofacial growth and development. It is affected in patients with Osteogenesis Imperfecta (OI) who present altered craniofacial development. The malocclusion present in 49 patients diagnosed with different types of OI aged between 4 and 18 was studied. The control group of healthy people was matched for age, sex, and molar class. To study the mixed and permanent dentition, the American Board of Orthodontics (ABO) discrepancy Index was applied. The primary dentition was evaluated with a Temporary Dentition Occlusion Analysis proposed for this study. The OI group obtained higher scores in the Discrepancy Index than the control group, indicating a high difficulty of treatment. The most significant differences were found in types III and IV of the disease. Regarding the variables studied, the greatest differences were found in the presence of lateral open bite in patients with OI, and in the variable “others” (agenesis and ectopic eruption). The analysis of primary dentition did not show significant differences between the OI and control groups. Patients with OI have more severe malocclusions than their healthy peers. Malocclusion is related to the severity of the disease and may progress with age.
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