By comparing the timing of the eruption of primary teeth in both sexes, it was found that eruption of almost all primary teeth took place earlier in boys than in girls and that the eruption process was shorter for boys than for girls. Most of the deciduous teeth erupted earlier in the right side in both sexes. No differences were found in the sequence of eruption of primary teeth between genders.

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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Osteogenesis Imperfecta (OI) is a disease that causes bone fragility and deformities, affecting both the cranial base and the craniocervical junction, and may lead to other neurological disorders. A retrospective cross-sectional study was carried out based on cephalometric analysis of the cranial base in a sample of patients with OI, in lateral skull radiographs and magnetic resonance imaging (MRI), comparing them with a sample of age-matched controls. When the different variables of the craniocervical junction were analyzed, significance was found in comparisons with the different age groups. All measurements of the variables studied stabilized as growth progressed. For most of the variables, the severity of the disease influences the measurements of the skull base, with statistically significant differences. Both age and severity of the disease are factors that directly influence the anatomy of the craniocervical junction in these patients and may serve as indicators in the early detection and prevention of other derived alterations.