Oro-Dental Manifestations in Different Types of Osteogenesis Imperfecta

Purpose: This study aimed at determining the characteristic oro-dental manifestations (ODMs) in patients with osteogenesis imperfecta (OI) in order to develop a therapeutic and follow up oro-dental management scheme. It also aimed at comparing the ODMs in Sillence different types of OI hoping to aid in the differential diagnosis. Subjects and Methods: This study was carried out on 49 Egyptian patients with OI. Diagnosis of each case was based on full history taking, three generation pedigree construction, clinical examination, and confirmative investigations. Cases were classified according to Sillence et al. (1979) into four types (I-IV), and the oro-dental examination was carried out. Then the data was collected, tabulated, and the descriptive statistics was performed in the form of percentages. Results: The results of this study revealed that the percentages of thick lower lip, high arched palate, long philtrum, highly attached labial frenum, thick upper lip, thick upper labial frenum, macroglosia, thick alveolar ridge, everted lower lip, prominent median palatine raphe (PMPR), enamel hypocalcification, bow upper shaped lip, long uvula, prominent premaxilla and dentinogenesis imperfecta (DI) were 57.1%, 53.1%, 49%, 46.9%, 44.9%, 32.6 %, 32.6 %, 30.6%, 28.6%, 28.6%, 26.5%, 24.5%, 24.5%, 22.4%, and 20.4%, respectively. The most common ODMs in the different studied OI types (I-IV) included: thick lower lip in 69% for type I, 50% for type III, and 55.5% for type IV. High arched palate was present in 76.9% for type I, 38.5% for type III, and 66.6% for type IV. Long philtrum was present in 30.8% for type I, 53.8% for type III, and 55.5% for type IV. The percentage of highly attached upper labial frenum was 38.4% for type I, 53.8% for type III, and 44.4% for type IV. Thick upper lip was present in 53.8% for type I, 38.5% for type III, and 55.5% for type IV. On the other hand the percentage of DI was 7.7% for type I, 26.9% for type III, and 22.2% for type IV. Conclusion: Although oro-dental examination should be routinely done in OI patients to detect and manage the common associated ODMs, it is difficult to differentiate between the Sillence different types of OI based on the clinical or radiographic features of ODMs.