The imperfect osteogenesis it is also called crystal bones. It is an inherited disorder that is characterized by an alteration in the connective tissue of the entire body, including the teeth.
It is caused by disturbances in the synthesis of type I collagen. The incidence of this disease is between 6 to 20 people per 100,000 new births.
The types of osteogenesis imperfecta
Osteogenesis imperfecta can be classified into four main types, according to the clinical characteristics it presents, being type II the most serious, since it is usually incompatible with life. Death occurs in the first months to a year of life and only in extremely rare cases does survival reach adolescence.
- Type I. It is the mildest type. Patients of this type present blue sclera, scoliosis and may present imperfect dentinogenesis. These patients are hearing impaired.
- Type II. It is the most serious form of the disease. Neonatal fractures occur and the long bones may appear to have been pressured.
- Type III. It is a severe osteogenesis. The sclera of these patients are not altered but there are severe deformities in the spine which cause short stature. In this type, dentinogenesis imperfecta mainly affects the primary dentition.
- Type IV. It is an autosomal dominant inheritance.
Manifestations of osteogenesis imperfecta and involvement in the oral cavity
The manifestations suffered by patients with this systemic alteration can be grouped into skeletal and non-skeletal manifestations. In the skeletal ones, the fragility of the bones, the reduction of the general bone mass and the possibility of causing fractures are mainly found. Regarding the non-skeletal manifestations, the sclera of the eyes are slightly bluish, the ligamentous hypermobility, alterations in the cranial sutures and imperfect dentinogenesis in the teeth.
Imperfect dentinogenesis is an autosomal dominant disorder in the development of dentin of hereditary origin. Teeth with this alteration are more yellowish or bluish-gray in color. In addition, the root length is less than usual. Imperfect dentinogenesis makes enamel detach easily. The teeth of the oral cavity most affected by this disease are the central incisors and the first molars. Radiologically, it shows a considerable reduction in the pulp chamber and also a decrease in the density of the dentin. The roots are small and sometimes show radiolucency at the periapx.
