Ostegenesis imperfecta (OI) is a group of genetic diseases with a wide spectrum of severity, ranging from very mild bone fragility to lethal forms. Without treatment the more severe forms have multiple fractures leading to progressive bone deformities with extreme shortness, frequent skeletal pain and immobilization.
AIMS. The overall aim of these studies were initially to find and later to optimize a symptomatic treatment of patients with OI to find and to monitor treatment.
METHOD AND MATERIALS. The study involves 15 persons with OI of which 9 were treated with bisphosphonates in infusions every three months (disodium pamidronate). 8 patients had dentinogenesys imperfecta. None of the 15 patients reviewed developed osteonecrosis of the jaw. In 46.5% of the cases the teeth had a restaurative treatment (26.6% while the patient had active bisphosphonate treatment); in 20% of the cases the teeth were extracted (none had active bisphosphonate treatment); in 13.2% of the cases the teeth had a not surgical parodontal treatment (6.6% while the patient had active bisphosphonate treatment); in 13.2% of the cases the teeth had a surgical parodontal treatment (none had active bisphosphonate treatment); in 13.2% of the cases the patiens had an implant surgery. Because no complications were reported, we inferred that no osteonecrosis of the jaw occurred.
CONCLUSIONS. One of the greatest challenges for the pediatric dentist is to provide adequate treatment to achieve functional and esthetic restoration in cases of diseases like OSTEOGENESIS imperfecta. Early diagnosis and treatment are essential for obtaining a favorable prognosis, any delay in intervention making the treatment even more complex. The aim is to achieve a good esthetic result and restore function along with preventive measures to establish a more favorable prognosis for such complex anomaly
