The present case report highlights a number of issues related to OI that are of relevance to a pediatric dentist. The patient’s short stature compared with age, delayed walking at 2 years of age, her multiple fractures during infancy, and digital deformity in hands and feet are all consistent with the general features of patients with OI 4.
In terms of the cardiac abnormality, this patient had ventricular septal defect. It is known that cardiac valvular abnormalities can be found in many patients of OI, which might be because of the decrease in myocardial collagen diameter and amount associated with a significant decrease in fiber and chamber stiffness. Myocardial collagen contributes directly toward ventricular stiffness at high and low loads and can influence stress-free state and myofiber architecture 9. When evaluating patients with OI, careful attention should be paid to cardiovascular findings and if valvular lesions are noted, patients should be instructed on the need for antibiotic prophylaxis for dental and surgical procedures 10.
The patient had normal white sclera of the eye and did not have DI. OI does not always have to be accompanied by DI. The reported prevalence of DI in patients with OI type I varies from 8 to 40%, from 43 to 82% in type III, and from 37 to 100% in type IV 7. The patient may experience severe delay of permanent teeth eruption because of the bone deformity or because of the prolonged use of bisphosphonates. Results from a study on rats suggested that administration of bisphosphonates during tooth development has the potential to inhibit tooth eruption and formation and to induce several types of dental abnormalities 11. Kamoun-Goldrat et al. 12 reported a mean delay of 1.67 years in tooth eruption in OI children who were under treatment with bisphosphonates, which may be attributed to altered osteoclastic activities.
A delay in dental development was observed in 21% of patients with type III OI, whereas accelerated development was noted in 23% of the patients with type IV OI. In addition, ectopic eruption occurred in 13 patients in a study that evaluated 40 children (age range, 1–17.5 years) with types III and IV OI 2. In the same study, about 70–80% of patients were described to have skeletal class III malocclusion 2. However, in contrast to this, our case showed a developing skeletal class II malocclusion. Anterior open bite, and anterior and posterior crossbites have also been reported 2,13.
There has been no mention in the literature of bone quality in panoramic or lateral cephalometric radiographs. In contrast, in this report, we have described osteoporotic bone quality in both jaws obvious in panoramic and lateral cephalometric radiographs. The mandible showed osteoporotic features including low-density bone trabeculation and very thin inferior cortical bone. In the lateral cephalometric radiograph, there was thickening of the superior orbital ridge (bossing), which reflects the abnormal facial appearance.
The patient is on infusion doses of bisphosphonate (Zoledronic acid 0.05 mg/kg over 30 min) every 3 months. Bisphosphonates are primary agents in the current pharmacological arsenal against osteoclast-mediated bone loss, because of osteoporosis, OI, and Paget’s disease of bone and bone malignancies. The use of bisphosphonates has been found to be associated with osteonecrosis of the jaw 14. Other studies concluded that despite long-term intravenous monthly treatment of bisphosphonate in children with severe OI, no clinical signs of osteonecrosis of the jaw were found in any of the patients following dental surgery 15. In a study of 15 patients with OI who underwent extraction of 60 primary teeth, none of them had osteonecrosis of the jaw. It was found that unlike adults, osteonecrosis of the jaw is not a pediatric disease, and that it occurs rarely, if at all, in children 16. Intravenous bisphosphonate treatment improves symptoms of chronic bone pain, recurrent fracture rate, and bone mineral density in children with OI. However, bisphosphonate treatment creates multiple sclerotic metaphyseal bands (zebra lines) on long bones that are parallel to the growth plates and correspond to the number of treatment cycles 17,18. This was observed in the hand radiograph of this patient (Fig. 8).
The goals of dental treatment in OI patients are the maintenance of both primary and permanent dentition, a functional occlusion, optimal gingival health, and overall appearance. In terms of the dental management of children with OI, it is noteworthy that many clinicians appear reluctant to provide dental care to such children. This could be because of the possible fear of causing maxillary or mandibular fractures because of bone fragility or the fear of the possibility of osteonecrosis of the jaw following dental surgery in patients undergoing biphosphonate therapy. These complications should not deprive the patients from receiving required dental interventions. It should be emphasized that these children should be handled gently with great attention when using behavior management techniques. Physical restraints and exertion of extra pressure on bone should be avoided. As anxiety can be an issue with these children, premedication for anxiolysis, such as nitrous oxide analgesia or midazolam, can be used for treatment in a clinical setting 19. Special attention should be paid to patient treatment under general anesthesia. This include proper positioning on the operating room table, for which egg crate foam is recommended 19. The dentist should be conservative in dental treatment. Prevention, restorations, and endodontic treatment are preferred to extraction or surgery. Considerations should be given to oral antibiotic prophylaxis 1 h before extraction especially in patients with cardiovascular findings 10. For OI patients who are under oral or intravenous treatment of bisphosphonates and to avoid the development of osteonecrosis of the jaw, some preventive measures should be taken into consideration. In case of dental surgery in patients who are treated with bisphosphonates, Barker and Rogers 14 have suggested the use of chlorhexidine mouthwash before and after extractions with careful follow-up until healing of the socket has completed. Schwartz et al. 16 suggested waiting 8–15 days after the last infusion of the bisphosphonates and to have a good radiograph of the surgical site to confirm healing before administration of more bisphosphonates. However, Khan et al. 20 concluded that osteoporosis patients receiving oral or intravenous bisphosphonates do not require a dental examination before initiating therapy in the presence of appropriate dental care and good oral hygiene.
Orthodontic treatment can be initiated but great care must be taken to prevent easy fracture of the brittle teeth and bone with orthodontic appliances 19. The objectives of orthodontic treatment in this patient are to reduce maxillary growth and enhance mandibular growth, and stop the tongue thrust habit that might have contributed to the proclination of upper incisors. The patient was found to be best managed by an orthognathic surgery team (including an orthodontist, a maxillofacial surgeon, a plastic surgeon, and a pediatrician).
The case presented showed the maxillofacial, clinical, and radiographic features that might be present in patients with OI. A pediatric dentist plays an important role in caring and diagnosing abnormal features in those patients. He/she should be careful when planning treatment for these patients. There should be close collaboration between a dentist and a pediatric endocrinologist and a cardiologist or physicians of any specialty dealing with these patients. Physicians treating these patients are highly encouraged to educate patients and their parents on the importance of prevention by maintenance of good oral hygiene and frequent semiannual dental examinations.
Conflicts of interest
There are no conflicts of interest.
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