PDF(Pubmed)
Abstract：
Osteopetrosis comprises a group of inherited disorders that are rare and result in abnormal bone structure. Bone remodeling is extremely inhibited because osteoclasts are nonfunctional or lacking. This condition causes overgrowth of bone with disappearance of the bone marrow, leading to aplastic anemia; obstruction of nerve passages in the skull leads to blindness and often hearing impairment. In most cases, osteopetrosis results in oral complications such as tooth deformation, hypomineralization, and delayed or absent tooth eruption. The only curative treatment is hematopoietic stem cell transplantation (HSCT). The main treatment of the oral complications during childhood and adolescence consists in protecting the erupted teeth against caries disease through prophylactic treatment aimed at optimal oral hygiene through frequent regular dental visits throughout life. Many patients with osteopetrosis require major oral rehabilitation to treat complications of the disease. Improved results of HSCT increase the likelihood that dental professionals will encounter patients with osteopetrosis.
In this case report, we show that individuals with osteopetrosis who have severe oral complications can be treated successfully if they are treated for osteopetrosis at an early age. The boy had his dental care in pedodontics, and regular multidisciplinary meetings were held for future treatment planning. At the age of 15, he was then referred for rehabilitation. The initial evaluations revealed no further growth in the alveolar bone. The rehabilitation was done stepwise, with extraction of malformed and malpositioned teeth. Initially, the patient received a removable partial denture followed by reconstruction of the width of the alveolar process, titanium implants, temporary fixed bridges, and finally screw-retained titanium-ceramic bridges with titanium frames for the upper and lower jaws.
The three-year follow-up after loading indicated a stable marginal bone level and optimal oral hygiene as a result of frequent professional oral hygiene care. The patient showed no signs of symptoms from the temporomandibular joint and has adapted to the new jaw relation without any functional or phonetical issues.
In this case report, we show that individuals with osteopetrosis who have severe oral complications can be treated successfully if they are treated for osteopetrosis at an early age. The boy had his dental care in pedodontics, and regular multidisciplinary meetings were held for future treatment planning. At the age of 15, he was then referred for rehabilitation. The initial evaluations revealed no further growth in the alveolar bone. The rehabilitation was done stepwise, with extraction of malformed and malpositioned teeth. Initially, the patient received a removable partial denture followed by reconstruction of the width of the alveolar process, titanium implants, temporary fixed bridges, and finally screw-retained titanium-ceramic bridges with titanium frames for the upper and lower jaws.
The three-year follow-up after loading indicated a stable marginal bone level and optimal oral hygiene as a result of frequent professional oral hygiene care. The patient showed no signs of symptoms from the temporomandibular joint and has adapted to the new jaw relation without any functional or phonetical issues.
摘要：
背景：石骨病包括一组罕见的遗传性疾病，并导致骨结构异常。由于破骨细胞无功能或缺乏，因此骨重塑受到极大抑制。这种情况导致骨骼过度生长，骨髓消失，导致再生障碍性贫血；颅骨神经通道阻塞会导致失明和听力损害。在大多数情况下,骨硬化导致口腔并发症，如牙齿变形，低矿化,牙齿萌出延迟或缺失。唯一的治愈性治疗是造血干细胞移植（HSCT）。儿童期和青春期口腔并发症的主要治疗包括通过预防性治疗来保护萌出的牙齿免受龋齿疾病的侵害,所述预防性治疗旨在通过一生中频繁的定期牙科就诊来实现最佳的口腔卫生。许多患有骨硬化症的患者需要主要的口腔康复来治疗该疾病的并发症。HSCT的改善结果增加了牙科专业人员遇到石骨症患者的可能性。
方法：在本案例报告中，我们表明，患有严重口腔并发症的石骨症患者，如果在早期接受石骨症治疗，可以成功治疗。这个男孩在儿科接受了牙齿护理，并定期举行多学科会议，以制定未来的治疗计划。15岁时，他被转介康复。初步评估显示牙槽骨没有进一步生长。康复是逐步进行的，拔除畸形和错位的牙齿。最初，患者接受了可摘局部义齿，然后重建了牙槽突的宽度，钛植入物，临时固定桥,最后是螺丝保留的钛陶瓷桥，带有上下颚的钛框架。
结论：负荷后的三年随访表明，由于频繁的专业口腔卫生护理，边缘骨水平稳定，口腔卫生最佳。患者没有表现出来自颞下颌关节的症状迹象，并且已经适应了新的颌骨关系，没有任何功能或语音问题。
方法：在本案例报告中，我们表明，患有严重口腔并发症的石骨症患者，如果在早期接受石骨症治疗，可以成功治疗。这个男孩在儿科接受了牙齿护理，并定期举行多学科会议，以制定未来的治疗计划。15岁时，他被转介康复。初步评估显示牙槽骨没有进一步生长。康复是逐步进行的，拔除畸形和错位的牙齿。最初，患者接受了可摘局部义齿，然后重建了牙槽突的宽度，钛植入物，临时固定桥,最后是螺丝保留的钛陶瓷桥，带有上下颚的钛框架。
结论：负荷后的三年随访表明，由于频繁的专业口腔卫生护理，边缘骨水平稳定，口腔卫生最佳。患者没有表现出来自颞下颌关节的症状迹象，并且已经适应了新的颌骨关系，没有任何功能或语音问题。
