Kos Marcin, Luczak Klaudiusz, Godzinski Jan, Klempous Jan
Department of Paediatric Surgery, Marciniak Hospital, Wroclaw, Poland.
J Craniomaxillofac Surg. 2004 Feb;32(1):10-5. doi: 10.1016/j.jcms.2003.07.009.
Fibrous dysplasia is a condition where fibrous tissue and bone with an altered structure replace normal bone. The craniofacial involvement is difficult to treat due to location, uncontrolled proliferation and compression of nerves. These result in facial asymmetry, pain, cranial nerve deficiencies, loss of vision or hearing, alterations in breathing, etc. The use of pamidronate, which inhibits the resorptive activity of osteoclasts may be an alternative to surgical intervention and may improve the results of treatment.
To evaluate the treatment of monostotic craniofacial fibrous dysplasia with pamidronate.
A prospective pilot study was undertaken in 6 children with progressive fibrous dysplasia located in the mandible (3 pts), maxilla (2 pts) or cranial base (1 pt). Surgery consisted only of a biopsy and implantation of gentamycin sponge into the biopsy bed in 3 cases in which an infection was initially suspected. All patients received pamidronate infusions (1mg/kg i.v. for 3 days, every 4-6 months). The changes in bone density and extension of the disease were evaluated by serial orthopantomograms and CT scans. Follow up time was 9-24 months.
Pain relief was achieved in all cases, decrease in swelling in 3, and stabilization in 3 cases. Radiological investigation revealed any progression but reduction in size and calcification of osteolytic lesions. The local bone density increased and there was no further spread of the disease in any of the patients. Inflammatory symptoms resolved in all cases. The only side effect was an increase in body temperature up to 38-40 degrees C.
Pamidronate appears to be an effective and well-tolerated therapeutic option for patients with fibrous dysplasia. In the future, the combination of the metabolic blockage of the dysplasia by pamidronate and limited non-mutilating surgical interventions are possible.
纤维性发育异常是一种纤维组织和结构改变的骨组织替代正常骨的病症。由于其位置、不受控制的增殖以及对神经的压迫，颅面部受累情况难以治疗。这些会导致面部不对称、疼痛、颅神经功能缺损、视力或听力丧失、呼吸改变等。使用抑制破骨细胞吸收活性的帕米膦酸盐可能是手术干预的一种替代方法，并且可能改善治疗效果。
评估用帕米膦酸盐治疗单发性颅面部纤维性发育异常。
对6例患有进行性纤维性发育异常的儿童进行了一项前瞻性初步研究，病变位于下颌骨（3例）、上颌骨（2例）或颅底（1例）。在最初怀疑有感染的3例病例中，手术仅包括活检并将庆大霉素海绵植入活检部位。所有患者均接受帕米膦酸盐输注（静脉注射1mg/kg，共3天，每4 - 6个月一次）。通过连续的全景X线片和CT扫描评估骨密度变化和疾病范围。随访时间为9 - 24个月。
所有病例均实现疼痛缓解，3例肿胀减轻，3例病情稳定。影像学检查未发现病情进展，但溶骨性病变的大小减小且出现钙化。局部骨密度增加，所有患者的疾病均未进一步扩散。所有病例的炎症症状均消失。唯一的副作用是体温升高至38 - 40摄氏度。
帕米膦酸盐似乎是纤维性发育异常患者一种有效且耐受性良好的治疗选择。未来，帕米膦酸盐对发育异常的代谢阻断与有限的非致残性手术干预相结合是有可能的。
