Micke O, Wagner W, Pötter R, Prott F J, Karbowski A
Klinik und Poliklinik für Strahlentherapie-Radioonkologie, Westfälische Wilhelms-Universität, Münster.
Strahlenther Onkol. 1994 Jun;170(6):347-51.
Osteogenesis imperfecta is a rare hereditary disease of connective tissue with a genetic defect in collagen synthesis. In osteogenesis imperfecta hyperplastic heterotopic ossification can be induced by hyperplastic callus formation caused by trauma or operation. Heterotopic ossifications can be found in numerous benign diseases. The successful use of low dose radiotherapy in the treatment of heterotopic ossifications is well-known from the literature.
We treated two children (a 13-year-old girl and a ten-year-old boy) with heterotopic ossifications of the lower extremities in osteogenesis imperfecta type IV (Lobstein) with a low dose irradiation (10 x 1 Gy, respectively 6 x 1 Gy) under megavoltage conditions.
After radiotherapy the children were painfree and the hyperplastic callus was considerably reduced. The previously immobilized patients could partly be mobilized. Thereby it could be contributed to the rehabilitation of the patients. New hyperplastic callus formation was not observed in the irradiated areas so far.
Analogous to the successful radiation of heterotopic ossifications in other benign diseases radiation therapy seems to be a successful treatment of hyperplastic callus formation in osteogenesis imperfecta. Despite the late risks of radiotherapy radiation treatment of benign diseases in children might be indicated.
成骨不全是一种罕见的结缔组织遗传性疾病，存在胶原蛋白合成的基因缺陷。在成骨不全中，创伤或手术引起的增生性骨痂形成可诱发增生性异位骨化。异位骨化可见于多种良性疾病。低剂量放疗成功用于治疗异位骨化在文献中已有记载。
我们对两名患有IV型（洛布斯坦型）成骨不全且下肢有异位骨化的儿童（一名13岁女孩和一名10岁男孩）在兆伏条件下进行了低剂量照射（分别为10×1 Gy和6×1 Gy）。
放疗后，患儿无痛，增生性骨痂明显减少。先前需固定的患者部分能够活动。这有助于患者的康复。到目前为止，在照射区域未观察到新的增生性骨痂形成。
与其他良性疾病中异位骨化放疗成功类似，放疗似乎是治疗成骨不全中增生性骨痂形成的一种成功方法。尽管放疗存在后期风险，但儿童良性疾病的放射治疗可能是必要的。
