Arponen Heidi, Vuorimies Ilkka, Haukka Jari, Valta Helena, Waltimo-Sirén Janna, Mäkitie Outi
Department of Orthodontics, Institute of Dentistry, University of Helsinki;
J Neurosurg Pediatr. 2015 Mar;15(3):313-20. doi: 10.3171/2014.11.PEDS14113. Epub 2015 Jan 10.
OBJECT: Cranial base pathology is a serious complication of osteogenesis imperfecta (OI). Our aim was to analyze whether bisphosphonate treatment, used to improve bone strength, could also prevent the development of craniocervical junction pathology (basilar impression, basilar invagination, or platybasia) in children with OI. METHODS: In this single-center retrospective study the authors analyzed the skull base morphology from lateral skull radiographs and midsagittal MR images (total of 94 images), obtained between the ages of 0 and 25 years in 39 bisphosphonate-treated OI patients. The results were compared with age-matched normative values and with findings in 70 OI patients who were not treated with bisphosphonates. In addition to cross-sectional data, longitudinal data were available from 22 patients with an average follow-up period of 7.6 years. The patients, who had OI types I, III, IV, VI, and VII, had been treated with zoledronic acid, pamidronate, or risedronate for 3.2 years on average. RESULTS: Altogether 33% of the 39 bisphosphonate-treated patients had at least 1 cranial base anomaly, platybasia being the most prevalent diagnosis (28%). Logistic regression analysis suggested a higher risk of basilar impression or invagination in patients with severe OI (OR 22.04) and/or older age at initiation of bisphosphonate treatment (OR 1.45), whereas a decreased risk was associated with longer duration of treatment (OR 0.28). No significant associations between age, height, or cumulative bisphosphonate dose and the risk for cranial base anomaly were detected. In longitudinal evaluation, Kaplan-Meier curves suggested delayed development of cranial base pathology in patients treated with bisphosphonates but the differences from the untreated group were not statistically significant. CONCLUSIONS: These findings indicate that cranial base pathology may develop despite bisphosphonate treatment. Early initiation of bisphosphonate treatment may delay development of craniocervical junction pathology. Careful followup of cranial base morphology is warranted, particularly in patients with severe OI.
目的：颅底病变是成骨不全症（OI）的一种严重并发症。我们的目的是分析用于增强骨强度的双膦酸盐治疗是否也能预防OI患儿颅颈交界区病变（基底凹陷、基底内陷或扁平颅底）的发生。 方法：在这项单中心回顾性研究中，作者分析了39例接受双膦酸盐治疗的OI患者在0至25岁期间获得的头颅侧位X线片和矢状面磁共振成像（共94张图像）的颅底形态。将结果与年龄匹配的正常参考值以及70例未接受双膦酸盐治疗的OI患者的检查结果进行比较。除横断面数据外，还获得了22例患者的纵向数据，平均随访期为7.6年。这些患有I型、III型、IV型、VI型和VII型OI的患者平均接受唑来膦酸、帕米膦酸或利塞膦酸治疗3.2年。 结果：在39例接受双膦酸盐治疗的患者中，共有33%至少存在1种颅底异常，扁平颅底是最常见的诊断（28%）。逻辑回归分析表明，严重OI患者（比值比22.04）和/或开始双膦酸盐治疗时年龄较大（比值比1.45）发生基底凹陷或内陷的风险较高，而治疗时间较长则风险降低（比值比0.28）。未检测到年龄、身高或双膦酸盐累积剂量与颅底异常风险之间存在显著关联。在纵向评估中，Kaplan-Meier曲线表明接受双膦酸盐治疗的患者颅底病变发展延迟，但与未治疗组的差异无统计学意义。 结论：这些发现表明，尽管进行了双膦酸盐治疗，颅底病变仍可能发生。早期开始双膦酸盐治疗可能会延迟颅颈交界区病变的发展。有必要对颅底形态进行仔细随访，尤其是严重OI患者。
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