Corsi Alessandro, Ippolito Ernesto, Robey Pamela G, Riminucci Mara, Boyde Alan
Department of Molecular Medicine, Sapienza University, Policlinico Umberto I, Viale Regina 324, 00161, Rome, Italy.
Department of Orthopaedic Surgery, University of Rome Tor Vergata, Rome, Italy.
Skeletal Radiol. 2017 Oct;46(10):1435-1439. doi: 10.1007/s00256-017-2698-2. Epub 2017 Jun 28.
Bisphosphonates (BPs) are currently used in the treatment of diverse bone diseases including fibrous dysplasia of bone (FD). In pediatric patients, a radiographic consequence of cyclical administration of BPs is the development of apo-, epi-, and meta-physeal sclerotic bands, otherwise known as zebra lines, which result from the temporary inhibition of osteoclastic activity at the time of drug treatment. We report here on a child with McCune-Albright syndrome (FD in addition to hyperfunctioning endocrinopathies and skin hyperpigmentation) treated with cyclical intravenous infusions of pamidronate in which conventional radiography, contact microradiography, histology, and backscattered electron image analysis demonstrated that zebra lines formed only where bone was normal, were arrested at the boundary between FD-unaffected and FD-affected bone where bone is sclerotic, and were absent within the undermineralized FD bone. Moreover, in spite of the treatment, the FD lesions continued to expand. This case report is unique because no previously published studies correlated the radiographic and the histologic features of BP-induced zebra lines in the metaphysis of an FD-affected long bone of the limbs.
双膦酸盐(BPs)目前用于治疗多种骨疾病,包括骨纤维发育不良(FD)。在儿科患者中,周期性使用BPs的影像学后果是干骺端、骨骺和骨干出现硬化带,即所谓的斑马纹,这是药物治疗时破骨细胞活性暂时受到抑制的结果。我们在此报告一名患有McCune-Albright综合征(除了内分泌功能亢进和皮肤色素沉着外还伴有FD)的儿童,该儿童接受了帕米膦酸的周期性静脉输注治疗,其中传统X线摄影、接触式微放射摄影、组织学和背散射电子图像分析表明,斑马纹仅在正常骨处形成,在FD未受影响和受影响骨之间的硬化骨边界处停止,并且在矿化不足的FD骨内不存在。此外,尽管进行了治疗,FD病变仍继续扩大。该病例报告很独特,因为之前没有发表的研究将BP诱导的斑马纹在四肢FD累及的长骨干骺端的影像学和组织学特征联系起来。