Kaplan F S, Strear C M, Zasloff M A
Department of Orthopaedic Surgery, University of Pennsylvania School of Medicine, Philadelphia.
Clin Orthop Relat Res. 1994 Jul(304):238-47.
To characterize the radiographic and scintigraphic features of modeling and remodeling in the heterotopic skeleton of patients who have fibrodysplasia ossificans progressiva, radiographs from 47 patients and radionuclide bone scans from 12 of those patients, all of whom had a confirmed diagnosis of the disease, were reviewed. A wide range of normal bone modeling and remodeling features was seen in the heterotopic skeleton of all but the youngest two (age, 1 year) of the 47 patients. Characteristic features of normal bone modeling identified on radiographs of the heterotopic skeleton included: (a) the development of tubular and flat bones with mature cortical and trabecular organization; (b) the presence of well defined cortical-endosteal borders enclosing medullary canals; and (c) the presence of metaphyseal funnelization in isolated ossicles or at sites of synostoses. Characteristic features of normal bone remodeling identified on radiographs of the heterotopic skeleton included: (a) the response of heterotopic bone to weight bearing stress with osteosclerosis of use and osteopenia of disuse, and (b) the resistance of heterotopic bone to fatigue failure with the absence of pathologic fractures and stress fractures. Radionuclide bone scans in 12 patients showed that remodeling of mature heterotopic bone occurred at a rate consistent with that of mature normotopic bone. This study documents the radiographic and scintigraphic features of a heterotopic skeletal system in 47 patients who have fibrodysplasia ossificans progressiva. These data provide additional support for the hypothesis that the genetic defect leading to the formation of a heterotopic skeleton involves normal skeletal morphogenesis at heterotopic sites.
为了描述进行性骨化性纤维发育不良患者异位骨骼中塑形和重塑的放射学及闪烁扫描特征，我们回顾了47例患者的X线片以及其中12例患者的放射性核素骨扫描结果，所有患者均确诊为此病。在47例患者中，除最年幼的两名患者（年龄1岁）外，其他患者的异位骨骼均呈现出广泛的正常骨塑形和重塑特征。在异位骨骼的X线片上确定的正常骨塑形特征包括：(a) 管状骨和平扁骨的发育，伴有成熟的皮质和小梁结构；(b) 存在界定清晰的皮质-骨内膜边界，包绕着骨髓腔；(c) 在孤立的小骨或骨融合部位存在干骺端漏斗状结构。在异位骨骼的X线片上确定的正常骨重塑特征包括：(a) 异位骨对负重应力的反应，即使用部位骨质硬化，废用部位骨质减少；(b) 异位骨对疲劳性骨折具有抵抗力，未出现病理性骨折和应力性骨折。12例患者的放射性核素骨扫描显示，成熟异位骨的重塑速率与成熟正常部位骨的重塑速率一致。本研究记录了47例进行性骨化性纤维发育不良患者异位骨骼系统的放射学及闪烁扫描特征。这些数据为以下假说提供了额外支持，即导致异位骨骼形成的基因缺陷涉及异位部位正常的骨骼形态发生过程。
