成骨不全:特殊表现

Special Findings in Osteogenesis Imperfecta

目的：观察成骨不全的特殊表现，探讨其形成机制和预后。材料与方法：对６８例成骨不全的临床与Ｘ线片资料进行回顾性分析。结果：本组成骨不全中，２３例有特殊表现，包括：①正常骺板变模糊或消失，骨骺、干骺区出现爆玉米花样钙化；②干骺端外展、增宽，骨骺见有多囊状透光区；③骨干不规则增粗，骨骺和干骺被粗大的骨小梁交织为蜂房状；④尺桡或胫腓骨间膜粘连、增生和骨化；⑤并行两长管骨之一的游离侧显示骨膜增生、骨化；⑥骨干骨折后骨痂生长稀少；⑦骨折后骨不连和假关节形成；⑧肿瘤样骨痂形成。结论：成骨不全的特殊表现多而复杂，与其病因有关，也是致残的主要因素。

Objective: To evaluate special findings in osteogenesis imperfecta and to study their formative mechanism and prognostic significance.Materials and Methods:The radiological features of 68 patients with osteogenesis imperfecta were retrospectively analyzed.Results:The following extraordinary X ray findings were found in 23 patients: (1) blurred or vanished epiphyseal plate, popcorn calcifications in epiphyses and metaphyses; (2) flared metaphyses, polycystic radiolucent areas in epiphyses; (3) irregularly broadened diaphyses, faveolate epiphyses and metaphyses due to coarse trabeculation; (4) adhesion, hyperplasia and ossification of ulna radius and/or tibia fibula interosseous membrance; (5) periosteal proliferation and ossification on lateral free border in one of parallel long bones; (6) rarefaction of callus to a fractured diaphysis; (7) nonunion and pseudoarthrosis formation of fracture site; (8) tumoral callus formation.Conclusion: Osteogenesis imperfecta has many extraordinary and complex X ray signs, which are related with its etiology. These changes are probably the main teratogenic causes.