保留骨骺的保肢手术临床研究

目的 介绍儿童及青少年保留骨骺的保肢手术方法，并讨论肿瘤复发率、转移情况及术后并发症与关节功能。方法1995年12月至2003年1月，对33例儿童及青少年肢体原发性恶性或侵袭性骨肿瘤进行保留骨骺的保肢手术，年龄8-16岁，平均12．2岁。股骨下端24例，胫骨上端9例；骨肉瘤23例，尤文肉瘤6例，软骨肉瘤2例，侵袭性骨母细胞瘤2例。Enneking外科分期：ⅠA期2例，ⅠB期2例，ⅡA期17例，ⅡB期12例。恶性肿瘤患者接受术前2-4个周期、术后6个周期的化疗。采用大段同种异体骨移植重建骨缺损，并用松质骨拉力螺钉将其与骨骺进行固定。结果29例患者随访资料完整。随访时间12～72个月，平均37．6个月。3例复发，分别于术后1年在骨骺处复发、术后15个月和30个月在原肿瘤位置的股动、静脉周围复发，复发率为10．3％。对复发病例行截肢术。共9例死亡，5年生存率为57．9％。4例患者发生5例并发症，发生率为17．2％。依据Enneking术后功能评价标准，优11例，良13例，可3例，差2例，总优良率为82．8％。结论四肢恶性骨肿瘤保留骨骺的保肢手术可以使患者获得较好的肢体功能，在严格掌握手术适应证和有效化疗的前提下，实施该手术是安全的。     

Solitary lucent epiphyseal lesions in children

We evaluated retrospectively the varying radiographic appearances of 15 solitary lucent epiphyseal lesions occurring in children. Imaging modalities used included plain films, conventional tomography, nuclear scintigraphy, and computed tomography. Forty percent of the lesions (6) were due to osteomyelitis. The remaining lesions included tuberculosis (1), foreign body granuloma (1), chondroblastoma (2), chondromyxoid fibroma (1), enchondroma (1), osteoid osteoma (2), and eosinophilic granuloma (1). Although the radiographic appearances of such lesions may be particularly characteristic, pathologic correlation is frequently necessary. The high incidence of osteomyelitis in our cases emphasizes its importance as a cause for a lucent epiphyseal lesion.     

Ossification and pseudoepiphysis formation in the “nonepiphyseal” end of bones of the hands and feet

Metacarpals, metatarsals, and phalanges were studied to assess the developmental morphology of secondary ossification in the nonepiphyseal ends of these bones as well as the formation of the pseudoepiphysis as an epiphyseal ossification variant. Both direct ossification extension from the metaphysis into the epiphysis and pseudoepiphysis formation preceded, and continued to be more mature than, formation and expansion of the classic epiphyseal (secondary) ossification center at the opposite end of each specific bone. Direct metaphyseal to epiphyseal ossification usually started centrally and expanded hemispherically, replacing both physeal and epiphyseal cartilage simultaneously. In contrast, when remnants of physis were retained, while juxtaposed epiphyseal cartilage was replaced, a pseudoepiphysis formed. There were three basic patterns of pseudoepiphysis formation. First, a central osseous bridge extended from the metaphysis across the physis into the epiphysis and subsequently expanded to create a mushroom-like osseous structure. In the second pattern a peripheral osseous bridge formed, creating either an osseous ring or an eccentric bridge between the metaphysis and the epiphysis. In the third pattern, multiple bridging occurred. In each situation the associated remnant physis lacked typical cell columns and was incapable of significantly contributing to the postnatal longitudinal growth of the involved bone. Pseudoepiphyses were well formed by 4–5 years and coalesced with the rest of the bone months of years before skeletal maturation was attained at the opposite epiphyseal end, which ossified in the typical pattern (i.e., formation of a secondary center de novo completely within the cartilaginous epiphysis). This process may also affect the development and appearance of ossification within the longitudinal epiphyseal bracket (delta phalanx).     

生长期儿童股骨干骨折髓内针内固定的远期观察

作者从1970～1985年收治生长期儿童股骨干骨折305例,其中切开复位髓内针内固定34例(38处股骨骨折),并进行了5～17年的长期随访观察,发现无1例出现术后早期并发症,12例(占33.3％)出现大粗隆发育受阻的晚期并发症。18例(22处股骨骨折)双下肢等长,13例患肢较健侧长0.8～1.4cm平均1.03cm,1例合并有下肢动脉损伤者出现下肢短缩。作者认为无论采用梅花型髓内针内固定,还是三棱型髓内针内固定都是可行的,但梅花型髓内针内固定有出现大粗隆发育受阻的危险。     

Juxta-articular hemangioma of long bone

We report on a rare case of an intraosseous hemangioma involving the proximal tibia in a 70-year-old man. Radiographically, the lesion was a well-defined osteolytic lesion with marginal sclerosis. The CT images demonstrated a well-defined osteolytic lesion with partial cortical breakthrough. T1-weighted MR images showed a hypointense lesion, while T2-weighted images revealed hyperintense areas, with internal, hypointense septa. Gadolinium-enhanced T1-weighted images showed lattice-like enhancement of the lesion. Received: 28 December 1999 Revision requested: 3 March 2000 Revision received: 23 May 2000 Accepted: 26 May 2000     

保留骨骺灭活再植术的实验与临床研究

目的探讨保留骨骺灭活再植术的可行性。方法在有效大剂量化疗保护下对2例儿童骨肉瘤患者行保留骨骺的灭活再植术。以18只新西兰大白兔为实验对象,行保留骨骺灭活再植术,于术后4、8、12周处死动物,行大体标本、影像学、病理组织学、扫描电镜观察及软骨各层厚度的测量。结果2例患者分别随访36个月和40个月,无复发转移,膝关节屈曲100°和110°。患肢较对侧短1.0cm和1.2cm。实验研究发现保留骨骺灭活再植术的灭活骨与保留的骨骺、灭活骨与截骨断端间术后8周即愈合;保留骨骺软骨钙化层的厚度随时间逐渐增厚,较正常薄;关节软骨面基本保持了正常的形态和结构。结论保留骨骺的灭活再植术为儿童骨肉瘤提供了一种新的保肢手术方法。     

Intraosseous leiomyosarcoma arising in the epiphysis of the distal femur

Herein, we present a rare case of intraosseous leiomyosarcoma arising in the epiphysis of the distal femur and showing unusual radiographic features. A 44-year-old man presented with a pain in the left knee joint. Computed tomography revealed an intraosseous lesion with slightly increased attenuation and a thin marginal sclerotic rim in the femoral medial condyle. The signal of the lesion was hypointense on T1-weighted magnetic resonance (MR) images and hyperintense on fat-suppressed T2-weighted MR images. After gadolinium administration, the signal of the lesion was moderately and diffusely enhanced. The histological diagnosis of leiomyosarcoma was made based on a preoperative core biopsy specimen. Microscopic examination of the resected specimen revealed an ill-defined intraosseous tumor composed of proliferated atypical and mildly pleomorphic smooth muscle cells permeating among the bone trabeculae with only focal destruction of the bone trabeculae and low mitotic activity, indicating low grade leiomyosarcoma. The bone trabeculae at the periphery of the tumor were mildly thickened and anastomosed with a rim of an increased number of osteoblasts. Systemic examination showed no tumorous lesions in other anatomical sites. Leiomyosarcomas rarely present in the bone as a diffuse intertrabecular growth, even in low grade tumors.     

儿童骨骺损伤

总结我院１９８０～１９８９年所收治的儿童骨骺损伤４６８例。随访时间１～８年。介绍了儿童骨骺损伤的Ｘ线分型、诊断要点及治疗方法。认为骨骺损伤的预后主要取决于原发损伤的类型，其次为受伤年龄和受伤部位，受伤年龄愈小或受伤部位为主要发育骨骺，将来所引起的继发畸形则愈大。     

Radiology of postnatal skeletal development

Thirty-one pairs of distal humeri were obtained from human cadavers ranging in age from full-term neonates to fourteen years. These were studied morphologically and roentgenographically. Specimen roentgenography using air/cartilage interfacing demonstrated both osseous and cartilaginous components of the epiphyses. These roentgenographic aspects of development are discussed and illustrated to provide a basic reference index.The supracondylar region is characterized by a fossa which initially is in both metaphysis and epiphysis, but migrates to the metaphysis completely within the first year. On either side of the fossa are osseous columns, which contrast with the broad metaphyseal bone above the columns. Within the fossa, anteriorly and posteriorly, are fat pads which may be elevated by intraarticular hematoma or reactive joint fluid. The physeal contour initially is transverse and smooth. Lappet formation progressively demarcates the epicondylar physeal regions, with the medial one becoming a functionally, but not histologically separate region.The capitellum is the first region to develop a secondary ossification center. This progressively expands into the trochlear portion of the epiphysis, a factor which predisposes to lateral condyle fracture propagation across the trochlear articular surface. The trochlea characteristically ossifies by multiple foci which fuse over time, often creating an irregular appearance to the developing ossification center. Epicondylar ossification tends to be from solitary foci. The lateral epicondylar center fuses with the capitellar center, whereas the medial epicondyle tends to be a functionally separate entity throughout development and does not normally fuse to the trochlear ossification center.     

Epiphyseal osteoblastoma-like osteosarcoma

Osteoblastoma-like osteosarcoma is a rare variant of osteosarcoma occurring in this instance in a highly unusual location: the lateral femoral condyle of a 13-year-old girl. The radiological features were non-aggressive and, although slightly unusual, were most suggestive of chondroblastoma.Presented at the Closed Meeting of the International Skeletal Society, Geneva, Switzerland, September 2003