Vascular changes in the periosteum of congenital pseudarthrosis of the tibia
Vascular changes in the periosteum of congenital pseudarthrosis of the tibia
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DOI：
10.1016/j.prp.2004.09.013
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发表时间：
2005-01-01
影响因子：
2.8
通讯作者：
Weber, M
中科院分区：
文献类型：
--
作者：
Hermanns-Sachweh, B;Senderek, J;Weber, M
先天性胫骨假关节（CPT）的病因和发病机制至今仍不清楚。受影响的胫骨表现出机械强度和成骨能力不足。CPT经常与1型神经纤维瘤病（NF 1; von Recklinghausen病）相关;然而，这两种疾病尚未在发病机制上联系起来。本研究提出了CPT的病理形态学研究结果下，特别考虑NF 1。因此，通过光学显微镜、免疫组织化学和电子显微镜研究了CPT（n = 4）伴（n = 3）和不伴（n = 1）神经纤维瘤病患者的样本。所有患者中最显著的发现是骨膜增厚，神经细胞聚集在小动脉周围，导致次全或完全闭塞。总之，血管化受损可导致成骨能力下降。神经纤维瘤病患者异常骨膜和皮肤神经纤维瘤超微结构的相似性可能表明这两种疾病的发病相关性。(c)2004年Elsevier GmbH。All rights reserved.
The etiology and the pathogenesis of congenital pseudarthrosis of the tibia (CPT) are still unknown. The affected tibia exhibits insufficient mechanical strength and osteogenetic capability. CPT is frequently associated with neurofibromatosis type 1 (NF1; von Recklinghausen's disease); however, both diseases have not yet been linked pathogenetically. This study presents the pathomorphologic findings of CPT under special consideration of NF1. Therefore, samples from patients operated on for CPT (n = 4) with (n = 3) and without (n = 1) neurofibromatosis were investigated by light microscopy, immunohistochemistry, and electron microscopy. The most striking finding in all patients was thickened periosteum with accumulation of nerval cells surrounding small arteries, causing subtotal or complete obliteration.In conclusion, impaired vascularization can result in decreased osteogenic capabilities. The similarity of ultrastructural findings in the abnormal periosteum and in skin neurofibromas of neurofibromatosis patients may indicate a pathogenetic association of both diseases. (c) 2004 Elsevier GmbH. All rights reserved.