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先天性胫骨假关节(congenital pseudarthrosis of the tibia, CPT)是由于发育异常所致的胫骨畸形，表现为胫骨成角畸形、囊肿或髓腔狭窄等，最终形成不能愈合的假关节。CPT是一种罕见的疾病，其发病率约占新生儿的1/140 000～1/250 000，主要特点是进行性胫骨和腓骨向前外侧弯曲、病理性骨折、进而发展成胫骨和/或腓骨假关节。该病常与1型神经纤维瘤病(neurofibromatosis type 1, NFn 1)有关，是小儿骨科最难治疗的疾病之一。CPT的病理及发病机制目前尚不完全清楚，存在多种热点研究方向，本文现就病理及发病机制这两方面的研究进展做一综述。n “,”As a deformity of the tibia caused by abnormal development, congenital pseudarthrosis of the tibia (CPT) is characterized by angular deformity of the tibia, cyst or stenosis of medullary cavity and eventually a false non-healing joint. It is a rare disease with an incidence of approximately 1/140, 000 to 1/250, 000 among neonates. There are progressive anterolateral curvature of the tibia and fibula, pathological fractures and subsequent development of the tibia and/or fibula pseudarthrosis. Often associated with neurofibromatosis type 1 (NFn 1), it is one of the most difficult pediatric orthopedic diseases to treat. Currently the pathology and pathogenesis of CPT are incompletely elucidated. With a large multitude of hot research directions, this review summarized recent advances of pathology and pathogenesis.n