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Klippel-Feil综合征影像学分型指导手术治疗的研究进展 被引量:1

Advances in imaging staging of Klippel-Feil syndrome for surgical treatment
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摘要 先天性颈椎融合综合征或先天性短颈综合征,即Klippel-Feil综合征(Klippel-Feil syndrome,KFS),1912年由法国神经学家Maurice Klippel和AndréFeil首次发现并描述。其典型特征是先天性的两个及以上颈椎节段异常融合或分隔不全,这种先天性的颈椎融合发生在妊娠第3~8周,由胚胎异常发育导致。全球范围内的新生儿发病率据估计为1/40000~1/42000(女性病例占60%)。Klippel-Feil综合征的典型临床“三联征”包括短颈、颈后发际线低以及颈部活动受限,但仅不足50%的KFS患者同时表现出上述“三联征”;而且KFS患者除两个及以上颈椎节段异常融合外,同时可伴有多种其他相关畸形。尽管KFS临床表现和相关畸形的范围很多,从无症状的单节段下颈椎融合患者到多节段颈椎融合、严重的先天性脊柱侧凸和多器官异常患者均可能出现,但是很大一部分患者直到外伤需要颈椎的影像学检查时才发现患有KFS。
作者 丁林垚 周非非 DING Linyao;ZHOU Feifei
出处 《中国脊柱脊髓杂志》 CAS CSCD 北大核心 2022年第6期564-568,共5页 Chinese Journal of Spine and Spinal Cord
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