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患者女,19岁.自幼耳聋,颈项短缩,近年来左侧颈.背及上肢酸痛,家族中无类似病症.检查:身材矮小,瘦弱;颈部短小,略斜向左侧,颈部活动严重受限;屈曲不足20°,伸展约20°,左、右侧屈都不足15°,左右旋转各约25°;活动时无疼痛,呼吸及吞咽动作正常;发际低,接近颈部;左侧臂丛神经牵拉试验阳性,颈椎椎间孔挤压试验左侧阳性,左上肢有神经根感觉传导障碍,大、小鱼际较对侧萎缩,肌力Ⅲ级;胸部摄片:心脏大血管外形及胸廓未见异常改变;静脉肾盂造影泌尿系未见异常.实验室检查无异常.X线检查:颈椎侧位片见C_(2～7)互相连合成一个椎块,椎间隙消失,有骨小梁通过相邻的椎骨,椎体变形缩小,前后径尤甚,附件也互相连合,棘突小,椎间孔的边缘光滑清楚,较正常小且近于卵圆形,颈椎向后弯凸(附图).正位片见颈椎向右弯凸.诊断:Klipple-Feil综合征.
Patient female, 19 years old. Deafness, neck shortening, left neck in recent years. Back and upper limb pain, no similar family disease. Check: short stature, thin neck, slightly oblique left, neck Activities are severely limited; flexion less than 20 °, stretching about 20 °, left and right flexor less than 15 °, about 25 ° rotation around; activity without pain, breathing and swallowing normal; hairline low, close to the neck ; Left brachial plexus traction test positive cervical left intervertebral extrusion test positive left upper extremity nerve root sensory conduction disorder, large and small fish than the contralateral contractions, muscle strength class III; chest radiography: X-ray examination: lateral cervical spine see C_ (2 ~ 7) connected to each other into a vertebral block, the intervertebral space Disappear, there trabecular bone through the adjacent vertebrae, vertebral body deformation narrowing, especially before and after the anteroposterior diameter, attachment also interlinked, spinous process is small, the edge of the intervertebral fossa smooth and clear, smaller than normal and near the oval, Cervical backward convex (with photos.) Orthographic films see cervical spine to the right. Diagnosis: Klipple-Feil syndrome.