Shimizu S, Kojima T, Morooka Y, Tanaka K, Nakagawa Y, Kuroki M
Department of Neurosurgery, Saiseikai Matsusaka General Hospital.
No Shinkei Geka. 1996 Oct;24(10):933-7.
The Klippel-Feil syndrome is one of the spinal malformations characterized by fusion of the cervical vertebrae. It is well known that the malformation can cause some neurologic disorders. However, an aneurysm in the vertebral artery associated with the Klippel-Feil syndrome is extremely rare, with only one case having been reported in the literature. We present a 39-year-old female with a sudden onset of disturbed consciousness. Lateral cervical x-ray films showed the Klippel-Feil syndrome and hypermobility between C1 and C2. MRI showed multiple infarctions in the posterior circulation, including bilateral thalami. Right vertebral angiogram identified the extracranial vertebral artery aneurysm as the source of the emboli. The patient gradually recovered with conservative therapy. The aneurysm was thought to be produced by chronic arterial trauma secondary to excessive movement between C1 and C2. We conclude that an extracranial vertebral artery aneurysm is a serious complication in a patient with the Klippel-Feil syndrome.
克-费综合征是一种以颈椎融合为特征的脊柱畸形。众所周知，这种畸形可导致一些神经功能障碍。然而，与克-费综合征相关的椎动脉动脉瘤极为罕见，文献中仅报道过1例。我们报告1例39岁突发意识障碍的女性患者。颈椎侧位X线片显示克-费综合征及C1和C2之间活动过度。MRI显示后循环多发梗死，包括双侧丘脑。右侧椎动脉血管造影确定颅外椎动脉动脉瘤为栓子来源。患者经保守治疗后逐渐康复。该动脉瘤被认为是由C1和C2之间过度活动继发的慢性动脉损伤所致。我们得出结论，颅外椎动脉动脉瘤是克-费综合征患者的一种严重并发症。
