Klippel-Feil症候群に合併した頭蓋外椎骨解離性動脈瘤の1例

Extracranial vertebral artery aneurysm complicating Klippel-Feil syndrome:case report

I．はじめに

　Klippel-Feil症候群は2つまたはそれ以上の頸椎の分節障害による奇形であるが，単に1つまたは2つの椎間が癒合したものでは症状を呈さないものが多い．しかし今回われわれは椎骨癒合上位での過剰運動部位で椎骨動脈損傷を生じ外傷性解離性動脈瘤を併発したと考えられるKlippel-Feil症候群の1例を経験したので報告する．

The Klippel-Feil syndrome is one of the spinal mal-formations characterized by fusion of the cervical ver-tebrae. It is well known that the malformation can cause some neurologic disorders. However, an aneu-rysm in the vetebral 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 ther-apy. The aneurysm was thought to be produced by chronic arterial trauma secondary to excessive move-ment between C1 and C2. We conclude that an ex-tracranial vertebral artery aneurysm is a serious com-plication in a patient with the Klippel-Feil syndrome.