Surgical Treatment of Klippel-Feil Syndrome with Cervical Spondylosis

ABSTRACT

We report a case of Klippel-Feil syndrome. The patient was a 37-year-old man who developed progressive motor weakness and on C4-5 and C6-7 segments combined with severe cervical stenosis, basilar impression and C1 assimilation. He showed progressive quadriparesis and respiratory difficulty. He had combined congenital anomaly of right side facial palsy of peripheral type and right side sensorineural hearing loss. In May 2002, we performed, via transoral approach, anterior fusion with Harm's mesh and hydroxyapatite from the clivus to the C3. But, one month after the operation, Harm's mesh with hydroxyapatite slipped anteriorly due to non-union. So, we removed the mesh and reoperated with fibular bone graft from the clivus to the C3. Simultaneously, posterior approach was performed with on-lay autologous rib bone graft and wiring from the occiput to the C2, 3, 4. One year after the operation, his motor weakness has been gradually improved and there is minimal difficulty in self-respiration.