J Korean Neurosurg Soc.  2004 Jan;35(1):116-118.

Surgical Treatment of Klippel-Feil Syndrome with Cervical Spondylosis

• Park KS
• Lee KS
• Kim YS
• Kuh SU

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.

Keyword

Klippel-Feil syndrome; Transoral approach; Congenital anomaly; Spine disease

MeSH Terms

Adult
Constriction, Pathologic
Cranial Fossa, Posterior
Durapatite
Facial Paralysis
Hearing Loss, Sensorineural
Humans
Klippel-Feil Syndrome*
Platybasia
Quadriplegia
Ribs
Spondylosis*
Transplants
Durapatite