Korean J Spine.  2015 Sep;12(3):225-229. 10.14245/kjs.2015.12.3.225.

Two Cases of Klippel-Feil Syndrome with Cervical Myelopathy Successfully Treated by Simple Decompression without Fixation

• Kim JB
• Park SW
• Lee YS
• Nam TK
• Park YS
• Kim YB

• KMID: 2288358
• DOI: http://doi.org/10.14245/kjs.2015.12.3.225

Abstract

Klippel-Feil syndrome (KFS) is a congenital developmental disorder of cervical spine, showing short neck with restricted neck motion, low hairline, and high thoracic cage due to multilevel cervical fusion. Radiculopathy or myelopathy can be accompanied. There were 2 patients who were diagnosed as KFS with exhibited radiological and physical characteristics. Both patients had stenosis and cord compression at C1 level due to anterior displacement of C1 posterior arch secondary to kyphotic deformity of upper cervical spine, which has been usually indicative to craniocervical fixation. One patient was referred due to quadriparesis detected after surgery for aortic arch aneurysmal dilatation. The other patient was referred to us due to paraparesis and radiating pain in all extremities developed during gynecological examinations. Decompressive C1 laminectomy was done for one patient and additional suboccipital craniectomy for the other. No craniocervical fixation was done because there was no spinal instability. Motor power improved immediately after the operation in both patients. Motor functions and spinal stability were well preserved in both patients for 2 years. In KFS patients with myelopathy at the C1 level without C1-2 instability, a favorable outcome could be achieved by a simple decompression without spinal fixation.

Keyword

Klippel-Feil syndrome; Deformity; Cervical vertebrae; Spinal cord compression

MeSH Terms

Aneurysm
Aorta, Thoracic
Cervical Vertebrae
Congenital Abnormalities
Constriction, Pathologic
Decompression*
Dilatation
Extremities
Female
Gynecological Examination
Humans
Klippel-Feil Syndrome*
Laminectomy
Neck
Paraparesis
Quadriplegia
Radiculopathy
Spinal Cord Compression
Spinal Cord Diseases*
Spine