Klippel-Feil Sequence Health Article

Treatment and management

If the individual has a very mild case of Klippel-Feil sequence, then the person can lead a normal life with only minor restrictions. These restrictions, such as avoiding contact sports that would place the neck at risk, are necessary because of the instability of the cervical spine. This is due to the increased motion between the fused cervical vertebrae.

Symptoms, such as pain, that occur with the arthritis and degeneration of the joints may also result. The individuals should be treated with pain medication and possible cervical traction. If neurological symptoms occur, the treatment of choice is fusion of the symptomatic area. However, due to the severe consequences of not having the preventive surgery, surgery is still the treatment most performed.

Prognosis

There have been reports of death following minor trauma because of injuries to the spinal cord in the cervical spine. Most commonly, individuals with Klippel-Feil will develop pain. Some diseases are acquired or occur because of the increased motion of the vertebrae. Degenerative disc disease, or destruction of the cushion like disc between the vertebrae, is also very common and affects the entire lower cervical spine. Spondylotic osteophytes, or bone spurs in the spine, form as a result of this degeneration. This laying down of new bone may lead to narrowing of the canal through which the spinal cord travels (spinal stenosis).

Because of the instability of the spinal cord, surgery may prevent a dangerous and fatal accident. Pain that originates in the neck and travels into the arms (radiculopathy) is common near the sites of the surgical fusion of vertebrae. One study found that 25% of the individuals who had surgery would have had neurological problems within ten years, therefore requiring additional surgery.

BOOKS

Guebert, Gary M., et al. "Congenital Anomalies and Normal Skeletal Variants." In Essentials of Skeletal Radiology, edited by Terry Yochum and Lindsay Rowe. 2nd ed. Baltimore: Williams & Wilkins, 1996.

Juhl J. H., A. B. Crummy, and J. E. Kuhlman, eds. Paul and Juhl's Essentials of Radiologic Imaging. 7th ed. Philadelphia: Lippencot-Raven, 1998.

PERIODICALS

Clarke, Raymond A., et al. "Familial Klippel-Feil Syndrome and Paracentric Inversion inv(8)(q22.2q23.3)." American Journal of Human Genetics 57 (1995): 1364–1370.

Clarke, Raymond A, et al. "Heterogenectiy in Klippel-Feil Syndrome: A New Classification." Pediatric Radiology 28 (1998): 967–974.

Hilibrand, A. S., et al. "Radiculopathy and Myelopathy at Segments Adjacent to the Site of a Previous Anterior Cervical Arthrodesis." Journal of Bone and Joint Surgery 81-A, no. 4 (1999): 519–528.

Nagashima, Hideki. "No Neurological Involvement for More Than 40 Years in Klippel-Feil Syndrome with Hypermobility of the Upper Cervical Spine." Archives of Orthopedic Trauma and Surgery 121 (2001): 99–101.

Thomsen, M. N., et al. "Scoliosis and Congenital Anomalies Associated with Klippel-Feil Syndrome Types I-Ill." Spine 22, no. 4 (1997): 396–401.

ORGANIZATIONS

National Institutes of Health (NIH). PO Box 5801, Bethesda, MD 20824. (800) 352-9424. nihinfo@Ood.nih.gov. <http://www.ninds.nih.gov/health>.

National Organization for Rare Disorders (NORD). PO Box 8923, New Fairfield, CT 06812-8923. (203) 746-6518 or (800) 999-6673. Fax: (203) 746-6481. <http://www.rarediseases.org>.

WEBSITES

KFS Circle of Friends support group. <http://www.fortunecity.com/millenium/bigears/99/kfs.html>.

KFS Connection Online, An online Klippel-Feil Support group. <http://members.aol.com/kfsconxpgs/links.htm>.

Jason S. Schliesser, D.C.