eMedicine Specialties > Orthopedic Surgery > Spine

Klippel-Feil Syndrome: Treatment

Author: J Andy Sullivan, MD, Clinical Professor of Pediatric Orthopedics, Department of Orthopedic Surgery, University of Oklahoma College of Medicine
Contributor Information and Disclosures

Updated: Jun 23, 2009

Treatment

Medical Therapy

Medical therapy for Klippel-Fein syndrome depends on the congenital anomalies present in the syndrome. Primary care physicians may not be familiar with all of the possible associated anomalies. Patients with genitourinary abnormalities are referred to a nephrologist or urologist. Patients with cardiovascular abnormalities are cared for by a cardiologist or primary care physician. Patients with auditory abnormalities are referred to an audiologist or otologist.

Surgical Therapy

Surgery is indicated for a variety of situations in patients with Klippel-Feil syndrome. As a result of fusion anomalies and the difference in growth potential of the anomalous vertebral bodies, deformity may be progressive. Instability of the cervical spine can develop because of craniocervical abnormalities. Instability of the cervical spine can also develop between 2 sets of fusion anomalies separated by a normal segment. Neurologic deficits and persistent pain are indications for surgery. Development of a compensatory curve in the thoracic spine may require surgical intervention or bracing. Symptomatic spinal stenosis may require decompression and fusion.

Koop and colleagues studied 13 cases of skeletally immature children who had a variety of disorders causing instability of the upper part of the cervical spine, from the occiput to C5.31 They looked at the efficacy of posterior arthrodesis and halo-cast immobilization. Although many of the study's patients did not have Klippel-Feil syndrome, the surgical indication was instability. Posterior arthrodesis with external immobilization by halo cast was carried out; in 2 of the patients, internal fixation with wire was utilized. Autogenous bone grafts provided solid arthrodesis in 12 patients. One patient treated with a rib allograft developed a pseudoarthrosis.

The investigators cautioned that the use of wires for fixation carries a risk of neural injury and often is not applicable in children with anomalous vertebrae. They stressed the need for delicate exposure, decortication using an air drill, and placement of an autologous iliac graft. They recommended mobilization by halo cast, which they thought would minimize the risk of neural damage and provide a reliable means of obtaining arthrodesis.

Preoperative Details

Patients must have a comprehensive workup to detect the previously mentioned anomalies. Adequate imaging studies must be obtained. Three-dimensional CT scan reconstruction often is useful.

More on Klippel-Feil Syndrome

Overview: Klippel-Feil Syndrome
Workup: Klippel-Feil Syndrome
Treatment: Klippel-Feil Syndrome
Follow-up: Klippel-Feil Syndrome
Multimedia: Klippel-Feil Syndrome
References
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References

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Further Reading

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Keywords

Klippel-Feil syndrome, Klippel-Feil disease, Klippel Feil, low hairline, short neck, cervical spine disorder, synkinesia, Klippel Feil syndrome

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Contributor Information and Disclosures

Author

J Andy Sullivan, MD, Clinical Professor of Pediatric Orthopedics, Department of Orthopedic Surgery, University of Oklahoma College of Medicine
J Andy Sullivan, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Medical Association, American Orthopaedic Association, Oklahoma State Medical Association, and Pediatric Orthopaedic Society of North America
Disclosure: Nothing to disclose.

Medical Editor

K Daniel Riew, MD, Mildred B Simon Distinguished Professor of Orthopedic Surgery, Professor of Neurologic Surgery, Washington University School of Medicine; Chief, Cervical Spine Surgery, Department of Orthopedic Surgery, Barnes-Jewish Hospital
K Daniel Riew, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Orthopaedic Association, AO Foundation, Cervical Spine Research Society, North American Spine Society, and Scoliosis Research Society
Disclosure: Medtronic Grant/research funds None; Medtronic Royalty Medtronic Vertex; Biomet Royalty Maxan anterior cervical plate; Osprey Royalty Interbody Graft; Osprey Ownership interest Consulting; SpineMedica Consulting fee Consulting

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

William O Shaffer, MD, Professor, Vice-Chairman and Residency Program Director, Department of Orthopedic Surgery, University of Kentucky at Lexington
William O Shaffer, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Orthopaedic Association, International Society for the Study of the Lumbar Spine, Kentucky Medical Association, Kentucky Orthopaedic Society, North American Spine Society, Southern Medical Association, and Southern Orthopaedic Association
Disclosure: DePuySpine 1997-2007 (not presently) Royalty Consulting; DePuySpine 2002-2007 (closed) Grant/research funds SacroPelvic Instrumentation Biomechanical Study; DePuyBiologics 2005-2008 (closed) Grant/research funds Healos study just closed; No present Industry grants or funds. None None

CME Editor

Dinesh Patel, MD, FACS, Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital
Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Association of Physicians of Indian Origin, American College of International Physicians, and American College of Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Mary Ann E Keenan, MD, Professor, Vice Chair for Graduate Medical Education, Department of Orthopedic Surgery, University of Pennsylvania School of Medicine; Chief of Neuro-Orthopedics Program, Department of Orthopedic Surgery, Hospital of the University of Pennsylvania
Mary Ann E Keenan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Orthopaedic Foot and Ankle Society, American Society for Surgery of the Hand, and Orthopaedic Rehabilitation Association
Disclosure: Nothing to disclose.

 
 
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