eMedicine Specialties > Orthopedic Surgery > Spine

Neuromuscular Scoliosis

Author: Matthew B Dobbs, MD, Associate Professor, Department of Orthopaedic Surgery, Washington University School of Medicine
Coauthor(s): Lawrence G Lenke, MD, Jerome J Gilden Professor of Orthopedic Surgery, Section of Spinal Surgery, Director of Residency Program, Washington University School of Medicine; Chief of Spinal Surgery, Department of Orthopedic Surgery, St Louis Shriners Hospital
Contributor Information and Disclosures

Updated: Apr 24, 2008

Introduction

Scoliosis is a common deformity in many types of neuromuscular diseases. It is generally most severe in nonambulatory patients. Severe curves of the vertebral column cause difficulties in sitting. Bracing neuromuscular curves does not affect the natural history of scoliosis and is not definitive treatment. Progressive curves require surgical correction and stabilization.

For excellent patient education resources, visit eMedicine's Bone Health Center and Back, Ribs, Neck, and Head Center. Also, see eMedicine's patient education article Scoliosis.

History of the Procedure

Surgical stabilization constitutes the mainstay of treatment for neuromuscular scoliosis.

Problem

Neuromuscular scoliosis can be defined as a coronal and sagittal plane deformity of the spine in patients with abnormalities of the myoneural pathways of the body. In neuromuscular spinal deformities, progression occurs much more frequently than in idiopathic scoliosis.

In addition, progression often continues into adulthood. The long-term effects of the spinal deformity in patients with neuromuscular conditions can be disabling. Loss of the ability to sit occurs, as does an accompanying decrease in overall function. In addition, pulmonary function is markedly affected.

Frequency

Because neuromuscular scoliosis has so many causes, the patterns and incidence vary greatly. However, the prevalence of spinal deformity in the patient with a neuromuscular disorder is much higher than in the general population. It ranges from  20% in children with cerebral palsy to 60% in patients with myelodysplasia. The prevalence rises to 90% in males with Duchenne muscular dystrophy. In general, the greater the neuromuscular involvement, the greater the likelihood and severity of scoliosis.

Etiology

Scoliosis associated with neuromuscular disorders has been classified by the Scoliosis Research Society into neuropathic and myopathic types.

The neuropathic conditions have been subdivided into those with upper and lower motor neuron lesions. The group with upper motor neuron lesions includes diseases such as cerebral palsy, syringomyelia, and spinal cord trauma; the group with lower motor neuron lesions includes poliomyelitis and spinal muscular atrophy. The myopathic conditions include arthrogryposis, muscular dystrophy, and other forms of myopathy.

Pathophysiology

The pathophysiology is not well understood. It seems logical to assume that scoliosis in these conditions is caused by muscle weakness, but this conclusion is difficult to support because some conditions are accompanied by spasticity and others by flaccidity. Furthermore, no consistent pattern of scoliosis is associated with a particular pattern of weakness.

Presentation

The evaluation of a patient with neuromuscular scoliosis entails a thorough assessment of all body systems. Accurate diagnosis of the underlying disease entity is essential and may require muscle biopsy.

Assessing nutritional status and pulmonary function is extremely important. The child's caregivers should be interviewed to gain an appreciation of the patient's functional level. The orthopedic examination includes assessment of all extremities and joints for contractures. Spinal deformity, decompensation, and shoulder balance are documented. Ambulatory status is also evaluated, and patients are classified as walkers, sitters, or nonsitters.

Indications

The 2 main indications for surgery are curve progression and deterioration in sitting ability.

Relevant Anatomy

Understanding the anatomy of the spine is crucial for safe and efficient exposure with a posterior approach. The incision is made from the spinous process above the most proximal vertebra to be instrumented to the most caudal extent of the proposed instrumented area. Identifying and staying in the midline is important so that muscle is not cut, which would lead to bleeding. The midline is identified by a thin line, which is actually the interspinous ligaments connecting the spinous processes.

Each vertebral level is exposed in a similar manner. An elevator is used to pull the soft tissue off of the spinous process, lamina, and transverse process of each respective level. To minimize blood loss, expose each segment completely the first time; do not leave soft tissue on the bone that will have to be removed later.

Contraindications

A preoperative assessment of respiratory competency, cardiac status, nutrition, possible feeding difficulties, seizure disorders, urologic status, and metabolic bone disease is necessary to ensure that the patient is healthy enough to tolerate surgery.

More on Neuromuscular Scoliosis

Overview: Neuromuscular Scoliosis
Workup: Neuromuscular Scoliosis
Treatment: Neuromuscular Scoliosis
Follow-up: Neuromuscular Scoliosis
Multimedia: Neuromuscular Scoliosis
References
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References

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  2. Bridwell KH, O''Brien MF, Lenke LG, et al. Posterior spinal fusion supplemented with only allograft bone in paralytic scoliosis. Does it work?. Spine. Dec 1 1994;19(23):2658-66. [Medline].

  3. Brook PD, Kennedy JD, Stern LM, et al. Spinal fusion in Duchenne''s muscular dystrophy. J Pediatr Orthop. May-Jun 1996;16(3):324-31. [Medline].

  4. Gersoff WK, Renshaw TS. The treatment of scoliosis in cerebral palsy by posterior spinal fusion with Luque-rod segmental instrumentation. J Bone Joint Surg Am. Jan 1988;70(1):41-4. [Medline].

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Further Reading

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Contributor Information and Disclosures

Author

Matthew B Dobbs, MD, Associate Professor, Department of Orthopaedic Surgery, Washington University School of Medicine
Matthew B Dobbs, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Orthopaedic Surgeons, and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Lawrence G Lenke, MD, Jerome J Gilden Professor of Orthopedic Surgery, Section of Spinal Surgery, Director of Residency Program, Washington University School of Medicine; Chief of Spinal Surgery, Department of Orthopedic Surgery, St Louis Shriners Hospital
Lawrence G Lenke, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Medical Association, American Orthopaedic Association, American Spinal Injury Association, Missouri State Medical Association, North American Spine Society, and Scoliosis Research Society
Disclosure: Nothing to disclose.

Medical Editor

Lee H Riley III, MD, Chief, Division of Orthopedic Spine Surgery, Assistant Professor, Departments of Orthopedic Surgery and Neurosurgery, Johns Hopkins University
Disclosure: Nothing to disclose.

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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