Syringomyelia
Syringomyelia is a chronic, often congenital, progressive disease of the spinal cord characterized by the development of fluid-filled cavities within the spinal cord substance.
The onset of the disease is manifested by a sensory defect arising first over the shoulders and back. Spasticity and weakness of the lower extremities may follow. Patients may experience vertigo, impairment of facial and lingual muscles, dysphagia, and other motor and sensory dysfunction. Thoracic kyphoscoliosis, often severe, occurs commonly as syringomyelia progresses.
In syringomyelia, as in a variety of other progressive neuromuscular diseases, pulmonary complications arise as a result of:
- restrictive lung disease secondary to spinal deformity and limited mobility;
- weakening of the respiratory muscles and cough; and
- aspiration of bacteria-laden oral secretions due to swallowing dysfunction.
The prevalence of restrictive lung disease, atelectasis, chronic aspiration, ineffective cough, and immobility affect the ability to clear secretions from the lungs.
To prevent or reduce the pulmonary complications associated with secretion retention, at-risk patients often require daily Airway Clearance Therapy. Without such therapy, impaired gas exchange, recurrent bacterial infections, progressive lung damage, and respiratory failure may result.
Sources
Fauci AS, Braunwald E, Isselbacher KJ, Wilson JD, et al., eds. Harrison’s Principles of Internal Medicine. 14th ed. New York: Mcgraw-Hill; 1998. 2387-2388. Small JA, Sheridan PH: Research priorities for syringomyelia: A National Institute of Neurologic Disorders and Stroke workshop summary. Neurology 1996; 46: 577.
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