Syringomyelia

Definition

Syringomyelia is a cyst-like collection of cerebrospinal fluid (CSF) that forms in the spinal cord. Over time, it may damage the spinal cord.

Alternative Names

Syrinx

Causes

The fluid-filled cyst is called a syrinx. The spinal fluid buildup may be caused by:

The fluid-filled cyst usually begins in the neck area. It expands slowly, putting pressure on the spinal cord and slowly causing damage.

Onset of syringomyelia is usually between 25 to 40 years old. Males are more affected than females.

Symptoms

If the condition is due to birth defects, there may be no symptoms until age 30 to 40 years. Symptoms of syringomyelia usually appear slowly and worsen over many years. In the case of trauma, the onset of symptoms may be as early as 2 to 3 months of age. If there are symptoms, they may include:

Exams and Tests

Your health care provider will perform a physical exam and ask about the symptoms, focusing on the nervous system. Tests that may be done include:

Treatment

There is no known effective treatment for syringomyelia. The goals of treatment are to stop the spinal cord damage from getting worse and to improve function.

Medicines and devices can help with pain.

Surgery may be needed to relieve pressure in the spinal cord. Physical and occupational therapy may be needed to improve muscle function and help maximize independence.

Ventriculoperitoneal shunting or syringosubarachnoid shunting may be needed. This is a procedure in which a catheter (thin, flexible tube) is inserted to drain the fluid buildup.

Other treatments are available to manage bowel and bladder problems and spasticity.

Outlook (Prognosis)

Without treatment, the disorder may get worse very slowly. Over time, it may cause severe disability.

Surgery usually stops the condition from getting worse. Nervous system function will improve in about 30% of the people who have surgery.

Possible Complications

Without treatment, the condition may lead to:

Possible complications of surgery include:

When to Contact a Medical Professional

Contact your provider if you have symptoms of syringomyelia.

Prevention

There is no known way to prevent this condition, other than avoiding injuries to the spinal cord. Getting treated right away slows the worsening due to this disorder.

References

Alobeidi F, Thurnher MM, Jäger HR. Non-tumoural spinal cord lesions. In: Adam A, Dixon AK, Gillard JH, Schaefer-Prokop CM, eds. Grainger & Allison's Diagnostic Radiology. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 50.

Benglis DM, Jea A, Vanni S, Shah AH, Green BA. Syringomyelia. In: Garfin SR, Eismont FJ, Bell GR, Fischgrund JS, Bono CM, eds. Rothman-Simeone and Herkowitz's The Spine. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 94.

National Institute of Neurological Disorders and Stroke website. Syringomyelia. www.ninds.nih.gov/health-information/disorders/syringomyelia. Reviewed September 23, 2023. Accessed July 3, 2024.

Roguski M, Groves ML. Adult syringomyelia. In: Winn HR, ed. Youmans and Winn Neurological Surgery. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 334.



Review Date: 6/13/2024
Reviewed By: Joseph V. Campellone, MD, Department of Neurology, Cooper Medical School at Rowan University, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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