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Tarlov Cysts


National Organization for Rare Disorders, Inc.

Synonyms

  • Perineural Cysts
  • Sacral Nerve Root Cysts

Disorder Subdivisions

  • None

Related Disorders List

Information on the following diseases can be found in the Related Disorders section of this report:

  • Arachnoid Cysts

General Discussion

Tarlov cysts are fluid-filled sacs that affect the nerve roots of the spine, especially near the base of the spine (sacral region). Individuals may be affected by multiple cysts of varying size. In most cases, Tarlov cysts do not cause symptoms (asymptomatic). However, symptoms can occur depending upon the size and specific location of the cyst. Symptoms sometimes caused by Tarlov cysts include pain, an inability to control bladder and bowel movements (incontinence), and weakness in the legs. Small, asymptomatic cysts can slowly increase in size eventually causing symptoms. The exact cause of Tarlov cysts is unknown. Tarlov cysts were first described in the medical literature in 1938.

Symptoms

Many cases of Tarlov cysts are not associated with symptoms (asymptomatic). However, Tarlov cysts can grow in size eventually compressing or damaging adjacent nerve roots (radiculopathy). The specific symptoms and their severity vary from case to case.

Symptoms that may be associated with Tarlov cysts include pain, leg weakness, sexual dysfunction such as impotence, and an inability to control bowel or bladder movements (incontinence) or painful urination (dysuria). Pain may affect the back and spread to the buttocks and legs (sciatica). Pain may be worsened by walking (neurogenic claudication). Symptoms may become progressively worse. In some individuals sitting or standing may worsen pain; in others sitting or standing may lessen pain. Additional symptoms that may be associated with Tarlov cysts include abnormal burning or prickling sensations (paresthesia) or numbness or decreased sensitivity (dysesthesia), especially in the legs or feet.

Causes

The exact cause of Tarlov cysts is unknown. Several theories exist including that the cysts result from an inflammatory process within the nerve root sheath or that trauma causes leaking of cerebrospinal fluid (CSF) into the area where a cyst forms. Some researchers believe that an abnormal connection exists between the subarachnoid space, which contains cerebrospinal fluid, and the area surrounding the affected nerves (perineural region). The connection eventually closes, but allows cerebrospinal fluid to leak out and cause a cyst. Because Tarlov cysts contain cerebrospinal fluid, researchers have speculated that increased CSF pressure may lead to an increase in tumor size and a greater likelihood of developing symptoms.

In many cases, individuals with asymptomatic Tarlov cysts developed symptoms following trauma or activities that raise cerebrospinal fluid pressure such as heavy lifting.

Affected Populations

Women are at a higher risk of developing Tarlov cysts than men. Fewer than 100 cases of symptomatic Tarlov cysts have been reported in the medical literature. Because these cysts often go unrecognized or misdiagnosed, determining their true frequency in the general population is difficult. However, the total number of Tarlov cyst patients (symptomatic and asymptomatic) is estimated at 4.6 to 9 percent of the adult population.

Related Disorders

Symptoms of the following disorders can be similar to those of Tarlov cysts. Comparison of symptoms may be useful for a differential diagnosis.

Various cysts and tumors may have similar symptoms to those associated with Tarlov cysts. This group includes meningeal diverticula, meningoceles, neurofibromas, schwannoma, and arachnoid cysts. These cysts and tumors may cause compression of the spinal cord or nerve roots. (For more information on these conditions, choose the specific cyst or tumor name in the Rare Disease Database.)

Arachnoid cysts are fluid-filled sacs that occur on the arachnoid membrane that covers the brain (intracranial) and the spinal cord (spinal). There are three membranes covering these components of the central nervous system: dura mater, arachnoid, and pia mater. Arachnoid cysts appear on the arachnoid membrane, and they may also expand into the space between the pia mater and arachnoid membranes (subarachnoid space). The most common locations for intracranial arachnoid cysts are near the temporal lobe (the middle fossa), near the third ventricle (the suprasellar region) and the area that contains the cerebellum, pons, and medulla oblongata (the posterior fossa). In many cases, arachnoid cysts do not cause symptoms (asymptomatic). In cases in which symptoms occur, headaches, seizures and abnormal accumulation of excessive cerebrospinal fluid in the brain (hydrocephalus) are common. The exact cause of arachnoid cysts is unknown. (For more information on this disorder, choose "arachnoid cysts" as your search term in the Rare Disease Database.)

Standard Therapies

Diagnosis
A diagnosis of Tarlov cysts is often made incidentally through x-ray investigation for other reasons. Magnetic resonance imaging of the lumbar region and computed tomography both reveal Tarlov cysts. During MRI, a magnetic field and radio waves are used to create cross-sectional images of the organ being studied. During CT scanning, a computer and x-rays are used to create a film showing cross-sectional images of the organ's tissue structure.

Treatment
Tarlov cysts that do not cause symptoms should be monitored periodically to see whether cysts increase in size or whether symptoms develop. There is no specific, accepted therapy for individuals with symptomatic Tarlov cysts. Treatment is directed toward the specific symptoms that are apparent in each individual and may include drugs, surgery and other techniques.

Certain drugs such as corticosteroid injections may provide temporary relief of pain. Tarlov cysts have been treated by procedures in which cerebrospinal fluid is drained from the cyst. Results from such procedures vary and, in most cases, the cysts eventually fill up with cerebrospinal fluid again.

Surgical removal of Tarlov cysts may be used to treat symptomatic individuals who do not respond to other forms of therapy. Debate exists in the medical literature as to the most appropriate surgical technique to treat individuals with symptomatic Tarlov cysts. Various techniques have been used with varying success rates and side effects. Surgical intervention depends upon numerous factors such as the progression of the disorder, the degree of nerve root compression, an individual's age and general health; and/or other elements. Decisions concerning the use of particular interventions should be made by physicians and other members of the health care team in careful consultation with the patient, based upon the specifics of his or her case; a thorough discussion of the potential benefits and risks; patient preference; and other appropriate factors.

Investigational Therapies

A procedure that uses a combination of substances that mimic blood clotting (fibrin glue) has been used to treat individuals with Tarlov cysts. Fibrin glue injection is a minimally invasive procedure that has benefited some individuals with symptomatic Tarlov cysts. After the cysts are drained, fibrin glue is used to seal or "glue" the cyst closed preventing the cysts from filling up again. More research is necessary to determine the long-term safety and effectiveness of this potential therapy.

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

References

JOURNAL ARTICLES
Tanaka M, Nakahara S, Ito Y, et al. Surgical results of sacral perineural (Tarlov) cysts. Acta Med. 2006;60:65-70.

Hefti M, Landolt H. Presacral mass consisting of a meningocele and a Tarlov cyst: successful surgical treatment based on pathogenic hypothesis. Acta Neurochir (Wien). 2006;148:479-83.

Langdown AJ, Grundy JR, Birch NC. The clinical relevance of Tarlov cysts. J Spinal Disord Tech. 2005;18:29-33.

Acosta FL Jr. Quinones-Hinojosa A, Schmidt MH, Weinstein PR. Diagnosis and management of sacral Tarlov cysts. Case report and review of the literature. Neurosurg Focus. 2003;15:1-7.

Voyadzis JM, Bhargava P, Henderson FC. Tarlov cysts: a study of 10 cases with review of the literature. J Neurosurg. 2001;95:25-32.

Paulsen RD, Call GA, Murtagh FR. Prevalence and percutaneous drainage of cysts of the sacral nerve root sheath (Tarlov cysts). AJNR Am J Neuroradiol. 1994;15:293-7.

Tarlov IM. Perineurial cysts of the spinal nerve roots. Arch Neurol Psychiatry. 1938;40:1067-74.

FROM THE INTERNET
National Institute of Neurological Disorders and Stroke. Tarlov Cysts Information Page. February 7, 2006. Available at: http://www.ninds.nih.gov/disorders/tarlov_cysts/tarlov_cysts.htm Accessed On: April 12, 2006.

Resources

National Institute of Neurological Disorders and Stroke (NINDS)
31 Center Drive
8A07
Bethesda, MD 20892-2540
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
Email: braininfo@ninds.nih.gov
Internet: http://www.ninds.nih.gov/

American Association of Neurological Surgeons
5550 Meadowbrook Drive
Rolling Meadows, IL 60008-3852
Tel: (847)378-0500
Fax: (847)378-0600
Tel: (888)566-2267
Email: info@aans.org
Internet: http://www.NeurosurgeryToday.org and http://www.AANS.org

Tarlov Cyst Disease Foundation
326 Norton Rd.
Knoxville, TN 37920
Tel: (865)577-4945
Fax: (865)577-4835
Email: contactus@tarlovcystfoundation.org
Internet: http://www.tarlovcystfoundation.org

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). CIGNA members can access the complete report by logging into myCIGNA.com. For non-CIGNA members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  4/11/2008
Copyright  2006 National Organization for Rare Disorders, Inc.



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