Tarlov Cysts
Tarlov Cysts
NORD gratefully acknowledges Philip R.
Weinstein, MD, Professor, University of California, San Francisco, Department
of Neurological Surgery, for assistance in the preparation of this report.
- perineural cysts
- sacral, lumbar, thoracic or
cervical nerve root cysts
- No subdivisions found
Summary
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. Generally, the
larger a Tarlov cyst is, the more likely it is to cause symptoms. Symptoms
sometimes caused by Tarlov cysts include pain in the area served by the
affected nerves, numbness and altered sensation, an inability to control
bladder and bowel movements (incontinence), impotence, and, rarely, weakness in
the legs. Small, asymptomatic cysts can slowly increase in size eventually
causing symptoms. The exact cause of Tarlov cysts is unknown, but they may
occur due to variation in normal development of the nerve sheath. Tarlov cysts
were first described in the medical literature in 1938.
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 or
nerves contained within the cyst (radiculopathy). The specific symptoms and
their severity vary from one individual to another and with location along the
spinal column.
Chronic pain is a
common with symptomatic Tarlov cysts. Pain from lumbo-sacral cysts may affect
the lower back, especially below the waist, and spread to the buttocks and
legs. Pain may be worsened by walking (neurogenic claudication). Symptoms may
become progressively worse. In some individuals sitting or standing may worsen
pain; recumbency may relieve pain. In some cases, pain can also affect the
upper back, neck, arms and hands if the cysts are located in the upper spine.
Pain may worsen when coughing or sneezing. Affected individuals have also
reported vulvar, testicular, rectal, pelvic and abdominal pain.
Because Tarlov cysts
can affect the nerves, symptoms relating to loss of neurological function can
also develop including leg weakness, diminished reflexes, loss of sensation on
the skin, and changes in bowel or bladder function such as incontinence or
painful urination (dysuria). Some individuals may have difficulty empting the
bladder and constipation has also been reported. Changes in sexual function
such as impotence can also occur.
Affected individuals
may also develop abnormal burning or prickling sensations (paresthesias) or
numbness and decreased sensitivity (dysesthesia), especially in the legs or
feet. Tenderness or soreness may be present around the involved area of the
spine.
Additional symptoms have been reported in the
medical literature including chronic headaches, blurred vision, pressure behind
the eyes, dizziness, and dragging of the foot when walking due to weakness of
the muscles in the ankles and feet (foot drop). Some individuals demonstrate
progressive thinning (erosion) of the spinal bone overlying the cyst.
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
injures the nerve root sheath and causes leaking of cerebrospinal fluid (CSF)
into the area where a cyst forms. Some researchers believe that an abnormal
congenital connection (communication) exists between the subarachnoid space,
which contains cerebrospinal fluid, and the area surrounding the affected nerves
(perineural region). The connection may remain or eventually close, after
allowing cerebrospinal fluid to leak out and cause a cyst. Because Tarlov cysts
contain cerebrospinal fluid, researchers have speculated that normal
fluctuations in CSF pressure may lead to an increase in cyst 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.
Some reports suggest that individuals with connective tissue disorders are at a
greater risk of developing Tarlov cysts than the general population.
More research is necessary to understand the
underlying mechanisms that ultimately cause the development of Tarlov cysts or
the onset of their symptoms.
Women are at a higher risk of developing
Tarlov cysts than men. The exact incidence or prevalence of symptomatic Tarlov
cysts in the general population is unknown. 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.
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.)
Diagnosis
A diagnosis of Tarlov cysts may be suspected based upon a thorough clinical evaluation, a detailed patient history with identification of characteristic symptoms and a neurological examination. A diagnosis may be confirmed by a variety of specialized tests. In some cases, a diagnosis of a Tarlov cyst is made incidentally through x-ray or MRI scan investigation undertaken for other reasons.
A diagnosis of Tarlov cysts may be suspected based upon a thorough clinical evaluation, a detailed patient history with identification of characteristic symptoms and a neurological examination. A diagnosis may be confirmed by a variety of specialized tests. In some cases, a diagnosis of a Tarlov cyst is made incidentally through x-ray or MRI scan investigation undertaken for other reasons.
Clinical Testing and
Work-Up
Magnetic resonance imaging (MRI) of the lumbar region and computed tomography (CT) can 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.
Magnetic resonance imaging (MRI) of the lumbar region and computed tomography (CT) can 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.
Another test, known as a myelogram, uses a
special dye called contrast material and x-rays as well as a CT scan to create
a picture of the subarachnoid space. During this test, the contrast fluid is
injected under local anesthesia into the spinal canal through a thin needle.
The dye allows certain structures such as the nerve roots and spinal canal to
be seen more clearly on x-ray. The size and location of the connection between
the cyst and the normal spinal fluid containing space can be demonstrated by CT
scan performed after the myelogram. Erosion of the sacrum or vertebral bone by
the cyst can also be shown.
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. The response to various
therapeutic options is highly individualized; what works for one person may be
ineffective for another.
Certain drugs such as
corticosteroid injections may provide temporary relief of pain. Some reports
have indicated that epidural steroid injections can provide long-standing pain
relief (up to 6 months) before the procedure needs to be repeated.
Non-steroidal
anti-inflammatory drugs (NSAIDs) may be prescribed to treat nerve irritation
and inflammation. A procedure known as transcutaneous electrical nerve
stimulation or TENS may also be used to relieve nerve pain. During this
procedure, electrical impulses are sent through the skin to help control pain.
Tarlov cysts have been
treated by procedures in which cerebrospinal fluid is drained from the cyst
(aspiration). Results from such procedures vary and, in most cases, the cysts
eventually fill up with cerebrospinal fluid again. In some cases, symptoms can
return within hours.
Several different
procedures, both surgical and nonsurgical, have been used that involve draining
a Tarlov cyst and then filling the cyst with another substance such as fibrin
glue, fat, or muscle. This prevents cerebrospinal fluid from refilling the
cysts and reduces pressure on the surrounding nerves.
A nonsurgical
procedure used to treat individuals with symptomatic Tarlov cysts uses a
combination of substances that mimic blood clotting (fibrin glue). 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.
Some individuals have experienced immediate relief after this procedure; others
reported delayed benefit. This procedure has led to short-term and long-term
relief of symptoms in some cases. Complications have been reported in cases
where the cyst communicates readily with the spinal fluid containing space.
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 different success rates and side
effects. Surgical intervention depends upon numerous factors such as the
progression of the disorder; the degree of nerve root compression; the size of
the connection between the subarachnoid space and the cyst; an individual’s age
and general health; and/or other factors. 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 is needed.
Very large cysts may
require direct surgical intervention to drain and then obliterate the
cyst. One surgical technique that has been used to treat symptomatic
Tarlov cysts is an operation that exposes the region of the spine where the
cyst is located by removal of overlying vertebral bone. The cyst is then sliced
open with one or more thin cuts (fenestrations) and drained of fluid. The cyst
wall is collapsed, circumferentially reinforced and sutured closed or the
cavity is packed full of another substance such as fat or tissue adhesive to
prevent it from refilling with cerebrospinal fluid.
In another procedure,
after surgery to expose and drain the cysts, a flap of nearby muscle tissue is
used to fill the cyst in order to prevent recurrence. A muscle flap is a
portion of muscle that can be transferred along with its blood supply to an
adjacent part of the body. The muscle flap is use to fill the decompressed cyst
and to prevent it from refilling with cerebrospinal fluid. Results of treatment
may be disappointing if irreversible nerve damage has already occurred.
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:
For information about clinical trials
conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
https://www.clinicaltrialsregister.eu/
Tarlov Cysts Resources
Please note that some of these organizations
may provide information concerning certain conditions potentially associated
with this disorder.
- Tarlov
Cyst Disease Foundation
- 326 Norton Rd.
- Knoxville, TN 37920
- Phone: (865) 577-4945
- Email: retahoneyhiers@tarlovcystfoundation.org
- Website: http://www.tarlovcystfoundation.org
Other Organizations
- Genetic
and Rare Diseases (GARD) Information Center
- PO Box 8126
- Gaithersburg, MD 20898-8126
- Phone: (301) 251-4925
- Toll-free: (888) 205-2311
- Website: http://rarediseases.info.nih.gov/GARD/
- NIH/National
Institute of Neurological Disorders and Stroke
- P.O. Box 5801
- Bethesda, MD 20824
- Phone: (301) 496-5751
- Toll-free: (800) 352-9424
- Website: http://www.ninds.nih.gov/
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