Tethered Cord Syndrome

NORD gratefully acknowledges Shokei Yamada, MD, PhD, FACS, Professor and Former Chairman,
Department of Neurosurgery, Loma Linda University, for assistance in the preparation of this report.

Synonyms of Tethered Cord Syndrome

TCS
tethered spinal cord syndrome

General Discussion
Tethered cord syndrome is a stretch-induced functional disorder associated with
the fixation (tethering) effect of inelastic tissue on the caudal spinal cord,
limiting its movement. This abnormal attachment is associated with progressive
stretching and increased tension of the spinal cord as a child ages, potentially
resulting in a variety of neurological and other symptoms. Due to the variation of the
growth rate of the spinal cord and the spinal column, the progression of
neurological signs and symptoms is highly variable. Some individuals present with
tethered cord syndrome at birth (so-called congenital), while others develop the
symptomatology in infancy or early childhood. Other individuals may not develop any
noticeable symptoms until adulthood. Although some authors call these cases
acquired, the majority of these cases are mostly developmental, corresponding to
the progressive development of excess fibrous connective tissue (fibrosis) in the
filum terminale. The filum terminale is a strand of tissue that bridges the spinal cord
tip and the tailbone (sacrum). The inelastic structures in children originated from
defective closure of the neural tube (the precursor of the spinal cord) during
embryonic development, eventually forming a condition known as spina bifida.
Because of its functional (physiological) nature, tethered cord syndrome can be
reversible if surgically treated in its early stage.

Signs & Symptoms

The specific symptoms, severity and progression of tethered cord syndrome vary
from one individual to another. In most cases, individuals experience symptoms
during childhood. In some cases, symptoms are stabilized in childhood, but become
apparent only in adulthood.
A high percentage of pediatric cases, with tethered cord syndrome show cutaneous
tufts of hair, skin tags, dimples, benign fatty tumors, skin discoloration or
hemangiomas. Additional symptoms include lower back pain that worsens with
activity and improves with rest (although rarely complained by young children,
because of inability to express pain), leg pain or numbness, difficulty walking (gait
disturbances), foot and spinal deformities, such as abnormal side-to-side curvature
of the spine (scoliosis) or hollow lowback (exaggerated lordosis), high-arched feet
and hammertoes, and less commonly difference in leg strength. Tethered cord
syndrome can also cause difficulties with bladder and bowel control. Affected
children may experience involuntary urination or defecation (incontinence) and
repeated urinary tract infections. Symptoms in children may be slowly progressive.
Adult onset of tethered cord syndrome was considered to be rare for many years, but
an increasing number of cases have been reported in recent years. This trend is due
to improvement in neurological examinations and in the interpretation of imaging
studies. Symptoms common to adult tethered cord syndrome include constant, often
severe back and leg pain, which may extend to the rectum and genital area in some
cases. Progressive sensory and motor deficits may affect the legs potentially
resulting in numbness, weakness or muscle wasting (atrophy) in the affected areas.
More than 50 percent of the affected individuals experience bladder and bowel
dysfunction, manifested by increased frequency or urgency of urination or
constipation. In some cases, a fluid-filled cavity (syrinx) is found, sometimes
associated with typical signs and symptoms syringomyelia, such as burning pain in
the analgesic (painless on examination) area, decreased motor function and loss of
muscle mass, or occasional headaches,

Causes
Tethered cord syndrome can be of a congenital (primary) origin or acquired
(secondary or developmental).
Various congenital anomalies, particularly spina bifida, are often associated with
congenital tethered cord syndrome. Spina bifida is a birth defect due to incomplete
closure of the posterior spinal cord and bony vertebral arch (lamina). Many cases
with this anomaly leave a portion of the spinal cord protruded through the spinal
canal, typically forming a myelomeningocele. Such birth defects, if located in the tail
(caudal) end of the spinal cord, can cause tethered cord syndrome. In others, where
the anomalous structure is attached to the wide area of the spinal cord, signs and
symptoms reflect local effects on the spinal cord, and not stretched-induced
dysfunction (tethered cord syndrome).
Types of spina bifida associated with tethered cord syndrome include an abnormal
connection of inelastic tissue to the caudal spinal cord, dermal sinus tract, which
extends from the intraspinal connective tissue to the skin (dermal sinus tract), a split
spinal cord (diastematomyelia), and a benign fatty mass or tumor (lipoma)
continuous to the spinal cord. The other fatty anomaly is a lipomyelomeningocele, in
which a lipoma extrudes from the spinal canal underneath the lining of the spinal
cord (meninges), but covered by normal skin.
In many individuals, tethered cord syndrome is caused mechanically by an inelastic
often-thickened filum terminale. This structure, which is composed of glial tissue (the
supportive structure of nerve cells) and covered by pia mater, is a delicate strand of
fibrous tissue, bridging the spinal cord tip and the sacrum (the tailbone). Due to its
high viscoelasticity, the filum allows movement of the spinal cord. If abnormal fibrous
tissue grows into the filum and replaces glial tissue, the filum loses its elasticity and
abnormally fixes (tethers) the spinal cord, and becomes the mechanical cause of
tethered cord syndrome. The inelastic filum is commonly thickened in children, but
found less frequently in adolescents and adults.
Genetic factors are involved in development of anomalous caudal spine and spinal
cord, e.g. myelomeningocele, and in some cases of lipomyelomeningocele. Since
tethered cord syndrome is a physiological disorder and develops only when it is
abnormally stretched, it cannot be connected to genetic factors, unless the
congenital susceptibility of spinal cord to oxidative metabolic impairment is proven.
Secondary causes of tethered cord syndrome include tumors, infection or the
development of scar tissue (fibrosis) connected to the spinal cord. Tethered cord
syndrome may develop as a complication of spinal surgery. Trauma to the spine
results in a band of scar formation attached to the spinal cord and can cause
tethered cord syndrome. However, some researchers believe that trauma alone is
not enough to cause the disorder. They propose that tethering and abnormal tension
were already present before the trauma, which worsened the condition.
Some researchers have speculated that some cases of tethered cord syndrome that
occur due to anomalies that can cause stretching of the spinal cord may have a
genetic basis or that some individuals are genetically predisposed to developing the
disorder in these specific cases. Although genetic factors are found in patients with
myelominingocele, more research is necessary to determine the exact role that
genetic factors play in the development of stretch-causing anomalies.
Pathophysiologically, neuronal dysfunction in tethered cord syndrome results partly
from inability for the spinal cord neurons to utilize oxygen, that is, the impaired
oxidative metabolism, partly due to lack of oxygen supply (ischemic effect), and
partly to ion channel dysfunction directly related neuronal membrane stretching. The
spinal cord consists of a long bundle of neuronal fibers (axons) and the interneurons
that connect sensory and motor fibers within the cord. During gestation, the spinal
cord is continuous to the brain and runs in the spinal canal to the tailbone area. In
general, the spinal cord is protected from external insult by two mechanisms; 1)
encased in the spinal column, that is, a rigid structure, 2) floating free in the spinal
fluid space of the spinal canal. In addition, the spinal cord is continuous to the filum
terminale, which is extremely extensible because of its high viscoelasticity. If the
spinal cord is tethered at its caudal end, and if the spinal cord is unable to grow as
fast as the vertebral column in childhood, the spinal cord is stretched beyond its
physiological tolerance. In turn, this causes various metabolic abnormalities in the
spinal cord and, ultimately, the various neurological symptoms of this disorder.
Normally, the spinal cord ascends in the spinal canal as the spinal column starts to
grow faster than the spinal cord at 9th weeks of gestation. Consequently, the spinal
cord is pulled upwards due to this growth difference. By three months of age, the tip
of the spinal cord reaches the normal level between T12 and L2 vertebrae. An
elastic, extremely extensible filum allows for the ascension of the less elastic spinal
cord. If the filum becomes inelastic in an embryo, then the spinal cord tip is anchored
and ceases to ascend. Compensatory to the stretching force, the lower
(lumbosacral) spinal cord naturally grows more than seen in normal subjects, and
becomes elongated. Associated with tethered cord syndrome, the elongated cord is
often noted in children, but less often in adults.
In most cases, the abnormal tension of the spinal cord increases over time, but
disturbing symptoms often develop quickly during a few weeks. Certain activities
such as flexing or extending the lower spinal column can put additional tension on
the spinal cord and often worsen tethered cord syndrome. Participation in physical
activities such as strenuous sports and ballet dancing with high kicks can worsen the
signs and symptoms. Special physical features such as abnormal curvature of the
spine (scoliosis and exaggerated lordosis) are the potential for symptomatic
acceleration. It should be warned that slight flexion of the lower (lumbosacral) spine
always aggravates back pain by spinal cord stretching.
Affected Populations
Tethered cord syndrome affects males and females in equal numbers. The exact
incidence of the disorder in the general population is unknown. In the past, the
diagnosis of tethered cord syndrome has been controversial and the disorder often
still remains unrecognized and underdiagnosed. After many years of skepticism,
tethered cord syndrome is now considered a distinct clinical entity. In order to help
clarify the situation, a proposed definition for true tethered cord syndrome limits that
this disorder to individuals who exhibit neurological signs and symptoms due to
inelastic structures anchoring the caudal end of the spinal cord. There are cases in
which individuals have the symptoms and signs similar to true tethered cord
syndrome, but have associated defects that cause compression and impaired blood
flow of the spinal cord, or congenital neuronal dysgenesis (failure of neuronal
development). For example, in myelomeningoceles and lipomyelomeningoceles that
are directly connected to the entire dorsal surface of lumbosacral spinal cord, their
neurological deficits are unrelated to spinal cord stretching (tethered cord syndrome).
Symptomatically, patients with these anomalies present with complete or nearly
complete paralsysis of lower limbs and total loss of bladder and rectal control.
The most important signs that can be found in late teenagers and adults are back
pain aggravated immediately on flexion of the lumbosacral spine, which elongates
the lumbosacral spinal canal, simultaneously stretching the lower spinal cord. The 3-
Bs sign is useful for initiating suspicion of tethered cords syndrome. They include 1)
bending slightly (over the sink), 2) Buddha sitting with legs crossed (like the Yoga
pose) and 3) Baby holding (or equivalent weight) at the waist level.

Related Disorders
Symptoms of the following disorders may present with sign and symptoms similar to
those of tethered cord syndrome. Comparisons may be useful for a differential
diagnosis. They are extradural lesions, intradural-extramedullary lesions,
intramedullary lesions extraspinal lesions, and peripheral neuropathy or myelpathy.
Children with spina bifida, particularly with myelomeningoceles, show a wide variety
of symptoms and physical findings depending on the severity of the defect.
Anomalies connected to the caudal end of the spinal cord (true tethered cord
syndrome) can be surgically repaired and result in excellent outcome from
untethering. Some others who present with aforementioned complete paraplegia
have no neurological benefit from the repair surgery. (For more information on this
disorder, choose spina bifida as your search term in the Rare Disease Database.)

Diagnosis
A diagnosis of tethered cord syndrome is made based upon identification of
characteristic signs and symptoms (see the symptom section) that can neurologically
locate the lesion to be above the attachment of the anomalies to the spinal cord. For
this purpose, a detailed patient history and a thorough clinical evaluation and
detailed MRI studies must be carried out. In children, typical imaging features such
as a low lying spinal cord and a thickened filum terminale is confirmed by special
imaging techniques such as magnetic resonance imaging (MRI) or computed
tomography (CT) scan and ultrasound studies.
An MRI uses a magnetic field and radio waves to produce cross-sectional images of
particular organs and bodily tissues. In addition, demonstration of spina bifida (bony
defect of the lamina) supports a diagnosis of tethered cord syndrome.
In late teenagers and adults, the displacement of the filum located posterior to the
cauda equina (a bundle of nerve roots that originate from the lower spinal cord) is a
consistent finding. This important feature is proved by the combination of MRI,
endoscopy and surgical findings. During CT scanning (a computer tomography) and
MRI special techniques are used to create cross-sectional images of vertebrae and
nervous system. In some cases, electromyography (EMG) and nerve conduction
studies may be used to assess nerve function. EMG is a test that records electrical
activity in skeletal (voluntary) muscles at rest and during muscle contractions. The
abnormalities in this examination are only shown in patients with an advanced stage
of tethered cord syndrome.

Standard Therapies
Treatment
In children, surgery to release "untether" the spinal cord is recommended to prevent
or reverse progressive neurological symptoms. The type of surgery varies depending
on the mechanical causes, such as an inelastic filum, myelomeningocele,
lipomyelomeningocele, and dermal sinus. Accordingly, the surgical prognosis varies
depending upon the presenting symptoms and tethering-producing anomalies. It has
been said that treatment for adult patients with tethered cord syndrome is
controversial However, it is clear that in both pediatric and adult patients who have
firm evidence of tethered cord syndrome, prompt surgical intervention results in
reversal, or at least stabilization, of symptoms in many cases.
Parents should talk to their physician and medical team about their child's specific
problems, associated symptoms and deformity of the spine and spinal cord. In an
individual with only minimum complaint his/her physician may advise conservative
treatment rather than surgery and will monitor the condition to see whether the
symptoms progress Many experts of tethered cord syndrome recommend against
surgery to individuals who present with the MRI finding of "cord elongation and
thickened filum" but have no symptoms. Some neurosurgeons may prefer cutting the
thickened filum in these cases for the prophylactic purpose.
The responses to treatment for tethered cord syndrome by repairing
myelomeningocele or removal of scarring formation, varies from one person to
another. After the repair, the spinal cord may become "retethered" and additional
surgery may be recommended.
In individuals with severe arachnoiditis (adhesion of the meninges to the spinal cord)
found by MRI or CT scan, careful evaluation of pain and neurological condition is
required to find if surgical treatment is warranted. At surgery, release of arachnoid
adhesion must be performed with meticulous technique. Or re-adhesion or extensive
scar formation might follow the surgery. To circumvent this problem, two special
surgical procedures have been advocated: 1) transection of the spinal cord to relieve
severe back and leg pain, and 2) shortening of the spinal column by resection of one
or two vertebrae to relieve spinal cord tension.

Investigational Therapies
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:
Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
For information about clinical trials sponsored by private sources, in the main,
contact:
www.centerwatch.com
Contact for additional information about tethered cord syndrome:
Shokei Yamada, MD, PhD, FACS
Professor and Former Chairman
Department of Neurosurgery
Loma Linda University
11234 Anderson. Loma Linda, CA 92354
E-mail: [email protected]
Consultant
Arrowhead Regional Medical Center
Department of Neurosurgery
400 N. Pepper Avenue, Colton, CA 92342-1819
Phone: 909-580-1366
Fax: 909-580-1363
Consultant
Kaiser Permanente Medical Center, Fontana
Department of Neurosurgery
99855 Sierra Avenue, Fontana, CA 92335
Contact: Daniel J. Won, MD, Associate of Dr. Yamada
Phone: 909-423-7582
Fax: 909-427-4570
Visiting Professor
Department of Neurosurgery
University of Mississippi Medical Center
2500 n. State Street
Jackson, MS 31296-4500
Jackson, MS

NORD Member Organizations

American Syringomyelia & Chiari Alliance Project, Inc.
o P.O. Box 1586

o Longview, TX 75606-1586

o Phone: (903) 236-7079

o Toll-free: (800) 272-7282

o Email: [email protected]

o Website: http://www.asap.org

Other Organizations
Birth Defect Research for Children, Inc.
o 976 Lake Baldwin Lane

o Orlando, FL 32814 USA

o Phone: (407) 895-0802

o Email: [email protected]

o Website: http://www.birthdefects.org

International Federation for Spina Bifida and Hydrocephalus

o Cellebroersstraat 16

o Brussels, B1000 Belgium

o Phone: 32025020413

o Email: [email protected]

o Website: http://www.ifglobal.org

March of Dimes
o 1275 Mamaroneck Avenue

o White Plains, NY 10605

o Phone: (914) 997-4488

o Email: [email protected] or [email protected]

o Website: http://www.marchofdimes.org and nacersano.org

NIH/National Institute of Neurological Disorders and Stroke

 o P.O. Box 5801

o Bethesda, MD 20824

o Phone: (301) 496-5751

o Toll-free: (800) 352-9424

o Website: http://www.ninds.nih.gov/

Spina Bifida and Hydrocephalus Canada

o Suite 647-167 av. Lombard Avenue

o Winnipeg

o R3B 0V3 Canada

o Phone: (204) 925-3650

o Toll-free: (800) 565-9488

o Email: [email protected]

o Website: http://www.sbhac.ca

Spina Bifida Association of America

o 4590 MacArthur Boulevard NW

o Suite 250

o Washington, DC 20007-4226

o Phone: (202) 944-3285

o Toll-free: (800) 621-3141

o Email: [email protected]

o Website: http://www.sbaa.org

References
JOURNAL ARTICLES
Gupta G, Heary RF, Michaels J. Reversal of longstanding neurological deficits after a
late release of tethered spinal cord. Neurosurg Focus. 2010;29:E11.
Filippidis AS, Kalani MY, Theodore N, Rekate HL. Spinal cord traction, vascular
compromise, hypoxia, and metabolic derangements in the pathophysiology of
tethered cord syndrome. Neurosurg Focus. 2010;29:E9.
Hertzler DA, DePowell JJ, Stevenson CB, Mangano FT. Tethered cord syndrome: a
review of the literature from embryology to adult presentation. Neurosurg Focus.
2010;29:E1.
Stetler WR Jr., Park P, Sullivan S. Pathophysiology of adult tethered cord syndrome:
review of literature. Neurosurg Focus. 2010;29:E2.
Skeik N, Jabr FI. Intermittent back and leg with numbness. Tethered spinal cord
syndrome. Am Fam Physician. 2008;78:869-870.
Yamada S, Won DJ, Pezeshkpour G, et al. Pathophysiology of tethered cord
syndrome and similar complex disorders. Neurosurg Focus. 2007;23:1-10.
Yamada S, Won DJ. What is the true tethered cord syndrome? Childs Nerv Syst.
2007;23:371-375.
Bassuk AG, Craig D, Jalali A, et al. The genetics of tethered cord syndrome. Am J
Med Genet A. 2005;132:450-453.
Argawalla PK, Dunn IF, Scott RM, Smith ER. Tethered cord syndrome. Neurosurg
Clin N Am. 2007;18:531-547.
Yamada S, Knierum DS, Mandybur GM, Schultz RL, Yamada BS. Pathophysiology
of tethered cord syndrome and other complex factors. Neurol Res. 2004;26:722-726.
Woods KRM, Colohan ART, Yamada S, Yamada SM, Won DJ: Intrathecal endoscopy
to enhance the diagnosis of tethered cord syndrome. J Neurosurg 2010;13: 477-483.
Yamada S: Tethered cords syndrome in children and adults. New York; Thieme, 2010
FROM THE INTERNET
National Institute of Neurological Disorders and Stoke. Tethered Spinal Cord
Syndrome Information Page. February 7, 2006. Available at:
http://www.ninds.nih.gov/disorders/tethered_cord/tethered_cord.htm Accessed:
10/13/10

Years Published
1990, 1999, 2007, 2010
The information in NORDs Rare Disease Database is for educational purposes only and is not
intended to replace the advice of a physician or other qualified medical professional.
The content of the website and databases of the National Organization for Rare Disorders (NORD) is
copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any
commercial or public purpose, without prior written authorization and approval from NORD.
Individuals may print one hard copy of an individual disease for personal use, provided that content is
unmodified and includes NORDs copyright.
National Organization for Rare Disorders (NORD)
55 Kenosia Ave., Danbury CT 06810 (203)744-0100

Report Index
Synonyms
General Discussion
Signs & Symptoms
Causes
Affected Populations
Related Disorders
Standard Therapies
Investigational Therapies
Supporting Organizations
References

Search Rare Diseases

Enter a disease name or synonym to search NORD's database of reports.

NORD's Rare Disease Database provides brief introductions for patients and their families to more than 1,200
rare diseases. This is not a comprehensive database since there are nearly 7,000 diseases considered rare in
the U.S. We add new topics as we are able to do so, with the help of rare disease medical experts.

If you are seeking information about a rare disease that is not in this database, we would suggest contacting
the Genetic and Rare Diseases Information Center (GARD) at the National Institutes of Health. NIH has the most
complete database of rare diseases in the U.S.

Representatives of patient organizations whose medical advisors are interested in assisting NORD in creating a
report on a disease not currently covered in this database may write to [email protected].

Log in

Username

Password
Login ?