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Tethered Spinal Cord Syndrome

(A Patient Information Service)

Congenital anomalies are the product of errors in "embryogenesis" (malformations consequent to errors in the developmental stages of the embryo) or the result of intrauterine events that affect embryonic and fetal growth (deformations and disruptions). As a general rule, it is apparent that the more complex the formation of a structure, the more opportunities for malformation. Some of the most serious neurological abnormalities affect the Brain (conditions that are reviewed elsewhere on this website) develop in the first two months of gestation and represent defects in neural tube (the embryonic precursor of the entire central nervous system) formation. The medical term for this is "dysraphia". Some of these affect the Spine and the Spinal Cord as well.

Modern investigative methods, such as amniocentesis and ultrasonography may provide an accurate in utero detection of many malformations. Genetic counseling for parents of a child with a major neurological abnormality is important, since the risk of a subsequent child's having such a defect is high. These parents frequently also need psychological help and support. Women who have had a pregnancy resulting in an infant or fetus with a neural tube defect should be advised that folic acid supplementation (4 mg/day) before conception and during early pregnancy may substantially reduce the risk of neural tube defects in subsequent pregnancies.

Tethered Spinal Cord Syndrome is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column.  The course of the disorder is progressive.  The term "tethered spinal cord" refers to any condition in which the spinal cord is attached to an immovable structure. Normally the bottom of the spinal cord, called the Conus Medullaris, floats freely within a pool of cerebrospinal fluid (CSF). There are, however, several pathologic conditions that cause this tethering. This leads to abnormal stretching of the Spinal Cord and its nerve roots with growth of the patient and movement, which over time can lead to the characteristic symptoms of tethered spinal cord, including muscle weakness, sensory loss, change in bowel or bladder control, and orthopedic deformity. The primary "causes" of tethered spinal cords include conditions such as: dermal sinus tract, Diastematomyelia (split spinal cord), Lipoma, tumor, myelomeningocele ("spina bifida"), tight Filum Terminale, past trauma or surgery on the spine.

TREATMENT

In children, symptoms may include observable surface lesions such as hairy patches, dimples, or fatty tumors (Lipomas) on the lower back; foot and spinal deformities; weakness in the legs; low back pain; scoliosis; and incontinence.  Tethered Spinal Cord Syndrome may go undiagnosed until adulthood, when sensory and motor problems and loss of bowel and bladder control emerge.  This delayed presentation of symptoms is related to the degree of strain placed on the spinal cord over time. Tethered Spinal Cord Syndrome appears to be the result of improper growth of the neural tube during fetal development, and is closely linked to Spina Bifida. Tethering may also develop after spinal cord injury and scar tissue can block the flow of fluids around the spinal cord.  Fluid pressure may cause cysts to form in the spinal cord, a condition called Syringomyelia.  This can lead to additional loss of movement, feeling or the onset of pain or autonomic symptoms.

The causes of tethered spinal cord are generally regarded to be errors in the normal development of the fetus. Many of these errors occur even before a woman knows that she is pregnant. Folate (Folic Acid) supplements for women in child bearing years can reduce the rate of some problems, such as open Spina Bifida, but many causes of tethered spinal cord are not affected by Folic Acid. As a consequence of increased awareness of this condition and its symptoms, and more sensitive MRI scanning, more tethered cords are being detected now than ever before.

Early surgery is recommended in children, to prevent further neurological deterioration. If surgery is not advisable, spinal cord nerve roots may be cut to relieve pain.  In adults, surgery to free up (release or "detether") the spinal cord can reduce the size and further development of cysts in the cord. An effective operation may abort the inevitable progression of the symptoms and may restore some function or alleviate other symptoms.

Organizations

Additional information is available from the following organizations: American Association of Neurological Surgeons
http://www.neurosurgerytoday.org/what/patient_e/tethered.asp

American Syringomyelia Alliance Project (ASAP)
P.O. Box 1586
Longview, TX 75606-1586
info@asap.org
http://www.asap.org
Tel: 903-236-7079 800-ASAP-282 (272-7282)
Fax: 903-757-7456

National Organization for Rare Disorders (NORD)
P.O. Box 1968
55 Kenosia Avenue
Danbury, CT 06813-1968
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291



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This page last edited on 2/20

All content ©2016 by Neurosurgical Consultants, P.A.
Author, Martin L. Lazar, MD, FACS
All Rights Reserved. See Usage Notices.