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

• Diastematomyelia
• Spina Bifida

Initial symptoms of Tethered Spinal Cord Syndrome are usually urological and may include the inability to control urination (incontinence) and repeated urinary tract infections. At birth, afflicted children present characteristic lesions on the lower back that often consist of tufts of hair, skin tags, dimples, or fatty tumors. Other symptoms may include weakness of the lower extremities, inability to control bowel function (fecal incontinence), low back pain, or a combination of these symptoms.

Adult onset of the disorder is very rare. When it does occur, the individual is initially asymptomatic. Later, the most common symptom is diffuse leg pain which may reach as high as the rectum. Progressive sensory and motor deficits may occur in the lower extremities, as well as bladder and bowel dysfunction.

Vertebral adhesions may play a role in Tethered Spinal Cord Syndrome. Such adhesions may arise as a consequence of Spina Bifida and may lead to a wide variety of orthopedic or urologic symptoms through “tethering” or compressing the nerve roots.

Children with benign skin tumors (cutaneous hemangiomas) on the lower back may also have tethered spinal cords. Tethered Spinal Cord Syndrome is also occasionally associated with diastematomyelia, a disorder characterized by diversion of the spinal cord by a midline septum during embryonic development.

Tethered Spinal Cord Syndrome affects males and females equally. First degree relatives of those with the malformation appear to be at slightly higher risk of developing it. Individuals with previously repaired defects of the neural tube are also particularly susceptible to this disorder.

The following disorders are often associated with Tethered Spinal Cord Syndrome.

Diastematomyelia is a rare and very serious spinal cord malformation. During embryonic development, a mix of genetic and/or environmental factors causes a longitudinal division of part of the spinal cord. Symptoms may include pain, weakness of legs and loss of control over urinary and fecal functions (incontinence). Surgery during infancy is often recommended. However, later in life, surgery is performed only if neurological symptoms develop.

Spina Bifida is a disorder that ranges in severity from being asymptomatic to presenting severe neurological disability. The failure of some bones to close leaves an opening through which some of the contents of the spinal canal can protrude or herniate. (For more information on this disorder, choose "Spina Bifida" as your search term in the Rare Disease Database.)

The appearance and recognition of surface lesions on the lower back at birth should lead to further testing for Tethered Spinal Cord Syndrome. Magnetic resonance imaging (MRI) is usually the technique of choice for identifying the tethered spinal cord. Pre-operative evaluation of potential sites of tethering, based on MRI findings, is very important for planning surgery. Removal of adhesions at the lower base of the spine through surgery is often recommended, and results are usually successful. Early management usually prevents neuromuscular, lower limb or urologic problems. It is usually best to treat the tethered cord before serious complications become apparent, as neurologic damage may not be reversible.

In cases where surgical release of the tethered spinal cord is ineffective, surgical severence of some spinal nerve roots may relieve pain.

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DICTIONARY OF MEDICAL SYNDROMES, 4th ed.: Sergio I. Magalini & Sabina C. Magalini; Lippincott-Raven Publishers, 1997. P. 145-46.

TETHERED CORD. H. Rafael; J NEUROSURG. (January,1999: 90(1)). Pp. 175.

THE USE OF GORE-TEX MEMBRANE FOR ADHESION PREVENTION IN TETHERED SPINAL CORD SURGERY: TECHNICAL CASE REPORTS. R.P. Aliredjo et al.; NEUROSURGERY. (March 1999; 44(3)). Pp. 674-77; Discussion: Pp. 677-78.

TETHERED CORD SYNDROME IN OCCULT SPINAL DYSRAPHISM: TIMING AND OUTCOME OF SURGICAL RELEASE. L. Cornette et al.; NEUROLOGY (June, 1999; 50 (6)). Pp. 1761-65.

CONGENITAL TETHERED SPINAL CORD SYNDROME IN ADULTS. B. J. Iskandar et al.; J NEUROSURG (June, 1998; 88 (6)). Pp. 958-61.

THE HUMAN TAIL. F. L. Lu; PEDIATR NEUROL (September, 1998; 19 (3)). Pp. 230-233.

UROLOGIC ASPECTS OF TETHERED CORD. R.C. Flanigan et al.; UROLOGY (January, 1989: issue 33 (1)). Pp. 80-82.

DIAGNOSIS OF TETHERED CORDS BY MAGNETIC RESONANCE IMAGING. W.A. Hall et al.; SURG NEUROL (July, 1988; 30 (1). Pp. 60-64

Lee GY, Paradiso G, Tator CH, Gentili F, Massicotte EM, Fehlings MG: Surgical management of tethered cord syndrome in adults: indications, techniques, and long-term outcomes in 60 patients. J Neurosurg Spine. 2006 Feb;4(2):123-31.