Tethered spinal cord syndrome

Tethered spinal cord syndrome
Classification and external resources
ICD-10	Q06.8
ICD-9	742.59
DiseasesDB	34471
MeSH	C16.131.666.680

Tethered spinal cord syndrome or occult spinal dysraphism sequence refers to a group of neurological disorders related to malformations of the spinal cord.^[1] The various forms include: tight filum terminale, lipomeningomyelocele, split cord malformations, dermal sinus tracts, dermoids, and cystoceles.^[1] All of the forms have in common the pulling of the spinal cord at the base of the spinal canal, literally a "tethered cord".^[1] The spinal cord normally hangs loose in the canal, free to move up and down with growth and bending and stretching, a tethered cord is held taut at the end. In children, a tethered cord can cause the spinal cord to stretch as they grow, in adults the spinal cord will stretch in the course of normal activity, usually leading to progressive spinal cord damage if left untreated.^[1] It is often associated with the closure of a spina bifida, although not always depending on the form it takes; for example it can be congenital such as in tight filum terminale, or the result of injury later in life.

Signs and symptoms

In children, symptoms may include lesions, hairy patches, dimples, or fatty tumours 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 or feeling, or the onset of pain or autonomic symptoms.

In adults, onset of symptoms typically include severe pain (in the lower back and radiating into the legs, groin, and perineum), bilateral muscle weakness and numbness, loss of feeling and movement in lower extremities, and bladder and/or bowel incontinence. Neurological symptoms can include a mixed picture of upper and lower motor neuron findings, such as amyotrophy, hyperreflexia, and pathologic plantar response, occurring in the same limb. Profound sensory changes, such as loss of pain, temperature, and proprioceptive sensations, are common. Last, progressive symptoms of a neuropathic bladder are noted on over 70% of adult patients, versus only 20% to 30% of children. These symptoms include urinary frequency and urgency, feeling of incomplete voiding, poor voluntary control, and urge and stress incontinence. Chronic recurrent infections are common and occasionally lead to nephrolithiasis (kidney stones), renal failure, or renal transplantation. Female patients also give a history of ineffective labor and postpartum rectal prolapse, presumably due to an atonic pelvic floor.

Treatment

Because neurological deficits are generally irreversible, early surgery is recommended when symptoms begin to worsen.^[2][3] In children, early surgery is recommended^[4] to prevent further neurological deterioration, including but not limited to chronic urinary incontinence. In adults, surgery to free (detether) the spinal cord can reduce the size and further development of cysts in the cord and may restore some function or alleviate other symptoms. Tethered cord syndrome is causally linked to Chiari Malformation and any affirmative diagnosis of TCS must be followed by screening for Chiari's several degrees. TCS may also be related to Ehlers-Danlos syndrome which should also be screened for upon a positive TCS diagnosis.

Other treatment is symptomatic and supportive. NSAIDs, opiates, synthetic opiates, Cox II inhibitors, off-label applications of tricyclic anti-depressants combined with anti-seizure compounds have yet to prove they are of value in treatment of this affliction's pain manifestations. There is anecdotal evidence that TENS units may benefit some patients.

Treatment may be needed in adults who, while previously asymptomatic, begin to experience pain, lower back degeneration, scoliosis, neck and upper back problems and incontinence and bladder control issues. Surgery on adults with minimal symptoms is somewhat controversial, for example Columbia University Dept of Neurosurgery says "For the child that has reached adult height with minimal if any symptoms, some neurosurgeons would advocate careful observation only,"^[1] however surgery for those who have symptoms is not controversial.

This syndrome was first noticed in the late 19th century and while information has been available for years, little widespread blind research has been done. More research has been called for and many studies have been done by doctors with great results. There is a low morbidity rate and no known complications have been documented other than those from any type of typical back surgery. The association of this condition with others has been noticed and also needs further research to understand the relations. Spinal compression and the resulting relief is a known issue with this disorder. Like with the early-onset form, this disease form is linked to the Arnold-Chiari malformation, in which the brain is pulled or lowers into the top of the spine.^[5]

Prognosis

The natural course of the disorder is progressive with age, but the aforementioned treatments can help prevent or sometimes relieve symptoms. With treatment, individuals with tethered spinal cord syndrome have a normal life expectancy. However, most neurological and motor impairments are irreversible.^[6]

Occult tight filum terminale

In this variation, the filum terminale (which joins the spinal cord to the backbone) thickens or hardens and causes downward pressure on the spinal cord. This can actually cause scoliosis as well as most of the other symptoms of the more typical tethered cord. This syndrome was first noticed and documented in the late 19th century.

Related disorders

• Kyphosis
• Brain herniation

References

1. ^ ^{a b c d e} Tethered Spinal Cord, Columbia University Dept of Neurosurgery
2. ^ http://www.neurosurgery.tv/tetheredcord.html
3. ^ Iskandar BJ, Fulmer BB, Hadley MN, Oakes WJ (2001). "Congenital tethered spinal cord syndrome in adults". Neurosurgical Focus 10 (1): e7. PMID 16749759. http://www.medscape.com/viewarticle/405679_1. 
4. ^ "Tethered spinal cord syndrome". National Institutes of Health. http://www.ninds.nih.gov/disorders/tethered_cord/tethered_cord.htm. Retrieved 2008-08-28. 
5. ^ Wehby MC, O'Hollaren PS, Abtin K, Hume JL, Richards BJ (2004). "Occult tight filum terminale syndrome: results of surgical untethering". Pediatric Neurosurgery 40 (2): 51–7; discussion 58. doi:10.1159/000078908. PMID 15292632. 
6. ^ Fitzgerald, Kevin. "Tethered spinal cord syndrome". http://www.answers.com/topic/tethered-spinal-cord-syndrome?cat=health. ^{[unreliable medical source?]}

Further reading

• Metcalfe PD, Luerssen TG, King SJ, et al. (October 2006). "Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale". The Journal of Urology 176 (4 Pt 2): 1826–9; discussion 1830. doi:10.1016/j.juro.2006.04.090. PMID 16945660. 
• Drake JM; Anthony Herndon, C. D.; Joseph, David B. (2007). "Surgical management of the tethered spinal cord--walking the fine line". Neurosurgical Focus 23 (2): 1–4. doi:10.3171/FOC-07/08/E8. PMID 17961008. 
• Bademci G, Saygun M, Batay F, et al. (2006). "Prevalence of primary tethered cord syndrome associated with occult spinal dysraphism in primary school children in Turkey". Pediatric Neurosurgery 42 (1): 4–13. doi:10.1159/000089503. PMID 16357495. 
• Fontes RB, Saad F, Soares MS, de Oliveira F, Pinto FC, Liberti EA (May 2006). "Ultrastructural study of the filum terminale and its elastic fibers". Neurosurgery 58 (5): 978–84; discussion 978–84. doi:10.1227/01.NEU.0000210224.54816.40. PMID 16639335. 
• Nazar GB, Casale AJ, Roberts JG, Linden RD (1995). "Occult filum terminale syndrome". Pediatric Neurosurgery 23 (5): 228–35. doi:10.1159/000120965. PMID 8688347. 

External links

• Chiari & Syringomyelia Foundation
• "Tethered spinal cord syndrome". Neurosurgery Today. http://www.neurosurgerytoday.org/what/patient_e/tethered_syndrome.asp. Retrieved 2007-11-03. 
• More Chirari information w/Tethered cord article
• National institute of Health page
• NPR show on it
• http://www.aans.org/education/journal/neurosurgical/Aug07/23-2-5-1214.pdf^{[dead link]} Recent research articles