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tethered spinal cord syndrome (occult spinal dysraphism sequence)
Etiology:
1) neural tube defect closely related to spina bifida
2) abnormal stretching of the spinal cord
Pathology:
- onset of symptoms related to the degree of strain on the spinal cord
Clinical manifestations:
1) lesions, hairy patches, dimples, or fatty tumors on the lower back
2) foot & spinal deformities
3) weakness in the legs
4) low back pain
5) scoliosis
6) urinary incontinence
7) fecal incontinence
8) may go undiagnosed until adulthood when symptoms emerge
Management:
1) early surgery (children) recommended to prevent further n neurological deterioration
2) spinal cord nerve roots may be cut to relieve pain (if surgery is not feasible)
3) other treatment is symptomatic & supportive
Prognosis:
1) with treatment, patients have a normal life expectancy
2) residual neurologic & motor impairments may remain
Related
spina bifida
General
neural tube defect
References
- NINDS Tethered Spinal Cord Syndrome Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Tethered-Spinal-Cord-Syndrome-Information-Page