Primary tethered cord syndrome

Primary tethered cord syndrome (also known as tethered spinal cord syndrome or tight filum terminale syndrome) is a congenital neurological condition in which the spinal cord is abnormally attached (tethered) to surrounding structures within the spinal canal, most commonly due to a thickened or shortened filum terminale. This tethering restricts the normal movement of the spinal cord within the spinal canal, and as the child grows, progressive stretching and tension on the spinal cord can lead to neurological, urological, and orthopedic deterioration. The condition primarily affects the nervous system, musculoskeletal system, and urinary system. Key clinical features include progressive lower extremity weakness, gait abnormalities, lower back pain, scoliosis, foot deformities (such as pes cavus or equinovarus), and bowel and bladder dysfunction (including urinary incontinence, recurrent urinary tract infections, and constipation). Cutaneous stigmata overlying the lower spine—such as a dimple, hairy patch, lipoma, or dermal sinus—may be present and serve as early diagnostic clues. Symptoms may present in childhood but can also manifest or worsen during periods of rapid growth or in adulthood. Neurological deficits can be progressive and irreversible if left untreated. Diagnosis is typically confirmed through magnetic resonance imaging (MRI) of the spine, which demonstrates a low-lying conus medullaris (below the L2 vertebral level) and/or a thickened filum terminale. The primary treatment is surgical untethering, in which the filum terminale is sectioned to release tension on the spinal cord. Early surgical intervention generally yields the best outcomes, particularly in preserving neurological and urological function. Postoperative monitoring is important, as re-tethering can occur in some patients, potentially necessitating repeat surgery. There is no curative pharmacological therapy; management is supportive and surgical.

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