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TETHERED CORD SYNDROME

 The tethered cord syndrome is a group of rare neurological disorder of the spinal cord present at birth.

It occurs when the spinal cord is abnormally attached to the tissue around the spine and limits the movement of spinal cord in the spinal column.

Tethered cord syndrome is a stretch induced functional disorder associated with the fixation effect of inelastic tissue on the caudal spinal cord, limiting its movements

This abnormal stretching causes

  • Progressive stretch of the spinal cord
  • Increased tension in the spinal cord
  • Increased strain and stretching of spinal cord can damage nerves
  • Increased stress with flexion and extension

CLINICAL PRESENTATION

GENERAL

  • Progressive leg weakness
  • Progressive sensory loss
  • Low back pain
  • Abnormal curvature of Spine-Scoliosis
  • Foot deformities
  • Spinal dysraphism (blemishes or coloured marks, hairy patch, dimple, subcutaneous lipoma, mass or grown under skin)
  • Difficulty in walking or running
  • Numbness and muscle weakness in lower limb
  • Loss of bladder or bowl control

SPECIALIZED

CUTANEOUS

  • Cutaneous Lipoma
  • Dermal sinus
  • Cutaneous Hemangioma
  • Deep dimple
  • Gluteal crease deviation

PAIN

  • Groin pain
  • Leg pain
  • Perineogluteal region radiating into lower limb
  • Increased by prolonged sitting and forward bending due to increased strain and stress on spinal cord

ORTHOPEADIC

  • Pes cavus
  • Hollow low back (exaggerated lordosis)
  • High arched feet
  • Hammertoes
  • Leg length discrepancy
  • Club foot
  • Trophic ulceration of foot
  • Atrophy of lower limb muscles
  • Scoliosis
  • Abnormal curvature of spine
  • Abnormal gait
  • sciatica

UROLOGICAL

  • Detrusor muscle hyperreflexia
  • Stress incontinence
  • Enuresis
  • Dribbling during urination
  • Involuntary urination and defecation
  • Repeated urinary tract infection
  • Constipation
  • Neuropathic bladder incontinence
  • Feeling of incomplete voiding
  • Nephrolithiasis

MOTOR

  • Mixed upper and lower motor neuron
  • Hyperreflexia
  • Lower limb spasticity
  • Loss of muscle strength and tone
  • Bilateral muscle weakness and numbness
  • Atonic pelvic floor in female patients
  • Loss of muscle mass
  • headaches

SENSORY

  • Loss of pain
  • Loss of sense of temperature
  • Loss of proprioceptive sensation
  • Loss of sensation in saddle area
  • Loss of sensation in distal portion of limb
  • Electric shock sensation through spine, arms, legs, trunk (Lhermitte’s sign)
  • Burning pain in analgesic area

CAUSES

  • Congenital (abnormal development of spine during fetal growth)
  • Spina bifida
  • Acquires after birth growth press into spinal cord (lipoma, tumor, cysts)
  • Excessively light or thick filum terminale
  • Spinal injuries
  • Post-operative scar tissue after spinal surgery
  • Infection or trauma to spinal cord
  • Diastematomyelia
  • Dermal sinus
  • Myelomeningocele
  • lipomyelomeningocele

DIAGNOSIS

  • Ultrasonography (infants, 8 weeks to 5 years)
  • MRI
  • Myelogram
  • CT scan or CAT scan
  • EMG
  • X-rays
  • Physical therapy examination

CLASSIFICATION

PRIMARY

  • Congenital tethered cord syndrome
  • The caudal spinal cord is tethered due to a thickened filum terminale
  • Improper canalization of neural tube during secondary neurulation causes preadipose cells to proliferate and differentiate leading to thickened filum terminale which causes ascension of conus medullaris resulting in abnormally elongated spinal cord.
  • Cutaneous lipomas are associated with TCS

SECONDARY

  • Acquired tethered cord syndrome
  • fibrotic thickening of filum terminale is caused by infection , fibrotic scaring, presence of benign tumor, lipoma, hemangioma, spinal surgery

DIFFERNTIAL DIAGNOSIS

  • Spondylolisthesis
  • Spinal disease
  • Spinal cord disorder
  • Herniated disc
  • Spinal cord tumors
  • Peripheral neuropathy
  • myelopathy

PATHOPHYSIOLOGY

  • TCS results from reduce oxygen uptake and utilization in spinal cord during fetal developments. Redox reaction occurs in hypoxic conditions.
  • Impaired oxidative metabolism
  • Lack of oxygenation to caudal end of spinal cord
  • Ischemia
  • Ion channel dysfunction

PHYSICALTHERAPY MANAGEMENT

Exercises are conducted

  • To regain as much movement as possible
  • Reduce atrophy
  • Make patient able to perform ADLs
  • Treat associated conditions e.g. (LBP, abnormal spine curvature, scoliosis, exaggerated lordosis, foot deformities)

MEDICATIONS

  • NSAIDs
  • Prescription opiods
  • Muscle relaxants
  • Antidepressents

SURGICAL TREATMENT

The surgical treatment includes;

  • Removal or release of tether (untethering)
  • Debulking of problematic tissue
  • Repair or release nearby structures
  • Spine shortening vertebral osteomy
  • Lumbosacral laminectomy

RELATED DISORDERS

  • Ehlers danlos syndrome , kippel feil syndrome
  • Arnold chiari malformation
  • Spina bifida
  • Extradural lesions
  • Intradural extramedullary lesions
  • Intramedullary lesions
  • Extraspinal lesions
  • Peripheral neuropathy
  • Myelopathy
  • myelomeningoceles
  • paraplegia

REFERENCES

  • NHS.Uk
  • National organization of rare disease (NORD)
  • Genetic and rare disease information center(GARD)
  • National institute of neurological disorder and stroke
  • Childerenshospital.org
  • thejns.org
  • radiopedia
  • Physiopedia

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