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Updated: Jun 24 2021

Syrinx & Syringomyelia

  • Definitions
    • Definitions
      • syringomyelia
        • a syrinx (fluid filled cavity) within the spinal cord that progressively expands and leads to neurologic deficits
      • syringobulbia
        • a syrinx within the brain stem that leads to neurologic symptoms
  • Epidemiology
    • Synringomyelia
      • incidence
        • prevalence of the disease is about 8.4 cases per 100,000 people
      • demographic
        • occurs more frequently in men than in women
        • usually appears in the third or fourth decade of life
  • Etiology
    • Pathophysiology
      • usually result from lesions that partially obstruct CSF flow including
        • craniocervical junction abnormalities
          • more than 50% related to Chiari malformations (CM)
            • herniated cerebellar tonsils act as pistons obstructing subarachnoid space at foramen magnum creating pressure wave in subarachnoid space with each heartbeat
        • spinal cord trauma
          • found in 4-10% of patients with spinal cord injury
          • may become apparent years after the injury
        • spinal cord tumors
          • 30% of patients with a spinal cord tumor develop a syrinx
        • post-infectious (e.g. meningitis)
          • due to scarring from arachnoiditis
    • Associated conditions
      • developmental scoliosis
        • 25% to 80% of cases of syringomyelia may have scoliosis
          • neural axis malformations found in 1.8% to 25% of patients initially diagnosed with idiopathic scoliosis depending on case series and referral patterns
      • Klippel-Feil deformity
      • Charcot's joints
        • occurs in < 5% with syringomyelia
        • often involves shoulder joint
  • Presentation
    • Syringomyelia
      • symptoms
        • symptoms usually begin insidiously between adolescence and age 45
        • occipital headache
          • sometimes exacerbated by Valsalva maneuver
        • back pain
        • radicular pain
        • neurologic deficits
          • syringomyelia often presents with a central cord syndrome
      • physical exam
        • reflexes
          • asymmetric abdominal reflexes
          • asymmetric and/or hyperactive reflexes
          • asymmetric sweating
          • spasticity
        • motor
          • asymmetric muscle bulk
          • weakness
        • sensory
          • deficits in pain and temperature sensation in a capelike distribution over the back of the neck
            • light touch and position and vibration sensation are usually not affected
        • deformity
          • lower extremity contracture and deformity
          • scoliosis
    • Syringobulbia
      • symptoms related to cranial nerve involvment
        • tongue weakness and atrophy (CN XII)
        • sternocleidomastoid & trapezius weakness (CN XI)
        • dysphagia and dysarthia (CN IX, CN X)
        • facial palsy (CN VII)
  • Imaging
    • Radiographs
      • relative lack of apical lordosis may indicate presence of syrinx
      • scoliosis series for evaluation of scoliosis
    • CT
      • not applicable for characterization of syrinx, only for associated scoliosis
    • MRI
      • diagnosis of syrinx made by MRI
      • obtain MRI with gadolinium enhancement to rule out associated tumor
      • indications for MRI in patients with scoliosis
        • abnormal curve (e.g. double curve, apex left)
        • neurologic deficit
        • infantile or juvenile age at onset
        • male gender with atypical or large curve
        • thoracic kyphosis >30 degrees
  • Histopathology
    • Gross pathology
      • cavitation of spinal cord gray matter
      • syrinx in continuity with or adjacent to central canal
      • inner layer of gliotic tissue
  • Differential Diagnosis
    • Hydromyelia
    • Glioependymal cysts
    • Myelomalacia
    • Cystic tumors
    • Persistent central canal
  • Treatment
    • Nonoperative
      • observation
        • indications
          • if asymptomatic, non-elarging syrinx, most pediatric neurosurgeons recommend against prophylactic surgery
          • orthopaedic management may include observation or bracing during neurosurgical and neurologic evaluations but results of bracing mixed
    • Operative
      • decompression of the foramen magnum and upper cervical cord +/- shunting
        • indications
          • cranial nerve deficits
          • extremity motor weakness
          • cerebellar findings
          • sensory deficit
        • technique
          • done prior to spinal arthrodesis
        • outcomes
          • effect of decompression on neurologic signs and symptoms is variable but supported due to propensity of deficits to progess
          • some evidence shows neurosurgery alone may improve spinal deformity
            • but scoliosis still likely to progress in large curves or children >8 years old
      • spinal fusion
        • indications
          • depends on age of presentation and curve characteristics
        • technique
          • timing of orthopaedic intervention debatable
            • most recommend waiting 3 to 6 months after neurosurgical decompression
  • Complications
    • In general, same as for idiopathic scoliosis
      • Increased risk of neurologic deterioration including paralysis if fusion done with undiagnosed syrinx
  • Prognosis
    • Natural history
      • not well defined
      • prognosis depends on degree of spinal deformity and neurologic deficits
      • multiple authors report resolution or improvement in syrinx size and neurologic deficits
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