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Spinal CSF leak

Summary

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  • Spinal CSF leak is characterised by spontaneous or traumatic leakage of cerebrospinal fluid from the spinal dura
  • Presents with orthostatic headaches, neck pain, and neurological symptoms
  • Diagnosis relies on clinical presentation, imaging findings, and CSF analysis

Pathophysiology

  • CSF leaks occur due to:
    • Dural defects or weakness
    • Traumatic injury to the spine
    • Iatrogenic causes (e.g., lumbar puncture, spinal surgery)
  • Results in intracranial hypotension and potential downward displacement of brain structures
  • Compensatory mechanisms include venous engorgement and subdural fluid collections

Demographics

  • Incidence: 5 per 100,000 per year
  • More common in females (2:1 ratio)
  • Peak age: 30-50 years
  • Risk factors:
    • Connective tissue disorders (e.g., Marfan syndrome, Ehlers-Danlos syndrome)
    • Bone spurs or osteophytes
    • Previous spinal surgery or intervention

Diagnosis

  • Clinical presentation:
    • Orthostatic headaches (worsening when upright, improving when supine)
    • Neck pain or stiffness
    • Tinnitus, hearing changes
    • Visual disturbances
    • Nausea and vomiting
  • CSF analysis:
    • Opening pressure typically low (<60 mm H2O)
    • Normal or slightly elevated protein levels
    • Normal glucose and cell count
  • Myelography:
    • Gold standard for localising CSF leaks
    • CT or MR myelography can be used

Imaging

  • MRI brain:
    • Diffuse pachymeningeal enhancement
    • Sagging of brain structures
    • Venous engorgement
    • Subdural fluid collections
  • MRI spine:
    • Extradural fluid collections
    • Meningeal diverticula
    • Nerve root sleeve cysts
  • CT myelography:
    • Contrast extravasation at leak site
    • High sensitivity for detecting small leaks
  • Digital subtraction myelography:
    • Useful for dynamic imaging of CSF flow
    • Can detect slow or intermittent leaks

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  • 70-year-old patient with orthostatic headaches and transient bilateral 6th nerve palsies. The patient had a multi-level thoracic laminectomy for a compressive arachnoid cyst many years prior.
  • CT myelography showed a rapidly filling small ventral epidural leak (red arrow).
  • More apparent on later phase imaging, the ventral leak was associated with a small osteophyte (blue arrow).

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  • A 70-year-old patient presented with tinnitus and dizziness.
  • MRI showed extensive superficial siderosis above and below the tentorium.
  • While the patient had no headache, given the distribution of siderosis, a CSF leak was suspected.
  • MRI of the spine showed a longitudinally extensive ventral epidural collection, indicating a CSF leak.

Treatment

  • Conservative management:
    • Bed rest
    • Hydration
    • Caffeine intake
  • Epidural blood patch:
    • Autologous blood injection into epidural space
    • Success rate: 30-70% after first attempt
  • Targeted patching:
    • Fibrin glue or blood patch at specific leak site
    • Guided by imaging findings
  • Surgical repair:
    • Reserved for refractory cases
    • Direct suturing of dural defect
    • Duraplasty with autologous or synthetic grafts
  • Follow-up imaging:
    • MRI brain to assess resolution of intracranial hypotension signs
    • Repeat myelography if symptoms persist

Differential diagnosis

Differential Diagnosis Differentiating Feature
Chiari malformation Cerebellar tonsillar descent below the foramen magnum without diffuse pachymeningeal enhancement or brain sag
Subdural haematoma (primary) Crescentic extra-axial collection with blood products but no pachymeningeal enhancement or brainstem sag
Diffuse dural disease (IgG4, neurosarcoidosis) Nodular or asymmetric dural thickening; no brain sag or engorged venous structures
CSF-venous fistula Myelogram shows filling of a paraspinal vein