What are the management options for Spontaneous Intracranial Hypotension (SIH)?

Management of Spontaneous Intracranial Hypotension (SIH)

The primary management approach for spontaneous intracranial hypotension should focus on identifying and treating the underlying CSF leak, with epidural blood patching (EBP) as the cornerstone of treatment for persistent symptoms. 1

Initial Evaluation and Management

Diagnostic Approach

• Brain and spine MRI with contrast is the first-line investigation for suspected SIH 1, 2
  • Look for characteristic findings: diffuse pachymeningeal enhancement, brain sagging, subdural collections, and spinal epidural fluid collections 2
• If MRI is positive for SIH, proceed with management algorithm
• If MRI is negative but clinical suspicion remains high, refer to a specialized neuroscience center 1, 2

Conservative Management (First 1-2 weeks)

• Bed rest in supine position
• Adequate hydration
• Caffeine supplementation
• Analgesics (acetaminophen/NSAIDs) for symptomatic relief 2
• Consider oral prednisone (1 mg/kg/day for 5 days with gradual withdrawal) as adjunctive therapy 3

Treatment Algorithm

Step 1: Conservative Management (1-2 weeks)

• If symptoms resolve: continue conservative measures and follow up
• If symptoms persist: proceed to Step 2

Step 2: Non-targeted Epidural Blood Patch

• High-volume (40-65 mL) autologous blood injected into the lumbar epidural space 1, 2
• Success rate approximately 77% 4
• If symptoms persist after 1-2 weeks, consider repeat non-targeted EBP 1
• If still symptomatic after repeat EBP: proceed to Step 3

Step 3: Referral to Specialist Neuroscience Center

• Multidisciplinary team evaluation
• Advanced imaging to localize CSF leak:
  • CT myelography
  • Digital subtraction myelography
  • Lateral decubitus CT or digital subtraction myelography 1

Step 4: Targeted Intervention Based on Leak Localization

• If Spinal Longitudinal Epidural Collection (SLEC) identified:

  • Perform targeted EBP or fibrin sealant patch at leak site 1

• If CSF-venous fistula (CVF) identified:

  • Consider targeted patching, surgery, or transvenous embolization 1

• If surgical intervention required:

  • Primary repair of dural defect 2

Management of Complications

Subdural Hematoma

• Small/asymptomatic: manage conservatively while treating the CSF leak
• Symptomatic with mass effect: burr hole drainage in conjunction with leak treatment 1

Cerebral Venous Thrombosis

• Prioritize EBP as initial treatment
• Consider anticoagulation on individual basis, balancing bleeding risks 1, 2

Superficial Siderosis

• Perform MRI with blood-sensitive sequences
• Manage in specialist center
• Offer non-targeted EBP or targeted treatment if leak site identified
• Consider deferiprone for symptomatic patients with untreatable leaks 1

Post-Treatment Care

After Epidural Blood Patch

• Monitor for 2-24 hours with basic physiological observations
• Maintain supine position (Trendelenburg for non-targeted EBP)
• Consider thromboprophylaxis during immobilization
• Clinical review before discharge
• Advise patients to:
  • Lie flat as much as possible for 1-3 days
  • Avoid bending, straining, heavy lifting, and strenuous exercise for 4-6 weeks 1
  • Seek urgent medical attention for concerning symptoms (severe back/leg pain, weakness, sensory changes, urinary/bowel dysfunction) 1, 2

Headache Management

• Focus primarily on treating the underlying CSF leak
• Use paracetamol/NSAIDs for symptomatic relief
• Opioids may be required for severe pain but avoid long-term use 1
• Be cautious with medications that potentially lower CSF pressure (topiramate, indomethacin) 1

Follow-up

• Clinical review after treatment
• For patients with persistent symptoms, evaluate for:
  • Comorbid primary headache
  • Post-procedural intracranial hypertension
  • Medication overuse headache 1

Pitfalls to Avoid

• Delaying diagnosis while treating for more common headache etiologies 2
• Performing lumbar puncture solely for diagnosis (may worsen condition) 2
• Failing to recognize serious complications like cerebral venous thrombosis 2
• Using medications that potentially lower CSF pressure in long-term management 1