Treatment of Intracranial Hypotension
The first-line treatment for spontaneous intracranial hypotension (SIH) is non-targeted epidural blood patch (EBP), which should be performed as early as possible after diagnosis. 1
Diagnostic Approach
Before initiating treatment, proper diagnosis is essential:
- MRI of the brain with contrast and whole spine is the first-line imaging
- Look for characteristic MRI findings: pachymeningeal enhancement, brain sagging, subdural collections
- Consider SIH in any patient with orthostatic headache (headache that worsens when upright)
Treatment Algorithm
1. Conservative Management
- Initial conservative measures for up to two weeks:
- Bed rest (lying flat as much as possible)
- Adequate hydration
- Caffeine
- Pain management with paracetamol/NSAIDs
- Avoid activities that increase CSF pressure (bending, straining, heavy lifting)
2. Epidural Blood Patch (EBP)
- Non-targeted EBP should be performed early and is the first-line interventional treatment
- For patients with persistent symptoms after conservative management
- Post-EBP care:
- Maintain supine or Trendelenburg position
- Lie flat as much as possible for 1-3 days
- Avoid bending, straining, stretching, twisting, heavy lifting for 4-6 weeks
- Monitor for complications (back pain, neurological symptoms, fever)
3. Advanced Diagnostic and Treatment Options
For patients not responding to initial non-targeted EBP:
- Refer to a specialist neuroscience center for multidisciplinary team discussion
- Advanced imaging to locate the CSF leak:
- CT myelography
- Digital subtraction myelography
- Lateral decubitus myelography (for CSF-venous fistulas)
- Based on imaging findings:
- Targeted blood patch at identified leak site
- Surgical repair of dural defect
- Transvenous embolization for CSF-venous fistulas
Management of Complications
Subdural Hematoma
- Small or asymptomatic hematomas: manage conservatively while treating the CSF leak
- Symptomatic hematomas with mass effect: may require burr hole drainage
Cerebral Venous Thrombosis
- EBP should be prioritized as initial treatment
- Anticoagulation may be considered on an individual basis, balancing bleeding risks
Superficial Siderosis
- Patients with SIH should have MRI with blood-sensitive sequences
- Treat the underlying CSF leak with targeted approaches
- Consider deferiprone for symptomatic patients where the leak cannot be found or treated
Symptomatic Management
- Headache management should focus primarily on treating the underlying CSF leak 1
- Pain relief options:
- Paracetamol and/or NSAIDs
- Opioids may be required short-term but avoid long-term use
- Avoid medications that potentially lower CSF pressure (topiramate, indomethacin)
- Caution with migraine preventives that reduce blood pressure
Follow-up Recommendations
Follow-up intervals:
- Early review: 24-48 hours after intervention
- Intermediate follow-up: 10-14 days after EBP or 3-6 weeks after surgery
- Late follow-up: 3-6 months after any intervention
Special Considerations
Asymptomatic Patients with Radiological Evidence
- Refer to a specialist neuroscience center
- Discuss potential long-term risks (particularly superficial siderosis)
- Consider investigation and treatment despite absence of symptoms
- If conservative approach chosen, offer clinical review and repeat neuroimaging every 1-2 years
Orthostatic Rehabilitation
- Consider for patients who have been bedbound
- Particularly important for those with orthostatic intolerance symptoms
- Rehabilitation should address both skeletal muscle deconditioning and autonomic postural responses
Refractory Cases
In cases where conservative treatment and EBP fail, surgical intervention may be necessary to repair the CSF leak site 2, 3. Specialized imaging techniques like radionuclide cisternography with the patient in upright position can help identify difficult-to-detect leaks.