Intracranial hypotension, also known as craniospinal hypotension is defined as cerebrospinal fluid (CSF) pressure <7 cm H2O in patients with clinical presentation compatible with intracranial hypotension, which are postural headache, nausea, vomiting, neck pain, visual and hearing disturbances, and vertigo. It most commonly results from CSF leaks somewhere along the neuraxis.
Intracranial hypotension can broadly be divided into:
- primary: usually referred to as spontaneous intracranial hypotension
- secondary: iatrogenic (lumbar puncture or surgery), over shunting due to diversion devices or traumatic
Spontaneous intracranial hypotension is typically encountered in middle age (30-50 years of age) and has a predilection for women (F:M, 2:1). Of interest, this is a similar demographic to pseudotumour cerebri, which is believed to be an unrecognised predisposing factor.
Epidemiology of secondary intracranial hypotension is variable and matches that of the underlying cause.
The condition often presents as a positional headache which is relieved by a recumbent position, usually within 15-30 minutes 12.
In cases of traumatic or iatrogenic intracranial hypotension, a history of abundant, clear rhinorrhea or otorrhea can sometimes be elicited. Such would be the case with a cerebrospinal fluid (CSF) leak following pituitary surgery or with basal skull fractures.
It is confirmed by assessing opening pressure on lumbar puncture (less than 7 cm H2O) 2. Note that when this is done fluoroscopically, it should be performed in a lateral position to allow for accurate measurement of pressure.
Occasionally presentation is more sinister, with even decreased level of consciousness and coma reported 3.
Intracranial hypotension most commonly results from CSF leaks somewhere along the neuraxis, and leads to alterations in the equilibrium between the volumes of intracranial blood, CSF, and brain tissue (Monro-Kellie hypothesis). A decrease in CSF volume leads to compensatory dilatation of the vascular spaces, mostly venous side due to its higher compliance.
Spontaneous intracranial hypotension (SIH) is usually the result of a CSF leak in the spine due to 9,12,13:
- spontaneous dural dehiscence of meningeal diverticulums (perineural cyst)
- secondary to degenerative dural tears (typically related to calcified thoracic disc protrusions)
- rarely due to congenital focal absence of dura (nude nerve root)
Much less frequently, but increasingly recognised, SIH can result from small medial sphenoid meningocoeles, often in patients with undiagnosed pseudotumour cerebri. It is also more commonly seen in connective tissue disorders, including Marfan syndrome, Ehlers-Danlos syndrome (type II), and autosomal dominant polycystic kidney disease (ADPKD). In many instances, the manifestation of these syndromes may be subtle or not fit into defined syndromes 12.
CSF leaks can also be iatrogenic, following lumbar puncture, surgery, secondary to over shunting, or trauma.
Imaging is crucial in both confirming the diagnosis of intracranial hypotension and identifying the location of the leak. The latter is discussed below in the Imaging Strategy section.
Described features of intracranial hypotension include:
- subdural collection
- cerebellar tonsillar herniation into the foramen magnum: tonsillar ectopia
- dural venous sinus distention
Most common qualitative finding is pachymeningeal thickening and enhancement followed by dural venous engorgement, tonsillar herniation, and subdural collection; but these features are not always present so quantitative findings would be of great help in making more accurate diagnosis based on MRI findings.
- qualitative signs
- pachymeningeal enhancement (most common finding): becomes less prevalent over time after the onset of symptoms; hence in patients with chronic duration of symptoms in whom clinically headache pattern also changes from orthostatic to atypical constant headache, the absence of dural enhancement may hinder the diagnosis of SIH 15
- increased blood volume in veins
- enlargement of the pituitary gland
- subdural effusions and eventual subdural haematomas
- diffuse brain swelling 3
- reduced CSF volume
- sagging brainstem and cerebellar tonsillar herniation
- droopy penis sign: perhaps only an Australianism to denote the shape of the downward drooping splenium of the corpus callosum
- decreased fluid within the optic nerve sheath 8
- quantitative signs
Identifying the site of CSF leakage can, especially in spontaneous cases, be challenging, and embarking upon imaging can be daunting as the leak can be located anywhere along the neuraxis and leaks can vary dramatically in their rate ranging from large volume rapid leaks to slow leaks to intermittent / inapparent leaks. As a result, no single examination is guaranteed to confirm and localise the abnormality.
Assuming the site is not likely to be cranial, then a suggested imaging strategy is as follows 9:
- MRI of the brain with contrast
- speculative lumbar epidural blood patch (in some cases)
- MRI spine and/or CT myelography: usually diagnostic for slow leaks
- depends on the speed of leak
- fast high volume leak: digital subtraction myelography or ultrafast / dynamic CT myelography
- no leak (?intermittent): MRI myelography (with intrathecal Gadolinium) or nuclear medicine myelography
1. MRI of the brain with contrast
MRI of the brain is an essential first step, needed to confirm the diagnosis of intracranial hypotension (see above) and rule out other unexpected pathology. In the setting of prior skull fracture or surgery then the addition of fat-saturated thin T2 weighted sequences are helpful in identifying an intracranial dural defect. This should also be added presumptively in patients who may have had undiagnosed pseudotumour cerebri (overweight young females) and targeted to the middle cranial fossa.
2. Speculative blood patch
If the diagnosis is beyond a doubt, with classic symptoms and imaging findings, and no likely localising history is present (i.e. no trauma, surgery, history of or suspicion of antecedent pseudotumour cerebri). Then a speculative non-targeted lumbar epidural blood patch is a reasonable next step without necessarily trying to identify/confirm the location of leak 9.
In many cases, non-targeted lumbar epidural blood patch will not result in a permanent cure, and further imaging is required.
3. CT myelography or MRI spine
If confirmation of leak needed (i.e. intracranial imaging and/or symptoms not definitive), and/or localisation is required, then further imaging is necessary.
CT myelography is useful in identifying the location of CSF leak and presence of calcified disc protrusions or osteophytes.
MRI of the spine is also useful, provided it is performed with fat-saturated T2 imaging allowing for the visualisation of accumulation of CSF in the epidural space. On T2 imaging reduced signal in the epidural space can also be seen but is subtle.
It should be noted that if the amount of leaked CSF is large then the distribution of fluid should not be interpreted as necessarily representing the site of leak 9. CSF in the epidural space can migrate significant distances and pool depending on patient position and anatomy.
A particular example of this is pooling of contrast / CSF at the C1/2 level posteriorly. This can be incorrectly attributed to a local leak, when in fact this is not the case. This is referred to as a false localising sign 10-12.
4a. Fast leaks
CT myelography can be repeated, with the lumbar puncture performed on the CT table using CT fluoroscopy.
Digital subtraction myelography has the best temporal resolution and with newer equipment allowing Dyna-CT also can obtain cross-sectional images.
4b. No / intermittent leaks
MRI myelography (with intrathecal Gadolinium) is not approved by FDA and is associated with neurotoxicity particularly if renal function or CSF hydrodynamics are compromised but it has been tried in some cases when other methods has failed to demonstrate the source of CSF leak 9. MRI myelography with intrathecal gadolinium has been shown to be more sensitive than CT in that respect 6.
Nuclear medicine myelography can also be employed, using intrathecal 111Indium-DTPA, with images obtained typically at 1, 2, 4, 24 and in some instances even 48 hours post injection 9. Localisation can be seen as focal accumulation of activity. In some cases, only indirect evidence of a leak being present 'somewhere' is available, which is only really useful in instances where the diagnosis of CSF leak remains uncertain. Indirect evidence includes 9:
- early accumulation of activity within the urinary tract (kidneys / bladder) at 4 hours
- absent activity over the cerebral convexities at 24 hours
- rapid loss of activity from within the CSF space
Cranial leak location
In most instances an MRI (see above) and CT of the base of skull with thin bone algorithm images is sufficient for identifying bony defects in the skull base, including acute or long-standing fractures, congenital bony defects (e.g. meningocoeles) or other focal deficiencies.
Treatment and prognosis
Non-targeted epidural blood patch is often used in cases of spontaneous intracranial hypotension, on the assumption that the leak is from the spine, with variable success 9. When successful, headaches resolve within 72 hours of intervention 12. Subdural effusions can resolve within a few days or weeks. Larger subdural collections often require far longer 12.
In cases where such speculative treatment fails, localisation of the CSF leak is required allowing for targeted epidural blood patch or surgical intervention 9.
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