Craniospinal Hypotension


Craniospinal Hypotension, also known as Intracranial Hypotension, is identified as the underlying cause of postural headaches in patients with superficial siderosis. This condition typically arises following a traumatic event, such as an accident or surgery, which also triggers Superficial Siderosis. Craniospinal Hypotension is characterized by cerebral spinal fluid leakage due to a breach in the dura, the outermost layer surrounding the brain and spinal cord. Every day, your body produces new spinal fluid. If there is a leak, however, your body may not be able to generate enough fluid to adequately support and cushion your brain, creating negative pressure within the brain cavity.

As an illustration, one superficial siderosis patient, Jennifer Soellner, experienced Craniospinal Hypotension as her initial symptom. Jennifer found it challenging to articulate her experiences to doctors, especially as she was also suffering from double sciatica at the time. She recalls the onset of pain as sudden and intense, originating at the base of her spine and shooting up to her brain, leading to severe headaches and loss of body control. Lying down seemed to stabilize her, but residual pain would persist for at least 24 hours. Her symptoms also included what seemed to be seizure-like eye movements, although without accompanying body convulsions.

Following her diagnosis, doctors discovered that iron deposits in her system, characteristic of superficial siderosis, were due to a rapid dural tear. Several attempts to patch the leak were unsuccessful. Ultimately, doctors at Mayo Clinic performed a laminectomy (removal of a portion of the vertebra) from T-3 to T-8 and repaired a significant tear in her dura. Since the operation, Jennifer has not experienced any recurrence of the excruciating pain that once pushed her close to thoughts of suicide.

Craniospinal Hypotension can be identified through signs such as postural headaches, nausea, vomiting, neck pain, visual and auditory disruptions, and vertigo. Diagnosis typically hinges on the positional component of the headache—whether the pain intensifies or diminishes with changes in the position of your head and body.

If the pain associated with Craniospinal Hypotension becomes unbearable, one treatment option is to consider a surgical repair of the dural leak.

Surgical Options

The surgical repair of a dural leak, often needed to manage craniospinal hypotension, involves identifying the location of the tear in the dura and then sealing it to prevent further leakage of cerebrospinal fluid (CSF).

Decoding Superficial Siderosis 15
Decoding Superficial Siderosis 15

The process begins with locating the dural tear, which can be challenging given its small size and potential location anywhere along the spine or skull base. Advanced imaging techniques are crucial in this step. These may include:

  1. Computed Tomography Myelography (CTM): This is an imaging technique where a contrast dye is injected into the spinal fluid, and then CT scans are taken to visualize the spinal canal and nerve roots. This can help identify any leaks or abnormal fluid collections.
  2. Magnetic Resonance Imaging (MRI): MRIs provide high-resolution images of soft tissues, including the brain and spinal cord, which can be useful in detecting any areas of fluid leakage.
  3. Digital Subtraction Myelography: This advanced imaging technique involves the injection of a contrast agent into the spinal canal, followed by a series of X-ray images. The images taken before the injection are subtracted from those taken after, thus enhancing the visibility of the CSF leak.

Once the site of the leak is identified, the surgeon can plan an appropriate approach for the repair. In many cases, a procedure called an epidural blood patch is attempted first, where the patient’s own blood is injected into the epidural space near the site of the leak, promoting clotting and sealing the tear.

If the blood patch procedure does not resolve the leak, or if the leak is large or in an area not suitable for a blood patch, a more invasive surgical repair may be necessary. This typically involves a laminectomy or other surgical procedure to access the dura directly. The tear is then repaired with sutures, and often a patch made from the patient’s own tissue, synthetic material, or a biological graft is placed over the tear to ensure a watertight seal.

The specific surgical approach will depend on the dural tear’s size, location, and nature. After surgery, patients are typically monitored closely for the resolution of their symptoms and to ensure the leak has been adequately sealed. As with all surgeries, there are potential risks and complications, and these should be discussed with the healthcare provider before proceeding with the operation.

Updated: June 21, 2023

Amrhein TJ, Befera NT, Gray L, Kranz PG. CT Fluoroscopy-Guided Blood Patching of Ventral CSF Leaks by Direct Needle Placement in the Ventral Epidural Space Using a Transforaminal Approach. AJNR Am J Neuroradiol. 2016 Jul 7;(37):1951-6.
Beck J, Ulrich CT, Fung C, Fichtner J, Seidel K, Fiechter M, et al. Diskogenic microspurs as a major cause of intractable spontaneous intracranial hypotension. Neurology. 2016 Aug 26;87(12):1220-6.
Sources: Superficial siderosis is a rare neurologic disease characterized by progressive sensorineural hearing loss, cerebellar ataxia, pyramidal signs, and neuroimaging findings revealing hemosiderin deposits in the spinal and cranial leptomeninges and subpial layer. The disease progresses slowly, and patients may present with mild cognitive impairment, nystagmus, dysmetria, spasticity, dysdiadochokinesia, dysarthria, hyperreflexia, and Babinski signs. Additional features reported include dementia, urinary incontinence, anosmia, ageusia, and anisocoria. Superficial siderosis MedGen UID: 831707 •Concept ID: CN226971 •Finding Orphanet: ORPHA247245
Living With SuperficialSiderosis Website PubMed Reference Library 
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