Dural ectasia

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Dural ectasia
Spinal cord
SpecialtyNeurology

Dural ectasia is widening or ballooning of the dural sac surrounding the spinal cord. This usually occurs in the lumbosacral region, as this is where the cerebrospinal fluid pressure is greatest,[1] but the spinal canal can be affected in any plane.[2]

Signs and symptoms

Common symptoms include lower back pain, headaches, weakness (myasthenia), numbness (hypoesthesia) above and below the involved limb,[2] leg pain,[3] and sometimes rectal and genital pain.[2] Bowel and bladder dysfunction, urinary retention or incontinence may occur.[4] Moderate-to-severe cases can cause radicular pain in the legs caused by nerve root compression.[5]

The symptoms are usually exacerbated by upright posture and often, but not always, relieved by lying down. Postural headaches can be related to spontaneous spinal cerebrospinal fluid leaks.[6] However, in many patients, dural ectasia is asymptomatic.[7]

Causes

The etiology of dural ectasia is unknown, but it has been suggested that is due to increased hydrostatic pressure,[8] general weakened connective tissue[9] or as a result of the pulsatile flow of cerebrospinal fluid on weakened spinal dura.[10]

Dural ectasia is common in

tumors or trauma.[14]

In neurofibromatosis type I, it has been theorized that local infiltration of the dura by plexiform neurofibromas leads to a weakening of the dural allowing the outpouching. A retrospective study found that a majority of dural ectasia were associated with nearby plexiform neurofibromas.[15]

Diagnosis

Dural ectasia is defined as a ballooning or outpouching of the dura with a dural volume greater than two standard deviations above the mean value in controls.

cysts,[18] anterior meningoceles, nerve root sleeve herniation and gradual erosion of the vertebral bodies (scalloping).[19]

Treatment

Dural ectasia can be asymptomatic, in which case no intervention is necessary. However, it is associated with chronic pain in patients with Marfan syndrome, suggesting it is a structural risk factor.[20] There is no medical consensus on how to manage symptomatic (painful) dural ectasia. The majority of patients are treated conservatively with pain control medications, physiotherapy, and other physical modalities, with often incomplete control of patients' pain.[9][15] Surgical repair of the dura may provide symptomatic relief for some patients.[5]

It has been reported that acetazolamide can be used to treat dural ectasia in individuals with Marfan syndrome, however, the only supporting evidence for this assertion is a small study of 14 patients which was not peer-reviewed or submitted for publication.[21][22] Moreover, several published cases of intracranial hypotension related to Marfan syndrome would warrant caution in using acetazolamide in these patients unless there is a clear indication, as it could lower intracranial pressure further.[23]

References

  1. ^
    PMID 21606066
    .
  2. ^
    PMID 18494231. Archived
    (PDF) from the original on 3 May 2021.
  3. ^
    PMID 19726741
    .
  4. PMID 12177551
    .
  5. ^
    PMID 16325702
    .
  6. PMID 30998996
    .
  7. ^
    PMID 17620463
    .
  8. PMID 17945136
    .
  9. ^
    S2CID 23015722
    .
  10. PMID 12177551
    .
  11. PMID 15695549
    .
  12. ISSN 2691-3895
    .
  13. PMID 12034948
    .
  14. ISSN 1479-2354
    .
  15. ^
    PMID 31350851
    .
  16. OCLC 460904299
    .
  17. PMID 15616116
    .
  18. PMID 19726741. Archived
    from the original on 4 May 2021.
  19. PMID 16641360
    .
  20. S2CID 7459589
    .
  21. ^ Ahn, Nicholas U; Sponseller, Paul D; Ahn, Uri M.; Nallamshetty, L.; Rose, Peter; et al. "Dural Ectasia in the Marfan Spine: Symptoms and Treatment". Archived from the original on 2007-09-26.
  22. PMID 30335315. Archived
    from the original on 3 May 2021.
  23. S2CID 5734726
    .
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