Dural ectasia

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Dural ectasia
Dissection of spinal cord.jpg
Spinal cord
Specialty Neurology

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]

Contents

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 Marfan syndrome, [3] occurring in 63–92% of people with the syndrome. [11] It may also occur in Ehlers-Danlos Syndrome, neurofibromatosis type I, [12] ankylosing spondylitis, [1] and is associated with spondylolisthesis, vertebral fractures, [13] scoliosis, 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. [9] It is usually identified by MRI or CT Scan, [7] which can be used to distinguish it from tumors. [16] Radiographs may also be used to identify secondary bone changes. [17] Associated signs include a lack of epidural fat at the posterior wall of the vertebral body, the presence of radicular 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. 1 2 Liu, CC; Lin, YC; Lo, CP; Chang, TP (June 2011). "Cauda equina syndrome and dural ectasia: rare manifestations in chronic ankylosing spondylitis". The British Journal of Radiology. 84 (1002): e123-5. doi:10.1259/bjr/45816561. PMC   3473624 . PMID   21606066.
  2. 1 2 3 Altman, A; Uliel, L; Caspi, L (March 2008). "Dural ectasia as presenting symptom of Marfan syndrome" (PDF). The Israel Medical Association Journal. 10 (3): 194–5. PMID   18494231. Archived (PDF) from the original on 3 May 2021.
  3. 1 2 Shirley, ED; Sponseller, PD (September 2009). "Marfan syndrome". The Journal of the American Academy of Orthopaedic Surgeons. 17 (9): 572–81. doi:10.5435/00124635-200909000-00004. PMID   19726741.
  4. ↑ Nallamshetty, L; Ahn, NU; Ahn, UM; Nallamshetty, HS; Rose, PS; Buchowski, JM; Sponseller, PD (August 2002). "Dural ectasia and back pain: review of the literature and case report". Journal of Spinal Disorders & Techniques. 15 (4): 326–9. doi:10.1097/00024720-200208000-00012. PMID   12177551.
  5. 1 2 Ho, Nicola CY; Tran, Joseph R; Bektas, Arsun (2005). "Marfan's syndrome". The Lancet. 366 (9501): 1980. doi:10.1016/S0140-6736(05)66995-4. ISSN   0140-6736. PMID   16325702.
  6. ↑ Böker, Tordis; Vanem, Thy Thy; Pripp, Are Hugo; Rand-Hendriksen, Svend; Paus, Benedicte; et al. (2019). "Dural ectasia in Marfan syndrome and other hereditary connective tissue disorders: a 10-year follow-up study". The Spine Journal. 19 (8): 1413. doi: 10.1016/j.spinee.2019.04.010 . hdl: 10852/74341 . ISSN   1529-9430. PMID   30998996.
  7. 1 2 Ha, Hong Il; Seo, Joon Beom; Lee, Sang Hoon; Kang, Joon-Won; Goo, Hyun Woo; Lim, Tae-Hwan; Shin, Myung Jin (2007). "Imaging of Marfan Syndrome: Multisystemic Manifestations". RadioGraphics. 27 (4): 1001. doi:10.1148/rg.274065171. ISSN   0271-5333. PMID   17620463.
  8. ↑ Demetracopoulos, Constantine A.; Sponseller, Paul D. (2007). "Spinal Deformities in Marfan Syndrome". Orthopedic Clinics of North America. 38 (4): 563–572. doi:10.1016/j.ocl.2007.04.003. ISSN   0030-5898. PMID   17945136.
  9. 1 2 3 Foran, Jared R. H.; Pyeritz, Reed E.; Dietz, Harry C.; Sponseller, Paul D. (2005). "Characterization of the symptoms associated with dural ectasia in the Marfan patient". American Journal of Medical Genetics Part A. 134A (1): 58–65. doi:10.1002/ajmg.a.30525. PMID   15690402. S2CID   23015722.
  10. ↑ Nallamshetty, Leelakrishna; Ahn, Nicholas U.; Ahn, Uri M.; Nallamshetty, Hema S.; Rose, Peter S.; Buchowski, Jacob M.; Sponseller, Paul D. (2002). "Dural Ectasia and Back Pain: Review of the Literature and Case Report". Journal of Spinal Disorders & Techniques. 15 (4): 328. doi:10.1097/00024720-200208000-00012. ISSN   1536-0652. PMID   12177551.
  11. ↑ Lacassie, HJ; Millar, S; Leithe, LG; Muir, HA; Montaña, R; et al. (April 2005). "Dural ectasia: a likely cause of inadequate spinal anaesthesia in two parturients with Marfan's syndrome". British Journal of Anaesthesia. 94 (4): 500–4. doi: 10.1093/bja/aei076 . PMID   15695549.
  12. ↑ Mutua, Victor; Mong’are, Newnex; Bundi, Brian; von Csefalvay, Chris; Oriko, David; Kitunguu, Peter (September 2021). "Sudden bilateral lower limb paralysis with dural ectasia in Neurofibromatosis type 1: A case report". Medicine: Case Reports and Study Protocols. 2 (9): e0165. doi: 10.1097/MD9.0000000000000165 . ISSN   2691-3895.
  13. ↑ Ho, Nicola C.; Hadley, Donald W.; Jain, Pawan K.; Francomano, Clair A. (2002). "Case 47: Dural Ectasia Associated with Marfan Syndrome". Radiology. 223 (3): 767–771. doi:10.1148/radiol.2233000971. ISSN   0033-8419. PMID   12034948.
  14. ↑ Grenier, Julie-Marthe; Wessely, Michelle A. (2007). "Magnetic resonance imaging of the spinal cord". Clinical Chiropractic. 10 (4): 205–217. doi:10.1016/j.clch.2007.10.001. ISSN   1479-2354.
  15. 1 2 Frim, David M; Warnke, Peter C; Tonsgard, James H; Yang, Carina W; Pytel, Peter; et al. (2020). "Dural Ectasia in Neurofibromatosis 1: Case Series, Management, and Review". Neurosurgery. 86 (5): 646–655. doi:10.1093/neuros/nyz244. ISSN   0148-396X. PMID   31350851.
  16. ↑ Stein, Diane Von; Crawford, Alvin H. (2009). "Neurofibromatosis×". In Errico, Thomas; Lonner, Baron S.; Moulton, Andrew W. (eds.). Surgical Management of Spinal Deformities. Philadelphia, PA: Saunders/Elsevier. p. 223. doi:10.1016/B978-141603372-1.50018-4. ISBN   978-1-4160-3372-1. OCLC   460904299.
  17. ↑ Habermann, Christian R.; Weiss, Florian; Schoder, Volker; Cramer, Miriam C.; Kemper, Joern; Wittkugel, Oliver; Adam, Gerhard (2005). "MR Evaluation of Dural Ectasia in Marfan Syndrome: Reassessment of the Established Criteria in Children, Adolescents, and Young Adults". Radiology. 234 (2): 535–541. doi:10.1148/radiol.2342031497. ISSN   0033-8419. PMID   15616116.
  18. ↑ Shirley, Eric D.; Sponseller, Paul D. (September 2009). "Marfan Syndrome" . Journal of the American Academy of Orthopaedic Surgeons. 17 (9): 572–581. doi:10.5435/00124635-200909000-00004. PMID   19726741. Archived from the original on 4 May 2021.
  19. ↑ Wakely, Suzanne L. (2006). "The Posterior Vertebral Scalloping Sign". Radiology. 239 (2): 607–609. doi:10.1148/radiol.2392040224. ISSN   0033-8419. PMID   16641360.
  20. ↑ Ahn, Nicholas U.; Sponseller, Paul D.; Ahn, Uri M.; Nallamshetty, Leelakrishna; Kuszyk, Brian S.; et al. (June 2000). "Dural Ectasia Is Associated With Back Pain in Marfan Syndrome". Spine. 25 (12): 1562–1568. doi:10.1097/00007632-200006150-00017. PMID   10851107. 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. ↑ Farzam, Khashayar; Abdullah, Muhammad (2022). "Acetazolamide". StatPearls. PMID   30335315. Archived from the original on 3 May 2021.
  23. ↑ Cheuret, E.; Edouard, T.; Mejdoubi, M.; Acar, P.; Pienkowski, C.; et al. (1 April 2008). "Intracranial hypotension in a girl with Marfan syndrome: case report and review of the literature". Child's Nervous System. 24 (4): 509–513. doi:10.1007/s00381-007-0506-3. PMID   17906865. S2CID   5734726.
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