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 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. ^ a b 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. ^ a b c 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. ^ a b 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. ^ a b 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. ^ a b 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. ^ a b c 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. ^ a b 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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