Dural ectasia
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]
Dural ectasia | |
---|---|
Spinal cord | |
Specialty | Neurology |
Signs and symptoms
Most common symptoms include lower back pain,[2] headaches,[2] weakness,[2] numbness (anesthesia) above and below the involved limb,[2] leg pain,[3] and sometimes there can be rectal[2] and genital pain.[2] Bowel and bladder dysfunction, urinary retention or even incontinence may occur. [4]
The symptoms are usually exacerbated by upright posture and often but not always relieved by lying down. However, in many patients it is asymptomatic.
Causes
It is common in Marfan syndrome,[3] occurring in 63–92% of people with the syndrome.[5] Dural ectasia may also occur in Ehlers-Danlos Syndrome,[1] neurofibromatosis type I,[1] ankylosing spondylitis,[1] and trauma.
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. [6]
Diagnosis
Diagnosis is usually conducted by MRI or CT Scan.
Treatment
Acetazolamide has been demonstrated in drug trials to relieve symptoms associated with dural ectasia in individuals with Marfan's syndrome. The majority of dural ectasia do not require surgery and can be treated conservatively. [7]
References
- 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.
- Altman, A; Uliel, L; Caspi, L (March 2008). "Dural ectasia as presenting symptom of Marfan syndrome" (PDF). The Israel Medical Association Journal : IMAJ. 10 (3): 194–5. PMID 18494231.
- 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.
- 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.
- Lacassie, HJ; Millar, S; Leithe, LG; Muir, HA; Montaña, R; Poblete, A; Habib, AS (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.
- Polster SP, Dougherty MC, Zeineddine HA, et al. Dural Ectasia in Neurofibromatosis 1: Case Series, Management, and Review. Neurosurgery. 2020;86(5):646–655. doi:10.1093/neuros/nyz244
- Polster SP, Dougherty MC, Zeineddine HA, et al. Dural Ectasia in Neurofibromatosis 1: Case Series, Management, and Review. Neurosurgery. 2020;86(5):646–655. doi:10.1093/neuros/nyz244