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LETTER TO THE EDITOR
Year : 2018  |  Volume : 7  |  Issue : 2  |  Page : 154-155

Dural ectasia associated with type-1 neurofibromatosis


1 Department of Radiology, Guru Gobind Singh Medical College and Hospital, Baba Farid University of Health Sciences, Faridkot, Punjab, India
2 Department of Medicine, Guru Gobind Singh Medical College and Hospital, Baba Farid University of Health Sciences, Faridkot, Punjab, India

Date of Web Publication6-Jun-2018

Correspondence Address:
Dr. Paramdeep Singh
Department of Radiology, Guru Gobind Singh Medical College and Hospital, Baba Farid University of Health Sciences, Faridkot, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.233847

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How to cite this article:
Singh P, Kaur R. Dural ectasia associated with type-1 neurofibromatosis. J NTR Univ Health Sci 2018;7:154-5

How to cite this URL:
Singh P, Kaur R. Dural ectasia associated with type-1 neurofibromatosis. J NTR Univ Health Sci [serial online] 2018 [cited 2019 Aug 18];7:154-5. Available from: http://www.jdrntruhs.org/text.asp?2018/7/2/154/233847



Sir,

A 45-year-old female presented with a history of recurrent bouts of unbearable back pain limited to the sacral area for the last 4 years. There was no radiation of pain that generally resolved spontaneously with rest. Lately, she also developed urinary incontinence. Physical examination revealed neurocutaneous stigmata of type 1 neurofibromatosis (NF-1), comprising many neurofibromas, axillary freckling, and conspicuous café au lait spots. Magnetic resonance (MR) imaging of the lumbosacral spine (LS) was done, and the T2-weighted sagittal MR images exhibited dural ectasia [Figure 1] in the sacral region. There was enlargement of the dura with thinning of the adjoining sacrum anteriorly and posteriorly along with scalloping of posterior sacral bodies. Axial T2-weighted MR images through the level of the ectatic region [Figure 2] revealed that the dura extended minimally into the adjacent soft tissues as well as into the presacral space. There were also associated lateral meningoceles/herniations of the nerve root sleeves in the sacral region [Figure 2]. Because the patient was symptomatic, the neurosurgical repair of the dura was contemplated.
Figure 1: Sagittal T2-weighted MR image of the lumbar and sacral spine reveals extensive dural ectasia in sacral region, with expansion of the dura and thinning of the adjacent sacrum

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Figure 2: Axial T2-weighted MR image showing dural ectasia minimally extending into the adjacent soft tissues as well as into the presacral space. Small lateral meningoceles/herniations of the nerve root sleeves are also visible

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Dural ectasia is widening of the dural sac and the spinal canal and is occasionally associated with enlarged nerve root sleeves. It is defined as the enlargement of the dural sac, with the anteroposterior diameter of the thecal sac at the S1 level more than that of the thecal sac at the L4 level. Dural ectasia may occur in the spinal canal in any level, nevertheless, the most frequent sites are the lumbosacral regions where the cerebrospinal fluid (CSF) pressure is highest. Consequently, the most frequent clinical symptoms are low backache, headache, weakness, decreased sensations, intermittent rectal pain, and pain in the genital area.[1] The symptoms are worse mainly in the supine position and are comforted by lying on the back. Dural ectasia is rare in general population and is usually associated with Marfan syndrome, NF-1,  Ehlers-Danlos syndrome More Details, ankylosing spondylitis, and may occur idiopathically or after surgery. Spondylolisthesis, scoliosis, and vertebral erosions and/or fractures may accompany this condition. This condition may also present as an anterior sacral meningocele or as a pelvic mass.[2],[3],[4] MR imaging is the modality of choice for the assessment of dural ectasia because this modality is not only noninvasive but also has a better soft tissue resolution and can generate excellent multiplaner views of the spine. Fattori et al.[5] classified the severity of dural ectasia into 4 grades, namely, grade 0, normal; grade 1, mild dural ectasia with associated findings of small radicular cysts and/or effacement of epidural fat at the posterior aspect of one vertebral body; grade 2, expansion of the dural sac with occurrence of large radicular cysts and effacement of epidural fat at the posterior margin of two or more vertebral bodies; and grade 3, severe expansion of the dural sac with an anterior sacral meningocele.

Dural ectasia may be confused with arachnoid cysts that are CSF filled cystic lesions frequently seen on MR examinations. When dural ectasia spread out into the pelvis as an anterior sacral meningocele, it can simulate a pelvic mass on clinical and ultrasound examinations.[1],[4] Possibility of the dural ectasia should be considered in a patient with expanded spinal canal on MR examination, particularly in the lower lumbar and sacral regions. To conclude, the clinical signs and symptoms along with the imaging findings in this patient implied dural ectasia associated with NF-1 syndrome. This case demonstrates the significance of considering the possibility of dural ectasia in patients with NF-1 who have low back pain, radicular pain in the buttocks or legs, leg weakness, or urinary incontinence.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Ho NC, Hadley DW, Jain PK, Francomano CA. Case 47: Dural ectasia associated with Marfan syndrome. Radiology 2002;223:767-71.  Back to cited text no. 1
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2.
Lundby R, Rand-Hendriksen S, Hald JK, Lilleås FG, Pripp AH, Skaar S, et al. Dural ectasia in Marfan syndrome: A case control study. AJNR Am J Neuroradiol 2009;30:1534-40.  Back to cited text no. 2
    
3.
Foran JR, Pyeritz RE, Dietz HC, Sponseller PD. Characterization of the symptoms associated with dural ectasia in the marfan patient. Am J Med Genet A 2005;34:58-65.  Back to cited text no. 3
    
4.
Forsnes EV, Segna RA. Dural ectasia presenting as an adnexal mass. Obstet Gynecol 1996;88:712-3.  Back to cited text no. 4
[PUBMED]    
5.
Fattori R, Nienaber CA, Descovich B, Ambrosetto P, Reggiani LB, Pepe G, et al. Importance of dural ectasia in phenotypic assessment of Marfan's syndrome. Lancet 1999;354:910-3.  Back to cited text no. 5
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