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Research ArticleSpine Imaging and Spine Image-Guided Interventions

Dural Ectasia in Marfan Syndrome: A Case Control Study

R. Lundby, S. Rand-Hendriksen, J.K. Hald, F.G. Lilleås, A.H. Pripp, S. Skaar, B. Paus, O. Geiran and H.-J. Smith
American Journal of Neuroradiology September 2009, 30 (8) 1534-1540; DOI: https://doi.org/10.3174/ajnr.A1620
R. Lundby
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S. Rand-Hendriksen
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J.K. Hald
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F.G. Lilleås
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A.H. Pripp
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S. Skaar
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B. Paus
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O. Geiran
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H.-J. Smith
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    Fig 1.

    Sagittal T2-weighted MR image of a patient with MFS showing a huge anterior meningocele herniating into the pelvis through a large sacral defect (arrows). The urinary bladder (asterisk) is compressed anteriorly.

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    Fig 2.

    Coronal T2-weighted MR image showing lateral meningoceles/herniations (arrows) of the nerve root sleeves at level S1 in a patient with MFS.

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    Fig 3.

    A and B, T2-weighted sagittal MR images of a patient with MFS showing craniocaudal and AP measurements of the vertebral bodies (white arrows, A and B) and AP measurements of the dural sac (black arrows, B). Scalloping is present at levels L5, S1, and S2.

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    Fig 4.

    ROC curves of DSR at levels L3-S1. Graph shows sensitivity versus (1-specificity) of DSR as a marker of MFS at levels L3-S1 when comparing Ghent-positive patients independent of DE and controls.

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    Table 1:

    Characteristics of the 4 study groups or the number and percentage of patients with the condition*

    CharacteristicsGhent Independent of DE (n = 73)Ghent Dependent on DE (n = 14)Not Fulfilling Ghent (n = 18)Control (n = 101)P Value
    Age (mean ± SD)39.4 (12.2)40.6 (17.1)36.1 (13.4)39.6 (12.9).74
    Females (No.) (%)45 (61.6)11 (78.6)11 (61.6)64 (63.4).68
    DSR L3 (mean ± SD)0.50b,c (0.11), n = 690.53c (0.06)0.43a (0.08), n = 140.45a,b (0.07)<.001
    DSR L4 (mean ± SD0.50b (0.12), n = 720.51b (0.07)0.41a (0.06)0.43a (0.07)<.001
    DSR L5 (mean ± SD)0.57b (0.18), n = 680.65b (0.20)0.39a (0.09)0.42a (0.08)<.001
    DSR S1 (mean ± SD)0.94b (0.69), n = 611.02b (0.77), n = 130.40a (0.12), n = 120.41a (0.13), n = 96<.001
    DSD sacrum > DSD L438 (55.1%), n = 694 (28.6%)1 (5.6%)5 (5.0%)<.001
    Herniation of nerve root sleeves
        Present in ≥1 level (No.) (%)53 (72.6%)10 (71.4%)2 (11.1%)1 (1%)<.001
        Present in level L59 (12.3%)0 (0%)0 (0%)0 (0%)<.001
        Present in level S135 (47.9%)8 (57.1%)2 (11.1%)0 (0%)<.001
        Present in level S248 (65.8%)8 (57.1%)1 (5.6%)1 (1%)<.001
        Present in level S330 (41.1%)5 (35.7%)0 (0%)0 (0%)<.001
        Present in level S48 (11%)1 (7.1%)0 (0%)0 (0%)<.004
    Odds ratio, ≥1 herniation of the nerve root sleeve265 (34–2029)250 (25–2458)12.5 (1–145)ref<.001
    Anterior meningocele27 (37%)2 (14%)00<.001
    Scalloping L1-S1 ≥1 level44 (61.1 %), n = 72 8 (42.9%) 1 (5.6%)4 (4%), n = 100<.001
    Perineural cysts11 (15.1 %) 1 (7.1%) 1 (5.6%)8 (7.9%).387
    Disk herniation beneath T12 5 (7%), n = 69 1 (7%) 2 (11%)6 (6%).890
    Caudal end of dural sac
        L5, L5/S1, S110 (14.5%) 0 (0%) 9 (50%)22 (21.8%)<.001
        S226 (37.7%) 9 (64.3%) 8 (44.4%)68 (67.3%)<.001
        S3, S4, S533 (47.8%), n = 69 5 (35.7%) 1 (5.6%)11 (10.9%)<.001
    • Note:—DE, dural ectasia; DSR, dural sac ratio; DSD, dural sac diameter.

    • * Study groups not containing similar letters (a, b, c, or d) for means were statistically different at the 5% level. Where n is specified in a cell, there were some observations missing and n is the actual number of observations. Missing values are due to incomplete anatomic coverage with CT, extreme scoliosis at MR, or the dural sac ending above the missed level.

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    Table 2:

    Prevalence of signs of enlarged dural sac in the 4 groups*

    CriteriaFulfilling Ghent (No.) (%)
    Independent of DE (n = 73)Dependent on DE (n = 14)Suspected MFS, Not Fulfilling Ghent (n = 18)Controls (n = 101)
    1) Anterior meningocele27 (37)2 (1400%0%
    2) DSD sacrum > DSD L438 (52)4 (29)1 (6)5 (5)
    3) Herniations of ≥1 nerve root sleeves53 (73)10 (71)2 (11)1 (1)
    4) DSR L5 > 0.48 (Oosterhof et al6)41 (56)13 (93)1 (6)19 (19)
    5) DSR S1 > 0.57 (Oosterhof et al6)45 (62)11 (85)1 (6)12 (12)
    Presence of ≥1 of above findings65 (89)14 (100)3 (17)24 (24)
    Only DSR L5 > 0.48 and/or S1 > 0.574 (5)4 (29)1 (6)18 (18)
    6) DSR S1 > 0.59 (current study)43 (59)11 (85)1 (6)7 (7)
    Presence of DE replacing criteria 4 and 5 with 663 (86)12 (86)3 (17)9 (9)
    • Note:—MES indicates Marfan Syndrome.

    • * * Expressed as number and percentage.

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American Journal of Neuroradiology: 30 (8)
American Journal of Neuroradiology
Vol. 30, Issue 8
1 Sep 2009
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Cite this article
R. Lundby, S. Rand-Hendriksen, J.K. Hald, F.G. Lilleås, A.H. Pripp, S. Skaar, B. Paus, O. Geiran, H.-J. Smith
Dural Ectasia in Marfan Syndrome: A Case Control Study
American Journal of Neuroradiology Sep 2009, 30 (8) 1534-1540; DOI: 10.3174/ajnr.A1620

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Dural Ectasia in Marfan Syndrome: A Case Control Study
R. Lundby, S. Rand-Hendriksen, J.K. Hald, F.G. Lilleås, A.H. Pripp, S. Skaar, B. Paus, O. Geiran, H.-J. Smith
American Journal of Neuroradiology Sep 2009, 30 (8) 1534-1540; DOI: 10.3174/ajnr.A1620
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