doi:10.1016/j.crad.2006.05.015 
Copyright © 2006 The Royal College of Radiologists Published by Elsevier Ltd.
Evaluation of three different measurement methods for dural ectasia in Marfan syndrome
E. Weiganga, 
, 
, N. Ghanemb, X.-C. Changa, H. Richtera, A. Frydrychowiczb, G. Szabóc, O. Dudeckd, W. Knirsche, P. von Samsona, M. Langerb and F. Beyersdorfa
aDepartments of Cardiovascular Surgery
bDiagnostic Radiology, University Hospital Freiburg, Germany
cDepartment of Cardiac Surgery, University Hospital Heidelberg, Germany
dDepartment of Radiology, Campus Rudolf-Virchow, Charité, University Hospital Berlin, Germany
eDivision of Paediatric Cardiology, Children's University Hospital Zurich, Switzerland
Received 21 April 2006;
revised 26 May 2006;
accepted 31 May 2006.
Available online 30 September 2006.
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Aim
Dural ectasia is a major diagnostic criterion for Marfan syndrome using the Ghent nosology. Our aim was to evaluate the efficacy of three different radiological methods previously proposed for the assessment of dural sac diameter in Marfan syndrome.
Methods
Marfan syndrome was diagnosed in our study using the Ghent criteria, disregarding dural ectasia as a criterion. Three proposed radiological methods were applied to measure dural sac diameter, examined for 41 patients (18 patients with and 23 without Marfan syndrome) by computed tomography or magnetic resonance imaging.
Results
Using Oosterhof's method, 94% of the patients with and 44% of the patients without Marfan syndrome fulfilled the criteria of dural ectasia. According to Villeirs, dural ectasia was diagnosed in 18% of the patients with and in none of the patients without Marfan syndrome. With Ahn's method, dural ectasia was found in 72% of the patients with and in 44% of the patients without Marfan syndrome. In only two patients with Marfan syndrome was dural ectasia diagnosed by all three methods.
Conclusion
Our results reveal overt discrepancy between the three methods of assessing dural ectasia. Considering the key role played by dural ectasia in reinforcing the diagnosis of Marfan syndrome according to the Ghent nosology, a standardized and reliable method should be sought.
Figure 1. Measuring method according to Oosterhof et al.1 (a) Measurement of diameters of vertebral bodies (VBD). (b) Measurement of dural sac diameters (DSD). Anteroposterior VBD and anteroposterior DSD measured perpendicular to long axis of vertebral body and dural sac.
Figure 2. Measuring method according to Villeirs et al.2 (a) Measurement at L1–4 (L3), transverse diameters of vertebral body, spinal canal and dural sac. (b, c) At L5, transverse diameters of only the spinal canal and dural sac. (d) Measurement at S1; transverse laterolateral diameter of spinal canal and dural sac, transverse (laterolateral) diameter of spinal canal (lls), vertebral body (LL) and dural sac (lld).
Figure 3. Measuring method according to Ahn et al.3 and 4 (a) Midline sagittal reconstruction from multi-source correlator tracker of case of Marfan syndrome and clear evidence of dural ectasia. Scalloping measured by average widths of superior and inferior endplates of vertebral bodies and subtracting width at midsection of vertebral body. (b) Evaluation of axial section at L 5 demonstrates nerve root sleeve diameter of 7.7 mm.
Figure 4. Dural ectasia measurement according to Oosterhof et al.,1 Villeirs et al.2 and Ahn et al.3 and 4; n = number of patients with Marfan syndrome and dural ectasia. The intersection illustrates corresponding diagnoses concerning dural ectasia.
Figure 5. Dural ectasia measurement according to Oosterhof et al.,1 Villeirs et al.2 and Ahn et al.3 and 4; n = number of patients without Marfan syndrome and with dural ectasia. The intersection illustrates corresponding diagnoses concerning dural ectasia.
Table 1.
Ghent nosologya
MFS, Marfan syndrome.
a Prerequisites for diagnosing Marfan syndrome in index patient by Ghent nosology: (a) negative family history and missing proof of FBN1-mutation, one major criterion present in at least two organ systems with participation of third organ system; (b) proof of FBN1-mutation, one major criterion in one organ system plus involvement of a second organ system, the FBN1-mutation must be known as causing Marfan syndrome within the family or worldwide; (c) for relative, one major criterion within family history plus genetic findings and one major criterion in one organ system with participation of a second organ system.
Table 2.
Dimensions of dura mater measured at L1–S1 in 41 participants using the method of Oosterhof et al. and CT- and MRI-based data
DSR, dural sac ratio.
Table 3.
Dimensions of dura mater measured in 37 participants using the method of Villeirs et al. and CT- and MRI-based data
MFS, 17 participants with Marfan syndrome; non-MFS, 20 participants without Marfan syndrome; SCI, spinal canal index; DSI, dural sac index.
Table 4.
Dimensions of dura mater measured in 41 participants using the method of Ahn et al. and CT- and MRI-based data
MFS, 18 participants with Marfan syndrome; non-MFS, 23 participants without Marfan syndrome; SCD, diameter of spinal canal.
Table 5.
Comparison of the Oosterhof, Villeirs and Ahn methods
MFS, participants with Marfan syndrome, number (%); non-MFS, participants without Marfan syndrome, number (%).
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