Evaluation of three different measurement methods for dural ectasia in Marfan syndrome☆

doi:10.1016/j.crad.2006.05.015

Clinical Radiology

Volume 61, Issue 11, November 2006, Pages 971-978

https://doi.org/10.1016/j.crad.2006.05.015 Get rights and content

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.

Introduction

According to the Ghent nosology, dural ectasia represents a major diagnostic criterion of Marfan syndrome (Table 1), occurring in 63% to 92% of adults with Marfan syndrome.1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11 Dural ectasia is defined as a dilatation of the dural sac with subsequent expansion of the spinal canal. Dural ectasia can develop at any level of the spinal column, but is mainly found within the lumbosacral region. Assessment of dural ectasia as proposed by Oosterhof et al. is based on cut-off values, i.e. anteroposterior vertebral body diameters (VBD) and anteroposterior dural sac diameters (DSD), at the levels of L3 and S1 as evaluated by magnetic resonance imaging (MRI).¹ Villeirs et al. defined dural ectasia as an increased ratio (>3.75) of transverse laterolateral diameter of dural sac (lld) and vertebral body (LL) on computed tomography (CT).² Ahn et al. defined the dural sac volume estimated by MRI as the gold standard of dural ectasia measurement.3, 4 The dural sac volume was measured caudal to the level of the inferior endplate of the L5 vertebral body. Alternatively, he analysed dural ectasia by CT and characterised it as a larger sagittal diameter of the spinal canal at the level of S1 (or below) than at the level of L4 (or above). In view of the large amount of false-negative diagnoses as compared with the gold standard (dural sac volume measurement by MRI), Ahn et al. developed additional criteria for the precise diagnosis of dural ectasia,3, 4 which required the presence of one major criterion and two minor criteria. Major criteria include a larger diameter of the spinal canal at the level of S1 than at the level of L4 and the existence of an anterior meningocele. Subordinate factors include scalloping at the level of S1 and increased nerve root sleeve diameter at L5.

The measuring method of Fattori et al.⁵ classifies the extent of changes in the dura mater as follows: 0 = normal, without alterations of the dural sac; 1 = mild dural ectasia with a bulge of the dural sac, absence of epidural fat tissue on the posterior part of one vertebra and small radicular cysts; 2 = moderate dural ectasia with a bulge of the dural sac, absence of epidural fat tissue on the posterior part of one or more vertebrae and large radicular cysts; 3 = extreme dural ectasia with anterior sacral meningoceles. This approach is based on descriptive, non-quantitative criteria and therefore was not used in our study.

As dural ectasia is a major diagnostic criterion for Marfan syndrome when using the Ghent nosology,¹² its identification can be decisive in diagnosis. The aim of this study was to investigate and compare the practicability and clinical usefulness of three radiological methods previously proposed for the assessment of DSD.

Section snippets

Materials and methods

Of 41 participants in our study, 30 underwent CT and 11 underwent MRI in order to detect dural ectasia, from January 2005 to August 2005 at the University Hospital Freiburg. The same measurements were obtained for all three of the above methods using MRI and CT. Marfan syndrome had already been confirmed in 18 cases (age 19 to 66 years) according to other criteria of the Ghent nosology (Table 1), and 23 vascular patients without Marfan syndrome were used as a control group (age 17 to 56 years).

Results

According to the method of Oosterhof et al., a diagnosis of dural ectasia was possible for 17 participants (94%) with, and for 10 participants (44%) without Marfan syndrome.¹ The average DSR for those with Marfan syndrome was above the cut-off value at the level of L3 and S1. For those without Marfan syndrome, the average DSR did not exceed the cut-off value at L3 and S1 (Table 2).

The method of Villeirs et al. assesses the SCI and DSI, the latter a sufficient criterion for the finding of dural

Discussion

The Ghent nosology proposes that dural ectasia is a major criterion of great importance in diagnosing Marfan syndrome.1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13 Our cases with Marfan syndrome met enough other criteria of the Ghent nosology, so that dural ectasia was not necessary for the diagnosis. The literature describes varying radiological measuring methods to detect dural ectasia.1, 2, 3, 4, 5 In this study, using CT and MRI, we evaluated the methods of Oosterhof et al.,¹ Villeirs et al.²

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Le diagnostic de syndrome de Marfan est porté à partir d’un ensemble de critères majeurs et mineurs, en rapport avec les différents systèmes impliqués selon la nosologie de Ghent. L’imagerie du rachis peut diagnostiquer à la fois une atteinte squelettique, notamment une scoliose et un spondylolisthésis et une atteinte neurologique, ectasie durale. L’objectif de notre travail était d’évaluer l’intérêt d’un examen du rachis par la radiographie conventionnelle et par une imagerie complémentaire scanner ou imagerie par résonance magnétique nucléaire chez les patients suspects de syndrome de Marfan et de modéliser la procédure d’imagerie la plus pertinente pour diagnostiquer un tel syndrome.
Évaluation de la sensibilité et de la spécificité de la radiographie standard du rachis lombosacré, en comparaison à l’imagerie en coupes pour la mise en évidence de l’ectasie durale dans un sous-groupe de 92 patients chez qui on suspecte un syndrome de Marfan. Analyse rétrospective sur la place de la radiographie standard dans le diagnostic chez 1992 patients admis à notre consultation.
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The diagnosis of Marfan syndrome (MFS) is defined by a combination of major and minor criteria, related to the different systems involved, according to the Ghent nosology of the spine. Spinal imaging can detect both skeletal (including scoliosis and spondylolisthesis) and neurological involvement (i.e. dural ectasia). The aim of the present study was to assess the interest of screening the rachis by conventional radiography CR and complementary imaging (computed tomography [CT] or magnetic resonance imaging [MRI]) in patients suspected of MFS, and to modelise the most relevant imaging procedure to diagnose MFS.
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Citation Excerpt :
Weigang and colleagues [35] and others have evaluated the efficacy of the MRI and CT criteria proposed for the diagnosis of DE (Table 1). Weigang and colleagues [35] found only two patients who had MFS who satisfied all criteria for DE using the three methods proposed by Villeirs and colleagues, Oosterhof and colleagues, and Ahn and colleagues in a group of 18 individuals who had MFS and 23 individuals who did not have MFS. Moreover, DE was found in up to 44% of normal controls.
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^☆: Presented at the 24th International Cardiovascular Surgical Symposium, 4–11 March 2006, Zürs, Austria.

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Evaluation of three different measurement methods for dural ectasia in Marfan syndrome☆☆

Aim

Methods

Results

Conclusion

Introduction

Section snippets

Materials and methods

Results

Discussion

Genet Med

Lancet

Lancet

Genet Med

Quantitative assessment of dural ectasia as a marker for Marfan syndrome

Radiology

Widening of the spinal canal and dural ectasia in Marfan’s syndrome: assessment by CT

Neuroradiology

Dural ectasia is associated with back pain in Marfan syndrome

Spine

Characterization of the symptoms associated with dural ectasia in the Marfan patient

Am J Med Genet A

Evaluation of three different measurement methods for dural ectasia in Marfan syndrome^☆☆