Evaluation of three different measurement methods for dural ectasia in Marfan syndrome☆☆
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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Presented at the 24th International Cardiovascular Surgical Symposium, 4–11 March 2006, Zürs, Austria.