Quentin Chuzel, Sophie Dupuis-Girod1, Matthieu Rousset, Charlotte Decharry2, Evelyne Decullier3, Jean-Baptiste Pialat4. 1. Service de Génétique Clinique, Hôpital Louis Pradel, Hospices Civils de Lyon, Bron. 2. Hôpital d'Instruction des Armées Desgenettes. 3. Service de Recherche Clinique, Hospices Civils de Lyon, Lyon. 4. Service de Radiologie Centre Hospitalier Lyon sud, Hospices Civils de Lyon, Pierre-Bénite, France.
Abstract
OBJECTIVE: The aim of this study was to reevaluate dural ectasia criteria in Marfan syndrome patients fulfilling the revised Ghent criteria. METHODS: Lumbar computed tomography scans of 19 Marfan patients and 30 matched control subjects were retrospectively assessed. Dural sac ratio (DSR), nerve root sleeve diameter, pedicle width, and a scalloping or meningocele presence were each assessed by 2 readers blinded from the diagnosis. Mann-Whitney-Wilcoxon tests compared the patient and control groups. Receiver operating characteristic curve analysis and multivariate models determined the optimal cutoff value. RESULTS: A DSR value greater than 0.69 at L5 (DSR-L5) such as L4 scalloping of more than 2.65 mm (scall-L4) and 6 or more vertebrae showing a scalloping of more than 3 mm (6-scall) were found very specific but with limited sensitivity. Multivariate model combining DSR-L5 + scall-L4 showed good positive predictive value, whereas model combining DSR-L5 + 6-scall showed good negative predictive value. CONCLUSIONS: Assessment of DSR and vertebral scalloping allows valuable depiction of dural ectasia in Marfan syndrome patients.
OBJECTIVE: The aim of this study was to reevaluate dural ectasia criteria in Marfan syndromepatients fulfilling the revised Ghent criteria. METHODS: Lumbar computed tomography scans of 19 Marfan patients and 30 matched control subjects were retrospectively assessed. Dural sac ratio (DSR), nerve root sleeve diameter, pedicle width, and a scalloping or meningocele presence were each assessed by 2 readers blinded from the diagnosis. Mann-Whitney-Wilcoxon tests compared the patient and control groups. Receiver operating characteristic curve analysis and multivariate models determined the optimal cutoff value. RESULTS: A DSR value greater than 0.69 at L5 (DSR-L5) such as L4 scalloping of more than 2.65 mm (scall-L4) and 6 or more vertebrae showing a scalloping of more than 3 mm (6-scall) were found very specific but with limited sensitivity. Multivariate model combining DSR-L5 + scall-L4 showed good positive predictive value, whereas model combining DSR-L5 + 6-scall showed good negative predictive value. CONCLUSIONS: Assessment of DSR and vertebral scalloping allows valuable depiction of dural ectasia in Marfan syndromepatients.