BACKGROUND AND PURPOSE: Dural ectasia (DE) is one of the major criteria of Marfan syndrome (MFS). Our aim was to establish the prevalence of DE in an adult population fulfilling the Ghent criteria for MFS and to assess definitions of DE. MATERIALS AND METHODS: One hundred five adults with suspected MFS were included. MR imaging at 1.5T was performed unless contraindicated; then CT was obtained. Lumbosacral anteroposterior vertebral body diameters (VBD) and dural sac diameters (DSD) were measured. Dural sac ratios (DSR = DSD/VBD) at levels L3 through S1 were calculated. Anterior meningoceles, herniations of nerve root sleeves, and scalloping were characterized. One hundred one sex- and age-matched patients were included as controls. RESULTS: We identified 3 patient groups: 1) fulfilling Ghent criteria independent of DE (n = 73), 2); fulfilling Ghent criteria dependent on DE (n = 14), and 3); and suspected MFS, not fulfilling Ghent criteria (n = 18). DE was found in 86% of group 1. At levels L4-S1, mean DSRs were significantly higher in group 1 than in group 3 and controls (P < .001). Herniations of the nerve root sleeves were present in 73% in group 1 versus 1% in controls. Anterior meningoceles were found in 37% and 14% in groups 1 and 2, respectively, but not in group 3 or controls. CONCLUSIONS: The diagnosis of DE on MR imaging or CT should be based on the presence of at least 1 of the following criteria: anterior meningoceles or nerve root sleeve herniation, DSD at S1 or below larger than DSD at L4, and DSR at S1 >0.59.
BACKGROUND AND PURPOSE:Dural ectasia (DE) is one of the major criteria of Marfan syndrome (MFS). Our aim was to establish the prevalence of DE in an adult population fulfilling the Ghent criteria for MFS and to assess definitions of DE. MATERIALS AND METHODS: One hundred five adults with suspected MFS were included. MR imaging at 1.5T was performed unless contraindicated; then CT was obtained. Lumbosacral anteroposterior vertebral body diameters (VBD) and dural sac diameters (DSD) were measured. Dural sac ratios (DSR = DSD/VBD) at levels L3 through S1 were calculated. Anterior meningoceles, herniations of nerve root sleeves, and scalloping were characterized. One hundred one sex- and age-matched patients were included as controls. RESULTS: We identified 3 patient groups: 1) fulfilling Ghent criteria independent of DE (n = 73), 2); fulfilling Ghent criteria dependent on DE (n = 14), and 3); and suspected MFS, not fulfilling Ghent criteria (n = 18). DE was found in 86% of group 1. At levels L4-S1, mean DSRs were significantly higher in group 1 than in group 3 and controls (P < .001). Herniations of the nerve root sleeves were present in 73% in group 1 versus 1% in controls. Anterior meningoceles were found in 37% and 14% in groups 1 and 2, respectively, but not in group 3 or controls. CONCLUSIONS: The diagnosis of DE on MR imaging or CT should be based on the presence of at least 1 of the following criteria: anterior meningoceles or nerve root sleeve herniation, DSD at S1 or below larger than DSD at L4, and DSR at S1 >0.59.
Authors: Benjamin B Vacula; Chelsea Gray; Michael P Hofkamp; Patrick T Noonan; Russell K McAllister; Kimberly A Pilkinton; Zhiying Diao Journal: Proc (Bayl Univ Med Cent) Date: 2016-10
Authors: Yskert von Kodolitsch; Julie De Backer; Helke Schüler; Peter Bannas; Cyrus Behzadi; Alexander M Bernhardt; Mathias Hillebrand; Bettina Fuisting; Sara Sheikhzadeh; Meike Rybczynski; Tilo Kölbel; Klaus Püschel; Stefan Blankenberg; Peter N Robinson Journal: Appl Clin Genet Date: 2015-06-16
Authors: M L A Daniels; J R Lowe; P Roy; M V Patrone; J M Conyers; J P Fine; M R Knowles; K R Birchard Journal: Clin Radiol Date: 2014-11-27 Impact factor: 2.350
Authors: Luigi Bassani; Christopher S Graffeo; Navid Behrooz; Vineet Tyagi; Taylor Wilson; Saul Penaranda; David Zagzag; Daniel B Rifkin; Mary Helen Barcellos-Hoff; Girish Fatterpekar; Dimitris Placantonakis Journal: Surg Neurol Int Date: 2014-01-21