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AJNR - American Journal of Neuroradiology

Published ahead of print on May 20, 2009
doi: 10.3174/ajnr.A1620

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American Journal of Neuroradiology 30:1534-1540, September 2009
© 2009 American Society of Neuroradiology

SPINE

Dural Ectasia in Marfan Syndrome: A Case Control Study

R. Lundby^a,g, S. Rand-Hendriksen^d,g, J.K. Hald^a, F.G. Lilleås^e, A.H. Pripp^b, S. Skaar^a, B. Paus^f, O. Geiran^c,g and H.-J. Smith^a,g

^aFrom the Department of Radiology (R.L., J.K.H., S.S., H.-J.S.)
^bSection of Biostatistics, Research Services Department (A.H.P.)
^cDivision for Cardiac and Respiratory Medicine and Surgery (O.G.), Oslo University Hospital, Rikshospitalet, Oslo, Norway
^dTRS National Resource Centre for Rare Disorders (S.R.-H.), Sunnaas Rehabilitation Hospital, Nesoddtangen, Norway
^eCurato A/S (F.G.L.), Oslo, Norway
^fDepartment of Medical Genetics (B.P.), Oslo University Hospital, Ullevaal, Oslo, Norway
^gFaculty Divison Rikshospitalet (R.L., S.R.-H., O.G., H.-J.S.), University of Oslo, Oslo, Norway.

Please address correspondence to Rigmor Lundby, MD, Department of Radiology, Oslo University Hospital, Rikshospitalet, Sognsvannsveien 20, NO-0027 Oslo, Norway; e-mail: rigmor.lundby{at}medisin.uio.no

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.