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Dural ectasia in Marfan syndrome: a case control study
R Lundby1, S Rand-Hendriksen, J K Hald
1Department of Radiology, Rikshospitalet, Sognsvannsveien 20, Oslo, Norway. rigmor.lundby@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.
Insights
Dural ectasia (DE) is common in Marfan syndrome (MFS) and can be diagnosed using specific imaging criteria. This study establishes prevalence and refines diagnostic definitions for DE in MFS patients.
Area of Science:
- Radiology
- Genetics
- Orthopedics
Background:
- Dural ectasia (DE) is a key diagnostic criterion for Marfan syndrome (MFS).
- Accurate diagnosis of DE is crucial for MFS management.
- Prevalence and diagnostic definitions of DE require further clarification.
Purpose of the Study:
- To determine the prevalence of DE in adults meeting Ghent criteria for MFS.
- To evaluate and propose standardized definitions for diagnosing DE.
- To assess imaging findings associated with DE in suspected MFS cases.
Main Methods:
- 105 adults with suspected MFS underwent MR or CT imaging.
- Lumbosacral vertebral and dural sac diameters were measured.
- Dural sac ratios and specific DE features (meningoceles, herniations) were analyzed.
Main Results:
- DE was identified in 86% of patients fulfilling Ghent criteria.
- Significantly higher dural sac ratios were observed in MFS patients compared to controls.
- Anterior meningoceles and nerve root sleeve herniations were frequent in MFS patients.
Conclusions:
- Proposed diagnostic criteria for DE include anterior meningoceles, nerve root sleeve herniation, or specific dural sac ratio thresholds.
- These criteria can aid in the diagnosis of DE in MFS.
- Imaging findings provide valuable insights into DE in MFS patients.
