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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
AJNR. American Journal of Neuroradiology
|May 23, 2009
Summary
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.
