PURPOSE: Marfan syndrome (MFS) is a genetic disorder of the connective tissue. 
Aortic root dilation is a main criterion of the Ghent Nosology. Dural ectasia 
and the presence of mitral valve prolapse (MVP) contribute to its systemic 
score. The purpose of this study was to investigate the frequency of dural 
ectasia and its correlation with cardiovascular manifestations in a pediatric 
study population.
PATIENTS AND METHODS: 119 pediatric patients with confirmed or suspected MFS 
were examined in the local Marfan Clinic. 31 children with MFS who underwent 
magnetic resonance imaging (MRI) were included. Each patient was evaluated 
according to the Ghent nosology. Echocardiography was used to measure the aortic 
root diameter and assess the presence of MVP and mitral regurgitation. Z-scores 
were calculated for the evaluation of the aortic root diameters. MRI was 
performed to determine the dural sac ratio (DSR).
RESULTS: The prevalence of dural ectasia was 90.3 %, of aortic root dilation 
32.2 %, of MVP 64.5 % and of mitral regurgitation 51.6 %. DSR at L5 correlated 
with the intraindividual z-scores (slope, 3.62 ± 1.5 [0.56; 6.68]; r = 0.17; 
p = 0.02; F = 5.84). Z-scores ≥ 2 were accompanied by dural ectasia in 100 %, 
MVP in 95 % and mitral regurgitation in 100 % of cases. MVP was accompanied by 
mitral regurgitation in 70 % of cases.
CONCLUSION: As the examined cardiac manifestations show a coincidence with dural 
ectasia in 95 - 100 % of cases, MRI for diagnostic dural sac imaging should be 
reserved for MFS suspicions with the absence of those manifestations in order to 
establish the diagnosis according to the Ghent criteria. Thus, the present study 
supports the recent downgrading of dural ectasia to a contributor to the 
systemic score.