Performance of a new quantitative method for assessing dural ectasia in patients with FBN1 mutations and clinical features of Marfan syndrome.
BACKGROUND
This study presents a comparison of established methods for measuring dural ectasia with a new quantitative method of assessing this clinical feature.


METHODS
Seventeen patients with an identified mutation in FBN1 were examined for dural ectasia. The results were compared with 17 age- and sex-matched controls. Our images were also evaluated using the two methods of quantifying dural ectasia, namely those of Ahn et al. and of Oosterhof et al.


RESULTS
With our method, 80% MFS1 patients and 7% controls fulfilled the criterion for dural ectasia. Using the method of Oosterhof et al., dural ectasia was found in 88% patients with MFS1 and in 47% controls. Using the method of Ahn et al. 76% patients with Marfan syndrome and 29% controls showed dural ectasia.


CONCLUSIONS
We present a novel quantitative method of evaluating MRT images for dural ectasia, which, in our own patient cohort, performed better than those previously described.
Söylen
Bahar
B
Institute of Human Genetics, Hannover Medical School, Carl-Neuberg-Strasse 1, 30625, Hannover, Germany.
Hinz
Kerstin
K
Prokein
Jana
J
Becker
Hartmut
H
Schmidtke
Jörg
J
Arslan-Kirchner
Mine
M
D000328:Adult; D004108:Dilatation, Pathologic / Q000175:diagnosis; D004388:Dura Mater / Q000473:pathology*; D005260:Female; D000071838:Fibrillin-1; D000071837:Fibrillins; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D007090:Image Interpretation, Computer-Assisted / Q000379:methods*; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis* / Q000235:genetics*; D008840:Microfilament Proteins / Q000235:genetics*; D008875:Middle Aged; D009154:Mutation; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D055815:Young Adult; D054392:Adipokines
D000071838:Fibrillin-1; D000071837:Fibrillins; D008840:Microfilament Proteins; C000605722:FBN1 protein, human; D054392:Adipokines