A comparison of the Berlin and Ghent nosologies and the influence of dural ectasia in the diagnosis of Marfan syndrome.
OBJECTIVE
To compare the Berlin and Ghent diagnostic criteria for Marfan syndrome and evaluate the utility of screening for dural ectasia in the diagnosis of Marfan syndrome.


METHODS
Review of clinical and radiographic data on 73 patients evaluated for Marfan syndrome at the National Institutes of Health.


RESULTS
Nineteen percent of patients diagnosed under the Berlin criteria failed to meet the Ghent standard. Dural ectasia was the second most common major diagnostic manifestation, and screening for dural ectasia established the diagnosis of Marfan syndrome in 23% of patients under the Ghent criteria.


CONCLUSIONS
Some patients are appropriately excluded from the diagnosis of Marfan syndrome by the Ghent criteria. Determination of dural ectasia is valuable in the diagnosis of Marfan syndrome.
Rose
P S
PS
National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland 20892-1852, USA. peterose@mail.mh.gov
Levy
H P
HP
Ahn
N U
NU
Sponseller
P D
PD
Magyari
T
T
Davis
J
J
Francomano
C A
CA
D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis* / Q000000981:diagnostic imaging; D009316:National Institutes of Health (U.S.); D011859:Radiography; D012189:Retrospective Studies; D014481:United States