Dural ectasia in children with Marfan syndrome: a prospective, multicenter, patient-control study.
The clinical diagnosis of Marfan syndrome in childhood is difficult, because symptoms may not have developed to their full expression until adulthood. The Ghent nosology for the diagnosis of Marfan syndrome classifies dural ectasia as a major diagnostic criterion. More than two thirds of adult patients with Marfan syndrome show dural ectasia, while the frequency in childhood is unknown. This prospective multicenter observational patient-control study was performed to identify pathologic changes of the lumbosacral spine in young patients with Marfan syndrome.


METHODS
Prospective clinical trial, multicentric, cross-sectional.


METHODS
MRI of the lumbosacral spine.


METHODS
Twenty patients with proven Marfan syndrome, 20 patients suspicious for Marfan syndrome and 38 healthy controls.


METHODS
Vertebral body diameter (VBD) from L1 to S1, dural sac diameter (DSD) from L1 to S1, dural sac ratio (DSR), qualitative assessment of the lumbosacral spine.


RESULTS
DSD and VBD in different age groups were higher in patients with proven or suspected Marfan syndrome than in healthy controls (DSD: L1, 6-8 years, P < 0.05). VBD related to body height showed a similar growth related increase in patients with proven or suspected Marfan syndrome and controls. DSD related to body height was elevated in patients with proven or suspected Marfan syndrome at different levels of the lumbar spine. DSD at levels L1, L5, and S1, and DSR at levels L5 and S1 of patients with proven Marfan syndrome were significantly higher (P < 0.05) than in controls.


CONCLUSIONS
Even during childhood pathologic changes inside the lumbosacral spine of patients with Marfan syndrome can be observed. Dural ectasia, which occurs at different levels of the lumbar spine, can be detected at levels L5 and S1 in up to 40% of patients with Marfan syndrome.
Knirsch
Walter
W
Division of Pediatric Cardiology, Children's University Hospital Zurich, Zurich, Switzerland. walter.knirsch@kispi.unizh.ch
Kurtz
Claudia
C
Häffner
Nicole
N
Binz
Gudrun
G
Heim
Peter
P
Winkler
Peter
P
Baumgartner
Daniela
D
Freund-Unsinn
Karin
K
Stern
Heiko
H
Kaemmerer
Harald
H
Molinari
Luciano
L
Kececioglu
Deniz
D
Uhlemann
Frank
F
D000293:Adolescent; D001827:Body Height; D002648:Child; D002675:Child, Preschool; D004108:Dilatation, Pathologic; D004388:Dura Mater / Q000473:pathology*; D005260:Female; D006801:Humans; D008159:Lumbar Vertebrae / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008382:Marfan Syndrome / Q000473:pathology* / Q000503:physiopathology; D011446:Prospective Studies