title: Dural ectasia in children with Marfan syndrome: a prospective, multicenter, patient-control study.
abstract: 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.
authors.affiliation: Division of Pediatric Cardiology, Children's University Hospital Zurich, Zurich, Switzerland. walter.knirsch@kispi.unizh.ch
authors.forename: Walter
authors.initials: W
authors.lastname: Knirsch
authors.forename: Claudia
authors.initials: C
authors.lastname: Kurtz
authors.forename: Nicole
authors.initials: N
authors.lastname: Häffner
authors.forename: Gudrun
authors.initials: G
authors.lastname: Binz
authors.forename: Peter
authors.initials: P
authors.lastname: Heim
authors.forename: Peter
authors.initials: P
authors.lastname: Winkler
authors.forename: Daniela
authors.initials: D
authors.lastname: Baumgartner
authors.forename: Karin
authors.initials: K
authors.lastname: Freund-Unsinn
authors.forename: Heiko
authors.initials: H
authors.lastname: Stern
authors.forename: Harald
authors.initials: H
authors.lastname: Kaemmerer
authors.forename: Luciano
authors.initials: L
authors.lastname: Molinari
authors.forename: Deniz
authors.initials: D
authors.lastname: Kececioglu
authors.forename: Frank
authors.initials: F
authors.lastname: Uhlemann
mesh_terms: 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