Dural ectasia in children with Marfan syndrome: a prospective, multicenter, patient-control study

American Journal of Medical Genetics. Part a
Walter KnirschFrank Uhlemann

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. Prospective clinical trial, multicentric, cross-sectional. MRI of the lumbosacral spine. Twenty patients with proven Marfan syndrome, 20 patients suspicious for Marfan syndrome and 38 healthy controls. 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. 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...Continue Reading

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Citations

Oct 2, 2007·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·E CheuretY Chaix
Mar 19, 2009·European Journal of Human Genetics : EJHG·Svend Rand-HendriksenOdd Geiran
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