Literature DB >> 24477527

Spinal imaging features in Japanese patients with Marfan syndrome: a case-control study.

Eri Hayashida1, Daisuke Utsunomiya, Akira Sasao, Tsuyoshi Yasuda, Toshinori Hirai, Hideaki Yuki, Seitaro Oda, Joji Urata, Akihiko Arakawa, Yasuyuki Yamashita.   

Abstract

PURPOSE: The purpose of our study was to evaluate the morphology of the lumbosacral spine, i.e. the dura and vertebral body shape, of Japanese patients with Marfan syndrome (MFS) by comparing it with sex- and age-matched controls.
MATERIALS AND METHODS: Spinal MR or CT images of 32 MFS patients and 32 controls were retrospectively reviewed. The anteroposterior dural sac diameter (DSD), anteroposterior vertebral body diameter (VBD), and vertebral body height (VBH) were measured from L1 to S1 levels and the dural sac ratio [DSR = (DSD/VBD)] and vertebral body aspect ratio [VAR = (VBH/VBD)] were calculated.
RESULTS: At each level, mean DSD and DSR were significantly higher in MFS patients; VBD was not. The cutoff values for DSR to differentiate between MFS patients and the controls were 0.59, 0.46, 0.42, 0.45, 0.47, and 0.47 from the level of L1 to S1. At a sensitivity of 93.8 % and a specificity of 84.4 % the cutoff value at S1 was most diagnostic. In MFS patients VAR was significantly higher at L3 and L4.
CONCLUSION: Our cutoff value for DSR >0.47 at S1 may help to identify MFS in the Japanese population. A square-like appearance of the L3 and L4 vertebral bodies is a supplementary finding in MFS patients.

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Year:  2014        PMID: 24477527     DOI: 10.1007/s11604-014-0285-1

Source DB:  PubMed          Journal:  Jpn J Radiol        ISSN: 1867-1071            Impact factor:   2.374


  21 in total

1.  MR evaluation of dural ectasia in Marfan syndrome: reassessment of the established criteria in children, adolescents, and young adults.

Authors:  Christian R Habermann; Florian Weiss; Volker Schoder; Miriam C Cramer; Joern Kemper; Oliver Wittkugel; Gerhard Adam
Journal:  Radiology       Date:  2004-12-22       Impact factor: 11.105

2.  Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene.

Authors:  H C Dietz; G R Cutting; R E Pyeritz; C L Maslen; L Y Sakai; G M Corson; E G Puffenberger; A Hamosh; E J Nanthakumar; S M Curristin
Journal:  Nature       Date:  1991-07-25       Impact factor: 49.962

Review 3.  The Marfan syndrome.

Authors:  R E Pyeritz
Journal:  Annu Rev Med       Date:  2000       Impact factor: 13.739

4.  The revised Ghent nosology for the Marfan syndrome.

Authors:  Bart L Loeys; Harry C Dietz; Alan C Braverman; Bert L Callewaert; Julie De Backer; Richard B Devereux; Yvonne Hilhorst-Hofstee; Guillaume Jondeau; Laurence Faivre; Dianna M Milewicz; Reed E Pyeritz; Paul D Sponseller; Paul Wordsworth; Anne M De Paepe
Journal:  J Med Genet       Date:  2010-07       Impact factor: 6.318

5.  Dural ectasia is associated with back pain in Marfan syndrome.

Authors:  N U Ahn; P D Sponseller; U M Ahn; L Nallamshetty; B S Kuszyk; S J Zinreich
Journal:  Spine (Phila Pa 1976)       Date:  2000-06-15       Impact factor: 3.468

6.  Quantitative assessment of dural ectasia as a marker for Marfan syndrome.

Authors:  T Oosterhof; M Groenink; F J Hulsmans; B J Mulder; E E van der Wall; R Smit; R C Hennekam
Journal:  Radiology       Date:  2001-08       Impact factor: 11.105

7.  Angiotensin II blockade and aortic-root dilation in Marfan's syndrome.

Authors:  Benjamin S Brooke; Jennifer P Habashi; Daniel P Judge; Nishant Patel; Bart Loeys; Harry C Dietz
Journal:  N Engl J Med       Date:  2008-06-26       Impact factor: 91.245

8.  Clinical and genetic analysis of Korean patients with Marfan syndrome: possible ethnic differences in clinical manifestation.

Authors:  E-H Yoo; H Woo; C-S Ki; H J Lee; D-K Kim; I-S Kang; P Park; K Sung; C S Lee; T-Y Chung; J R Moon; H Han; S-T Lee; J-W Kim
Journal:  Clin Genet       Date:  2009-10-23       Impact factor: 4.438

9.  Prevalence of dural ectasia in 63 gene-mutation-positive patients with features of Marfan syndrome type 1 and Loeys-Dietz syndrome and report of 22 novel FBN1 mutations.

Authors:  B Söylen; K K Singh; A Abuzainin; K Rommel; H Becker; M Arslan-Kirchner; J Schmidtke
Journal:  Clin Genet       Date:  2009-01-20       Impact factor: 4.438

10.  Dural ectasia and the Marfan syndrome.

Authors:  W E Stern
Journal:  J Neurosurg       Date:  1988-08       Impact factor: 5.115

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