title: Diffusion Tensor Imaging and Fiber Tractography in Children with Craniosynostosis Syndromes.
abstract: OBJECTIVE
  Patients with craniosynostosis syndromes caused by mutations in FGFR-2, FGFR-3, and TWIST1 genes are characterized by having prematurely fused skull sutures and skull base synchondroses, which result in a skull deformity and are accompanied by brain anomalies, including altered white matter microarchitecture. In this study, the reliability and reproducibility of DTI fiber tractography was investigated in these patients. The outcomes were compared with those of controls.
  
  
  METHODS
  DTI datasets were acquired with a 1.5T MR imaging system with 25 diffusion gradient orientations (voxel size = 1.8 × 1.8 × 3.0 mm(3), b-value = 1000 s/mm(2)). White matter tracts studied included the following: corpus callosum, cingulate gyrus, fornix, corticospinal tracts, and medial cerebellar peduncle. Tract pathways were reconstructed with ExploreDTI in 58 surgically treated patients with craniosynostosis syndromes and 7 controls (age range, 6-18 years).
  
  
  RESULTS
  Because of the brain deformity and abnormal ventricular shape and size, DTI fiber tractography was challenging to perform in patients with craniosynostosis syndromes. To provide reliable tracts, we adapted standard tracking protocols. Fractional anisotropy was equal to that in controls (0.44 versus 0.45 ± 0.02, P = .536), whereas mean, axial, and radial diffusivity parameters of the mean white matter were increased in patients with craniosynostosis syndromes (P < .001). No craniosynostosis syndrome-specific difference in DTI properties was seen for any of the fiber tracts studied in this work.
  
  
  CONCLUSIONS
  Performing DTI fiber tractography in patients with craniosynostosis syndromes was difficult due to partial volume effects caused by an anisotropic voxel size and deformed brain structures. Although these patients have a normal fiber organization, increased diffusivity parameters suggest abnormal microstructural tissue properties of the investigated white matter tracts.
authors.affiliation: From the Departments of Plastic and Reconstructive Surgery and Hand Surgery (B.F.M.R., Y.L., I.M.J.M.) b.rijken@erasmusmc.nl.
authors.forename: B F M
authors.initials: BF
authors.lastname: Rijken
authors.affiliation: Image Sciences Institute (A.L.), University Medical Center, Utrecht, the Netherlands.
authors.forename: A
authors.initials: A
authors.lastname: Leemans
authors.affiliation: From the Departments of Plastic and Reconstructive Surgery and Hand Surgery (B.F.M.R., Y.L., I.M.J.M.).
authors.forename: Y
authors.initials: Y
authors.lastname: Lucas
authors.affiliation: Department of Biostatics (K.v.M.), Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: K
authors.initials: K
authors.lastname: van Montfort
authors.affiliation: From the Departments of Plastic and Reconstructive Surgery and Hand Surgery (B.F.M.R., Y.L., I.M.J.M.).
authors.forename: I M J
authors.initials: IM
authors.lastname: Mathijssen
authors.affiliation: Radiology (M.H.L.), Erasmus Medical Center/Sophia Children's Hospital, Rotterdam, the Netherlands.
authors.forename: M H
authors.initials: MH
authors.lastname: Lequin
mesh_terms: D016880:Anisotropy; D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000209:etiology* / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D003398:Craniosynostoses / Q000150:complications* / Q000473:pathology*; D056324:Diffusion Tensor Imaging / Q000379:methods*; D005260:Female; D006801:Humans; D008297:Male; D009434:Neural Pathways / Q000473:pathology*; D015203:Reproducibility of Results; D013577:Syndrome