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Deep venous remodeling in unilateral Sturge-Weber syndrome: Robust hemispheric differences and clinical correlates

  • Csaba Juhász
    Correspondence
    Corresponding Author: Csaba Juhász, MD, PhD, Professor of Pediatrics and Neurology, Wayne State University School of Medicine, 3901 Beaubien St., Children’s Hospital of Michigan, Detroit, MI, 48201, Tel: 313-966-5136; Fax: 313-576-9920,
    Affiliations
    Department of Pediatrics, Wayne State University School of Medicine, Children’s Hospital of Michigan, 3901 Beaubien St., Detroit, MI, 48201

    Department of Neurology, Wayne State University School of Medicine, 4201 St. Antoine St., Detroit, MI, 48201

    Translational Imaging Laboratory, Children’s Hospital of Michigan, 3901 Beaubien St., Detroit, MI, 48201
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  • Aimee F. Luat
    Affiliations
    Department of Pediatrics, Wayne State University School of Medicine, Children’s Hospital of Michigan, 3901 Beaubien St., Detroit, MI, 48201

    Department of Pediatrics, Central Michigan University, 1280 East Campus Dr, Mt Pleasant, MI, 48858
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  • Michael E. Behen
    Affiliations
    Department of Pediatrics, Wayne State University School of Medicine, Children’s Hospital of Michigan, 3901 Beaubien St., Detroit, MI, 48201

    Translational Imaging Laboratory, Children’s Hospital of Michigan, 3901 Beaubien St., Detroit, MI, 48201
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  • Nore Gjolaj
    Affiliations
    Department of Pediatrics, Wayne State University School of Medicine, Children’s Hospital of Michigan, 3901 Beaubien St., Detroit, MI, 48201

    Translational Imaging Laboratory, Children’s Hospital of Michigan, 3901 Beaubien St., Detroit, MI, 48201
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  • Jeong-Won Jeong
    Affiliations
    Department of Pediatrics, Wayne State University School of Medicine, Children’s Hospital of Michigan, 3901 Beaubien St., Detroit, MI, 48201

    Department of Neurology, Wayne State University School of Medicine, 4201 St. Antoine St., Detroit, MI, 48201

    Translational Imaging Laboratory, Children’s Hospital of Michigan, 3901 Beaubien St., Detroit, MI, 48201
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  • Author Footnotes
    ∗ present address: Department of Neurology, NYU Langone School of Medicine, 223 East 34th St., New York, NY, 10016
    Harry T. Chugani
    Footnotes
    ∗ present address: Department of Neurology, NYU Langone School of Medicine, 223 East 34th St., New York, NY, 10016
    Affiliations
    Department of Pediatrics, Wayne State University School of Medicine, Children’s Hospital of Michigan, 3901 Beaubien St., Detroit, MI, 48201

    Department of Neurology, Wayne State University School of Medicine, 4201 St. Antoine St., Detroit, MI, 48201

    Translational Imaging Laboratory, Children’s Hospital of Michigan, 3901 Beaubien St., Detroit, MI, 48201

    Department of Neurology, NYU Langone School of Medicine, 223 East 34th St., New York, NY, 10016
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  • Author Footnotes
    ∗∗ present address: Division of Neuroradiology, Johns Hopkins University School of Medicine, The Johns Hopkins Hospital, 1800 Orleans St., Baltimore, MD, 21287
    Ajay Kumar
    Footnotes
    ∗∗ present address: Division of Neuroradiology, Johns Hopkins University School of Medicine, The Johns Hopkins Hospital, 1800 Orleans St., Baltimore, MD, 21287
    Affiliations
    Department of Pediatrics, Wayne State University School of Medicine, Children’s Hospital of Michigan, 3901 Beaubien St., Detroit, MI, 48201

    Translational Imaging Laboratory, Children’s Hospital of Michigan, 3901 Beaubien St., Detroit, MI, 48201

    Department of Radiology, Wayne State University School of Medicine, 4201 St. Antoine St., Detroit, MI, 48201

    Division of Neuroradiology, Johns Hopkins University School of Medicine, The Johns Hopkins Hospital, 1800 Orleans St., Baltimore, MD, 21287
    Search for articles by this author
  • Author Footnotes
    ∗ present address: Department of Neurology, NYU Langone School of Medicine, 223 East 34th St., New York, NY, 10016
    ∗∗ present address: Division of Neuroradiology, Johns Hopkins University School of Medicine, The Johns Hopkins Hospital, 1800 Orleans St., Baltimore, MD, 21287

      Abstract

      Objective

      Enlarged deep medullary veins (EDMVs) in patients with Sturge-Weber syndrome (SWS) occur during the early disease course and may provide compensatory venous drainage for brain regions affected by the leptomeningeal venous malformation (LVM). We evaluated the prevalence, extent, hemispheric differences, and clinical correlates of EDMVs in SWS.

      Methods

      Fifty children (median age: 4.5 years) with unilateral SWS underwent brain MRI prospectively including susceptibility-weighted imaging (SWI); children ≥2.5 years of age also had a formal neurocognitive evaluation. The extent of EDMVs in the affected hemisphere was assessed on SWI by using an EDMV hemispheric score, which was compared between patients with right and left SWS and correlated with clinical variables.

      Results

      EDMVs were present in 89% (24/27) of right and 78% (18/23) of left SWS brains. Extensive EDMVs (score >6) were more frequent in right (33%) than in left SWS (9%; p=0.046) and commonly occurred in young children with right but not with left SWS. Patients with EDMV scores >4 (n=19) had rare (less than monthly) seizures, while 35% (11/31) of patients with EDMV scores ≤4 had monthly or more frequent seizures (p=0.003). In patients with right SWS and at least two LVM-affected lobes, higher EDMV scores were associated with higher IQ (p<0.05).

      Conclusions

      Enlarged deep medullary veins are common in unilateral SWS, but extensive EDMVs appear to develop more commonly and earlier during the disease course in right hemispheric SWS. Deep venous remodeling may be a compensatory mechanism contributing to better clinical outcomes in some patients with SWS.

      Key words

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