Abstract
Background
Enlarged deep medullary veins (EDMVs) in patients with Sturge-Weber syndrome (SWS)
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 magnetic
resonance imaging prospectively including susceptibility-weighted imaging (SWI); children
aged 2.5 years or older also had a formal neurocognitive evaluation. The extent of
EDMVs 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 of 27) of right and 78% (18 of 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 SWS. Patients with EDMV
scores >4 had rare (less than monthly) seizures, whereas 35% (11 of 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 intelligence quotient (P < 0.05).
Conclusions
Enlarged deep medullary veins are common in unilateral SWS, but extensive EDMVs appear
to develop more commonly and earlier in right hemispheric SWS. Deep venous remodeling
may be a compensatory mechanism contributing to better clinical outcomes in some patients
with SWS.
Keywords
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Article info
Publication history
Published online: November 24, 2022
Accepted:
November 20,
2022
Received:
June 19,
2022
Footnotes
Conflict of interest: none.
Funding: This work was supported by the National Institutes of Health (grant numbers NS041922 and NS065705).
Identification
Copyright
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