Sturge-Weber syndrome with spontaneous intracerebral hemorrhage in childhood

Case report

Madoka Nakajima M.D., Ph.D., Hidenori Sugano M.D., Ph.D., Yasushi Iimura M.D., Takuma Higo M.D., Hajime Nakanishi M.D., Ph.D., Kazuaki Shimoji M.D., Ph.D., Kostadin Karagiozov M.D., Ph.D., Masakazu Miyajima M.D., Ph.D., and Hajime Arai M.D., Ph.D.
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  • Department of Neurosurgery, Juntendo University School of Medicine, Tokyo, Japan
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A girl aged 2 years 10 months suddenly went into a deep coma and demonstrated left hemiplegia. At birth, she had exhibited a left-sided facial port-wine stain typical of Sturge-Weber syndrome (SWS) and involving the V1 and V2 distributions of the trigeminal nerve. Computed tomography showed a right thalamic hemorrhage with acute hydrocephalus. Magnetic resonance imaging with Gd enhancement 8 months before the hemorrhage had shown a patent superior sagittal sinus (SSS) and deep venous system. Magnetic resonance imaging and MR angiography studies 2 months before the hemorrhage had revealed obstruction of the SSS and right internal cerebral vein (ICV). Given that a digital subtraction angiography study obtained after the hemorrhage did not show the SSS or right ICV, the authors assumed that impaired drainage was present in the deep venous system at that stage. The authors speculated that the patient's venous drainage pattern underwent compensatory changes because of the occluded SSS and deep venous collectors, shifting outflow through other cortical venous channels to nonoccluded dural sinuses. Sudden congestion (nearly total to total obstruction) of the ICV may have caused the thalamic hemorrhage in this case, which is the first reported instance of pediatric SWS with intracerebral hemorrhage and no other vascular lesion. Findings suggested that the appearance of major venous sinus occlusion in a child with SWS could be a warning sign of hemorrhage.

Abbreviations used in this paper:DSA = digital subtraction angiography; ICV = internal cerebral vein; SSS = superior sagittal sinus; SWS = Sturge-Weber syndrome.

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Contributor Notes

Address correspondence to: Madoka Nakajima, M.D., Ph.D., Department of Neurosurgery, Juntendo University, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan. email: madoka66@juntendo.ac.jp.

Please include this information when citing this paper: published online October 25, 2013; DOI: 10.3171/2013.9.PEDS133.

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