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September 10, 2013; 81 (11) NeuroImages

Extensive subarachnoid venous angiomatosis with hydrocephalus in phacomatosis pigmentovascularis

Li-Wen Chen, Yi-Shan Tsai, Jung-Shun Lee, Yi-Fang Tu, Chao-Ching Huang
First published September 9, 2013, DOI: https://doi.org/10.1212/WNL.0b013e3182a43ba6
Li-Wen Chen
From National Cheng Kung University College of Medicine, Tainan, Taiwan.
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Yi-Shan Tsai
From National Cheng Kung University College of Medicine, Tainan, Taiwan.
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Jung-Shun Lee
From National Cheng Kung University College of Medicine, Tainan, Taiwan.
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Yi-Fang Tu
From National Cheng Kung University College of Medicine, Tainan, Taiwan.
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Chao-Ching Huang
From National Cheng Kung University College of Medicine, Tainan, Taiwan.
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Citation
Extensive subarachnoid venous angiomatosis with hydrocephalus in phacomatosis pigmentovascularis
Li-Wen Chen, Yi-Shan Tsai, Jung-Shun Lee, Yi-Fang Tu, Chao-Ching Huang
Neurology Sep 2013, 81 (11) 1020-1021; DOI: 10.1212/WNL.0b013e3182a43ba6

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An 8-month-old boy with cutaneous vascular malformations and dermal melanocytosis (Mongolian spots, figure, A) on the face and trunk was diagnosed with phacomatosis pigmentovascularis type 2. He had normal neurodevelopment, but progressive macrocephaly (figure, B). Linear brain ultrasonography showed extensive venous angiomatosis in the prominent subarachnoid space (figure, C and D). MRI revealed cortical sulcal widening, prominent leptomeningeal vessels in an enlarged subarachnoid space (figure, E and F), and communicating hydrocephalus (figure, F). Neurologic involvement in phacomatosis pigmentovascularis is uncommon except in Sturge-Weber and Klippel-Trenaunay syndromes.1,2 Communicating hydrocephalus due to subarachnoid angiomatosis may be underdiagnosed in phacomatosis pigmentovascularis, and should be considered in cases of progressive macrocephaly.

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Figure Cutaneous findings, head circumference changes, and neuroimages

Nevus flammeus and pigmentary abnormalities (A). Progressive macrocephaly (B). Prominent vasculatures in the subarachnoid space (white arrowheads) under linear (C) and power Doppler sonography (D) and 3D steady-state acquisition sequence MRI (E). Clustered leptomeningeal vessels in the enlarged subarachnoid space and nonobstructive ventriculomegaly on enhanced T1-weighted image (F).

Footnotes

  • Author contributions: All authors participated in the study concept and design and figure acquisitions. The manuscript was drafted by Dr. Chen and Dr. Tsai and revised by Dr. Huang. All the authors made an intellectual contribution to the final manuscript.

  • Study funding: No targeted funding reported.

  • Disclosure: The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.

  • © 2013 American Academy of Neurology

References

  1. 1.↵
    1. Hall BD,
    2. Cadle RG,
    3. Morrill-Cornelius SM,
    4. Bay CA
    . Phakomatosis pigmentovascularis: Implications for severity with special reference to Mongolian spots associated with Sturge-Weber and Klippel-Trenaunay syndromes. Am J Med Genet 2007;143A:3047–3053.
    OpenUrl
  2. 2.↵
    1. Fernández-Guarino M,
    2. Boixeda P,
    3. de Las Heras E,
    4. Aboin S,
    5. García-Millán C,
    6. Olasolo PJ
    . Phakomatosis pigmentovascularis: clinical findings in 15 patients and review of the literature. J Am Acad Dermatol 2008;58:88–93.
    OpenUrlCrossRefPubMed

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