Hemiventriculomegaly and Hemicranial Expansion in Klippel-Trenaunay-Weber Syndrome: A new pathophysiological concept of progressive unilateral hydrocephalus

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Annual Review of Hydrocephalus
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Abstract

A new concept of progressive unilateral hydrocephalus is described. A 6-month-old female with Klippel-Trenaunay-Weber syndrome presented extremely rare clinical features with unilateral ventriculomegaly, hemihypertrophy and hemicranial expansion all on the same side. CT cisternography revealed a mildly disturbed cerebrospinal fluid circulation but the foramen of Monro was fully patent. Continuous intraventricular pressure monitoring demonstrated a very mildly elevated base line pressure and almost no pressure wave appearance but a remarkably increased pulse pressure, a pathogenetic role of which is suggested in explaining the unilateral ventriculomegaly exhibited by this case with a nearly normal mean pressure of the cerebrospinal fluid. (J Pediatr Neurosci 3: 21–27, 1987)

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© 1990 Springer-Verlag Berlin Heidelberg

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Oi, S., Urui, S., Matsumoto, S. (1990). Hemiventriculomegaly and Hemicranial Expansion in Klippel-Trenaunay-Weber Syndrome: A new pathophysiological concept of progressive unilateral hydrocephalus. In: Matsumoto, S., Sato, K., Tamaki, N., Oi, S. (eds) Annual Review of Hydrocephalus. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-11152-9_95

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  • DOI: https://doi.org/10.1007/978-3-662-11152-9_95

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-11154-3

  • Online ISBN: 978-3-662-11152-9

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