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Hydrocephalus in Vein of Galen Malformation

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Pediatric Hydrocephalus

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Abstract

Vein of Galen aneurysmal malformation (VGAM) represents up to 30% of vascular malformations in the pediatric age group. Described in the late 1980s, this entity was associated with a high rate of hydrocephalus and poor prognosis. This work presents the hydrocephalus physiopathology in patients with a VGAM and the modern care of this frequent complication.

If aqueduct stenosis may explain the abnormal accumulation of cerebrospinal fluid in some patients harboring a VGAM, high venous pressure is the most likely predominant physiopathologic mechanism: immature Pacchioni granulations cannot drain the brain during infancy, and the elevated venous pressure leads to a congestive status of the brain.

Thus, the first-line treatment is nowadays embolization, ideally at 5 months. Embolization normalizes venous pressure and treats the hydrocephalus. If emergency treatment is required, endoscopic third ventriculostomy can resolve mechanical compression of the aqueduct. Ventriculoperitoneal shunting, associated with poor outcomes, is not a first-line therapy.

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Author information

Authors and Affiliations

  1. Neurosurgery Department, Sainte-Anne Hospital, Paris, France

    Marc Zanello

  2. Paris V, René Descartes University, Paris, France

    Marc Zanello & Michel Zerah

  3. Pediatric Neurosurgical Department, Hôpital Necker-Enfants Malades, AP-HP, Paris, France

    Michel Zerah

  4. Department of Pediatric Neurosurgery, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Lyon, France

    Federico Di Rocco

  5. Université Claude Bernard, Lyon, France

    Federico Di Rocco

Authors
  1. Marc Zanello
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  2. Michel Zerah
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  3. Federico Di Rocco
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Corresponding author

Correspondence to Federico Di Rocco .

Editor information

Editors and Affiliations

  1. Pediatric Neurosurgery, Santobono Pausilipon Children’s Hospital, Naples, Italy

    Giuseppe Cinalli

  2. Division of Pediatric Neurosurgery, Acıbadem University, School of Medicine, Istanbul, Turkey

    M. Memet Özek

  3. Pediatric Neurosurgery, Hôpital Necker-Enfants Malades Université René Descartes-Paris V, Paris, France

    Christian Sainte-Rose

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Zanello, M., Zerah, M., Di Rocco, F. (2019). Hydrocephalus in Vein of Galen Malformation. In: Cinalli, G., Özek, M., Sainte-Rose, C. (eds) Pediatric Hydrocephalus. Springer, Cham. https://doi.org/10.1007/978-3-319-27250-4_67

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  • DOI: https://doi.org/10.1007/978-3-319-27250-4_67

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-27248-1

  • Online ISBN: 978-3-319-27250-4

  • eBook Packages: MedicineReference Module Medicine

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