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Hydrocephalus in vein of Galen malformation: etiologies and therapeutic management implications

  • Clinical Article - Pediatrics
  • Published:
Acta Neurochirurgica Aims and scope Submit manuscript

Abstract

Background

Up to now, only little is known about hydrocephalus (HC) in vein of Galen malformation (VGM). We want to present the different etiologies and our long-term experience (1992–2015) in the management of HC.

Methods

Out of 44 treated children with VGM, we retrospectively reviewed all cases with HC. We analyzed the etiologies, our treatment results and complications.

Results

Twenty-one children (48 %) presented either with HC or developed it over time. In 21 % of those cases, high venous pressure was presumably the sole cause. Until 2009, seven of them received ventriculoperitoneal (VP) shunting; six of those resulted in severe postoperative complications. The remaining children have been treated successfully by endovascular embolization. Five out of the 44 children (11 %) developed HC after intraventricular hemorrhage. In four cases, those children were treated with positive results by using transient external ventricular drainages. In one case a VP shunt with highest valve pressure was inserted. Another four children (9 %) presented with aqueductal stenosis-related HC caused by either dilated venous outflow or space-occupying coil masses after embolization. The latter case was successfully treated by ventriculocisternostomy, whereas endovascular treatment decreased the venous outflow in size and thus resolved the HC in the other cases. In the remaining cases (7 %), atrophy due to melting brain syndrome led to HC ex vacuo.

Conclusions

HC in VGM is a common phenomenon with several etiologies requiring different treatments. In most cases, embolization of the VGM as sole treatment is completely sufficient in order to decrease high venous pressure. However, certain other causes of HC should be treated in an interdisciplinary setting by specialized neurosurgeons.

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Acknowledgments

The authors would like to thank Dr. Eva Rütz and Cynthia Schmidt cand. med. for editorial assistance.

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Authors and Affiliations

  1. Department of Radiology and Neuroradiology, Sana Kliniken Duisburg, Zu den Rehwiesen 9, 47055, Duisburg, Germany

    Dan Meila, Dominik Grieb, Katharina Melber & Friedhelm Brassel

  2. Department of Diagnostic and Interventional Neuroradiology, Medical School Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Germany

    Dan Meila & Heinrich Lanfermann

  3. Department of Orthodontics, University of Mainz, Mainz, Germany

    Collin Jacobs

  4. Department of Neurosurgery, University Hospital Essen, Essen, Germany

    Homajoun Maslehaty

  5. Department of Neurosurgery, University Hospital Düsseldorf, Düsseldorf, Germany

    Athanasios Petridis

  6. Department of Neurosurgery, Sana Kliniken Duisburg, Duisburg, Germany

    Rashad El Habony & Martin Scholz

Authors
  1. Dan Meila
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  2. Dominik Grieb
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  3. Katharina Melber
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  4. Collin Jacobs
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  5. Homajoun Maslehaty
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  6. Athanasios Petridis
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  7. Rashad El Habony
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  8. Heinrich Lanfermann
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  9. Martin Scholz
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  10. Friedhelm Brassel
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Corresponding author

Correspondence to Dan Meila.

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Funding

No funding was received for this research.

Conflict of interest

All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

For this type of study formal consent is not required.

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Informed consent was obtained from all individual participants included in the study.

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The authors have no financial relationships relevant to this article to disclose.

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Meila, D., Grieb, D., Melber, K. et al. Hydrocephalus in vein of Galen malformation: etiologies and therapeutic management implications. Acta Neurochir 158, 1279–1284 (2016). https://doi.org/10.1007/s00701-016-2836-y

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  • DOI: https://doi.org/10.1007/s00701-016-2836-y

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