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Spontaneous thrombosis of vein of Galen malformation managed with surgical resection: a case‑based review

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Abstract

Introduction

Vein of Galen malformation (VOGM) is an exceptionally uncommon form of congenital intracranial vascular malformations. It is highly unusual for this lesion to spontaneously thrombose. The clinical presentation of a patient may range from being asymptomatic to critically ill. The underlying pathophysiological mechanisms that cause spontaneous thrombosis are still poorly understood.

Methods and results

The literature on spontaneous thrombosis of VOGM was systematically reviewed, analyzed, and summarized with a focus on its pathophysiology, types, clinical presentations, diagnosis, management, and outcomes. It was also illustrated with a case presentation. The case presents an unusual presentation and location of a VOGM in a 2-year-old boy who was successfully treated with surgical resection.

Conclusions

A handful of cases of thrombosed VOGM have been reported worldwide where surgery was used to treat the condition. Low-flow fistulas of the mural type are prone to spontaneous thrombosis, have delayed clinical presentations, and are typically diagnosed in young children. Among the many possible manifestations, hydrocephalus is by far the most common. In the absence of blood flow, MRI is the diagnostic test of choice. Depending on the patient’s symptoms, surgery to either remove the aneurysm or divert the cerebrospinal fluid usually results in a good prognosis.

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Availability of data and materials

Datasets are available on demand through emailing the corresponding author Y. H. K. at yakhormi@jazanu.edu.sa.

Abbreviations

VOGM:

Vein of Galen malformation

CT:

Computed tomography

MRI:

Magnetic resonance imaging

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Authors and Affiliations

  1. Department of Surgery, Faculty of Medicine, Jazan University, Jazan, Saudi Arabia

    Yahya H. Khormi

  2. Department of Neurosurgery, King Fahd Central Hospital, Jazan, Saudi Arabia

    Yahya H. Khormi, Alshaymaa M. Arishy & Mostafa M. E. Atteya

Authors
  1. Yahya H. Khormi
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  2. Alshaymaa M. Arishy
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  3. Mostafa M. E. Atteya
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Contributions

Performing surgery, study concept, and design: Y.K.; data acquisition and analysis: Y.K., A.M.A.; writing of the manuscript, figure design, table design, critically revising the manuscript, and review of submitted manuscript: all authors; study supervision: Y.K.

Corresponding author

Correspondence to Yahya H. Khormi.

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Ethics approval

All procedures performed in the 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 standard. Informed consent to participate and consent for publication were obtained from the patient included in this report.

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Khormi, Y.H., Arishy, A.M. & Atteya, M.M.E. Spontaneous thrombosis of vein of Galen malformation managed with surgical resection: a case‑based review. Childs Nerv Syst 39, 2521–2526 (2023). https://doi.org/10.1007/s00381-023-05974-5

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