Understanding Adult Vein of Galen Malformation

A Vein of Galen malformation is a rare congenital vascular shunt usually diagnosed in neonates. However, exceptional cases involve adults presenting with late-onset neurological symptoms. Recently, clinicians documented a unique case of a 47-year-old female in Puerto Rico. She presented with progressive cognitive decline and new-onset seizures. Furthermore, she had a background of chronically compensated heart failure. On admission, her Glasgow Coma Scale score was 6, indicating severe neurological distress. This clinical scenario illustrates how these malformations can remain hidden until middle age.

Diagnosis and Imaging of Vein of Galen Malformation

Computed tomography (CT) imaging initially revealed intraventricular hemorrhage and extensive parenchymal calcifications. Additionally, the patient exhibited signs of hydrocephalus. Consequently, surgeons placed an external ventricular drain urgently to manage intracranial pressure. Subsequent digital subtraction angiography (DSA) and CT angiography confirmed a choroidal Vein of Galen malformation. Notably, this subtype is typically more severe than the mural type because it involves multiple arterial feeders. Imaging remains the gold standard for differentiating these vascular abnormalities from other neurodegenerative conditions.

Management of Vein of Galen Malformation

Workup for cerebrovascular abnormalities is crucial for patients with unexplained neurodegeneration at atypical ages. Early diagnosis of a Vein of Galen malformation can significantly impact clinical management and survival. While endovascular embolization is the standard treatment for many, some families may opt for conservative measures depending on the prognosis. In this specific case, the family chose to withdraw care given the severity of the brain injury. Nevertheless, this case highlights that clinicians should consider vascular shunts in the differential diagnosis of adult cognitive decline.

FAQ on Vein of Galen Malformations

What is the difference between mural and choroidal VOGM?

Choroidal Vein of Galen malformations involve multiple arterial feeders from choroidal and pericallosal arteries. They are generally more complex and severe compared to the mural type, which features fewer fistulae and often presents later in childhood.

Why do adults present differently than infants?

Neonates often present with high-output heart failure due to high-volume shunting. In contrast, adults may have lower-flow fistulae or compensated systems. Consequently, they often experience symptoms like headaches, seizures, or progressive cognitive decline over several years.

Disclaimer: This content is for informational and educational purposes only and does not constitute medical advice or a professional relationship. Always seek the advice of a qualified healthcare provider for any questions regarding a medical condition. Refer to the latest local and national guidelines for clinical practice.

References

1. Rivera-Rivera E et al. Adult presentation of a vein of Galen malformation as cognitive decline in a 47-year-old multiparous female: illustrative case. J Neurosurg Case Lessons. 2026 May 18. doi: undefined. PMID: 42150193.


2. Gupta AK, et al. Evaluation, management, and long-term follow up of vein of Galen malformations. J Neurosurg. 2006 Jul;105(1):26-33.


3. Sagazzi A, et al. Vein of Galen Malformations in adults. Clin Neurol Neurosurg. 2023 May;228:107671.