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Spontaneous Regression Of A Vein Of Galen Aneurysmal Malformation In A Pediatric Patient: Illustrative Case

Kumar, K. K. 2021Other/Unknown

Kumar, K. K., Fornoff, L. E., Dodd, R. L., Marks, M. P., & Hong, D. S. (2021). Spontaneous regression of a vein of Galen aneurysmal malformation in a pediatric patient: illustrative case. Journal of neurosurgery. Case lessons, 1(8), CASE20171. https://doi.org/10.3171/CASE20171

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Abstract

BACKGROUND: Vein of Galen aneurysmal malformations (VGAMs) are rare congenital intracranial vascular lesions that represent 30% of all pediatric vascular anomalies. These lesions are associated with severe manifestations, including congestive heart failure, hydrocephalus, and spontaneous hemorrhage. The mainstay of management is medical stabilization followed by endovascular embolization of the lesion. Although VGAM was first reported in 1937, there are few published cases demonstrating spontaneous regression of the lesion. OBSERVATIONS: The authors report the case of a 31-month-old female who presented with an incidentally found VGAM. After initial evaluation, including magnetic resonance imaging and angiography, the patient was lost to follow-up. Upon her return to the clinic at age 12 years, the previously identified VGAM was absent, indicative of involution of the lesion. The patient remained asymptomatic and met appropriate developmental milestones during this interval. LESSONS: This report adds a rare case of the spontaneous resolution of VGAM to the literature. This case may suggest the presence of VGAMs that are asymptomatic, undetected, and regress within the pediatric population. Future studies may benefit from identifying imaging and angiographic findings predictive of spontaneous regression. There may be a role for conservative management in particular cases of asymptomatic and medically stable children with VGAMs.

Case Details

Disease Location

Brain venous system

Personal Characteristics

31-month-old female

Clinical Characteristics

Incidentally found vein of galen aneurysmal malformations (vgam). MRI was notable for a large vgam with as- sociated hydrocephalus due to compression of the cerebral aqueduct by a feeding artery. Cerebral angiography was then performed, identifying a vascular shunt with multiple feeders arising from the medial and lateral posterior choroidal arteries. Also a single-hole fistula used by all of the arterial feeders along the anterior aspect of the vein of galen aneurysmal dilatation.

Remission Characteristics

MRI/magnetic resonance angiography performed 12 years later, revealed the absence of the previously identified vgam, thought to represent delayed involution of the lesion

Treatment & Mechanisms

Proposed Remission Mechanisms

None reported

Clinical Treatment

None reported

Non-Clinical Treatment

None reported