Vein of Galen aneurismal malformations - clinical characteristics, treatment and presentation: Three cases report

Abstract

BACKGROUND The vein of Galen aneurismal malformations (VGAM) are rare arteriovenous malformations of the embryonic choroid plexus. They represent about 30% of paediatric neurovascular disorders and show diverse characteristics. The VGAM is constituted by a midline dilated venous structure that receives blood from abnormal macroscopic or microscopic arteriovenous shunting vessels. Two types of VGAM exist, the choroidal and the mural. The treatment represents a challenge with the therapeutic objective to preserve the normal brain development without creating new neurological deficits. CASE SUMMARY We present three cases of VGAM in the early postnatal period and their treatment. All patents were treated with the endovascular technique, which was successful. According to our experience, the endovascular technique is a safe and efficient mode of VGAM treatment. CONCLUSION The objective of treatment aims to child’s normal neurological development. A proper selection of patients and a thorough diagnostic workup is of vital importance. When the endovascular treatment is performed, the primary aim is not a complete VGAM exclusion at one time, which could produce a sudden reversal of blood flow with consequent venous infarction and ischemia. The aim is therefore to occlude as much of the VGAM as needed to relieve the congestive cardiac failure, to gain time and to create the conditions for a normal maturation of the neurovascular system. With the use of endovascular techniques, which represent not only the first choice of treatment but also the only safe therapeutic modality, the natural history of VGAM and their risks may be avoided safely.

Vein of Galen Aneurysmal Malformations in Pediatric Patients Submitted to Endovascular Treatment: Report of 3 Cases

“…A score between 8 and 12 indicates the need for urgent embolization, and between 12 and 21, a more stable condition, which allows the endovascular treatment to be postponed for 5 months, the age at which it is best tolerated. 8 We can give the 1st patient a Bicêtre score of 14, the 2nd, of 20, and the 3rd a score of 10, which, indeed, is consistent with the outcome in each case. It was not possible to offer patient 3 an early treatment, since he was referred to the neurosurgery service at 1 year of age.…”

Vein of Galen Malformation Manifesting as High-Output Heart Failure

“…In patients with the vein of Galen malformation, the placenta and the arteriovenous shunt are both draining blood from the fetal choroid plexus in utero, but with removal of the placenta, there is decreased resistance and thus greater blood flow across the shunt [7]. This leads to increased venous return to the right atrium and thus increased preload, which can lead to pulmonary hypertension and congestive heart failure.…”

“…Upon this removal, there is an automatic increase in the baby's systemic vascular resistance and increased exposure to oxygen leads to vasodilation in pulmonary arteries and a subsequent decrease in the pulmonary vascular resistance. In patients with the vein of Galen malformation, the placenta and the arteriovenous shunt are both draining blood from the fetal choroid plexus in utero, but with removal of the placenta, there is decreased resistance and thus greater blood flow across the shunt [ 7 ]. This leads to increased venous return to the right atrium and thus increased preload, which can lead to pulmonary hypertension and congestive heart failure.…”

Coexisting arachnoid cyst and tentorial sinus: A therapeutic dilemma

“…The vein of Galen is a midline venous structure that is formed by the embryonic remnant of the caudal median prosencephalic vein when it joins the internal cerebral veins. [ 5 ] Vein of Galen aneurysmal dilations can occur secondary to outflow obstruction and present as an incidental finding or as focal neurological deficits, seizures, or intracranial hemorrhage. [ 5 ] However, after careful review of the angiography, a medial tentorial sinus was considered to be the most probable diagnosis.…”

“…[ 5 ] Vein of Galen aneurysmal dilations can occur secondary to outflow obstruction and present as an incidental finding or as focal neurological deficits, seizures, or intracranial hemorrhage. [ 5 ] However, after careful review of the angiography, a medial tentorial sinus was considered to be the most probable diagnosis. Medial tentorial sinus is a normal anatomical variant that is responsible for the venous drainage of the superior cerebellar surface into the straight sinus.…”