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

Objective To report three cases of vein of Galen aneurysmalmalformation (VGAM) in pediatric patients treated at the hemodynamics lab of Hospital Santa Isabel (HSI) in Blumenau, state of Santa Catarina, Brazil, from 2006 to 2020. Clinical presentation, endovascular treatment, and postprocedure evolution to date are included. Case description Three children aged 5 to 12 months with cardiac, respiratory, or neurological damage in the neonatal stage, were referred to the neurosurgery service and diagnosed with VGAM. The three patients underwent endovascular embolization of themalformation, with different clinical evolution throughout outpatient follow-up. Conclusion Vein of Galen aneurysmal malformations are uncommon vascular abnormalities that, until the advent of endovascular embolization, were associated with high morbidity and mortality rates. Its prognosis is linked with initial clinic, early diagnosis, and timely surgical correction.

High Output Cardiovascular Physiology and Outcomes in Fetal Diagnosis of Vein of Galen Malformation.

Vein of Galen aneurysmal malformation (VGAM) is a rare anomaly associated with poor outcomes from high output cardiac failure and neurologic complications. Studies addressing fetal cardiovascular status and outcomes in this population are limited. A single-center retrospective review was conducted on patients with a prenatal diagnosis of VGAM who underwent a fetal echocardiogram between January 2015 and July 2019. Fetal echocardiographic data, brain magnetic resonance imaging (MRI) findings and outcomes were collected. Nine fetuses [median gestational age at echocardiogram 34 (1.1) weeks] were included. All patients had superior vena cava dilation and reversal of diastolic flow in the transverse aortic arch. Median cardiothoracic (CT) ratio was 0.39 (0.09). Right ventricular (RV) and left ventricular (LV) dysfunction was present in 66% and 11% fetuses, respectively. Four out of five patients that underwent postnatal endovascular neurosurgical interventions at our center were alive at follow-up (mean 2.7 years). Of the non-survivors (n = 5), 3 received comfort care because of severe brain damage and died in the neonatal period. Non-survivors more commonly had > mild tricuspid regurgitation (TR) (40% vs. 25%) and > mild RV dilation (60% vs. 25%). Combined cardiac index (CCI) was higher in non-survivors when compared to survivors (672.7 vs. 530.2 ml/kg/min, p = 0.016). Fetuses with significant parenchymal damage on brain MRI tended to have a higher CCI than those without (979.8 vs. 605.0 ml/kg/min, p = 0.047). RV dysfunction, TR and elevated CCI are more commonly seen in non-survivors with VGAM. A higher CCI is seen in those deemed untreatable due to significant parenchymal volume loss. Future multicenter studies are needed to assess for prenatal prediction of outcomes in this high-risk population.

The use of adenosine in the treatment of a high-flow vein of Galen malformation in an adult.

The treatment of high-flow vein of Galen aneurysmal malformations (VGAM) remains a therapeutic challenge for the neurosurgeon and the neurointerventionalist, as it is associated with high morbidity and mortality rates despite recent advances in open cranial surgery and interventional neuroradiology. A 37-year-old patient presented with a history of non-specific headaches. He had a history of heart failure since birth that was caused by an untreated VGAM. Intravenous boluses of adenosine were injected as an attempt to slow down the arteriovenous shunting of a VGAM prior to endovascular treatment. Adenosine can be a very useful adjunct in patients with extremely high arteriovenous shunting. n-butyl cyanoacrylate (n-BCA) should be the embolic material of choice due to its quick polymerization and adhesive properties.

Teaching neuroimages: vein of Galen aneurysm mimicking pineal mass in a young adult.

A 22-year-old man presented with intractable progressive headaches over 2 months. Noncontrast head CT and contrast-enhanced MRI scan revealed a hyperdense pineal-based mass (figure, A and B). Arteriogram was normal (figure, C and D).

Regression of cerebral calcifications after endovascular treatment in a case of vein of Galen arteriovenous malformation.

The authors present the case of an infant harboring a vein of Galen arteriovenous malformation with conspicuous cerebral calcifications that progressively regressed after staged endovascular obliteration of the lesion. The role of venous hypertension and hydrocephalus secondary to the arteriovenous shunt are discussed to explain the formation and regression of the cerebral calcifications.

Endoscopic treatment of hydrocephalus due to aneurysm of the vein of Galen: case report and literature review.

Aneurysms of the vein of Galen are uncommon vascular malformations. They are most frequently seen in infants and children, leading to heart failure and hydrocephalus. Exceptionally, they are detected in adults. Several theories have been proposed to explain hydrocephalus in these patients: obstruction of the cerebral aqueduct, impaired absorption of CSF after subarachnoid hemorrhage, passive ex-vacuo mechanism, or thrombosis of an aneurysm. Hydrocephalus has been treated mainly with cerebrospinal shunt procedures, but also direct surgery, radiosurgery and embolisation of the malformation have proved to be effective. We report the case of a partially thrombosed ectasia of the vein of Galen in a 44-year-old male, with huge hydrocephalus successfully treated with an endoscopic third ventriculostomy.