Endovascular management of vein of Galen aneurysmal malformations presenting in the neonatal period

BACKGROUND AND PURPOSE: Neonates with vein of Galen aneurysmal malformations (VGAMs) presenting with cardiac failure have high morbidity and mortality, and outcomes are significantly better in those presenting in later childhood. Neurologic outcomes in survivors are perceived to be uniformly poor, which may lead to the neonate being denied treatment. We assessed outcomes of modern neonatal intensive care and endovascular embolization in a consecutive series of such neonates presenting with cardiac failure. METHODS: Between 1996 and 1998, five infants (three male, two female) were diagnosed with symptomatic VGAMs in the first week of life, four of whom had intractable, high-output cardiac failure and underwent initial endovascular treatment. There were 15 endovascular procedures and one neurosurgical clipping in these five patients. Transarterial and transvenous routes were required, using multiple embolic agents. We emphasized the use of sonographically guided, percutaneous transtorcular-venous-access, moveable-core guidewire as an embolic agent; routine MR imaging; and MR angiography. RESULTS: Immediate outcomes included control of cardiac failure with normal neurologic function in four (80%) patients and one (20%) death from intractable cardiac failure. On follow-up examination, three (60%) infants showed no evidence of neurologic abnormality or cardiac failure; one (20%) infant showed moderate developmental delay. Two have had no further shunting on angiography, one has minimal flow, and one is awaiting follow-up imaging. CONCLUSION: Endovascular therapy with modern neuroanesthetic and neurointensive care can provide good outcomes even in the highest-risk neonates with VGAMs and cardiac failure. If medical management of cardiac failure fails, and there is no evidence of gross cerebral parenchymal damage on imaging, urgent endovascular treatment is feasible and can reduce the almost-100% mortality otherwise expected, without invariably severe morbidity. Use of multiple embolization strategies in multiple stages usually is necessary in these patients, and novel approaches and embolic agents may be necessary.     

Antenatal diagnosis of vein of Galen aneurysmal malformation: MR study of fetal brain and postnatal follow-up

About 20 cases of prenatal diagnosis of vein of Galen aneurysmal Malformation (VGAM) have been described. We present a case diagnosed prenatally by Doppler ultrasonography. Prenatal MRI and postnatal radiological studies including post-treatment MRI and MRA, were carried out.     

Transfemoral venous embolization of vein of Galen malformations

Three infants with vein of Galen malformations, all presenting with congestive heart failure, underwent a total of five embolization procedures that employed a percutaneous transfemoral venous approach to catheterize the vein of Galen. In one instance, direct retrograde catheterization of feeding arterial pedicles to the vein of Galen and embolization of the fistulous connections was achieved via this route. The indications for transfemoral venous treatment included persistent symptoms despite transarterial and transtorcular embolization in one patient, an unsuccessful transarterial embolization attempt (complicated by catheter fracture) in another, and the inadvisability of transarterial embolization because of an excessive number of feeding arteries in a third. Complete obliteration of the malformation was achieved in one patient and significant flow reduction in the other two. Vein of Galen perforation with the catheter tip complicated one procedure. All three patients were stable after clinical follow-ups (9-12 months). The transvenous route to the vein of Galen can be undertaken from a transfemoral approach, obviating surgical exposure of the torcular Herophili. In addition, we introduce the concept of direct retrograde catheterization of the feeding arteries to the vein of Galen malformation by a transfemoral venous approach, a procedure that has not been reported previously.     

MR angiography of the vein of Galen malformation

Vein of Galen malformation is a rare congenital disorder. It has been studied with most of the imaging modalities; however, there is no report in the literature on magnetic resonance (MR) angiography of the condition. This article describes two patients with the malformation studied by MR angiography—MR arteriography and MR venography.     

Spontaneous disappearance of an aneurysmal malformation of the vein of Galen

We describe spontaneous disappearance of an aneurysmal malformation of the vein of Galen (AMG), an anomaly that occurs most often in children, with a high mortality due to the accompanying severe cardiac failure. Spontaneous thrombosis of an AMG is rare. In this case, the AMG has been closed for about 5 years, without radiological evidence of thrombosis, and no active treatment. Proposed mechanisms of spontaneous thrombosis include slow flow shunts and severe obstruction of the venous outflow. However, in this case, the disappearance of the AMG without evidence of thrombosis indicates a different mechanisms, namely, obstruction of the feeding artery.     

Vein of Galen technique for occluding the aneurysmal sac of pulmonary arteriovenous malformations

In seven of 255 consecutive patients (2.7%) who underwent pulmonary arteriovenous malformation (PAVM) embolization at our center between July 1, 1996, and July 1, 2000, the feeding artery was considered too short for safe occlusion with use of standard stainless-steel coils or detachable balloons. These patients were successfully treated with use of a modified vein of Galen technique similar to the one used for managing intracranial vein of Galen aneurysms. In this article, the authors report their experience with this technique in safely occluding the aneurysmal sacs of PAVMs in patients with short (<2 cm) feeding pulmonary arteries.     

Cognitive and functional status after vein of Galen aneurysmal malformation endovascular occlusion

AIM:To study the clinical outcomes of treating vein of Galen aneurysmal malformations(VGAM),we assessed our patient cohort using standardized cognitive and functional measures.METHODS:A retrospective review of patients with VGAM treated by a single practitioner between 2003 and 2009 was performed for this study.In addition to routine clinical assessment,all patients were evaluated for cognitive and functional impairment using validated measures including the Neurobehavioral Rating ScaleRevised,the Bicêtre outcome score,and the Barthel index.RESULTS:Five patients underwent combined transarterial and transvenous embolization of their VGAM during the study period.VGAMs were classified based on angioarchitecture as either choroidal(1/5) or mural(4/5) according to the classification scheme of Lasjaunias.In total,13 embolization procedures were performed consisting of 1 to 3 treatment stages per patient.Complete or near complete occlusion was achieved in 4 patients,while subtotal occlusion was achieved in 1 patient.During follow-up(median 62.6 mo),all patients were either unchanged or cognitively and neurologically intact.CONCLUSION:VGAM can be safely and effectively treated by staged transarterial and transvenous embolization.Using this strategy,excellent long-term cognitive and functional outcomes can be achieved.     

Three-dimensional MR visualization of the intracisternal course of the cranial nerves V-VIII by virtual cisternoscopy

Purpose:
A post-processing protocol for 3D visualization of the cranial nerves V-VIII along their intracisternal course is presented. Material and Methods:
Six healthy volunteers underwent MR imaging (1.5 T) to obtain high-resolution heavily T2-weighted data sets (3DFT CISS) with isotropic voxels (0.5 mm³). The data sets were post-processed by using volume rendering software in order to visualize the intracisternal courses of the cranial nerves V-VIII as well as their root entry zones. The data acquisition and post-processing protocol was then applied in 14 patients with a suspected neural compression syndrome according to the clinical findings as well as cross-sectional images and evaluated with respect to image quality and diagnostic value by two neuroradiologists, using a five-point scale. Results:
Virtual cisternoscopy allowed a comprehensive intracisternal 3D visualization of the affected cranial nerves in 12/14 patients. The mean post-processing time amounted to 13.1/5.6/13.7 min for the cranial nerves V/VI/VII and VIII. The mean score for image quality was 4.2, that for diagnostic value 4.1. 2D and/or 3D reference images were indispensable for appreciating the spatial information provided by virtual cisternoscopy. Conclusion:
The data acquisition and post-processing protocol presented here allows comprehensive and standardized intracisternal 3D visualization of the cranial nerves V-VIII in a routine setting as a complementary imaging procedure.     

Deep venous drainage in great cerebral vein (vein of Galen) absence and malformations

Summary We report two types of venous patterns associated with great cerebral vein (vein of Galen) absence or unavailability. Developmental venous anomalies or vein of Galen arteriovenous malformations (VGAM) serve as an illustrative material. A diencephalic pattern that collects the thalamo-striate veins into the tentorial sinus is recognized in most VGAM. A telencephalic arrangement connecting the striate veins with the rostral afferents to the basal vein is less frequent. Both patterns reproduce embryonic stages preceeding the development of the great cerebral vein, thus confirming Raybaud's hypothesis that in VGAM the pouch is not the vein of Galen but the medial vein of the prosencephalon. The prognostic value of each pattern can then be appreciated and the therapeutic strategies rationalized; some unexplained complications of the venous approach for non selected VGAM can thus be avoided.     

Neurogenic stunned myocardium after embolization in two children with vein of Galen aneurysmal malformation

Introduction

This study reports the first cases of neurogenic stunned myocardium in two children with vein of Galen aneurysmal malformation after interventional treatment.

Patients

Two newborns with vein of Galen aneurysmal malformation and high output cardiac failure developed a severe reversible left ventricular dysfunction shortly after embolization, concurrently with acute hydrocephalus.

Results

There was a resolution of the cardiac symptoms of left ventricular dysfunction within a few days under treatment with milrinone and dobutamine.

Conclusions

Reversible left ventricular dysfunction is observed in adult patients mainly after subarachnoid hemorrhage and is called neurogenic stunned myocardium (NSM). Other forms of brain injuries have also been identified accounting for this condition in adults. In pediatric population especially with specific cerebral diseases, NSM may be underdiagnosed.     

Successful treatment of neonatal aneurysmal dilatation of the vein of Galen: the role of prenatal diagnosis and trans-arterial embolization

Summary We report a neonate with aneurysmal dilatation of the vein of Galen diagnosed prenatally by color Doppler sonography and MRI at 37 weeks' gestation. The child was treated by transarterial embolization of microcoils 2 and 8 days after birth. The aneurysmal dilatation of the vein of Galen markedly decreased after embolization and the patient is developing normally at 2 years 5 months of age. Prenatal diagnosis and early intervention by transarterial embolization produced in a good outcome in this patient.     

Spontaneous thrombosis of a vein of Galen aneurysmal malformation: possible effects of contrast media

A 5-year-old boy with macrocephaly and mental retardation was referred for radiologic evaluation. After cranial CT and MR imaging, the diagnosis of mural type vein of Galen aneurysmal malformation was established by angiography. Two weeks later, preembolization angiography revealed complete thrombosis of the malformation. Although it is a very rare event, vein of Galen aneurysmal malformation may spontaneously thrombose following diagnostic angiography. Possible effects of contrast media on thrombosis were discussed. Received: 27 April 1999; Revised: 29 September 1999; Accepted: 28 January 2000     

Adult choroidal vein of Galen malformation.

We report staged embolization and stereotactic radiation in a true adult choroidal vein of Galen malformation. Management dilemmas and their resolutions are also discussed.     

In utero diagnosis of a vein of Galen aneurysm by ultrasound

Summary A large midline supratentorial cystic lesion with associated cardiomegaly was seen in utero by ultrasound during the 34th week of pregnancy. The provisional diagnosis of a vein of Galen aneurysm was confirmed with CT and angiography after birth.     

Case report: in utero diagnosis of a vein of Galen aneurysm using colour flow Doppler

We report the ante-natal diagnosis of a vein of Galen aneurysm using colour flow Doppler at 33 weeks gestation. Ultrasound demonstrated a midline, tubular, echo-free structure superior to the thalamus extending posteriorly. Venous flow was demonstrated within the structure using colour flow Doppler. The diagnosis was confirmed by post-natal sonography. We believe this to be the first ante-natal diagnosis of this condition using colour flow Doppler.     

Angiographic findings in two cases of aneurysmal malformation of vein of Galen prior to spontaneous thrombosis: therapeutic implications.

Two cases of aneurysmal malformations of the vein of Galen with later spontaneous thrombosis are reported. Angiograms before thrombosis in both cases showed mural type aneurysmal malformations of the vein of Galen with slow arteriovenous shunts and associated stagnation of contrast in the venous sac secondary to severe outflow restriction. Based on these angiographic findings, the patients were managed conservatively and the arteriovenous malformations of the vein of Galen thrombosed with good clinical outcomes. Twenty cases of spontaneous thrombosis previously reported in the literature are reviewed.