Malformation of the great vein of Galen with neonatal heart failure. Report of two cases

The clinical and patholgoical findings in two neonates with the malformation of the great vein of Galen are given. They both reported with serious neonatal heart failure suggesting congenital heart disease. In one of them cardiac catheterization revealed a foetal pattern of circulation causing cyanosis. A bruit and in one of them a thrill over the skull gave the clinical diagnosis of an intracranial arteriovenous aneurysm. They died 48 and 144 hours after birth in spite of medical treatment. The outlook for patients having malformation of the great vein of Galen and suffering neonatal heart failure treated conservatively, seems hopeless.     

Vein of Galen aneurysmal malformation. Different clinical expressiveness. Three case reports

The vein of Galen aneurysmal malformation (VGAM) is a rare cerebro-vascular disorder in neonates. It is characterized by an abnormal direct communication between one or several cerebral arteries and the vein of Galen. It may appear in the neonatal period or afterwards. Three cases of patients affected by VGAM with different clinical expression are presented. Two of them were treated successfully with endovascular embolization. It was not possible to provide the same treatment to the third patient for an intractable congestive heart failure already existing when VGAM was diagnosed.     

Vein of Galen aneurysmal malformation: a neonatal case with unusual evolution]

We report on a neonatal patient case with a cerebral vascular dilation consistent with a vein of Galen malformation diagnosed on an antenatal doppler ultrasound examination. Antenatal diagnosis was confirmed by fetal MRI scan. After delivery, cerebral MRI scan found the same dilatation and showed no cerebral parenchymal damage. The infant's condition was stable and particularly there was no congestive cardiac failure. Before discharge at ten days of age, clinical examination and particularly cardiac examination was normal. At 14 days of age, symptoms in relation to a congestive cardiac failure appeared. Echocardiography showed a high output cardiac failure with no cardiac malformation. Infectious laboratory tests were normal. Evolution was initially favourable after digitalo-diuretic treatment. Unfortunately, death occurred at 36 days of age due to intractable cardiac failure. This case highlights that, unusually, heart failure can occur long after the first days of life in cases of Galen vein aneurysmal malformation. The optimal time for vascular embolization in neonatal cases of Galen vein aneurysmal malformation is discussed.     

An unusual cause of cardiac failure in a neonate

We report the case of a newborn admitted with signs of congestive cardiac failure with prominent and pulsatile cervical veins. Echocardiography showed a structurally normal heart, right-to-left ductal flow, and reversed diastolic flow in the proximal descending aorta. A computed-tomography scan of the head showed a vein of Galen arteriovenous malformation. This highlights the importance of considering an intracranial cause in the differential diagnosis of neonatal congestive heart failure.     

Arteriovenous malformation of the vein of Galen: treatment in a neonate.

Heart failure in a 2-day-old infant was not readily explained by clinical examination. Cardiac catheterization suggested an intracranial arteriovenous (AV) fistula, and cerebral arteriography showed a malformation of the vein of Galen. The major feeding arteries were surgically obliterated. At age 27 months, the boy has normal mentation but moderate left hemiparesis. Review of the literature disclosed 39 other infants with AV malformations of the vein of Galen producing heart failure before age 3 months. Most of them were boys, and had cyanosis, a systolic murmer, cranial bruit, cardiomegaly, and right bentricular hypertrophy. Only three of the 13 who had surgery for their malformation survived. Removal of the malformation is difficult; obliteration of the nutrient vessels, using the operating microscope, is the currently accepted treatment.     

Cerebral AV fistula with cardiac insufficiency in newborns (author's transl)

A case of arteriovenous aneurysm of the great vein of Galen with congestive heart failure in a newborn infant is presented. Efficiancy of medical management and stop of growth of the head circumference point towards a good prognosis. Patients with this malformation have been divided into four clinical groups presenting with different symptoms in different ages. Anatomy, hemodynamics, clinical picture and diagnosis are considered.     

Management and outcome of vein of galen malformation in childhood

The vein of Galen malformation (VGM) is a rare congenital cerebral vascular malformation. In most cases it already has been diagnosed prenatally. The impact of heart failure due to the arteriovenous shunt is relevant for the prognosis.Prognosis of neonatal symptomatic VGM with early heart failure is severe. Therefore an interdisciplinary cooperation is necessary and it is important to treat patients in a clinic with a perinatal center and a neuroradiology and neurosurgery that is experienced in treating such patients. The genesis, the pathophysiology, the diagnostic agents, the therapeutic agents and the prognosis of VGM will be described.     

Arteriovenous malformations of the vein of Galen

Vein of Galen arteriovenous malformations encompass a diverse group of vascular anomalies that share a common feature: dilatation of the vein of Galen. Although clinical presentations are highly variable, depending on age of presentation, signs and symptoms overlap between age groups. The association of heart failure and cranial bruit constitutes the most striking clinical presentation in neonates. However, less severe and fulminant modes of presentation are frequent in older infants, children, and adults. Treatment approaches consist of symptomatic treatment of heart failure on the one hand and of surgery or endovascular treatment on the other. The results of the latter have improved in recent years, opening up a broad spectrum of new possibilities. We present the case of an asymptomatic 15-day-old neonate who presented an arteriovenous malformation of the vein of Galen and who was treated with endovascular occlusion of the arterial afferents. An excellent result was obtained with no evidence of neurological abnormalities.     

Aneurysm of the vein of Galen with neonatal cardiac insufficiency. Success of early neurosurgical treatment

The vein of Galen malformation is a rarely recognized cause of congestive heart failure in the newborn. This report describes a newborn who developed severe cardiac failure in the second week of life. An aneurysm of the vein of Galen was noted on the computerized tomography scan, confirming the clinical diagnosis of cerebral arteriovenous fistula. The vascular abnormality was well shown by cerebral arteriography. At the age of four weeks, surgery was carried out by clipping the afferent arteries. The patient is non a three years old infant with moderate developmental delay and hypotonia.     

Evaluation of vein of Galen arteriovenous malformation in newborns by two dimensional ultrasound,pulsed and colour Doppler method

Two babies with severe cardiac failure caused by a large arteriovenous malformation of the vein of Galen were evaluated with 2-dimensional ultrasound, pulsed and colour Doppler techniques. In both cases similar findings were recorded: dilated right cardiac chambers with high cardiac output in superior vena cava and ascending aorta; retrograde diastolic flow in descending aorta and continuous forward flow in arch arteries reflecting low resistance to flow in cerebral arteries; recirculation of microbubbles through the superior vena cava after passage unchanged through the cerebral malformation following contrast injection of saline into a peripheral vein or an arterial ombilical line. In addition, colour Doppler study of the brain clearly showed the malformation and the dilated straight sinus. Arteriovenous malformation of the vein of Galen can be noninvasively and easily detected by ultrasound studies of the heart and the brain.     

Rare basal vein fistula with dilated vein of Galen

The authors present a rare case of arteriovenous fistula (AVF) of the basal vein of Rosenthal draining into a dilated vein of Galen managed by transarterial endovascular embolization. A male infant born at full term following a normal pregnancy and delivery with congestive heart failure, on investigation with MR imaging and MR angiography was found to have a basal vein of Rosenthal fistula with a dilated vein of Galen. His congestive heart failure was treated medically, and the AVF was managed electively at 10 months of age with successful transarterial endovascular embolization. The authors discuss the embryological aspects related to the pathological entity and the various clinical presentations, investigations, and management options. Management is primarily endovascular embolization; microsurgery is performed for a few selected cases, and radiosurgery has a limited role in older patients. Endovascular embolization is a safe and effective way to manage this malformation, with an excellent outcome if the AVF is eliminated by proper embolization at the fistulous point.     

Cardiac failure caused by arteriovenous malformation in the area of Galen's vein. Treatment by endovascular embolization

The authors report a case, in a 7-month-old girl, of cerebral arteriovenous malformation with a vein of Galen ectasia, complicated by a congestive heart failure and hydrocephalus. The clinical diagnosis was carried out by fontanelle auscultation and confirmed by: cerebral echography. D?ppler exploration, computerized tomography and cerebral angiography. A cerebral embolization permitted reduction of cardiac insufficiency and stabilisation of the hydrocephalus.     

Spontaneous resolution of marked dilatation of cerebral duro-venous system in newborn presenting with fetal hydrops

A newborn with antenatal diagnosis of fetal hydrops at 36 wk of gestation, presented with congestive heart failure (CHF) and generalized edema. Computed tomographic angiography showed marked dilatation of cerebral duro-venous system including vein of Galen (VOG), straight sinus, torcula and transverse sinus without evidence of arteriovenous fistulae at the vein of Galen. Dilatation of duro-venous system resolved with concomitant improvement in biventricular function and CHF with decongestive therapy. Such entity should be differentiated from more serious conditions like VOG malformation and venous sinus thrombosis.     

Congenital cardiac anomalies with vein of Galen malformations in infants

Published reports and personal experience are reviewed relating to patients under 1 year of age diagnosed with a vein of Galen malformation and congenital heart disease. Including five patients from this institution, a total of 23 patients (12 neonates) with congenital heart disease and a vein of Galen malformation have been reported. Six of these had sinus venosus atrial septal defect and nine had aortic coarctation.     

Vein of Galen aneurysmal malformation associated with an endoglin gene mutation

A child with vein of Galen aneurysmal malformation (VGAM) presented with cardiac failure in the neonatal period. The family history revealed his mother to have hereditary hemorrhagic telangiectasia. The child underwent an endoglin genetic analysis after the newborn period, which eventually demonstrated an endoglin mutation. The pathogenesis of VGAM is currently unknown. The findings of this case suggest that an endoglin mutation might be linked with VGAM.     

Congenital cardiac anomalies with vein of Galen malformations in infants

Published reports and personal experience are reviewed relating to patients under 1 year of age diagnosed with a vein of Galen malformation and congenital heart disease. Including five patients from this institution, a total of 23 patients (12 neonates) with congenital heart disease and a vein of Galen malformation have been reported. Six of these had sinus venosus atrial septal defect and nine had aortic coarctation.

     

Arteriovenous malformation of the vein of Galen in children

Arteriovenous malformation of the vein of Galen (VGM) is a unique vascular anomaly in children. Its extra parenchymal location allows for aggressive endovascular treatment. Depending on the age of the child, the clinical presentation of VGM varies from congestive heart failure to hydrocephalus. The deleterious consequences to the brain depend on many factors, the most important of which is the volume of blood shunting through the malformation and away from the brain parenchyma (“steal” phenomenon). High flow in a fetus with a developing brain will lead not only to brain ischemia but also to multiple organ failure. At the other end of the spectrum, a small malformation can be well tolerated. Treatment should be tailored to each individual case. Received/accepted: 20 December 1996     

Arteriovenous malformation of the great cerebral vein (of Galen) in a newborn

This is a report of a case with an arteriovenous (a.v.) malformation of the great cerebral vein. Applicable diagnostic procedures are reviewed. The initial diagnostic procedure in the approach to an a.v. malformation of the great vein of Galen is cerebral ultrasound.The findings in our patient are compared with those reported in the literature.     

Congestive heart failure in a newborn infant with arteriovenous malformation of the vein of Galen: diagnosis using color-coded Doppler image echography

Arteriovenous malformations of the vein of Galen are rare disorders that may appear in the newborn period with severe congestive heart failure mimicking many intrinsic cardiac defects. Using combined two-dimensional ultrasound and color-coded blood flow mapping arterio-venous aneurysm of the vein of Galen and congenital atrial septal defect could be diagnosed in an newborn with congestive heart failure. In addition to the presented clinical value of the new two-dimensional color Doppler echography physiological aspects of intracranial arteriovenous fistula in infancy discussed.     

Rapid normalization of marked dilatation of the cerebral duro-venous system in a newborn infant mimicking a great vein of Galen varix

A newborn infant with a marked dilatation of the cerebral duro-venous system is presented. The patient was diagnosed as having a vein of Galen aneurysmal varix by a cranial ultrasound examination immediately following delivery. Computed tomographic angiography on the following day, however, showed a marked dilatation of the cerebral duro-venous system, including the great vein of Galen, superior sagittal sinus, torcular herophili and transverse sinuses. There were no arteriovenous fistulas at the vein of Galen. Dilatation of the duro-venous system and concomitant heart failure subsided rapidly after intravenous administration of indomethacin for the treatment of the patent ductus arteriosus on the fourth day of life. Dilatation of the duro-venous system in a newborn infant should be differentiated from any form of vein of Galen aneurysm.