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.     

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.     

Magnetic resonance demonstration in the newborn of generalized cerebral venous dilation with spontaneous resolution.

The authors present serial magnetic resonance (MR) images of an infant with cardiac failure and generalized cerebral venous dilation, which was initially misdiagnosed in the first week of life on cranial ultrasound as a vein of Galen malformation. At 3 months of age, repeat MR imaging demonstrated complete resolution of this marked cerebral venous distension with no evidence for cerebral injury. This case illustrates the value of MR in the identification of this disorder and its distinction from more serious conditions, such as vein of Galen malformation and venous sinus thrombosis. Complete resolution of the venous dilation and the lack of definable parenchymal injury suggest a good prognosis for this disorder.     

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.     

Periventricular calcifications in a newborn associated with aneurysm of the great vein of Galen

Summary A newborn infant who presented with heart failure was diagnosed to have an aneurysm of the vein of Galen. Skull radiographs and CT scan demonstrated multiple periventricular calcifications. This case illustrates a new diagnostic possibility for the radiologic finding of periventricular calcifications in the newborn. It also establishes the importance of performing good skull roentgenographic series in all neonates with unexplained heart failure.     

Neonatal intraventricular hemorrhage due to an intracranial arteriovenous malformation: a case report.

A full-term newborn infant with intraventricular hemorrhage due to a small arteriovenous malformation draining into the vein of Galen is presented. Other reported series are reviewed, and differences between this case and the usual pattern of morbidity are discussed. The use of computerized tomography is demonstrated.     

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.     

A new case of aneurysm of Galen's vein. Use of echography. Treatment difficulties

A new case of intracranial arteriovenous malformation was observed in a newborn infant presenting as severe congestive heart failure, massive cardiomegaly and intracranial murmur. Cerebral echotomography through the fontanelle showed a large echo-free structure behind the third ventricle. Cerebral arteriography confirmed the vein of Galen aneurysm. A neurosurgical therapy clipping the nutrient vessels had not allowed this child to survive. Diagnostic value of ultrasound examination is emphasized. Surgical therapy is discussed.     

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.     

Subclavian-artery-to-innominate-vein fistula presenting with congestive failure in a newborn infant

Congestive heart failure associated with intrathoracic arterio-venous fistula has not, to our knowledge, been reported previously in a newborn infant. A 2 X 4 mm arterio-venous fistula between the subclavian artery and innominate vein presented on the first day of life with heart failure and required surgical intervention. The uncommon location of the fistula produced potentially misleading radiologic findings. When the infant was 2 days old the fistula and a 12 mm diameter persistent ductus arteriosus were ligated; following this, heart failure regressed.     

Endovascular remodeling technique for vein of Galen aneurysmal malformations--angiographic confirmation of a connection between the median prosencephalic vein and the deep venous system

It is commonly believed that in vein of Galen aneurysmal malformations (VGAMs) venous structures normally constituting the deep or Galenic venous system, such as the internal cerebral vein (ICV) and the basal vein of Rosenthal, are not connected to the vein of Galen. In this report, the authors describe 2 cases of successfully treated VGAM in which drainage of an ICV into the vein of Galen was confirmed by follow-up angiography. Two mural types of VGAM were treated using transarterial glue embolization when 1 child was 5 months and the other was 6 months old. The postoperative outcomes for these babies were complete cures. Follow-up digital subtraction angiography obtained after 12 months (Case 1) and 6 months (Case 2) confirmed that the shrunken median prosencephalic vein connects with the deep venous system. The possibility of normal deep Galenic venous drainage must be considered in endovascular management of VGAM. The goal of endovascular intervention is to close only the ventral component of the dilated median prosencephalic vein.     

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.     

Urgent and emergent embolization of lesions of the head and neck in children: indications and results

Indications for and results and complications of embolization of lesions of the head and neck were analyzed retrospectively. The procedures were performed since 1980 on an emergent or urgent basis in 30 infants and children by an experienced interventional neuroradiologist in Bicetre, France. Indications for embolization included hemorrhage, occular occlusion, respiratory obstruction, CNS complications or potential complications, interference with nutrition, and functional impairment related to the effect of the lesion on the developing facial skeleton and teeth. The specific lesions included seven hemangiomas (palpebral, subglottic, and nasal) and 20 vascular malformations (maxillofacial, auricular, dural, cerebral [including three vein of Galen malformations] and spinomedullary). Embolization was efficacious in 28 of 30 patients. Hemangiomas (potentially involutive tumors) responded dramatically with arrest of the proliferative phase and shrinking of the mass. Combined hemovascular lymphatic malformations (hemolymphangiomas) of the tongue demonstrated a variable decrease in size. High-flow evolutive arteriovenous malformations involving the teeth and dura were controlled but required multiple embolizations. One infant with a vein of Galen arteriovenous malformation died. Three local complications occurred in two patients. No cerebral ischemic or femoral artery complications occurred.     

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.     

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.     

Seizures in the preterm infant: effects on cerebral blood flow velocity, intracranial pressure, and arterial blood pressure

The relationship of neonatal seizures to changes in the cerebral circulation was studied in 12 premature newborn infants. The objectives of the study were to determine whether important alterations in cerebral hemodynamics occur with neonatal seizures and whether such alterations relate to systemic hemodynamic events. Blood flow velocity in the anterior cerebral arteries was measured by a transcutaneous Doppler technique. A marked increase in cerebral blood flow velocity was documented with seizures in every patient. The prominent changes in the cerebral circulation occurred despite the fact that 10 of the 12 infants had only subtle seizures and all 12 were receiving mechanical ventilation at the time of the seizures. Accompanying the increase in cerebral flow velocity was a marked increase in intracranial pressure. The cerebral hemodynamic changes appeared to reflect directly changes in systemic hemodynamic events, that is, a marked increase in blood pressure at the time of seizures. The increase in cerebral blood flow velocity with seizures, an apparent adaptive physiologic response in older individuals, may be maladaptive in the newborn infant with certain vulnerable capillary beds, such as the germinal matrix in the premature infant or the margins of an infarct in the asphyxiated infant.     

Developmental deep venous system anomaly associated with congenital malformation of the brain

We report a rare case of developmental deep venous system anomaly. The great vein of Galen and the straight sinus were absent. Both internal cerebral veins and the basal veins of Rosenthal drained into a large frontal interhemispheric falcine vein, which eventually drained into the superior sagittal sinus. The patient also had an associated neuronal migration anomaly.     

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.     

Cerebral injury in association with profound iatrogenic hyperglycemia in a neonate.

The adverse effect of hyperglycemia on neurological outcome following cerebral ischemia has been established in both experimental and adult human studies. However, there is a paucity of data to assess this risk in the human infant. This case is the first to describe severe and prolonged iatrogenic hyperglycemia in association with ischemia in an infant who sustained a major cerebral lesion. The topography of the predominant cerebral injury, i.e., bilateral parieto-occipital cortex and subcortical white matter, shown by magnetic resonance imaging was similar to that documented in the hypoglycemic infant, suggesting a similarity in regional cerebral vulnerability to altered glucose metabolism. This case extends recognition of the risk of severe hyperglycemia in association with cerebral ischemia to the newborn and suggests also that the posterior cerebral predilection for injury is similar in both hypoglycemia and hyperglycemia.