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.     

Ultrasound detection of congenital Arteriovenous aneurysm of the great cerebral vein of Galen

Using combined echoencephalography and Doppler flow determination the diagnosis Arteriovenous aneurysm of the great cerebral vein of Galen could be made in two infants. Without vascular surgery one patient died, the other recovered completely. CT scanning confirmed the diagnosis. Invasive methods such as cerebral angiography were avoided.Case histories, neuropathological findings, ultrasound method and results are presented. Pathogenesis, clinical signs, treatment and prognosis are discussed. With the ultrasound method presented, the nature and location of the vascular anomaly were demonstrable, thus additional higher risk diagnostic methods could be avoided or planned more purposefully.     

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.     

MALFORMATION OF THE GREAT VEIN OF GALEN WITH NEONATAL HEART FAILURE Report of Two Cases

ABSTRACT. The clinical and pathological 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.     

Prenatal MR findings in a case of aneurysm of the vein of Galen

We describe a case of aneurysm of the vein of Galen (AVG), which was diagnosed by intrauterine US, MRI and MRA. The baby girl was born at 35 weeks' gestation. She had severe clinical symptoms at birth and died at 29 h of age from intractable congestive heart failure. Intrauterine US detected an intracranial aneurysm and cardiomegaly due to excessive arteriovenous shunting. Intrauterine MRI (SSFSE) confirmed the diagnosis of AVG, and intrauterine MRA (2D-TOF) successfully demonstrated the anatomical structure of the AVG. MRA may be a useful additional sequence to evaluate AVG, and 2D-TOF is thought to be an appropriate technique for scanning fetal AVG. Received: 1 November 1999 Accepted: 27 March 2000     

Cerebral arteriovenous malformation in a neonate: treatment by embolisation.

A neonate with an aneurysm of the vein of Galen was treated by embolisation using Giant-urco coils. Doppler ultrasound examination showed that blood flow in the internal carotid artery decreased while that in the pericallosal artery increased after occlusion, suggesting a ''steal phenomenon'' with blood directed preferentially towards the aneurysm.     

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.     

Vein of galen aneurysm: MRI with a fast gradient refocusing pulse sequence

A case of vein of Galen aneurysm, studied with a fast gradient refocusing pulse MRI sequence, is presented. Fast MRI sequences allowed the acquisition of additional hemodynamic information that was not available with either routine MRI or angiography.     

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.     

A vein of Galen aneurysm with an abnormal drain system: MRI findings

The case of premature girl with a vein of Galen aneurysm is reported. The lesion was detected by ultrasonography. MRI provided complete anatomical information, obviating the need for initial angiography for preoperative evaluation of the malformation. Venous drainage through a persistent embryonic falcine sinus and associated atresia of the straight sinus could be identified, while venous thrombosis and parenchymal damage could be excluded.     

Diagnosis of an aneurysm of the great cerebral vein of Galen using duplex sonography

The combination of two-dimensional sonography of the skull and pulsed Doppler measurements (= Duplex-scan-technique) was used to detect an aneurysm of the vein of Galen in a three months old infant, who presented with clinical signs of congestive heart failure. Excellent correlation of sonography with computed tomography and intravenous digital subtraction angiography could be demonstrated. A short review of the pathological and clinical findings of this lesion is given. The value of Duplex-scanning in the diagnostic approach of arterio-venous malformations of the brain in comparison with other radiological methods is discussed.     

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.     

Cerebral arteriovenous malformations (CAVM)]

Two infants with endocranic A-V malformations and irreversible picture of congestive cardiac failure are presented: the first is a newborn with a very large angioma, the second is a newborn with a large aneurysm of the vein of Galen. A review of the literature is presented: the salient age-related features required to make a diagnosis are discussed.     

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.     

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.     

Spectrum of clinical presentations of vein of Galen aneurysm

Aneurysm of the vein of Galen is a rare intracranial vascular malformation. It is known to have diverse manifestations and varying severity. Four cases with different modes of presentation and outcome are reported. A mortality of 50 per cent was encountered. Among the survivors, one had neurologic sequelae whereas the other had attained age-appropriate developmental milestones. The former was a rare case of spontaneous thrombosis of the aneurysm while the latter was a boy who underwent therapeutic embolization.     

Aneurysm of the vein of Galen as a possible cause of congestive heart failure: a report of 5 new cases

We report on five newborns with an arterio-venous malformation of the vein of Galen. All newborns were cyanotic and in congestive heart failure without any evidence of congenital heart disease. Congestive heart failure in these cases was mainly due to an almost two-fold increase in cardiac output of approximately 8 l/min/m2 (normal: 4.5 l/min/m2). According to previous reports, mortality is very high in patients with this malformation when becoming symptomatic during infancy, and therapy by surgery or embolization is only successful in 10-30%. While three of our patients died shortly after diagnosis because of untreatable heart failure, the other two were operated on either by subtotal ligation of the draining vein or by ligation of 4 arterial feeders. In the first case secondary thrombosis of the aneurysm occurred and cardiac failure subsided. In the second case a large shunt remained and a balloon-embolization was performed successfully. However, in both patients severe neurologic defects occurred, the severity of which remains to be assessed later since the post-operative observation period is only 2 and 5 months, respectively.     

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.     

Aneurysm of the vein of Galen

We report a case of a vein of Galen Aneurysm in a neonate in which MR imaging provided precise anatomic information, including feeding and draining vessels. MRI may become the most definitive imaging procedure in vein of Galen malformations.