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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.

Autopsy↗

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.

Cerebral Angiography↗

Efficacy and safety of two different galenic formulations of a transdermal clonidine system in the treatment of hypertension.

The efficacy and safety of two different galenic formulations of transdermal clonidine systems were compared in 905 patients with mild to moderate essential hypertension during a 12 weeks observation period. 449 patients were treated with the matrix controlled clonidine transdermal (C-TD) system and 456 patients with the membrane controlled clonidine system (C-TTS). The mean blood pressure was reduced from 168/102 mmHg to 146/87 mmHg in the C-TD and from 169/102 mmHg to 148/88 mmHg in the C-TTS group. There was no difference in the response rate. The pattern of systemic side-effects appeared to correspond to those seen during oral clonidine treatment but with a lower frequency and intensity. The C-TD patch improved skin tolerability and the drop-out rate due to skin reactions was 2.2 times lower compared with the C-TTS group. Local skin reactions led to withdrawal in 35 of 449 C-TD treated and in 79 of 456 C-TTS treated patients. In conclusion, the improved carrier system for the transdermal clonidine treatment reduced local side-effects which in turn may further enhance acceptability and compliance in treated patients.

Administration, Cutaneous↗

A new haemodynamic factor in cerebral AVM. Aspiration from the venous system demonstrated in two cases of pedonculo-Galen AVM successfully cured by occlusion of the superior longitudinal sinus.

Using a Doppler Mark 500 of ATL we measured the systolic and diastolic velocity of the red cells into the main arteries of the neck (diastolic rate cm/sec) thus approaching the flow in two cases of upper brain stem AVM with a unique drainage into the superior longitudinal sinus through an enlarged vein of Galen and a falcine sinus. Because of the desperate clinical condition of the children it was decided to stop the high blood flow by closing the aspiration coming from the low pressure of the venous system above and below the site of union between the malformation and the superior longitudinal sinus. An intra-operative and post-operative measurement of the diastolic rate showed a radical effect of the closure of the superior longitudinal sinus. The demonstration of this aspirative factor has allowed us to classify an AVM as a haemodynamic push-pull malformation.

Cerebral Angiography↗

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

The case of a 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.

Carotid Arteries↗

Varied CT appearance of aneurysms of the vein of Galen in infancy.

An aneurysm of the vein of Galen can have a varied CT appearance in infancy. The classical picture is a spherical posterior third ventricular mass with the density of circulating blood contiguous with a dilated straight sinus and with uniform contrast enhancement. With varying degrees of thrombosis of the aneurysm which may occur in late infancy, the mass can change in density and the dilated straight sinus disappear. With total thrombosis, a precontrast hyperdense rim develops with a low density. The rim enhances but the low density center does not change with contrast infusion. Our experience in three patients with this spectrum of CT findings will be demonstrated and correlated with clinical findings and angiography.

Brain↗

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↗

[Consequences of galenic differences and outcome of clinical trials with budesonide and 5-aminosalicylic acids for therapy of Crohn disease].

Budesonide in both galenic forms is suitable for the treatment of a flare of Crohn's disease of up to moderate activity. The same holds true for 5-amino-salicylates, although they are less effective. Topical steroids delay but do not prevent relapses. Aminosalicylates may be used in the postoperative situation for prevention of relapse but are not significantly effective after drug-induced remission.

Anti-Inflammatory Agents↗

Aneurysmal malformation of the vein of Galen in three patients: clinical and radiological follow-up.

We studied three patients with aneurysmal malformations of the vein of Galen: all underwent embolisation followed by MRI and conventional angiography; two also underwent postembolisation magnetic resonance angiography (MRA). MRI was performed before treatment in two patients, MRA in only one, diagnosed prenatally. Two patients had normal appearances on follow-up MR studies and were completely cured by embolisation. However, the last patient, after two embolisations, despite the stabilisation of the clinical condition, underwent surgery and died. We think MRI is mandatory before endoarterial treatment, to assess the conditions of the brain. Angiography is mandatory only at the time of endovascular treatment, while MRA and MRI have a role in follow-up. Endoarterial embolisation remains the treatment of choice, and surgery is not advisable.

Cerebral Angiography↗

Neonate with vein of Galen malformation and heart failure: serial changes in plasma B-type natriuretic peptide following endovascular embolization.

We report a neonate with vein of Galen malformation (VGM) who developed congestive heart failure (CHF) early after birth. Serial changes in plasma B-type natriuretic peptide (BNP) following an endovascular embolization procedure for VGM were mirrored in his clinical CHF status. The plasma BNP level markedly increased to 1800 pg/ml (normal, <100 pg/ml) in accordance with the severity of CHF. It rapidly decreased to 356 pg/ml during the first week after endovascular embolization for VGM. In the following 3 weeks there was an unexpected upward trend in plasma BNP despite echocardiography revealing normal biventricular function. After additional evaluation and treatment for CHF, BNP decreased again and the patient's clinical status concurrently improved. The patient was discharged with a normal BNP level. Monitoring serial plasma BNP provides valuable information regarding the need for additional evaluation or treatment of newborns with CHF and may be used to document improvement.

Central Nervous System Vascular Malformations↗

Severe pulmonary hypertensive vascular disease in two newborns with aneurysmal vein of galen.

Arteriovenous malformation of the vein of Galen (AVG) is a rare entity in the newborn with a high morbidity and mortality. We present two cases of fatal AVG with persistent pulmonary hypertension of the newborn and significant pulmonary hypertension documented by autopsy histopathology. The pathophysiology is reviewed and a proposed mechanism of the association between AVG and pulmonary hypertension is discussed.

Cerebral Veins↗

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.

Adult↗

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.

Cerebral Veins↗

Vein of Galen vascular malformations in infants: clinical, radiological and therapeutic aspect.

A series of 14 vein of Galen vascular malformations diagnosed in the pediatric populations and treated at the Hospital for Sick Children-Necker, Paris, between 1988 and 1994 is presented. Five of the patients were diagnosed in the neonatal period, of whom 4 presented with life-threatening, intractable cardiac decompensation and high-flow arteriovenous fistulae. Embolization was performed on vital indications in 4 patients during the first week after birth. One embolization failed with fatal outcome. Of the 3 who were embolized, 2 succumbed within 1 week and 1 survived with marked improvement of cardiac symptoms. The older children presented with hydrocephalus and neurologic symptoms. The 10 patients older than 1 year were embolized. These procedures were successful in 90 %, with hemodynamic stabilization and improvement of clinical symptoms. In this group the mortality rate was 10 %. The total mortality rate was 29 %. Hydrocephalus was secondary to a compression of the Sylvian aqueduct in 44 % of cases. Five patients had ventricular drainage before embolization followed by a staged elective embolization. Transarterial embolizations were performed in 11 patients, whereas 2 patients were embolized via the transvenous route.

Arteriovenous Fistula↗

Digitate intravascular appendages and aneurysm of the vein of Galen.

Peculiar intravascular appendages were found in a case of aneurysm of the vein of Galen. Such appendages consisted of finger-like polypoid growths which protruded within the lumen of ectatic cerebral veins and arteries. They were solid, relatively large, and non-branching. Histologically, their structure was comparable to that of a vessel wall, but was concentrically laminated around the longitudinal axis. Some appendages were associated with thrombi. It is suggested that appendages of this kind probably represent secondary vascular malformations, that they are likely to develop in reaction to abnormal hemodynamic strains upon the vessel walls, and that they might be thrombogenic and, therefore, potentially beneficial for patients with arteriovenous shunts.

Cerebral Veins↗

Congenital cystic adenomatoid malformation type 4 and aneurysm of the vein of Galen: a rare coincidence or possibly related association.

As far as the prognosis is concerned, it is important to diagnose the exact type of congenital cystic adenomatoid malformation (CCAM) in order to exclude associated anomalies, as well as the risk of development of malignancies in later life. The frequency of associated malformations of CCAM type 4 is unknown. We report a 4-month-old boy with CCAM type 4 and aneurysmal malformation of the vein of Galen (AVG). Although AVG is the most frequent arteriovenous malformation during childhood, this is the first case report, to our knowledge, of CCAM type 4 in association with an anomaly. In addition, we speculate that the relation between CCAM type 4 and AVG appears to be more likely a possibly related association rather than a rare coincidence, as both malformations develop at the same stages of embryonic life, and pathogenetically, apoptosis play a significant role in both entities. However, further studies are needed to validate this speculation placing emphasis on the association of the two anomalies, otherwise a rare coincidence cannot be excluded.

Cystic Adenomatoid Malformation of Lung, Congenita↗