Very rare: < 1/25,000 deliveries. Complex cerebrovascular malformation produced by a pseudoanevrysmal dilation of the bulb of Galen associated with one or more arteriovenous fistulae that make communicate branches of the carotid or the vertebrobasilar artery with the vein of Galen. This malformation drains usually in the right sinus, more rarely in the longitudinal sinus.
Embryologically, it is due to the absence of differentiation of arterial and venous systems at the level of the prosencephalic vein of Markowski. As a result of increased flow and pressure, the vein of Galen is arterialized and expands.
Angiographically, there are two forms which can coexist:
- a form known as 'mural': the shunt is unique with 'full channel' arteriovenous communication
- a choroidal form: fistulas are multiple; the symptomatology tends to be more severe.
History:
- 50 % in the neonatal period:
- heart failure due to the high cardiac output: its severity is directly correlated with the importance of the arteriovenous shunt; there is often an associated pulmonary arterial hypertension (isolated echocardiographic signs of PH due to the increased venous return through the fistula) which does not respond to the inhalation of NO (nitric oxide)
- hydrocephalus caused by cerebral venous hypertension
- a continuous murmur is often heard on the skull
- in the older child: macrocrania with hydrocephalus, sometimes convulsions or psychomotor delay; sometimes: cerebral hemorrhage
Treatment: repeated arterial embolizations (coils or biological glue) by femoral arterial access, according to the Bicetre criterias:
- symptomatic infant: emergency
- newborn and paucisymptomatic infant: wait until 4-5 months of age
|
|
cardiac function |
cerebral function |
respiratory function |
hepatic function |
renal function |
|
5 |
normal |
normal |
normal |
- |
- |
|
4 |
minor overloading without treatment |
subclinical
|
tachypnea, no feeding problems |
- |
- |
|
3 |
cardiac failure, stable |
intermittent neurologic signs |
tachypnea, feeding problems
|
normal |
normal |
|
2 |
cardiac failure, unstable |
isolated convulsion |
assisted ventilation,
|
hepatomegaly, normal hepatic function |
transient anuria |
|
1 |
assisted ventilation |
epilepsy |
assisted ventilation,
|
moderate or transient hepatic failure |
unstable diuresis under treatment |
|
0 |
unresponsive
|
permanent neurologic signs |
assisted ventilation, desaturation |
coagulation disorders, severe cytolysis |
anuria |
Bicêtre criterias: < 8 : no treatment ; 8-12 : embolisation during the neonatal period ;
score > 12 : embolisation around 5 months of age (Neurosurgery 2006 ; 59 S3 : S184-94)
Anesthetic implications:
heart failure or intracranial hypertension; in the newborn: echocardiography to verify the absence of pulmonary arterial hypertension; check the total dose of IV contrast media given during each session of embolization.
References :
- Ashida Y, Miyahara H, Sawada H, Mitani Y, Maruyama K.
Anesthetic management of a neonate with vein of Galen aneurysmal malformations and severe pulmonary hypertension.
Pediatr Anesth 2005; 15: 525-8.
- Berenstein A, Ortiz R, Niimi Y, Elijovich L et al.
Endovascular management of arteriovenous malformations and other intracranial arteriovenous malformations in neonates, infants and children.
Childs Nerv Syst 2010; 26: 1345-58
- Madhaoui N, Merchaoui SN, Fekih M, Bianco CF, Yacoubi MT, Seboui H.
Forme sévère d’un anévrisme de la veine de Galien compliquée d’encéphalomalacie de révélation anténatale.
Rev Méd Périnat 2011; 3: 148-51.
- Smith A, Abruzzo T, Mahmoud M.
Vein of Galen malformation and high-output cardiac failure.
Anesthesiology 2016; 125:597
- Setlur K, Singh S, Verma A.
A masquerader of neonatal persistent pulmonary hypertension.
J Pediatr 2021 ; 233 : 281-2
Updated: June 2021