Frequently Asked Questions

The parent support group is pleased to have had the support and expertise of Dr Wendy Taylor and Professor Karel Ter Brugge in the past to provide their expert medical knowledge in this specialist area. The FAQ page has been devised with their help, and this page will be updated regularly.

Is the condition hereditary
No it is not, in that the chances of having two children with the same condition seem, so far, to be unknown. However, the incidence of VGM does vary across different ethnic groups and is more common in caucasian children. The incidence in Ireland has been higher than in mainland UK for example. Therefore there seems to be a possibility of a predisposition to the condition within certain population groups. (Back to top)

Why do some children have more procedures than others?
VGM is quite a broad-spectrum condition. Some children just have a single av fistula whilst others can have very many. Each of fistulae has to be sealed off and this takes time. Therefore it is safer to treat these children in several stages, as it would take too long in one sitting. (Back to top)

Does the delay in disconnecting all the feeders harm my child?
The doctors are aware of the situation and will be carefully documenting the scans and development of your child. Sometimes it is necessary to treat in sessions quite close together. It is often not necessary to repeat the procedure more regularly than every few months. Once the child is a little larger, the sessions can be spaced even further apart. As long as your child continues to develop normally there is no problem. (Back to top)

My child is cured, but will he/she be normal?
If you child has always developed normally there is no reason to think that he/she would not continue to do so. If your child has any damage to the brain, then this is likely to be a stable situation and your child will develop with this fixed level of damage.
In a study in London it was discovered that fine motor skill (such as peg board tests) is nearly always reduced in these children and many of them are clumsy children. Why this is, is not known. (Back to top)

My child has seizures (epilepsy)- is this related??
One of the effects of untreated VGM can result in brain damage that may be associated with epilepsy. However, epilepsy is a relatively common condition and it is possible that the two conditions occur in the same child and not be causally related. Epilepsy can be caused by many things. (Back to top)

Can there be a spontaneous cure without treatment?
This does occur, but it is very rare. (About 2-3%). (Back to top)

This condition seems to be treated in different ways by different doctors. Why?
This is a difficult question! In the USA there used to be strict controls on the use of various agents, which meant that only one or two places had the licence to use them. Therefore other substances, such as coils were used instead of glue.
Before the development of endovascular techniques, the only option was surgery, and this was a very difficult procedure to perform. The outcomes were not very good.
Even now, the techniques used by the major centres require a high level of skill. As these conditions are rare, it makes sense that these cases are centralised into units that have accumulated the expertise to deal with them. (Back to top)

Is the condition related to other abnormalities?
Some children do have other abnormalities. These can be abnormalities of the great vessels coming out of the heart. Some children may have genital abnormalities. (Back to top)

Can my child play sport?
Children with a true vein of Galen malformation are not at risk of haemorrhage into the brain and there is no reason why they should be prevented from playing any sport. Most affected children seem to be a little clumsy however and hand eye coordination is often reduced, making some sports more difficult for the child.
In contradistinction to this, it would be advisable to avoid fairground rides with high G forces such as roller coasters, because if there is any restriction on blood flowing in the veins in the brain the effects of these forces are uncertain and we have certainly seen complications in children with other brain vascular abnormalities in these circumstances. (Back to top)

Tonsils or Dental extractions?
Some children with VGM utilise alternate pathways for brain drainage and these routes can involve pathways through the orbit and back of the pharynx. You should always consult your specialist before any such procedures are performed to ensure that he/she is satisfied that is no increased risk of haemorrhage. (Back to top)

Is it possible that one leg can be shorter than another, caused by threading a catheter to do the embolisation?
If femoral arterial puncture is done at neonatal age and not performed by experts then this femoral artery may be damaged and become occluded (blocked off) resulting in less blood going directly to the involved leg eventually resulting in interference with growth of that leg. (Back to top)

Do large malformations stunt growth?
in my experience such relationship is very rare. Normally there does not appear to be a direct relationship between the size of the Malformation and growth of the child. V of G M extremely rarely in my experience are found to be associated with growth delay. (Back to top)

How common is it for a patient with a VGM to also have Patent Ductus Arteriosus? (When the heart valve stays open after birth to allow for shunting of extra blood) What takes priority in treating these 2 conditions, and how do they affect one another?
It is hard to give numbers. In my experience it is not common to have clinically significant coexisting conditions. The patent ductus may worsen the heartfaillure and if the vein of galen malformation is not treated may produce faillure to thrive of the child. Vein of Galen should be treated first as it tends to have a positive impact on the inclination to close spontaniously the patent ductus arteriosis. (Back to top)

Is there an ongoing research programme for the condition?
Most of the clinical research is done by the group in Bicetre Paris France (Prof Lasjaunias).Basic science research with respect to VGM is to my knowledge not done by any team. (Back to top)

What if some of the malformation persists?
There are different types of VGM. Some of these have very many small feeders, which would be impossible to completely close off, simply because of the time and risk involved. The important factors in assessing this malformation are the rate of the shunt and the adequacy of venous drainage. Unfortunately these two factors are linked in early life as the veins in the head are still developing at birth. However, if a child is not in heart failure then it is good to wait until that child is bigger for technical reasons. What has to be watched is how the shunt is interacting with the veins.
If a child is normal and developing normally and their veins are stable than a risk/benefit analysis about further treatment has to be made. Repeated embolisation may not bring much benefit and only brings risk. This is a fine judgement that only people who deal with this issue regularly can make. There are many children who are happy and healthy who have residual shunts. There are adult patients who have residual shunts. The risk of a remaining shunt very long term is probably not known, but in a 'true' galenic malformation there is NOT an increased risk of haemorrhage. (Back to top)

Does the size of the VGM decrease in size completely after a successful embolisation?
Nearly all the time does a complete shrinkage occur although sometimes this process may take up to several years to be completed. (Back to top)

Can vomiting be a regular occurrence with VGM children?
No, vomiting can be a sign of raised pressure inside the head but such evolution is very rare in VGM patients
Additional Information:
Vomiting in any young child is fairly common. The commonest reason is infectious viral agents and children quickly recover. Children who have contact with other children will often pick these viruses up, and especially so in hospital environments. Children who have been affected by illness, for any reason, and hospitalised are often more prone to periods of illness and this will include these sorts of virus infections. Vomiting can also be a sign of drug incompatibility and if a child is on multiple medications it may be wise to check that the drug levels are correct for that child. Vomiting can also be a sign of increased pressure within the head, but is often accompanied by other symptoms, such as increased drowsiness and headache.
In any situation where there has been prolonged vomiting, it is better to contact your local paediatrician who can work through the different causes ( and there are many others than these three). It is important to keep your child well hydrated by giving lots of drinks and electrolyte drinks can be obtained over the counter. Often ice cubes and/or ice cream is good because the cold stimulates the gut to work more effectively in the right direction. (Back to top)

Any possible side effects with booster immunisations for tetanus, polio, diphtheria with VGM children and teenagers?
None as far as impact on the VGM is concerned (Back to top)

If a child has been successfully embolised by coils can problems occur later on in life (This question has been asked by several parents where their children have been embolised with coils)?
As you know we are not in favour of using coils for VGM treatment. As far as delayed effects that may occur after coiling one should not be overly concerned. Coils may be extruded from the VGM into the adjacent tissues but that would unlikely result in clinical symptoms. Coils in AVF are known to recanalize in time and therefore follow up is recommended. (Back to top)

What is the average occurrence of VGM?
Statistics re VGAM are not easily available due to the heterogenious presentation (from death to symptomatic)and referral patterns (easy cases locally managed and difficult ones being referred to certain centres) (Back to top)

If a baby is diagnosed in utero, is there any precautions that could be taken by the mother? Are there heart medications she can take that will control congestive heart failure in the baby and is bed rest advised?
VGAM can certainly be diagnosed in utero with ultrasound or MRI and more and more of our patients are diagnosed in that fashion.There is no impact on the mother and no meds can be given in reliable fashion to the mother that will have benefit to the child. (Back to top)

If the case is extreme, should the mother travel by Air to her doctor, and if so, at what point in the pregnancy should the travel occur? Would the change in the cabin pressure of an aeroplane affect the condition of the baby in the womb?
Mothers should have no restrictions with respect to activity or traveling or type of delivery. But knowing that there is a baby with VGAM in utero makes us to organise that the delivery is done in a centre for high risk deliveries neonatal ICU and access to paediatric interventional neurorad team. (Back to top)

Why are feeding tubes required in so many VGM patients and If food can be kept down with tubes why not by mouth?
The most physical effort a new-born baby makes is to feed. If a baby is in incipient or actual heart failure it is much better to remove this effort and substitute it with a tube. Some centres go further and insert a tube directly into the stomach. This is known as a gastrostomy and is certainly more comfortable for the baby if feeding by this route is likely to be prolonged. (Back to top)

Is there a connection with Autistic behaviour and VGM We have found some association with autism?
This is not necessarily causative; babies who suffer horrible experiences when they are very new also have an association. It is not known at all whether there is a true link. (Back to top)

Could birth marks have a link with VGM?
It is difficult to give a definite answer. In principle vascular birth marks are very common and VGMs are not, therefore their coexistence is believed to be fortuitous. On the other hand we have started to see a number patients who do have both. As blood vessels to the brain and the face have a common origin it would not be impossible that an abnormal trigger produced both skin and intracranial vascular malformations. For the moment their coexistence seems not related. (Back to top)

Are patients with Oster-Webber-Rendu at increased risk of VGMs? Could there be any connection between the two conditions if a parent has some symptoms of Oster-Webber-Rendu?
Rendu-Osler-Weber/Osler-Weber-Rendu is a condition which affects blood vessels in many organ systems. It has a variable pattern of presentation and depends on more than one gene mutation. However, it is well recognised that this condition can cause problems with blood vessels in the brain….these problems have NEVER, however, been linked with VGM. If you seem to have some of the symptoms of ROW, genetic analysis will be able to confirm this. ROW can affect the lung, gut, skin and brain and can be responsible for medical problems that will require close medical follow up. If you have this condition as a parent, you may transmit the risk to any child so it is important to know for you and your children. I do not yet know of any cases of VGM and ROW combined- but as far as we know with all available medical evidence there is no genetic link. (Back to top)

Is it common to have a third vetriculostomy for large ventricles due to Hydrocephalus?
It is not common that any form of treatment is needed for hydrocephalus, but occasionally it is really necessary. The indications for treatment are when there are symptoms of acute hydrocephalus, which include headache ( hard to be certain in a young baby), vomiting and an abnormal eye movement known as 'sun setting', when the eyes look down. Third ventriculostomy is a treatment which leaves the baby without any tubing, which can be a source of complication and future problems such as infection. Many children with VGM have big ventricles, but this is not because they are under increased and dangerous pressure and it can be harmful to treat children when the pressure is normal. Therefore it is really important to know what the indication is. It is possible to measure intracranial pressure in a very simple way by measuring the pressure through the ears. Not all centres will have this facility so it may take some research to find it. If the pressure is not so elevated and the baby is well I would recommend that any procedure is avoided. Third ventriculostomy involves a procedure wherby it is made possible for excess fluid to be drained. It involves, inevitably, some damage to the brain- but there is always a risk/benefit assessment and if the baby is sick it could be a good way to help. It takes longer to be effective than placing a tube into the ventricles ( which also involves some damage). (Back to top)

Can there be long term health issues like Bipolar or Schizophrenia?
No-one knows about this. Children who have had a traumatic birth and post natal period may present with some manifestations of autism. Whether this is due directly to the condition or hospitalisation is not known. We have no information on schizophrenia or bipolar disorder. It is really important, if possible, for websites like this to help collect any information about these problems because it may be helpful in the future. (Back to top)

Immunisations for Tetanus, Polio and diphtheria have already been covered in the FAQ but wondered if there was any other vaccines that should be avoided in particular TB ?
There is no contraindication to any vaccination for children with this disorder. (Back to top)

Is it common for a child who has been coiled to have difficulty breathing and is there a procedure to correct this? Also is it common for a young VGM embolised infant who also fits, to become exhausted by very little exercise?
It is unlikely that the method of treatment impacts the capability of the children afterwards. If the management of the VGM was performed to diminish heartfaillure then the impact is usually that the children are less tired and have less difficulty with breathing or excercises. Perhaps if the treatment was incomplete then such symptoms may continue. Additional medical therapy might be needed or further endovascular treatment. (Back to top)

How do I ask a question that is not covered in this FAQ?
Although we are unable to answer your questions direct, we will pass any questions of a general nature to our contacts who will do the best to answer them. You can submit a question via the contact form. (Back to top)