The two UK centres for treatment of Vein of Galen malformation meet each year to discuss cases and share experience. This year Great Ormond Street hospital hosted the meeting. A particular highlight was the attendance of Dr Karel Ter Brugge, the internationally renowned expert from Toronto, who participated in the case discussions and gave a lecture.

The morning session opened with a review of all cases treated at Glasgow and London since the last meeting. Particular themes which emerged were the use and interpretation of magnetic resonance imaging (MRI), drug regimes before and after procedures, and the use of genetic tests. There is increasing evidence that a small proportion of cases of Vein of Galen malformation may be caused by a genetic mutation. If true (more research is needed) then it will be important to identify cases which are caused by a mutation because it means the disease may affect other pregnancies in the family. Even if this is true, familial cases are extremely rare, with almost all cases being sporadic.

After lunch Dr Ter Brugge gave a lecture to a packed seminar room. His talk covered all malformations of veins and arteries in the brain, not just the Vein of Galen malformation. He emphasised the difference between, on the one hand, the malformations which present in infancy or childhood, and on the other hand, those which present in adulthood. The childhood malformations have different origins and different treatments – as is often heard in children’s hospitals “Children are not just small adults!”. Dr Ter Brugge also encouraged his audience to think about what was happening in the wall of the blood vessel: for some types of malformation (not Vein of Galen) we can fix or block the abnormal flow of blood but the disease process continues in the wall itself.

The final seminar of the day reviewed a number of difficult problems in the management of Vein of Galen malformation. Again there was good discussion between the experts from Glasgow, London and Toronto.

A quick announcement: We have finally got the new forum up and running following the poll that we ran on here a few months ago. We invite all of you that were interested in participating in forum discussion to visit: the new forum here

You will need to sign up and register to post on the new board. There is a suggestions forum where you can make any suggestions for how YOU would like to see the forum grow and develop. Although we may not be able to respond immediately, please rest assured we are listening and your input is very welcome. This site and the forum were set up to help YOU and without YOU we would not be able to continue to grow and give support to countless families around the world.

2011 was an excellent year for this website, with over 25000 visitors and an average of 2100 visitors per month. Hopefully we can continue to improve the site in 2012 and we will continue to try and ensure our site is the number one resource for information and support for this devastating condition. Having a vibrant and active user community on the forums will be another way in which we can deliver that. Please note however that if you don’t use it, it will be closed for inactivity by the owners of the forum hosting space.

Your feedback is always welcome, so please either post on the forum or send us a message through the content page on this site.

thanks!

The VOG Team

Born October 31st, 2010

I was pregnant for the first time and had my third ultra-sound scan on August 27, 2010 (29th week), when my gynaecologist told me that there would be an “abnormality in my child’s brain”. I was totally devastated and very afraid. He presumed that my son had an arachnoidal cyst and so we were sent to the antenatal unit at the University Clinic in Frankfurt to have a more specific ultra-sound scan.

The doctor who did the first test could not find out what my baby had. So she called her boss who was also not able to figure out what was wrong with my baby. Then the Department Head came and said that my little one suffered from Vein of Galen Malformation. I had never heard of this before and he explained that it was a very rare disease; only one out of 25,000 women gave birth to a baby with this problem. He told me that our boy would have a survival chance of 60 percent until his birth. He could die due to heart failure or a bleeding aneurysm. If he would survive until his birth, he would die later on without medical intervention. It was the worst day of my life. The doctor informed us that it would be possible to medicate the malformation after the birth. Years ago they had sent a family to specialists in Paris, where the mother had a C-section and the aneurysm was later clipped. The child had survived but still suffers from seizures, which is a “good” progress regarding the diagnosis Vein of Galen Malformation. Our only thought was that our baby could also survive if he made it to the birth.

Every week we went to the University clinic to monitor his heart condition and the size of the aneurysm. Fortunately, our boy was so tough that both his heart and the aneurysm remained stable. Three weeks after the horrible diagnosis, the Frankfurt doctor found a German specialist who was able to help us. They sent us to the neuro-radiologist Dr. Friedhelm Brassel based at the Duisburg Wedau Clinic, who is one of the leading specialists in treating Vein of Galen Malformation. He told us that he had almost 30 years of experience in treating this kind of malformation and uses the embolisation method rather than the clipping one. More specifically, the combination of a trans-venous and trans-arterial method using a “kissing microcatheter technique”.

There was a chance that our boy could be treated successfully after birth. The ideal time for the intervention was three months after birth. If any difficult conditions arose between the birth and the planned date, they would have to do the intervention earlier which would increase the risks. So we stayed optimistic.

The plan was that I would have a C-section two weeks before the expected due date (November 12th) in order to have the best conditions in case an immediate intervention were necessary. The Frankfurt team worked closely with the Duisburg team in order to offer us the best support. Luckily, over the weeks that followed, my boy’s condition remained stable. A few days before we were to go to Duisburg, I left some amniotic fluid, which meant that I had to go to Duisburg immediately. However, I had to continue to wait in Duisburg since my waters hadn’t really ‘broken’. Two days prior to the planned C-section, I started having contractions and the C-section had to be done earlier.

Fortunately everything went well. Little Vincent was born at 4.31 pm on October 31, 2010. He was taken directly to the intensive care unit for new born babies so that they could check his functions and see if he would manage the new circulatory situation due to the malformation and his heart. He was stable and I was allowed to see my little boy for a few hours after the birth. Vincent had to stay in an incubator for some days in order to be monitored. He did really well and no intervention was necessary which meant that we were permitted to go home after two weeks. Dr. Brassel said that the best date for intervention would be three months after birth, so the date was set for January 31st, 2011. To support his heart, we had to give him Lanitop® and Aldatone®. He also had to be monitored weekly by a paediatric cardiologist.

Back home we had a wonderful time, in spite of the angst we had concerning the necessary intervention. Our paediatrician was also a cardiologist and monitored Vincent with weekly ultra-sound scans. Furthermore we had a check-up in Duisburg just before Christmas and everything seemed well, too. The aneurysm and the heart were still stable. We waited for the 31st January.

Everything went well – until mid January, when Vincent began vomiting often and his eyes rolled down. At first we thought that it was normal for babies to frequently vomit and look strange. Vincent was our first child and our family and friends also confirmed that he would be OK. As we went to our paediatrician on January 13th to have another ultra-sound – he had been on holiday from Christmas until that day – he immediately called the Duisburg team in order to bring the intervention date forward. The aneurysm had grown rapidly and due to the high pressure, Vincent had developed a hydrocephalus as well as the sundown phenomenon.

We were mortified and blamed ourselves for the delay in our reaction. Vincent had his first embolisation on January 20th, which took more than nine hours. Dr. Brassel and his team closed three of four feeding vessels of the malformation using platinum coils. It was very risky but all went well without any complications.

Afterwards Dr. Brassel intended to close the malformation completely in order to minimize the pressure of the aneurysm which led to the hydrocephalus. Since Vincent had several infections and we had to wait in hospital, the second embolisation took place on February 7th. Dr. Brassel was also able to close the last feeding vessel – again without any complications. We were so happy. It was unbelievable. He had saved our son’s life.

Vincent quickly recovered and we went home five days later. A follow up check-up was planned several months later by means of an MRI and angiogram. The date was set for the beginning of May, but Vincent had been improving so well that it was put off until September 14th. We went back to Duisburg and Dr. Brassel told us that Vincent was cured. The MRI and the angiogram both revealed that the aneurysm had already been absorbed and there was no feeding vessel left.

Vincent has been improving in leaps and bounds. During the first three months following the embolisations, he had just a few motoric delays, but for several months now he has been getting better much faster than normal and is a very active and happy child – he makes us unbelievable happy. We are so grateful for the wonderful support from Dr. Brassel and his team who did a terrific job. It is still unbelievable that such a highly skilled therapy is possible. We thank Dr. Brassel and his team for saving the life of our son Vincent. We thank all the people involved, the doctors, specialists and nurses, who supported us in both the worst and happiest time of our lives. Our little boy Vincent is just a wonder.

We hope that our story may help families that are in a similar situation. It is so important to act appropriately and immediately in order to find the right people who can help.

Even though I am still quite affected by the experiences I made, I would be happy to assist with information – as much as possible.

Please contact me via this email-address: vincent.l.koch[at]web.de

Other useful related Information:
The website of the Wedau Duisburg Clinic:

Contact List for Duisburg Clinic

Main Contact Person:
Mrs Friebe, secretary of Prof. Dr. Brassel:
Tel. 0049 / (0) 203 733-2701
E-Mail: f.brassel[at]klinikum-duisburg.de

Further Information concerning the treatment of Vein of Galen Malformation (only in German):

Visitors to this site who used the old site will know that there was a popular message board on there. When we moved to this site, we created a new message board but this was not used anywhere near as regularly and was unfortunately removed by the forum hosting company due to inactivity.

After thinking about this, and inspired by Kate Da Silva’s comment on the “did you know we have a forum” post. We thought we’d put it to the vote. It is you, the users of this site, that the forum would be for after all. If you want it, I will happily create a new one. If you guys think this site does the job well enough then again please vote.

We would encourage you all to please vote and welcome any comments.

Thanks very much,

The Vein Of Galen Support team

I hope this is useful for everybody!

Graham (site admin).

Born October 13th, 2010

Myself Salini and my husband Ajay Kumar got married in Aug 2006.I became pregnant for the 1st time in April 2007. But it was a vascicular mole. 2nd pregnancy was in Dec 2007. That was a twin pregnancy. Scanning in the 2nd month revealed a retarded growth. In both the cases there were minor bleeding also. For the 3rd time we consulted an infertility specialist in the home town but that too was detected as a vascicular mole. It was in Oct 2009.All the three pregnancies were aborted in the 2nd month itself.

By the time we had conducted whatever tests doctors had suggested and nothing was wrong. I became pregnant in all these cases without adopting any medical aids such as IUI,IVF etc… Then we consulted another very famous infertility doctor nearby. This time
also I got conceived in the normal way. But in the 1st month itself there was some bleeding. Doctor was a bit worried on that. He said if I missed that one also he would conduct a laproscopy the next time. But I didn’t miss that one which was the 4th pregnancy in 4 years. Everything progressed well upto the 7th month. Scanning on the 29th week revealed that my baby has got one vein in the brain enlarged which is called Vein of Gallen Aneurysm.The size of the vein was 5.8*2.2cm. We told the matter to our cousin who is a paediatrician. He contacted several cardiologist and neurosurgeons in the country and gave me this site address.

Each enquiry acertained the fact that we had very less chance of getting our baby healthy or even alive. I kept on searching the site. I could never imagine God letting me lose my baby. I started believing the stories of Mia Stewart from UK and that of Kai Westvig from South Africa which were published in the site. It was said that they had survived this disease miraculously.

We came to know that surgery(embolisation) can be done only if our baby become at least 6 months old after birth. Another scanning on the 32nd week revealed that the vein had a size of 5.4*.2.2cm.. I made a statistic on the survival chances of my baby on studying the stories posted in the web. I found that 3 among the 12 babies detected with the disease prior to their birth are still surviving. I started to believe that my baby has 1/4 th survival chance.

I delivered my little one normally at 11.38am on 13th Oct 2010. She was a beautiful baby girl. Though she cried at the time of birth her breathe stopped soon and was rushed to the NICU. At that time the heart beat rate was much high. Doctor administered medicines to bring down the beat rate. Scanning of the head also was done. The terrible beating of the heart could clearly be seen. The pumping of blood through the blood vessel from heart to brain was also clearly visible through the neck. Her body seemed to throw itself up with each beating of the heart. The sight was unbearable. We visited the ICU many times a day. She never opened her eyes. As hours passed by things got worsened. We told the doctors to do what they felt as the best.

On 15 Oct 2010 by 6.30 in the evening my baby was weaned of the ventilation and she passed away at 8.45pm on the same day. She didn’t wait to get named or photographed. The following is what is said in the report of our child:

”This baby was born in our hospital at term with a birth weight of 2.8 kg. Antenatally baby was diagnosed to have ‘Vein of Gallen Malformation with cardiomegally.’

After birth baby needed resuscitation with IV intubation and Positive pressure ventilation and baby had evidence of cardiac failure. Baby subsequently was transfered to NICU and started on ventilatory support,Inotropes and Antibiotics. Chest X-ray showed Cardiomegaly. USG-brain showed a large 3.5*3 cm aneurismal dilation in central part of cerebrum with effacement of ventricles. Turbulent flow seen through it. USB-abdomen was normal. The bad prognosis was explained to the parents. There was nothing to offer medically or surgically to the baby’s problem.The baby was weaned off ventilation and she expired at 8.45 pm. On 15-10-10

Case of death: Congestive cardiac failure secondary to Vein of Gallen Malformation”

The stories in the website and its team of doctors were very much helpful to us at the time of our extreme agony. I will remember with gratitude all those service forever. I publish our story also with the hope that it may help someone in one way or the other. We wish the very best to anyone visiting the site.Be strong and fight till the end and let God take the final decision.

Thank you very much for everything,

Salini Ajaykumar

saliniajaykumar[at]gmail.com

I found out that I was expecting identical twins at my 12 week scan and with no history of twins in our family this came as a very huge shock. Due to the complications that twin pregnancies can come with I was scanned again at 16 weeks and it was at this point it was discovered that I had a condition called Twin to Twin Transfusion which can put both babies life at risk depending on the severity. Initially mine was only thought to be mild but they made a decision to monitor/scan me regularly to keep a close eye on the babies. At 20 weeks we were told that we were expecting twin boys who we named Alfie and Charlie and which we were over the moon about. At a scan at 25 weeks at my local hospital the sonographer picked up an ‘abnormality’ in one of the Alfie’s brain – we were obviously devastated and immediately started to worry about what was wrong and what the future would hold. We were referred to our local fetal medicine unit in Newcastle where we were first told of the diagnosis of Vein of Galen Malformation. It felt like a very dark day and what had already been a pregnancy fraught with difficulty just seemed to become that much harder. We were given a lot of information by the doctors but none were experts and we felt lost. We left the hospital and came home and began to do a lot of online research which probably didn’t help as although there were many positive outcomes there were also those that weren’t.
We continued to be monitored weekly not only for Vein of Galen malformation but also the Twin to Twin Transfusion which was still putting both babies life’s at risk. I was given a choice about where Alfie could be treated and came to decision that we would go to London to Great Ormond Street Hospital as we had family and friends who would be able to support us through what was to come.
I travelled to London for the first time at 32 weeks pregnant as the Twin 2 Twin transfusion had started to cause complications for the boys. I had been told by local hospital that usually they would deliver the babies at this point but Alfies VOG made this not a straight forward decision. I arrived at UCHL to meet a large team of doctors including Dr Stefan Brew who would discuss what was the best plan for the boys. They basically told us that if the boys were delivered at this point and Alfie was born unwell then he would have very little chance of survival. We were told they had previously operated on 5 babies born pre 36 weeks all who had died. We were given the choice as to whether deliver the boys immediately, but we knew that the best chance of both babies surviving was try and get my pregnancy to 36 weeks.
We travelled home on the Monday and I was to be scanned every few days. By the Thursday things had deteriorated to the point that we had already been told if it got to this stage they would have no choice but to deliver the babies. So we were sent immediately back to London and by Friday the team was ready to deliver the boys. They were born at 33 weeks gestation on 07/08/09 Alfie weighing 4lb 1 and Charlie 4lb 2. I got to hold Alfie very briefly before he was taken to intensive care at Great Ormond Street. Alfie was doing very well initially and Dr Brew had told us he would like to wait 4 weeks so that Alfie could grow and gain weight and he would remain at Great Ormond Street till they could perform the first embolisation at 37 weeks. However Alfie’s health started to deteriorate 1 week later and he started to show early signs of heart falure. This was one of the worst days of our lives because we knew his only chance of survival was to have the embolisation but history dictated that he stood a very slim chance of survival. He was taken down to theatre at lunchtime and waiting to hear whether or not he had survived was one of the darkest moments of our lives. We cried and cried when we told he was on his way back from theatre and although he suffered a small bleed to the brain post op he defied all the odds and began to recover. Dr Brew said that he would still need further surgery as he had not managed to seal the artery but wanted wait another 6 months to allow Alfie to grow. So after 5 weeks in hospital we took our boys home.
Alfie was doing really well at home and all the doctors involved were really pleased with his progress. In January 2010 we travelled to London for his second embolisation. This again went very well and Alfie was discharged from hospital within 2 days. His development was deemed as ‘normal’ and he was doing everything his brother could do and more. Following another MRI in November 2010 the team at Great Ormond Street were hopeful that they had potentially sealed the artery and he was booked in for Angiogram in January 2011 which would give an accurate answer as to whether he was ‘cured’. In February 2011 we were given the news that we have dreamed of and that the artery is totally sealed and Alfie will require no further treatment. Dr Brew has told us to go away and live our lives as normal and that hopefully Alfie will have no memory of what he has been though.
Alfie is the happiest little boy you could ever wish to meet he laughs at anything and anyone and is as mischievous as any little boy his age. We are extremely blessed that he is in our lives and we are looking forward to the future and will be eternally grateful to Dr Stefan Brew and all those involved in saving our sons life – he truly is a miracle.

Or you can just go here to jump direct to the forum itself.

Sign up is free but you will need a valid email address in order to complete the registration process and to fully identify you as the person that signed up. Once you have registered, you will be able to chat to other people about their experiences with Vein of Galen malformation.

We hope that the readers of our site find the forum a useful resource. If anyone has any suggestions as to anything else we could do to improve the site, or any information you require that you cannot find here, please contact us and we will try our best to help.

Karls Story

In 1975 I had meningitis, the diagnosis was made at the Cambridge military hospital in Aldershot. At the same time the doctor in question heard a noise. Thinking it was my heart I was sent to the Brompton, finding the heart not to be at fault I was then sent to the Maudsley, who diagnosed an AVM of the vein of Galen. It was at this point that I was sent across to the National. Things went well and my condition was controlled naturally. My life changed as the hormones imbalanced with puberty, it was at this point that our hand was forced into the dramatic step of fitting a shunt system. After a period of illness and several shunt revisions, I once again stabilised.

My life continued relatively uneventfully until, at the age of 25 I started to deteriorate. I was then introduced to Dr Taylor; my first embolisation took place in 1998, the results of which were amazing.

I Have Seen As A Child

Waking up after the procedure was quite an experience, a perceptual change had occurred. The way that I perceived sound had altered: before sound was fragmented (like a pie chart) and flat, now it is three-dimensional; before I had noises inside my head, which had now gone. I now needed more sound and had filled the gap with what I can only describe as electrical noise (when a TV searches for a signal).

Vision had altered with heightened sensitivity to the magenta end of the spectrum. Edge definition had increased giving me a fuller and clearer image. The two senses described above had a profound influence on distance judgement. Since leaving hospital I have become very aware of the weight of my head, it is apparent that the left side is heavier, the side of the valve. The valve itself is still working though with less fluid and more slowly. I have always been conscious of the CSF flow through this shunt and it has never really bothered me, however with the reduced pressure the shunt fills with fluid and remains so, giving me the sensation of an ear full of water. This sensation can last for hours, only easing when all channels open at once, my artificial drain and what I must presume is the natural route. The event is not comfortable when combined with a noticeable change in pressure. The pressure change is only apparent when I am in an upright position.

Another of my changes is the way that I experience motion, travelling in a car was always such a violent experience whereas now it is smoother. Headaches I thought would be a problem, however this appears not to be the case. I have a certain number of uncomfortable feelings going on inside my head, but nothing on the scale that I used to experience. The feelings themselves consist mainly of specific pressure points, the size of one maybe two fingertips pushing outwards. These have occurred above the eyes and both old and current shunt sites; they also seem to occur in a large number of different places across the back of my skull. The altered blood flow had given me fluctuating blood pressure, this extra pressure led to a number of nosebleeds, these have since calmed down.

Trusting what is in essence a new body has been hard. My sense of balance has improved to the extent that I am now able to stand on one leg. This skill was achieved with a certain amount of trepidation as I still have a tremor down the right side of my body.

________________________________________

This has been written by a young adult with a vein of Galen malformation, considered too dangerous to treat in the past…..
Dr Wendy Taylor
Consultant Neuroradiologist

Born July 18th, 2005

This story is about our baby boy, Kai Da Silva. We were living the perfect life, I had an amazing pregnancy – and our son seemed well. When Kai started nursery at 6 months – he got a nasty virus called Rota-Virus – which involved vomiting and diarrhoea for three weeks – within February 2006 I must have taken him to see the GP around 4-5 times as well as went to the local hospital a few times to get him checked out by paeditricians at St. Helier A&E. On the third visit to the A&E, after noticing a bulging fontanel, a young doctor, Kirsty Schmidt, checked Kai’s head size. In the last few months no professionals had once offered to measure his head, even although it looked larger than normal. Kai was diagnosed on 20th of March having malformed Vein of Galen which was causing obstructive hydrocephalus. Our nightmare was only just beginning.

When Kai was diagnosed, there were no neuro-radiologists in the country who could perform the embolisation – they were all away at a conference in Australia.. We had also heard via Lynn Mussard and Vicki Rainbow, having daughters with the same malformation – that we should push to go to Paris – as Prof. Pierre Lasjuanias was the most experienced person in this particular treatment/field. The website was such an enormous help to us as were Lynn and Vicki, they guided us, advised us and told us what to do and how to get to Paris. We are very thankful to both of them and the website as we would never have found them without it, it was like a shining star for us – which led us to where we were going….

We managed to get funding from the Department of Health for the embolisation in Paris. The wait to get to Paris – was painfully long – it was a month in total – and as the time passed Kai’s head just grew and grew. From measuring 51.1cm from diagnosis – to measuring 53.5cm on day of arrival in Paris – one helluva growth for a babies head for one month. The whole procedure and build up was very hard – but Kai was a star and basically did not show any signs of being unwell in himself before the emobolisation. The surgery was a success although seeing him in intensive care for 3 days nearly broke us to pieces – seeing Kai on life-support to help him breathe when waking up from his deep sleep was gut wrenching . We went home 6 days after we arrived in Paris – hoping that Kai was now on the mend. We were told by Prof. Pierre Lasjuanias that Kai was now cured. This, however, was not to be the case.

We took Kai to get his head measured regularly but after one month I went for the weekly measurement and his head had grown 0.6cm in just over one week. This was not supposed to be happening – we called Great Ormond Street straight away and managed to get an earlier appointment – the following week – still not good enough as his head was growing fast again. By the time we got to the appointment Kai’s head had grown another 0.7cm in less than one week…..They did an MRI scan and told us that the hydrocephalus was worse and that they would need to operate on Kai tomorrow morning. The neurosurgeon, Sylvia Gatscher carried out a Third Ventriculostomy the next morning at 9:30am. The surgeon was happy with the brain surgery but told us that they could not give us the all clear for 6 months to come. Yet again we wait in anticipation for good news of our son’s recovery and eventually and hopefully the all clear.

We are home again now – but now our lives are wraught with worry and fear. Kai is a fighter and I really hope he can keep fighting until they manage to stabilise the hydrocephalus. The third ventriculostomy only carries a 50/50 chance of success owing to the malformation of Kai’s vein of galen. They think his veins and arteries got damaged from the Vein of Galen malformation. We called Paris to tell them about Kai’s Third Ventriculostomy, they commented that this is also very rarely needed after being embolised for Vein of Galen malformation. If this does not work then Kai will need further brain surgery and will have to have a shunt put in – the one thing we never really wanted for him. We live in hope that this may not have to happen.

The support we have received from family and friends has been amazing and we thank God every day for our little miracle. We just wish that soon – this will end – or become easier – the day they tell us our son is getting better is the day we will be able to relax again and not live with this horrible worry hanging over us.

God Bless you Kai, you little fighter, heart as big as an ocean!!