Vein of Galen Malformation
The story of
“Fraser (an alias)”
Meg Seow
Auckland City Hospital
E-mail Meg Seow with questions or comments relating to this presentation and/or
Vein of Galen Malformation: MSeow@radiology.co.nz
Fraser Just 2 Hours Old
The story of
“Fraser (an alias)”
Fraser was born on the 24th of April, 2006 in Lower Hutt Hospital. Fraser’s
parents, Belinda and Steve (an alias) described this as “the happiest moment of
both of our lives.” Fraser was 3.6kg and a beautiful and health baby. He was
perfect.
For the first day and a half of Fraser’s life, he was very sleepy and didn’t want to
be breastfed. The nurses and midwives were not concerned. They told Belinda
“not to worry about this as it was common for new babies.” Belinda and Steve
were not worried because they trusted nurses.
On the night of the 25th of April 2006, Belinda and Steve’s whole happy world
was to come crashing down. When Belinda was trying to breastfeed Fraser,
another midwife popped into the room. She thought that Fraser looked like he
was breathing a little fast. She counted his respiratory rate. She then called the
registrar straight away to assess Fraser.
Soon after the assessment, Fraser was taken into the special care baby
unit. Some chest X-ray films were taken, and the on-call consultant
pediatrician was called in to examine Fraser.
The consultant pediatrician told Belinda and Steve that “Fraser is going into
heart failure and needs to be transferred to the NICU at Wellington Hospital
right away.”
Belinda started to scream. She thought that this was the end of her little
baby’s life. She describes this as:
“I was very scared and distraught. The thing I wanted most in the
world had been taken away from me. I felt as though I was no
longer a mum. I couldn’t do anything to help my baby and I wasn’t
in control of what was happening to Fraser.
I was nothing.”
Fraser was transferred to Wellington Hospital that night. He had an
echocardiogram. It identified severe heart failure.
Fraser’s diagnosis was made at 1am that night. It was Patent Ductus
Arteriosis” but there was something else going on with Fraser. Further
exploration was required so he was scheduled to have a MRI scan next.
Wellington NICU
In the mean while, Fraser was commenced on Diuretics and went under
close observations. Later the next day, Fraser had a MRI scan. It showed
the complexity of the problem.
Fraser was intubated.
The on-call doctor spent a lot of time on the phone and consulting with the
doctors in the Starship Children’s Hospital in Auckland.
Finally, Fraser’s primary diagnosis was made. It was “Vein of Galen
Malformation”. This was the real cause of the heart failure.
Belinda and Steve are told that “Vein of Galen Malformation” is treatable,
but more than likely Fraser could die.
Belinda and Steve are devastated and terrified.
They are also told that Wellington Hospital don’t have the doctors or the
equipment to help Fraser, so now he needs to be transferred to Auckland
by the Life Flight Air Ambulance.
Fraser and his family are flown to Auckland on the 28th of April, 2006 and
Fraser is admitted to Level 3, NICU at National Woman’s Hospital in
Auckland.
So, what is “Vein of Galen”?
• This is a very rare condition affecting the blood
vessels of the brain.
• The abnormality occurs during the embryonic (6
to 11 weeks) development and results in
abnormal communications between the arteries
and the veins of the developing brain.
• The capillaries which normally connect arteries
to veins and function to slow blood flow allowing
for the drop off of oxygen and nutrients to the
brain are missing.
• Since “vein of Galen
malformations” lack
capillaries, blood flow
could be fast and this in
turn increase the work of
the heart.
• Blood drainage is towards
a single deep draining
vein, which becomes
markedly enlarged.
• It affect all races.
• It affect boys and girls
equally.
• The cause of it is
unknown
• It occasionally detect on
antenatal ultrasound
(from 25weeks gestation).
• It commonly diagnosed
during neonatal period.
• Statistically, occurs one
case each year in
population of about three
million people (1case
/year/ 3millions)
Clinical signs and symptoms
• High output congestive
heart failure
Decreased resistance
Tachypnoea
Respiratory distress
Cyanosis
High blood flow in the lesion
Often require ventilation support
• Developmental delay
• Hydrocephalus
Investigation (Imaging)
• Initial investigation = seek cause of heart
failure (eg: Echocardiogram)
• Transfontanelle ultrasound
• MRI
• When Vein of Galen is confirmed by MRI,
Angiography would be performed at the
time of Embolisation
Considerations for treatment
• Infant often die if the high output CHF is
the presenting feature.
• Large shunt = Rapid deterioration with
progressively worsening cardiac failure
• Smaller shunt = Present later with cardiac
failure and failure to thrive.
So when should we treat the patient?
Consideration for Treatment
• If the child can be managed medically, it is best to wait
until aged 5 or 6months old.
• Embolisation of a neonate is a high risk procedure.
There are some limitation of the procedure (amount of
contrast medium, flush solution can be given to baby).
• Surgical attempts at closure of the shunt have high
mortality or severe morbidity. Embolisation is the only
way to treat VOGM at this stage.
• Large shunt with many feeding vessels will need several
embolisation sessions.
So why Fraser was treated so soon?
Fraser’s score
1 (cardiac) + 5 (cerebral) + 2 (respiratory) + 2 (hepatic) + 1 (renal) = 11
For emergency treatment 8/ 21 < score> 12 / 21
Score less than 8 = Not for treatment
Score more than 12 = Medical management until age over 5 months old.
Treatment
• Recently, prognosis of
patients with “Vein of
Galen” has improved,
largely due to
improvements in
endovascular treatments
and techniques.
• These technique involve
the use of the catheter
that is inserted in to a
feeding artery to block off
the supply by using coils
and glue like substances.
Fraser’s Progress
(2nd Embolisation)
• A week after the 2nd
embolisation, Fraser
came off from ventilation
• A month later, the family
went home together
(discharge from hospital)
3rd embolisation
The family celebrate
their Fraser’s first
Christmas together.
4th Embolisation
• After the forth
embolisation, Belinda and
Steve saw a huge leap
forward in Fraser’s
development.
• He came off from his
diuretics.
• His size of the heart was
normal and the pressure
in his lung had decreased.
5th Embolisation
• Fraser is doing better
than ever. He is starting
to take few steps while
holding on and now he
is able to speak few
words.
• Total of 99% of feeder
were shut down.
• Fraser is now 16
months old.
Team Management
• Team approach is critical
to successful
management
Neonatology
Peadiatric
Cardiology
Fetal Medicine
Fetal medicine
Neonatology
Pediatric cardiology
Intensive care
Neurologist
Neurosurgeon
Interventional Neuroradiology
Patient
Interventional
Neuroradiology
Neurosurgery
Intensive care
Neurologist
By preparing this presentation, I have learned many things.
I communicated with Belinda and Steve via weekly e-mails.
Belinda wrote to me:
“We have learned to celebrate every success,however
small it
is and to treasure every day that we have
together. Some days it is
really hard, but we get there. We get there with support from each other, our
families, friends and the many wonderful professionals
who
are on Fynn’s “team”.”
I have also learned how I and my profession could impact on someone’s life
without realizing it.
I am in a very powerful profession.
I am a nurse.
Belinda wrote:
“Thanks so much for everything that you have done for our little
Fraser. I remember the first time we met you on the morning of
Fraser’s very first procedure. You really helped to ease our worries
and keep doing so before each procedure. When we see you we
know that you will be in there with him and it helps us feel a little
better! It is so nice for us to have the continuity of having you there
caring for him and us each time. A big thank you from us all.”
She also wrote:
“There are times when I really thought that Fynn wouldn’t make it to
one week or even one month old, but like the surgeons told us the
first time that we met them, and every time we’ve met them, “Take
each day as it come”. It has taken us a long time to be able to do
just that.”
To conclude my presentation, I would like to share a poem.
Belinda found this poem in a book about living with a disabled child. She
reads it time to time.
Belinda wishes to share this poem with you all…
“Welcome to
Holland”
by Emily Perl Kingsley
©1987 Emily Perl Kingsley. All rights reserved*
I am often asked describe the
experience of raising a child with a
disability – to try to help people who have
not shared that unique experience to
understand it, to imagine how it would feel.
It’s like this ………
When you’re going to have a baby, it’s like
planning a fabulous vacation trip - to Italy.
You buy a bunch of guide books and make
your wonderful plans. To the Coliseum. The
Michelangelo David. The gondolas in Venice.
You may learn some handy phrases in Italian.
It’s all very exciting.
After months of eager anticipation, the day
finally arrives. You pack your bags and off you
go. Several hours later, the plane lands. The
stewardess comes in and says,
“Welcome to Holland”.
“Holland?!?” you say.
“What do you mean
Holland?? I signed up
for Italy! I’m supposed
to be in Italy. All my
life I’ve dreamed of
going to Italy”.
But there’s been
changes in the flight
plan. They’ve landed
in Holland and there
you must stay.
The important thing is
that they haven’t taken
you to a horrible,
disgusting, filthy place,
full of pestilence, famine
and disease. It’s just a
different place.
So you must go out
and buy new guide
books. And you must
learn a whole new
language. And you will
meet a whole new group
of people you would
never have met.
It’s just a different
place. It’s slower-paced
than Italy, less flashy than
Italy. But after you’ve
been there for a while
and you catch your
breath, you look
around….. and you begin
to notice that
Holland has windmills….
And Holland has tulips.
Holland has Rembrandts.
But everyone you
know is busy coming and
going from Italy… and
they’re all bragging about
what a wonderful time
they had there. And for
the rest of your life, you
will say “yes, that’s where
I supposed to go. That’s
what I had planned”.
And the pain of that
will never, ever, ever,
ever go away…
because the loss of
that dream is a very
very significant loss.
But, if you spend your
life mourning the fact
that you didn’t get to
Italy, you may never
be free to enjoy the
very special, the very
lovely things, about
Holland.
Thank
you