Dandy-Walker Malformation

[jordanac]

Hi all -

It's has been awhile since I have posted to this board. I am pregnant with twin - boy and girl - due in the summer. A week and a half ago I had my mid-pregnancy ultrasound. The radiologist mentioned in his report of our little girl that he could not see a clearly defined vermis on the cerebellum and that he was stating this could be Dandy-Walker Malformation. There was not a cyst present nor was there hydrocephalus.

Does anyone have a child with Dandy-Walker Malformation?

We are in the process of obtaining a second opinion.

My 2.5 year old is special needs - has seizures - just now trying to walk - doesn't talk. So I know the reality that this could bring - I don't feel terribly upset but I want to know for sure and plan accordingly. Any thoughts would be appreciated!

Thanks -
jordana

[MaryB69]

I found out last year that I have Dandy-Walker Variant. I had no clue that I even had it until I had an MRI.

[2boyzmama]

I know one person IRL with a child with DW. He had sudden severe hydrocephalus as an infant and required a shunt, and has some delays, but is otherwise "normal". He's 16 years old now, and has only required one shunt replacement.

I also know one other person IRL with a child with Chiari Malformation, which is sometimes similar in presentation I believe??? I'd have to google both again, I'm not very well read in either

[hummingmom]

Originally Posted by jordanac

Hi all -

It's has been awhile since I have posted to this board. I am pregnant with twin - boy and girl - due in the summer. A week and a half ago I had my mid-pregnancy ultrasound. The radiologist mentioned in his report of our little girl that he could not see a clearly defined vermis on the cerebellum and that he was stating this could be Dandy-Walker Malformation. There was not a cyst present nor was there hydrocephalus.

Hi!

I can't write much now, but we dealt with this diagnosis with my DD2 (now 18 months old), and I wanted to emphasize something that I wish we'd known in the beginning:

Dandy-Walker malformation -- whether the classic type, or the variant -- can't be diagnosed by ultrasound or CT scan. It's essential to have an MRI, and the film has to be read by a neurologist or neurosurgeon, not a radiologist.

In my DD's case, the MRI (which was done when she was a month old) showed that the vermis was present after all; it was just pushed out of place by a larger than usual cisterna magna. This is considered to be a benign anatomical variant. Some people consider it to be the very mild end of a "Dandy-Walker spectrum," but our neurosurgeon doesn't agree, since it's not associated with any of the symptoms that are commonly found with DWM or DWV.

OTOH, if your baby has any kind of cerebellar malformation, that can be a marker for other stuff going on. My DD turned out to have a congenital heart defect. But that's certainly not a given; many of these children turn out to have no problems. When I was first looking into this, I remember reading one study in which they did autopsies on babies who'd been aborted due to a prenatal diagnosis of Dandy-Walker, and the majority turned out to be perfectly normal. Seems like there was some overzealous CYA going on there.

Of course, this isn't to say that it's no big deal. I think there is (or was?) a mother on here whose daughter has DWM, and is dealing with a lot of medical and developmental issues. But there's a very wide range of outcomes, even when the diagnosis is confirmed. I found this page to be a pretty helpful in explaining that.

In any case, I hope you can find the information and support to help with whatever challenges might come your way.

[glamourpup]

Like the previous poster, my perinatologist swore up and down that my son (now 1 year old) had a severe Dandy-Walker malformation, diagnosed via ultrasound. He pointed out all of the features of it, we had probably 5 ultrasounds and on each one he pointed it out again.

We then traveled to Children's Hospital Boston to have various testing done (my son has a chromosome abnormality), including a fetal MRI. That MRI as well as one done after he was born conclusively proved that he didn't have a Dandy-Walker malformation, the structures of his brain were entirely normal. No one has been able to tell us what that doctor saw, and it isn't like he ever apologized.

All this to say that I would strongly recommend a fetal MRI to know for sure. I wish you much luck!

[Mamatohaleybug]

Hello! Congratulations on your new babies on the way. That's very exciting!

My daughter has DWM. She is now 6. Her situation hasn't been nearly as bad as we originally thought it would be. She is quite typical now and our lives have gotten much, much easier this past year. There is reason to have great hope for the future.

She was diagnosed prenatally and the diagnosis was correct. At birth she had a large cyst, no vermis visible, very little cerebellum visible but only mild hydrocephalus. Two weeks later her hydrocephalus became quite serious and she had two shunts placed. She has had two revisions to her shunt systems. Her more recent scans show that her cerebellum "grew" quite a bit after her surgery. The major damage/differences are to her occipital lobes, her corpus callosum and of course, her cerebellum and vermis. She has a diagnosis of static encephalopathy. She has a history of tonic clonic seizures, currently has serious migraines and strabismus (and an eye going lazy that we have been desperately trying to "save"). She has sensory processing disorder. She also has some behavioral challenges (similar to those seen with ADHD and ODD) probably due to executive dysfunction. She has a diagnosis of Intermittant Explosive Disorder but I'm not sure that's going to stick. She's really improving in her coping skills and isn't nearly as explosive now.

Haley has always had some mild to moderate issues with her development and conventional therapies have been quite helpful. Over the years we've dealt with minor speech delays (mostly poor muscle tone in her mouth), moderate motor delays (she can attain skills but the quality is always pretty poor - she has problems especially with balance, trunk, hip, shoulder and arm tone, motor planning, coordination, etc), some cognitive delays, social/emotional and behavioral problems and lots of visual processing issues. She is of normal intelligence and is even gifted in some areas. She has had a practically perfect kindergarten year, with an IEP (but very few supports in place).

If you have any specific questions, please let me know. I'm always happy to talk about my sweet daughter.

[hummingmom]

Hi mamatohaleybug! I'm pretty sure it was your family I was thinking of. I think I read some of your posts back when my little girl was a newborn. It's great to hear that your daughter is doing so well now.

Even though the neurosurgeon rejected the diagnosis of DWM, it's still on DD's chart ("they just keep copying it from the previous page," so I'm told ), so every time she goes into the hospital, I have to explain everything all over again. As a result, I've turned into somewhat of a PR person for this condition, especially with the nurses, many of whom seem to have a very negative attitude about it. I'm guessing that this is due to outdated information in their training, or maybe just the fact that they tend to see the sickest kids. So I'll say something like, "it's common for people with Dandy Walker to have learning disabilities, but many have normal intelligence, and some are able to go on to university and have a professional career. I've even heard of a couple of cases where they became RN's." That makes them change their tune pretty fast, LOL. (And it's true... though even if it weren't, I would have been tempted to make it up. )

[hungry]

Dandy-Walker syndrome (DWS), or Dandy-Walker complex, is a congenital brain malformation involving the cerebellum and the fluid filled spaces around it. The Dandy-Walker complex is a genetically sporadic disorder that occurs one in every 25,000 live births, mostly in females.

Dandy Walker malformation is the most severe presentation of the syndrome. It can be treated by shunts.

[lindalovely85]

hi i am 18 weeks pregnant on my first baby, i had a my scan on wednesday and the doctor found signs of dandy walker on my babys brain but the rest of her organs seem to be okay. i never heard of this before and am really worried how it will affect my baby. i have been given the impression that she will be severly disabled or mentally retarded if she does have it and told that i might have to make a decision on terminating the pregnancy (which i could not bare to do) even tough the doctor has never come accross this herself. is this the case and has anyone else been told this ?

[2boyzmama]

Originally Posted by lindalovely85

hi i am 18 weeks pregnant on my first baby, i had a my scan on wednesday and the doctor found signs of dandy walker on my babys brain but the rest of her organs seem to be okay. i never heard of this before and am really worried how it will affect my baby. i have been given the impression that she will be severly disabled or mentally retarded if she does have it and told that i might have to make a decision on terminating the pregnancy (which i could not bare to do) even tough the doctor has never come accross this herself. is this the case and has anyone else been told this ?

Hi Linda! Congratulations on your pregnancy!

You should start a new thread introducing yourself, you'll probably get more responses specific to your situation that way This is a great forum, lots of really smart people here! If you start a new thread, be as specific as you're comfortable with, list what tests have been run and what the test report says exactly. We can help you decipher teminology.

I'm sorry that it sounds like you are being given "worst case scenario" information. It is very possible to live a normal or very close to normal life with Dandy Walker. It doesn't always have to mean severe disability or mental retardation. But of course it all depends on the extent of it. Know also that there have been mamas on here who were told during pregnancy that their child would have some kind of malformation, but at birth there was none to be found, or it was much less severe than was previously thought. There is always hope!!! If you don't want to terminate, then don't. Period. And don't let anyone talk you into it. It is a decision you and your partner/family should make, and once the decision is made, be firm in it. Ask the drs to note it in your chart that you don't wish the topic to be brought up again.

[Mamatohaleybug]

My 7 year old has Dandy Walker (and hydrocephalus with two shunts). It's been an interesting ride but she is a bright, vivacious, sweet child. She does have some challenges but I'd do it all over again in a heartbeat.

The doctors give us the worst case scenarios. It stinks because it causes so much anxiety. Our daughter's birthparents were also offered termination and refused. We didn't know if she would survive her birth.

I have many old posts about our challenges if you look through my previous posts. If you have ANY questions, I'd be happy to answer whatever I can.

My daughter is doing really, really well right now and I'm enjoying every minute of it. There is hope!!!

Best wishes!

[mommy2one0326]

My daughter has DWM and Hydrocephalus. She was diagnosed by ultrasound around 34 weeks...I was then sent to the hospital for a MRI which confirmed it. I was given the worst case scenario as well...My daugther will be 3 in march and is a normal developing child with a few problems...one is her eyes are messed up so she needs glasses and she had some slight problems with gross motor skills. she did have a shunt placed 5 days before her first birthday and has some problems with balance....Good luck.

[APToddlerMama]

Originally Posted by lindalovely85

hi i am 18 weeks pregnant on my first baby, i had a my scan on wednesday and the doctor found signs of dandy walker on my babys brain but the rest of her organs seem to be okay. i never heard of this before and am really worried how it will affect my baby. i have been given the impression that she will be severly disabled or mentally retarded if she does have it and told that i might have to make a decision on terminating the pregnancy (which i could not bare to do) even tough the doctor has never come accross this herself. is this the case and has anyone else been told this ?

A friend of mine was told her baby had DWM (and I believe there was no "maybe"--it was just "this baby has DWM and you need to decide what you want to do"). Well, the baby is a year old now and she in fact does not have DWM at all. The great thing about motherhood is that you will be worried a billion times over about things that don't come to pass. If your baby does end up having special needs, you will have the strength you need to parent your child and meet your child's needs and she will still be a huge blessing to you. From what I've heard from my friend, there is a huge range of abilities that a child with DW could have, including it being so mild that the average person would not know there was anything atypical at all about the child.

[avaughn]

2 month old with dandy walker

Hi, My 2 month old son was diagnosed with Dandy Walker Syndrome on a fetal ultrasound at 20 weeks. This diagnosis was confirmed by Fetal MRI. All of his other organs were check and came to be okay. The dr.'s seems to be pushing me towards termination but I continued with the pregnancy anyway. Ethan was breech and delivered at full term by c-section. His apgar scored were 10 and he only had very suble physical abnormalities. (small chin, crooked toe, sacral dimple on back and low set ears) At birth the hospital ultrsounded his soft spot and took a look at his brain. They came into my room the day after birth and told me he had full blown DWM with all elements in the brain associated. They were "WRONG". I was terribly upset and had to take him down to the Childrens hopital to see a neurosurgeon. The neurosurgeon said he did not have all 3 brain associations and only a underdeveloped vermis. No hydrocephalis as of yet. He was diagnosed then with a "variant". We later came to find out Ethan has 2 Chromosome deletions also. He has been to every Dr. to check out everything from eyes to ultrasounds of his spine and kidneys. All is checking out well. Ethan is a normal weight at 2 month and is eating, smiling and tracking things with his eye! I also have another older son and in comparison Ethan seems to be following all the same milestones as my first some did. He is a great joy in my life and I am glad I decided to have him. Only Ethan will tell us what he is going to be able to do and not do. Time will tell. Each case of DW seems very individual. I have hope for my son even though the Dr.'s are not giving me any postive feedback. Hang in there to all you mom's with DW children. I will keep updating Ethan's progress as he grows. His next appointment is this Monday to have his breathing checked( he has a stidor wheeze). Fingers crossed.

[2boyzmama]

Originally Posted by avaughn

Hi, My 2 month old son was diagnosed with Dandy Walker Syndrome on a fetal ultrasound at 20 weeks. This diagnosis was confirmed by Fetal MRI. All of his other organs were check and came to be okay. The dr.'s seems to be pushing me towards termination but I continued with the pregnancy anyway. Ethan was breech and delivered at full term by c-section. His apgar scored were 10 and he only had very suble physical abnormalities. (small chin, crooked toe, sacral dimple on back and low set ears) At birth the hospital ultrsounded his soft spot and took a look at his brain. They came into my room the day after birth and told me he had full blown DWM with all elements in the brain associated. They were "WRONG". I was terribly upset and had to take him down to the Childrens hopital to see a neurosurgeon. The neurosurgeon said he did not have all 3 brain associations and only a underdeveloped vermis. No hydrocephalis as of yet. He was diagnosed then with a "variant". We later came to find out Ethan has 2 Chromosome deletions also. He has been to every Dr. to check out everything from eyes to ultrasounds of his spine and kidneys. All is checking out well. Ethan is a normal weight at 2 month and is eating, smiling and tracking things with his eye! I also have another older son and in comparison Ethan seems to be following all the same milestones as my first some did. He is a great joy in my life and I am glad I decided to have him. Only Ethan will tell us what he is going to be able to do and not do. Time will tell. Each case of DW seems very individual. I have hope for my son even though the Dr.'s are not giving me any postive feedback. Hang in there to all you mom's with DW children. I will keep updating Ethan's progress as he grows. His next appointment is this Monday to have his breathing checked( he has a stidor wheeze). Fingers crossed.

Hi avaugh, and WELCOME!!! I'm so happy to hear Ethan is doing so well, proves that each child is an individual, and even the best predictions are just that...predictions.

You should start your own thread and introduce yourself, you'll find that this special needs community is very welcoming, non-judgemental, and really helpful!!! All of our kids are different in their diagnoses, but we all have something in common...we're just trying to do the best we can!

[naruladrug]

Thank you so much for your posting,it gives me and my partner great hope.We had our baby "diagnosed" when only 17 weeks pregnant however the consultant didnt give us a medical term,just said baby has no brain nor cerebeller and offered a termination.We decided we needed a 2nd opinion and paid to see private consultant who was and is amazing,he took so much time and effort to research our babys problem and has now even taken me and all antenatal care on.The NHS consultant seemed only to see problems,wheras other consultant is so positive because he has worked as fetal med specialist out in Australia and has seen great triumphs and success stories of babies with DWS.

[hummingmom]

Since this thread is active again, I just wanted to share an update about our daughter who's now three years old.   She was originally diagnosed with Dandy Walker Variant, then the diagnosis was changed to mega cisterna magna, which isn't associated with any delays.   I think the truth is somewhere in between.   She does have a bit of trouble with balance, and has been slow to develop gross motor skills; when she's on the playground, she seems more like a young two year old.  On the other hand, her intellectual, fine motor, and social skills are normal, even quite advanced in some areas.    (She has a great sense of humor and sometimes almost adult-like poise.   This evening, she peered up at me and asked, "Mommy?  Can I please sit on this chair and tell you about my hurt finger?") 

We've done some conventional and alternative physical therapies, but couldn't see that they made much difference in her motor development.   It's my understanding that this is often the case with cerebellar issues.  The brain has to make new pathways to learn certain skills, and this can take a while.   On the up side, these children often continue to make lots of progress in the long term; the trick is to keep them motivated, and keep giving appropriate challenges.     Ordinary parent-child gymnastics classes have been very helpful for our daughter, probably more so than all the PT put together.    When she's a bit older, we'll be looking into hippotherapy (therapeutic horseback riding).   We also try to do lots of active games at home; it helps that she has two older siblings to keep up with.    I'd love to hear about any other ideas that families have found helpful for balance and coordination. 

Also wanted to point out a beautiful video, for anyone who hasn't seen it:  Dandy-Walker Children

[ephesians611]

I have a 2 1/2 year old with Dandy walker Malformation - he had a shunt placed at 3 months old.  He is affected a great deal by all of this with hearing loss, vision loss and is beginning to have seizures.  He is also very developmentally delayed - weighing in at about 8 months old developmentally, in his speech, and motor development.  But this little boy is so full of love and is happy all the time.  He is our pride and joy!  We were told at the beginning that with DWM that it could go any way, from slight symptoms right to the other end of the spectrum, so we just take everything from day to day.