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Is it advisable to continue with pregnancy when having fetus showing ventriculomegaly?

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Posted on Tue, 19 May 2015
Question: Dear Doctor,

I am french so please excuse my english.
My daughter was discovered to have severe ventriculomegaly at 32 weeks (last ultrasond at 22 weeks was ok) and an MRI at 33 weeks showed her ventricles to be 18mm and 21mm with no other problems. Toxoplasmosis and CMV are both negative. We are waiting for amniocentesis results. We should meet the neurologist next week and a new MRI is programmed in ten days to see the evolution. I am really worried because I am at 34 weeks and I do not imagine to interrupt my pregnancy at this stage. I just pray for that ventriculomagly to regress.

The litterature about severe ventriculomegaly is poor.

Could you please tell me If my reasoning is appropriate ?

If it is confirmed that the ventriculomegaly is "isolated", then the prognosis depends on the development of the size of the ventricles:
- If the size of the ventricles increase, I think the prognosis is very bad
- If the size of the ventricles remain stabe, I don't know what to expect
- If the size of the ventricles regress, can we expect a good neurological development ?
Or is the dilatation has reached a stage that leaves anyway side effects?

My baby moves a lot and easily respond to stimuli (she comes immediately in contact with the hands). So I have trouble imagining that she does not grow well.

Best regards,

XXXX
doctor
Answered by Dr. Olsi Taka (1 hour later)
Brief Answer:
Read below.

Detailed Answer:
I read your question carefully and I can only begin to imagine the anguish you must be feeling during these weeks. I am really sorry about that.

Your english is pretty good by the way, I hope mine is adequate as well :)

You seem to have done some research by yourself and your conclusions are well founded. The reason there is less research on outcome severe ventriculomegaly unfortunately is that there is high rate of complications and pregnancy termination which makes for a low rate of survival.

In evaluating a severe ventriculomegaly first step is excluding associated abnormalities. The fact MRI hasn't found any is a good thing but still chromosomal abnormalities can be found in the amniocentesis report. Also, as accurate as MRI is there is still the chance of what were considered isolated cases being found to have undetected brain abnormalities after birth (about 27,8% from a british study).

If we assume that it is indeed isolated, there is still a high risk of complications, with a higher rate of still birth or neonatal death (around 30-45%). In those children who make it there is still the chance of disability. The numbers vary, most of them show a chance of normal development or mild handicap of about 50-60% (completely normal 10-50%), while 40-50% still had severe disability.
The assumption you made before that the evolution on follow up MRI might be a good indicator is correct, however I fear that since your baby's ventricle size is well above the severe megaly threshold (of 15mm) even if there is reduction in size there is the risk of disability.

I hope I didn't confuse you with too many numbers, but as you surely know it is a very delicate issue and since a clear cut prediction can't be made I prefer to let the numbers speak.

I hope to have been of help.
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Olsi Taka (17 hours later)
Yes, it is a real difficult period. We were ready to receive this child. Everything was fine and suddenly, everything changed for no reason.

Thank you very much of your clear answer. Yes it has been of help.

It confirms what I was thinking, even if I didn’t know the numbers.

I aslo fear that this so-called “isolated” ventriculomegaly is not isolated.

If no others abnormalities are found, how can this dilatation can be explained ?

According to me (and I am not a physician), this can be due to:
- an internal bleedind a few weeks ago (but in that case, it should regress ?)
- a malformation of the central nervous system not visible on the MRI
- a malformation of the interventricular foramen (is it visible on the MRI ?)
- other hypotheses ?

Are the numbers you gave to me the results of your own experience and/or of published studies?
Would you mind to give me the main references you rely on? I uploaded the references I have found so far (most of the time, I only have access to the abstract).

We should meet the neuropaediatrician tomorrow. I will share its opinion with you.

Best regards,

XXXX
doctor
Answered by Dr. Olsi Taka (2 hours later)
Brief Answer:
Read below.

Detailed Answer:
That is an impressive list of articles, at this rate you'll become an expert in the field :)

My answer was of course based on studies I read. As I said before you don't have many cases with severe ventriculomegaly followed for a long term, often, even when it seems isolated, mothers choose to terminate pregnancy. Even those number I mentioned were small groups, once cases with associated abnormalities, terminated pregnancies and perinatal death were excluded only groups of about 10 cases remained to be followed for their long term development.
Some of the studies I was based on you seem to have already encountered by yourself main ones were from P. Gaglioti et al, Kennelly et al, Hannon et al. One that wasn't included was from XXXXXXX et al (The Journal of Maternal-Fetal & Neonatal Medicine XXXXXXX 2001, Vol. 10, No. 4: 258–263.)
I might have access to some articles, not all of them but some, if you tell me what interest you the most and provide an email address I can see if I can get them and send them (can't upload here and I don't think it'd be allowed anyway). I am not suggesting you really should, it seems you already know your options by now, the rest is more a matter of percentages, they can vary somewhat but still no major differences, shouldn't influence much your individual decisions.

Regarding your questions on the cause. An internal bleeding can be absorbed but it can still leave adherences which can cause narrowing of the aqueduct connecting the ventricles. A hemorrhage on the brain surface can also damage the structures which absorb the cerebrospinal fluid (CSF) with more fluid being produced than absorbed. Genetic malformations of brain and the CSF circulation system undetected by MRI are certainly another possible cause. Other causes may be infections although the two common ones have been excluded in your case. The cause can remain undetermined in a percentage of cases though.

I remain at your disposal to discuss your neuropediatrician recommendations with you if you need to.
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Olsi Taka (2 days later)
Thank you very much for your answer.

We met the neuropediatrician yesterday. To sum up, even if we don't have all the analyses, he thinks that this dilatation may be due to an hemmorhage in the ventricules that arrived suddendly. He says that if the dilatation regresses, it could be of good pronostic because there is no other problem on the IRM. But he told us that he can not totaly exclude disabilities.
So he suggests to wait for the remaining results and check the evolution by ultrasond (tomorrow) and IRM (last week) . He also spoke about 2 neurosurgery postnatal methods : ventriculocisternostomy (in case of stenosis of the aquaduct of Sylvius, which is not our case) and a ventriculoperitoneal shunt (but it seems too early at this stage to think about it).

Is-it possible to have your e-mail adress so I can give you some news later ?

This is mine: YYYY@YYYY

Thank you very much to have answered my questions.

Best regards

XXXX
doctor
Answered by Dr. Olsi Taka (6 minutes later)
Brief Answer:
Of course.

Detailed Answer:
Thank you for keeping me updated. Yes of course you can have my email address which is YYYY@YYYY . I hope you'll write with some good news.
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
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Dr. Olsi Taka

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Is it advisable to continue with pregnancy when having fetus showing ventriculomegaly?

Brief Answer: Read below. Detailed Answer: I read your question carefully and I can only begin to imagine the anguish you must be feeling during these weeks. I am really sorry about that. Your english is pretty good by the way, I hope mine is adequate as well :) You seem to have done some research by yourself and your conclusions are well founded. The reason there is less research on outcome severe ventriculomegaly unfortunately is that there is high rate of complications and pregnancy termination which makes for a low rate of survival. In evaluating a severe ventriculomegaly first step is excluding associated abnormalities. The fact MRI hasn't found any is a good thing but still chromosomal abnormalities can be found in the amniocentesis report. Also, as accurate as MRI is there is still the chance of what were considered isolated cases being found to have undetected brain abnormalities after birth (about 27,8% from a british study). If we assume that it is indeed isolated, there is still a high risk of complications, with a higher rate of still birth or neonatal death (around 30-45%). In those children who make it there is still the chance of disability. The numbers vary, most of them show a chance of normal development or mild handicap of about 50-60% (completely normal 10-50%), while 40-50% still had severe disability. The assumption you made before that the evolution on follow up MRI might be a good indicator is correct, however I fear that since your baby's ventricle size is well above the severe megaly threshold (of 15mm) even if there is reduction in size there is the risk of disability. I hope I didn't confuse you with too many numbers, but as you surely know it is a very delicate issue and since a clear cut prediction can't be made I prefer to let the numbers speak. I hope to have been of help.