Lymphatics FAQ

Are there any guidelines for hydrops in pregnancy?
Our baby was born with extreme hydrops (increased fluid around the pregnancy). The obstetrician basically did a quick “Google” search as he had no guidelines for pregnancies for Noonan Syndrome and didn’t find anything that could go wrong but we wondered if there were plans to write any guidelines? The problem is that hydrops occurs in a lot of different conditions so the problem for the obstetrician is what the underlying cause is. They may look for a number of causes – infections for example – and Noonan Syndrome comes a long way further down the line and sometime doing the test can take some time in getting the results. Certainly, a number of labs will offer the PTFN11 test for Noonan Syndrome if they think that’s a possible diagnosis in the womb. Hydrops is due to lymphatic problems so there have been a number of examples where Noonan Syndrome cases have presented with hydrops. Hopefully there a paper will be produced on this and particularly regarding prognosis and what’s best to do in the prenatal period. Question asked and answered at our family’s day 2015
How common is Lymphoedema in children with Noonan Syndrome?

Is watching and waiting the best treatment for a mild Lymphoedema or is it worth using compression garments early on to prevent it getting worse?

Lymphatic problems are very common – hydrops, swelling of the feet at birth, web neck, they’re all lymphatic, so they’re all very common. I think some children with Noonans and adults may have lymphoedema and not realised that that might be the problem or even their intestinal problems. If there’s some Lymphoedema, once it starts there is the possibility of it increasing and, some centres tend to treat people at an early stage with light garments – we don’t want to make life unbearable so they should be comfortable and easy to put on and we work with the patient to try to optimise that because if they don’t like wearing it, especially a child, they won’t comply so there is an in-between round for it but we would advocate early use of compression garments. It doesn’t cure it; it doesn’t stop it progressing but it may slow down and stabilise it.

Question asked and answered at our family’s day 2016

Is there a link between Hydrops and lymphedema?

I understand that hydrops is quite common in Noonan’s children. Our daughter’s 5 and had hydrops when I was pregnant. I just wondered if there’s a high chance of those that did suffer with hydrops of developing lymphoedema problems later in life?

We’ve only seen about 15 people with Noonan’s and lymphoedema and some of those had hydrops but not all – and then I’ve seen many children with hydrops who haven’t developed lymphoedema so there’s no clear correlation at the moment.    

Question asked and answered at our family’s day 2016

Is there a link between Hydrops and lymphedema?

I understand that hydrops is quite common in Noonan’s children. Our daughter’s 5 and had hydrops when I was pregnant. I just wondered if there’s a high chance of those that did suffer with hydrops of developing lymphoedema problems later in life?

We’ve only seen about 15 people with Noonan’s and lymphoedema and some of those had hydrops but not all – and then I’ve seen many children with hydrops who haven’t developed lymphoedema so there’s no clear correlation at the moment.    

Question asked and answered at our family’s day 2016

Is there any research happening to link chylothorax with cardiac issues?

Our daughter suffers from spontaneous chylothorax as well as pulmonary stenosis and ASD. She’s had open heart surgery which was very successful. I was wondering if in the medical world is there any more research being done on whether there is any connection to the chylothorax issues within her lungs to cardiac issues or are they totally separate situations?

There’s certainly association with chylothorax and cardiac surgery with individuals who have had open heart surgery who then develop chylothorax. We also know there is an increased prevalence of lymphatic problems in Noonan Syndrome. There is a lot of research going on in terms of the work Sahar is doing and the fact that we can now image lymphatics in a better way. We’ve talked about Noonan’s having some implications for growth and potentially over-growth, over-thickening of vessels. There’s a possibility that certain situations lead to over-growth of lymphatic vessels which then don’t do their job very well. There’s an excellent link now with Philadelphia with Professor Max Itkin who is an expert in interventional radiology to actually get a picture of the whole lymphatics of the body, not just the lower part of the body and then ways to intervene in certain circumstances.

Question asked and answered at our family’s day 2018