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Children and Families

If your child has been diagnosed with Paediatric or Primary Lymphoedema, this section is for you. I had no idea what it would be like for a family whose child has paediatric or primary lymphoedema when I initially started my journey with lymphoedema. When I was originally diagnosed with lymphoedema, neither the terms "secondary" or "primary" lymphoedema were familiar to me.

I didn't learn about primary lymphoedema until 2014, when I started an adult support group and a woman joined who said she and her family had been affected by it for generations. I still didn't fully grasp the impact that this must have on their lives. In addition, my great granddaughter was born in the latter half of 2016 with a cystic hygroma, a type of lymphatic malformation (LM) (CH). It was then that I realised the scope of the problems that families could confront.

The parents of a child with a lymphatic malformation are likely to feel lost and alone after hearing the news. When there is no family history of a complication during pregnancy, the mother may begin to blame herself and wonder if she could have done anything differently.

There are also those families where lymphoedema is genetic.

Our goal is to equip and empower families so that they can speak up for themselves. We intend to equip parents and guardians with the knowledge necessary to manage and provide self-care for their children with Paediatric and Primary Lymphoedema (PPL) as our understanding of PPL grows.

Lymphatic Malformations


Paediatric Lymphoedema and Primary Lymphoedema is caused by alterations (mutations) in genes responsible for the development of the lymphatic system and therefore leads to the lymphatic system breaking down.  The "faulty" genes cause the parts of the lymphatic system responsible for draining fluid to not develop properly or not work as they should which can then cause swelling and tissue changes. 

The swelling will appear as a mass or lumps these growths are benign and not cancerous. In the case of some lymphatic malformations the lymphatic vessels did not develop properly in the womb.  Primary lymphoedema often runs in families, although not every child born to someone with this condition will develop lymphoedema themselves.

Lymph Fluid is a clear pale-yellow colour


The lymphatic system forms part of the immune system that identifies and fights off invaders, such as bacteria and viruses consisting of a network of vessels within the body through which lymph circulates, and many lymph nodes filter the lymph fluid that  helps the body fight infection.  Often the lymphatic vessels do not develop properly in the womb.  Nobody knows why this happened, nobody is to blame, and this could not have been prevented.  These malformations grow because the lymph in the vessels do not drain well, they then swell causing a flare-up.  They often look red, swollen and can feel tender if touched.

  • macrocystic lymphatic malformations (cystic hygromas or lymphangiomas) have large stretched vessels and cysts filled with lymph, blood from internal bleeding or both

  • microcystic lymphatic malformations are spongy have have small vessels and tiny cysts

NORD (National Organisation for Rare Diseases) describes lymphatic malformations as:

"Lymphatic malformations are rare, non-malignant masses consisting of fluid-filled channels or spaces thought to be caused by the abnormal development of the lymphatic system."




Interventional radiology (IR) is a radiology specialty which provides minimally invasive image-guided diagnosis and treatment of disease. Although the range of procedures performed by interventional radiologists is broad, the unifying concept behind these procedures is the application of image guidance and minimally invasive techniques to minimise risk to the patient. There are many uses for interventional radiology but in our case used for the treatment of vascular and lymphatic malformations.

This procedure is carried out by a radiologist who is highly skilled in anatomy and image guidance.




Interventional Radiology (IR) is less painful than having regular surgery. Your child will receive a tiny amount of anaesthetic or sedation so that your child will be comfortable and safe.  The incisions or cuts will be small and should leave no scars.  Recovery time for your child will be shorter and it is unlikely that your child will be kept in hospital overnight.  

 IR treatments are image-guided, they can be very precise. 


This helps doctors prevent as much damage as possible to surrounding tissue, organs, and skin.





Parents will obviously be anxious when children go into hospital for any procedure and this will often be projected to your child, try not to let your anxieties transfer to your child.

You know your child better than anyone.

Talk to your child, explain to them what is going to happen, reassure them everything will be safe.  When you take them into hospital let them take their favourite soft toy or their snuggle blanket.


Lymphatic sclerotherapy is a procedure used to treat lymphatic malformations. Lymphatic malformations (sometimes referred to as cystic hygromas) are a collection of small cysts. These are congenital, that is, they were present when your child was born.


(When Ezmae-Kate has this procedure, she has gone into hospital around 07.30 a.m. she has often been out of hospital around 2.00 p.m.) Expand

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