McKenna's Ongoing Journey With Her Cystic Hygroma (Sept. 24th 2008)

So it's been about 7 months since McKenna's surgery to remove her Cystic Hygroma.

Here are some statistics for you: 1 in 6000 to 16,000 is the estimate I've seen for live births with a Cystic Hygroma. Most Cystic hygromas (50-65%) are evident at birth, with 80-90% of CHs presenting by age 2 years. Some authors believe that all CHs are present at birth, although they may have not yet fully manifested at that time. 75% are on the head and neck. 20% are in the axillary region where McKenna's was. McKenna's presented at 3 months of age. Karyotypic abnormalities are present in (60-80%) of the cases diagnosed fetally. Over the summer we had McKenna's chromosomes tested and she has no abnormalities.

Yes-- Rachael's baby Gracie Anne had a Cystic Hygroma which caused a back-up of fluid in her body and subsequently caused her death. Testing showed that Gracie had Turner's Syndrome. So the genetic counseling we have received has been that they are not connected. Even though pathologically they are the same thing. McKenna's is isolated and Gracies was caused by her Turners Syndrome.

Here is an explanation for what a Cystic Hygroma is: Simply, much like your vascular system, there is a certain path in which lymphatic fluid flows and is processed throughout your body. In LM (Lymphatic Malformation) patients, the natural flow of the lymphatic system has been disrupted branching off in different directions into dead ends or like streets in our neighborhoods, a cul-de-sac, if you will. Some of the cul de-sacs create large pockets of fluid. There is no "cure" for Lymphatic Malformation. In some cases, of localized LM, surgical resection or sclerotherapy can eliminate the condition without reoccurrence. However, treatment is aimed at keeping the condition under control not curing it. When the large mass is removed there are lymphatic channels or vessels left behind. Remember the cul-de-sac's? Well, at the end of these vessels are dead ends or cul-de-sac's. The pressure builds and new vessels begin to branch off trying to reach back to the lymphatic system. They meet a dead end and the process repeats itself over and over. This happens just adjacent to the site of treatment and may eventually run back into the site of treatment. Reoccurrence is really the body's continued effort to fix it's lymphatic system.

The problem with these malformations are:
1. They can become infected.
2. They can hemorrage
3. They can invade and compromise surrounding bodily structures.
4. They can be disfiguring

We were told that McKenna would have a 90% chance that nothing else would happen or be detected after her surgery. Unfortunately Friday Sept. 12th I noticed a bump under Mckenna's collar bone near her shoulder on her right side. This *is* the same side her Hygroma was on. She has since had an ultrasound and we've met with and discussed this new development with her surgeon.

The surgeon feels/hopes it is connected with her original mass.

The reason we hope this is connected to her original mass is because a separate area of damaged/deformed lymph tissue is a disturbing thought. Meaning she could possibly have much more than just an isolated issue. If that were the case this condition would be called "Diffuse Lymphangiomatosis" This is a disease that depending on how it presented could be very serious and even fatal. The surgeon recommended we have a CT scan done of Mckenna's torso and abdomen to make sure there are no other visible malformations deep inside her body. He used the phrase, "for our peace of mind" . . .
He is also recommending Sclerotherapy for this new site. He feels there are so many blood vessels and nerve endings there that we would be taking less of a chance damaging something if we went with injections.

So-- We have to decide what to do. Do we proceed with sclerotherapy? Do we do the CT scan? Do we get a second opinion?

Also we found out-- someone dropped the ball and sent out our annual Medicaid forms to the wrong address and we were dropped. We are in the process of "hopefully" getting re-approved for coverage. Pray that works out for us.

We appreciate your prayers for clarity and peace in our decision making. And of course for healing for McKenna!

Love,
Amber