Our Questions
These are real questions from people who have LM and their families. The questions were compiled by CJ (MySpace Support Group) and presented to doctors from Boston by her in 2008. These are the answers she got from them.
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1. Cystic Hygroma and Lymphangioma are they the same thing?
Cystic Hygroma and Lymphangioma are the same condition.
2. How are they alike and different?
Cystic Hygroma is typically used to describe large masses in the head/neck and occasionally in the axillary. These are often macro cystic lesions and may be mixed with micro cystic lesions. Lymphangioma is typically used to describe micro cystic lesions and may be found on the skin, bones, lungs and many places throughout the body.
3. Is Lymphatic Malformation the category of our medical condition and Cystic Hygroma and Lymphangioma the condition, itself?
No, Lymphatic Malformation is the condition.
4. There are many terms would you please clarify?
When Cystic Hygroma and Lymphangioma were first identified they were believed to be tumors. In medical terminology OMA means tumor. Tumors are foreign invasions to the body. Since Cystic Hygr-OMA, Lymphangi-OMA and even, Cystic Lymphangi-OMA were thought to be foreign invasions of the lymphatic system these were the terms used to describe them. In recent years, they have been found not to be a foreign invasion but, a deformity of the lymphatic system. Malformation means deformity or abnormality therefore, the term Lymphatic Malformation has become the new and more appropriate terminology.
Simply, much like your vascular system, there is a certain path in which lymphatic fluid flows and is processed throughout your body. In LM 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-sac create large pockets of fluid (macro cystic) and other cul-de-sac create small pockets of fluid (micro cystic). Both pockets are a part of the same system just one is larger than the other so, Cystic Hygroma and Lymphangioma are, in essence, the same deformity just one being small and one being large. Because they are part of the lymphatic system, itself, they are not foreign invaders so, they are not tumors.
In an effort to help educate my doctors and to ensure I reach the largest LM population possible you will notice I use the old terminology along with the new. Whenever, I write my condition down on medical forms I write it like this:
I have Lymphatic Malformation often called Cystic Hygroma and Lymphangioma. (I have been given both diagnosis so, I write both. ) You may consider writing, "I have Lymphatic Malformation often called Cystic Hygroma" or "I have Lymphatic Malformation often called Lymphangioma". I do this because it will ensure no matter what doctor, old school or new school, who reads my medical file will know exactly what I am talking about. Old doctors may also, receive an education on terminology and may even research the term Lymphatic Malformation because they have never heard it put that way before. So, not only would you be ensuring any doctor knew exactly what condition you had, you may even be educating some of them to the new terminology. For the purposes of our group I am going to continue to use the combination of new and old terms. It will help reach people who are only familiar with the old terminology and it will help them understand the information as it relates to their condition.
5. What are the feelers or tentacles off of Cystic Hygroma that our doctors describe?
Remember the cul-de-sac above? Well, as the lymph fluid rushes into the cul-de-sac it has no where to go. Eventually, pressure builds and it decides to find an alternative route out of there so, it will branch off attempting to reach back to the lymphatic system. That branch ends in another cul-de-sac and it branches off again, repeating this process over and over. These are the "feeler's" or tentacle's" described by our doctors. Basically, they are the lymphatic system constantly trying to correct itself.
6. Why is there such a broad use of terms?
7. Why does Lymphatic Malformation reoccur after treatment?
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. For this reason, sometimes your doctor may advise you to watch and wait. They may de-bulk the large compromising mass and if reoccurrence occurs, advise you to watch and wait. Sometimes it's best to leave it alone if you are not having any problems or compromise of other organs to avoid making it shift into a direction that will create greater problems.
For example: I personally had an 8 pound mass removed from my right axillary last year. We left a portion of it alone that was in close proximity to my brachial plexus. The mass adjacent to my brachial plexus is sitting there behaving itself so, we are watching and waiting. If it begins to compromise my brachial plexus that can create a world of trouble and it will need to be addressed. So, why didn't we just remove it before it enters that area? Well, if we did that, the residual mass may have no where to go but into my brachial plexus and we may have a real problem on our hands.
8. What body systems can be affected by Lymphatic Malformation?
9. Do I have to be concerned if I have Lymphatic Malformation in one part of my body that I may get it in another?
10. Should Cystic Hygroma patients be worried about getting Lymphangioma and vice versa?
It is the same condition. Some patients are more prone to having macro cystic forms while others are prone to having micro cystic forms. Many patients have a combination of the two.
11. Are there tests or assessments that should be made annually for Lymphatic Malformation patients? What Are they?
There is currently, no "best practice" established for LM patients. Each presentation of LM is unique to the individual therefore, medical management and evaluations are going to be based on each individual patients need. It is recommended that LM patients be seen annually by a Lymphatic Malformation expert. That the expert evaluate the patient, work up specific area's and offer recommendations for testing, treatment and ongoing care.
13. Why do our children get unexplained fevers with no accompanying
symptoms?
14. Why do we get skin lesions and what are they?
The skin lesions are called vesicles. Remember those cul-de-sac's? Well, when the pressure builds and those lymphatic vessels begin to branch off, some branch off up toward the skin. When they do that, clusters of blistery looking lesions are created on the skin. That's why you can have perfectly healthy beautiful skin one day and see a vesicle the next.
15. If that's the case does this mean wherever I have a skin lesion I have LM below internally?
16. Why do our skin lesions hemorrhage?
The lymphatic system is related to the vascular system. It collects fluid and caries it through the lymph nodes back to the vascular system. This accounts for the blood supply required for hemorrhage. The hemorrhaging in the vesicles is thought to be caused by infection or trauma.
17. Why do some hurt and others do not?
18. Why is termination the number one recommendation of doctors when Cystic Hygroma is diagnosed in utero?
This is a HUGE problem that the Lymphatic Malformation experts are aware of and just as disturbed by as we are. They are and have been trying to change the mind set of the OB community through lectures and education. The experts are just as frustrated by this as we are. When I talked to the experts about this I told them the OB community believes there is little or no hope for Lymphatic Malformation patients. The experts said they know and don't understand why that is the OB communities belief because, with proper treatment from the beginning, wonderful results are being achieved and LM patients are living fulfilling contributing "normal" life. They DO become adults and they ARE viable lives.
* When and if, we have a broadcast devoted to LM, I hope you will all go back to the OB doctors who gave you little or no hope and particularly the ones who recommended termination and invite them to join the discussion so that they may become educated to this FACT.
19. Why are there no Lymphatic Specialists?
20. Why is it difficult to find doctors in the adult community who are knowledgeable in treating this condition and how do we change that?
21. Why aren't there specialized clinics for Lymphatic Malformations?
22. Why are chromosomal disorders often associated with Cystic Hygroma yet, most people we meet do not have one?
23. What is the mortality rate of natural births associated with Lymphatic Malformation?
24. What is the mortality rate of people who survive infancy?
25. Do Lymphatic Malformation patients have a shorter life expectancy?
26. Does puberty, pregnancy or menopause effect Lymphatic Malformation? How and why?
27. What is the difference between Cystic Hygroma and Hydrops?
28. Why do doctors treat us as all other patients before they look to Lymphatic Malformation as the cause to other symptoms? Wouldn't it be more cost effective and less time consuming to rule out a connection with our pre-existing medical condition? 29. Why aren't there best practices for Lymphatic Malformation patients that OBGYN's and Pediatrician are aware of and educated on? 30. What needs to change or happen in order to ensure first line care for newborns with Lymphatic Malformation is accessible to all doctors? 31. What, if any, is the relationship between Lymphatic Malformation and the vascular system? 32. Do Lymphatic Malformations tend to throw clots? I need to get more clarification on this answer. I was told they tend to throw clots. I am not sure if that is in every patient or in some patients. Their theory is, the Lymphatic Malformation likes to deplete platelets in order to make clots. Again, the verdict isn't in on this one yet, let me get more clarification. 33. Should Lymphatic Malformation patients be monitored for DVT? 34. Should special precautions or procedures be used post op to prevent or monitor DVT? 35. Are the immune systems of Lymphatic Malformation patients really weaker than the rest of the population? 36. Should Lymphatic Malformation patients take prophylactic antibiotics? 37. What are the benefits to LM patients of participating in a Vascular Clinic? 38. Why has little progress been made in medicine where Lymphatic Malformation patients are concerned in the last 40 years? What may be done to change that? 39. What advice would you give to adult patients seeking medical management for Lymphatic Malformation? 40. Sclerotherapy vs Surgery what are the pro's and con's of each? Why use one over the other? 41. There are many sclerotherapy treatments available are any methods safer than others or more effective? 42. What are the advantages and disadvantages to the different sclerotherapy agents? 43. If Lymphatic Malformation is not a tumor should LM patients be receiving chemotherapy or radiation? I need to get more clarification on this. The experts in Boston do not use chemotherapy as a sclerosing agent. I think Bleomyacin is a form of chemo being used by some facilities as a sclerosing agent. Until we receive clarification on this, if this treatment was being recommended for my child, I would ask a few questions. It is my belief that chemotherapy is used to fight foreign invaders of the body like some cancers and tumors. If the lymphatic system is a needed system and part of my body then, it is not a foreign invader. If it is not a foreign invader then, why are we using chemotherapy to fight this deformity of a needed body system? Is the chemotherapy fighting only my deformity, itself, or is it fighting my lymphatic system which, I need? If they could not give me satisfying answers to these questions I would seek a second opinion and an LM expert opinion. This is one of the sclerotherapy treatments, I personally, am on the fence about. My personal choice would be Alcohol or OK432. 44. What are the common mistakes made in first line treatment of newborns with Lymphatic Malformation? What should be avoided? 45. What advice would you give to doctors providing first line treatment of newborns with Lymphatic Malformation? What should be the first steps? 48. Where do you see the future of our care going?