… an introduction and overview
As obvious and gross (in a medical sense) as it is, every doctor who saw me from birth through adulthood had no professional curiosity to better understand its life and medical consequences. It was my own advocacy that drove me to finally locate the small group of doctors whose curiosity exceeded the others’ lazy implementation of healthcare. And even so, the wonderful doctors who finally diagnosed me didn’t have instructive materials that better defined my condition or common sense explanations of the whys and what fors.
So in my own words, Klippel-Trenaunay syndrome is a congenital and developmental, non-inheritable syndrome that is clinically identified using imaging technologies and is foundationally-based on an aggregation of vascular malformations, which may also include lymphatic malformations. These malformations, in and of themself, are counter-productive and singularly, or in combination, impair and modify the development, normal use, and long-term function of correlated muscular, neurological, skeletal, and internal life systems.
Complications from respective vascular and lymphatic malformations are known to cause chronic and degenerative venous insufficiency, progressive varicosity of the veins, recurrent stasis ulcers, blood pooling, lymphatic pooling and swelling, bleebs, rectal bleeding, G-I bleeding, life-threatening cellulitis, mild to gross hypertrophy (limb or skeletal enlargement), unequal limb development (throughout childhood maturation), compromised internal organs, peripheral neuropathy, systemic muscular atrophy (over time), and/ or other related chronic and degenerative conditions including periodic reports of complications with pregnancy and child-birth. Most find their condition unique due to differing trigger points during the gestation process, yet commonality of conditions clearly point to abnormal development and maturation, and then (in or around puberty) the beginning of life-long degenerative conditions which directly correlates to time of use of limbs or sitting, wherein such activity interferes with gravitational assistance to impaired vascular and lymphatic circulation.
In particular candidates whose condition includes the absence of, or severe incompetence of, deep veins are advised NOT to wear prescription compression garments except under the strictest care from a vascular surgeon as constriction of the peripheral veins most often causes serious neurological damage in the area of application. Likewise, any medical procedure that removes or closes down peripheral veins can not be undertaken except as the result of deep vein studies performed at the bequest of K-TS experienced vascular surgeons or intervention radiologists. To do otherwise is to risk the amputation of limbs when said peripheral veins are the only competent vascular pathway for returning blood to the heart. This syndrome advises “caution” over “expediency”, and “track record” over “genius”.
The most visible feature of Klippel-Trenaunay Syndrome is the appearance of a Port Wine Stain. While not the predominant feature that causes the complications noted above, it is a strong indicator of where (internal and deeper) vascular, lymphatic and related systems are compromised. Please note that my condition is lower limb involved, others have upper limb, trunk, and/or neck and head involvement. I’ve been told that my condition, which is bi-lateral, is less frequent. Apparently, most of those affected are not bi-lateral. It bears repeating, do not confuse K-TS with vascular tumors, which are classified as Hemangiomas and clinically marked quite differently and respectively diagnosed and treated quite differently.
It’s best to understand K-T as fundamentally your young body screwing up during the gestation process and constructing your base plumbing wrong. Specifically, it is usually your return plumbing that is messed up. Depending on when the “vascular trigger” turns on and off your version of K-T will take on very different topologies and thus complications. A quick review of human biology will remind you that the vascular, lymphatic, neurological, muscular and skeletal systems develop simultaneously and interactively. In so much as the vascular system takes on a very special role during these times, its proper construction is key to the proper development of the other systems. For us, this is the big oops! That is why for some of us muscular-skeletal complications can be so dramatic.
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Note: This condition is not properly defined in the class of condition called “hemangioma” or “dermatological birthmark”. Some with this condition may also have additional complications with Arterial-Venous Malformations, and if these complications are more prevalent are better classified under a different condition called Sturge-Weber Syndrome or Parkes-Weber Syndrome.