WE HAVE A DEFINITION PROBLEM. DOCTORS AND LAYPERSON’S ALIKE CALL
ALL OF THE BELOW “PORT WINE STAINS” (PWS) AS IF OUR CARE & TREATMENT WILL BE ALIKE
– – – THE MEDICAL PROFESSION COULD HELP US BY CORRECTING THE SCIENTIFIC RECORD – – –
WE OVERGENERALIZE AT OUR PERIL
. . . Capillary malformations developed via different genetic mutations or
different classical syndromes are not universally the same biologically and thus
warrant careful examination and condition-based targeted interventions, including
NO INTERVENTION in many cases. See Boston Children’s Hospital up-front explanation.
The medical profession only marginally complains about misdiagnosis
based on stale and incongruent terms. THOSE BORN RARE WHO LIVE
WITH THE CONSEQUENCES ARE PROPER TO ASK FOR
CONSIDERATION ON THIS POINT.
THE INTERNATIONAL SOCIETY FOR STUDY OF VASCULAR ANOMALIES, THE ISSVA,
LISTS AS FEW AS 13 POSSIBLE DIAGNOSIS FOR CAPILLARY MALFORMATIONS AS A DISTINCT
CONDITION, 5 AS A COMBINED CONDITION, AND 6 AS A KEY COMPONENT IN LEGACY CONGENTIAL SYNDROMES.
Is this a Port Wine Stain? No. It is a different Congenital Vascular Malformation – one that has no capillaries, Arteries and Veins are shunting directly into each other. These are medically referred to as High Flow malformations. Capillary malformations more properly are in the Low Flow group.
Is this best diagnosed as singularly a Port Wine Stain? Likely not. More likely, this is a Combined Vascular Malformation. The flatter visual (red-pink) is the Capillary Malformation component which when alone carries the common name Port Wine Stain. The second component, the blister-like entities or more commonly called “blebs” which are outgrowths of Lymphatic Malformations. When these burst these weep lymphatic fluid – can include redish fluids (blood cells) as well. Doctors following the ISSVA Classification system for Vascular Anomalies seem to be diagnosing this person with CLM, maybe even CLVM if underlying Vein Malformations were indicated. Using the term Port Wine Stain would in our view belie the complexity of the various interactions between no less than two vascular components which are congenitally malformed.
Is this a Port Wine Stain? No. It’s reported to be a Superficial Segmented Infantile Hemangioma, one of several different Hemangioma diagnostic classifications. Hemangioma is an all too often misapplied diagnosis to those of us with Port Wine Stains. Per the ISSVA a Hemangioma is NOT a Vascular Malformation – its medically in a different diagnostic class which is medically termed Blood Tumors. We hope to develop a section on these to help those who face medical professionals or support groups who use visual cues to diagnose. It’s common among those of us who have Combined and Singular Vascular Malformations to say our symptoms, our conditions, are not skin deep. With cause. With frustration at misdiagnosis and delayed proper diagnosis.
WHAT WOULD YOU DO IF THIS WAS YOUR PWS PATIENT?
Expert Birthmark Doctor – “I can’t help you.”
Another Birthmark Expert, “We can’t do anything for you.”
A multi-disciplinary Vascular Anomaly team properly diagnosed and did a successful intervention.
Please know your experts depth of knowledge and constructive experience with Congenital Vascular Malformations, and therein scientifically differentiated syndromes & symptoms
By way of analogy, not every white car we see is a Chevy. In our community of congenital anomalies, a capillary malformation when tested for root cause returns distinct causes ranging from Sturge Weber, to Klippel Trenaunay, to CLOVES, to PROTEUS, to M-CM, to Simple Capillary Malformations, to any other 34 plus diagnostic classifications.
AND this doesn’t take in to account the OTHER FAMILY of red-blue artistry by Mother Nature – Hemangiomas! These we don’t expand on in our website because these are not classified as Congenital Vascular Malformations.
SYNTHESIS – ANALYSIS – FEEDBACK – EDUCATION
work product is a personal perspective
The Capillaries are very small vessels that are the road system that connects a major one-way road, the arteries, to another one-way road, the veins. When these are not formed properly these can take on a number of different anomalies. These can form in excess looking much like Rat’s nest. They can form in larger sizes. They can not form at all, or sparsely, both of these causing arteries and veins to connect directly. They can form as dead ends trapping blood.
All in all the above malformations tend to bring blood closer to the surface of our skin where we can see the blood. Warm indicates red blood. Pink indicates cooling blood. Blue-Purple indicates cold blood. Oxygenated shows as red blood. Deoxygenated shows as blue-purple blood. My wife says I have a built in Temperature Gauge.
The capillary vessels are like the tiny roots of plants in that these are designed to deliver and pick up blood from surrounding tissues and muscles; as well as return it to the heart. As an aside, as a kid I enjoyed freaking out my friends by pressing out the blood in these red to purple areas to prove my skin was not painted or stained. My laymen’s objection to calling Capillary Malformations a “Stain” or a “Birthmark”.
As for me these Capillary Malformations did not and still don’t hurt. My bio-chemist father used to say Capillaries, Veins and Lymph Vessels do not have nerves in them. The pains I was feeling he said came form pressure on nerves in surrounding tissues. These Capillary Malformations sure get 98% of the world’s attention though. They are rather obvious to the naked eye. I consider mine the least of my concerns. That said, there certainly are variations that are problematic. Please get to a doctor who explains these realities in medical terms and makes critical distinctions. Interventions are best when informed and cautious.
Ask your physician if your pain is caused by Capillary Malformations or other underlying Vascular Anomalies and associated pressures on your nerves. Goes for Lymphatic Vessel Malformations as well. Pain when present is indicative of a bigger set of medical issues that deserve distinct attention.
- DermNet NZ – New Zealand
- OTOLARYNGOLOGY & OPHTHALMOLOGY INSIGHT
- RADY’S CHILDREN HOSPITAL – USA, CA, San Diego
- MOUNT SINAI
- BOSTON CHILDREN’S HOSPITAL
- MAYO CLINIC, USA, Minnesota, Rochester
- JOHN HOPKINS HOSPITAL – USA, Maryland
- INTERNATIONAL SOCIETY FOR STUDIES OF VASCULAR ANOMALIES
SUPPORT – RESOURCES
- CLOVES SG
- PROS SG
- M-CM SG – Netherlands
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