An arteriovenous malformation (AVM) is a tangle of abnormally connected arteries and veins. As normal vessels course throughout the body or other parts of the body, they supply, they divide into smaller and smaller branches. By the time they reach their destination, they have branched into many tiny vessels called capillaries. Capillaries have the diameter of a human hair. In normal vessels, there are so many capillaries that blood flow within them is slow and under low pressure. The exchange of nutrients occurs at the capillary level.
When a child has an AVM, there are no capillary
vessels to slow down the flow of blood. An AVM is
best described as a high-flow vascular anomaly with
multiple, low resistance shunts that short circuit
(bypass) the capillary bed.
From birth through age 5, Kenny’s nose became red
and bulbous due to the accelerated pressure of blood
supply feeding the malformation. If an AVM is not
controlled, more vessels become involved; the
vessels continue to increase in volume and size. The
increase in blood flow during a growth phase will
increase the size of all other tissues around it. It
is the increased volume in thin walled vessels like
veins that cause them to get really big, really
fast. Nasal AVMs must be watched to prevent any
harmful effects to the underlying tissue or growth
of the nasal bone.
Sometimes they ulcerate
and bleed. Treatment for excessive bleeding and
management of nasal AVMs is explained in Breslow’s
book, FUNNY FACE.
Kenny has a genetic form of AVM called CM-AVM;
caused by mutation in the RASA-1 gene.
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In 2002, after a severe bleed, Kenny underwent a pre-operative embolization to seal as many feeder blood vessels as possible. He then had the majority of his nose resected; leaving the septum, cartilage, mucosa, and columella. A skin graft was taken from his groin and sutured to his face to “make” a temporary nose. That skin has stretched and grown with the growth of his face. While a few procedures were necessary to control bleeding/bony overgrowth to the nose, Kenny has been stable.
Kenny receives ongoing care from Drs. Alejandro Berenstein (St. Luke’s Roosevelt Medical Center) and Barry Zide (NYU Medical Center) both in New York City.
Kenny has also had the benefit of care from Maria Garzon MD (Columbia Presbyterian Medical Center, Department of Dermatology), Francine Blei MD, (Stephen Hassenfeld Center for Cancer and Blood Disorders), John Mulliken MD (Children’s Hospital of Boston), and Mark Sultan MD (Plastic Surgeon, NY).
Kenny’s mother, Deb Breslow, is a regular speaker at the annual benefit for Dr. Berenstein’s innovative Vascular Biology Lab which performs ongoing research for the treatment of rare vascular conditions (AVMs, aneurysms). |
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Dr. Alex Berenstein (honoree), Kenny Breslow, Michael Cipriano, President, Needler's Foundation In 2007, at the age of 10, Kenny had the opportunity to speak to 400 attendees at the Needler’s Foundation benefit honoring Dr. Alex Berenstein as ‘Man of the Year’ at Tavern on the Green in NYC. |
| Useful Links | |
| National Foundation for Facial Reconstruction | Arteriosclerosis, Thrombosis, and Vascular Biology |
| www.aboutfaceinternational.org/ | |
| http://www.davidroche.com/ | |
| http://www.stephendaleproject.com/index.html | |
| http://groups.msn.com/vascularbirthmarksupport/researchmaterialsannouncementsreferralsetc.msnw | |
| Important Articles | |
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RASA1: variable phenotype with capillary and arteriovenous malformations Lawrence M. Boon, John B. Mulliken and Miikka Vikkula
Current Opinion in
Genetics and Development 2005, 15:265-269 Full text provided by www.sciencedirect.com Large Arteriovenous malformations of the Face: Aesthetic Results with Recurrence Control Bradley, James P. MD; Zide, Barry M. DMD, MD; Berenstein, Alejandro MD; Longaker, Michael T. MD Plastic & Reconstructive Surgery: Volume 103(2) February 1999 pp 351-361 Full text provided by www.plasreconsurg.com |
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