Vascular Birthmarks Foundation Hemangiomas  |  Port Wine Stains  |  Vascular Malformations  
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1994 - 2014
Celebrating 20 years with
75,000 networked into treatment

Dr. Linda Rozell-Shannon, PhD President and Founder

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Ask the VBF Experts

Dr. Stuart Nelson, VBF Co-Medical Director and International Port Wine Stain Laser Specialist
Dr. Nelson will answer your questions concerning the diagnosis and treatment of Port Wine Stains.

 

Dr. Gregory Levitin, Hemangioma and Malformations Surgeon, NYC and LA
Dr. Levitin will answer your questions regarding the surgical treatment of all vascular birthmarks and tumors.

 

Dr. Robert Rosen, Vascular Lesions of Arms and Legs Interventional Radiologist
Our expert for all non-brain AVMs and vascular lesions of the arms and legs, Dr. Rosen welcomes your questions.

 

Dr. Roy Geronemus, NYC and International Laser Specialist
If you have a question or concern about laser treatments in general, contact Dr. Geronemus.

 

Dr. Aaron Fay, Hemangioma and Malformation Eye Surgeon
Dr. Fay will answer your questions about orbital birthmarks.

 

Corinne Barinaga, VBF Family Services Director
Corinne Barinaga, our Administrative Director, will answer emails concerning family advocacy, treatment questions, or physician referral.

 

Dr. Martin Mihm, VBF Co-Medical Director and Research Director
Dr. Mihm is coordinating and directing research regarding vascular birthmarks and tumors.

 

Dr. Darren Orbach, Pediatric Neurointerventionalist for AVMs and PHACE
VBF is proud to welcome Dr. Orbach!

 

Dr. Anne Comi, Sturge Weber Syndrome Specialist
One of the leading experts on Sturge Weber Syndrome, Dr. Comi will be responding to your questions concerning this syndrome.

 

Dr. Alex Berenstein, Malformations and AVM Interventional Radiologist
Ask Dr. Berenstein your questions regarding interventional radiology.

 

Dr. Kami Delfanian, KTS Treatment Specialist
Send your questions concerning KT Syndrome to Dr. Delfanian.

 

Dr. Barry Zide, NYC Hemangioma and Malformations Surgeon
If you have a question or concern about hemangioma and vascular malformation treatment in general, contact Dr. Zide.

 

Dr. Joseph Edmonds, Lymphatic Malformations Surgeon
Ask Dr. Edmonds your questions related to Lymphatic Malformations.

 

Anna Duarte, M.D., Florida Expert
Ask our expert Dr. Duarte, your questions about receiving treatment in Florida.

 

Dr. Orhan Konez, Interventional Radiologist
Questions regarding reading and interpreting films and treating malformations with sclerotherapy or embollization can be sent to Dr. Orhan Konez.

 

Dr. Milton Waner, Hemangioma and Malformations Surgeon
Email Dr. Waner with questions regarding hemangiomas and other vascular lesions.

 

Dr. Steven Fishman, Internal Lesions Surgeon
Ask Dr. Fishman your questions about liver and other internal vascular lesions.

 

Rafael Ortiz, MD, Neuro-endovascular Surgeon
Ask Dr. Ortiz your questions about vascular tumors of the head and neck region, cerebral and spinal arteriovenous malformations, treatment of craniofacial vascular lesions (venous, lymphatic, AVMs, hemangiomas) in adults and children.

 

Dr. Calil, Lymphatic Malformation Surgeon
Dr. Calil will answer your questions about Lymphatic Malformations.

 

Elissa-Uretsky Rifkin, M.Ed. CMHC Midwest Developmental Specialist
A trained developmental specialist and is on the board of VBF. Send questions concerning hemangiomas and this topic to Elissa.

 

Dr. Stavros Tombris, European Surgeon
Fr. Tombris treats all forms of hemangomas, port wine stains and malformations.

 

Dr. Stevan Thompson, Military (Tricare) Surgeon
Dr. Stevan Thompson has joined us to answer questions concerning the treatment of vascular birthmarks in the military.

 

Dr. Helen Figge, Pharmacist
If you or your child has a vascular birthmark and you have a question regarding a prescription drug, please ask Doc Helen Figge.

 

Dr. Linda Rozell-Shannon, VBF President and Founder
Dr. Linda Rozell-Shannon is the leading lay expert in the world on the subject of vascular birthmarks.

 

Lex Van der Heijden, CMTC Foundation
If you or your child has CMTC, please contact Lex with your questions.

 

Leslie Graff, East Coast Developmental Specialist
Leslie is a trained developmental specialist. Send questions concerning port wine stains and this topic to Leslie.

 

Linda Seidel - Make-up Expert
Ask Linda Seidel your questions about make-up.

 

Nancy Roberts - Make-up Specialist
Ask our expert Nancy Roberts, Co-Creator of Smart Cover Cosmetics (www.smartcover.com), your questions about make-up.

 

Eileen O'Connor, Adult Living with PWS

 

Laurie Moore, Make Up Expert from Colortration
Laurie Moore, from www.colortration.com will answer makeup related concerns.

 

Alicita, Spanish Expert
Ask our expert Alicita, your questions in Spanish.

 

Dr. Thomas Serena, Wound Care Expert

 

Sarina Patel, Young Adult Advocate

 




 

What Our Families Are Saying About Us

 

"We relied on the Vascular Birthmarks Foundation to provide us with the information, the contacts, the resources, and the support that we needed to get through this difficult time. Their theme, "We are making a difference" couldn't be more accurate. For us, it was all the difference in the world."
Jill Brown

 


Hi Linda
Just a note to say how wonderful I found the interview of you and Capital 9 news. Thanks so much for your devotion.
Gina

 




Treatment Options


Treatment Options

AAD Summer 1999 James F. Nigro, MD New York, New York July 31, 1999

Diagnosis and Management of Hemangiomas and Vascular Malformations in Childhood

I. Nomenclature of hemangiomas and vascular malformations

a. The major obstacle to the understanding and management of vascular birthmarks

b. Mulliken and Glowacki demonstrated that there are only two major types of vascular birthmarks based on differences in the following categories:
-Clinical
-Histologic
-Hematological
-Radiological
-Skeletal

c. Histologic features are the most important differences
-Hemangiomas have plump endothelia, increased mast cells, and multilaminated basement membranes
-Malformations have flat endothelia, normal mast cell numbers, and a thin basement membrane

d. Modern Nomenclature
-Hemangiomas are superficial, deep, or combined and may be proliferating or involuting
-Vascular malformations may be capillary, venous, arterial, lymphatic, or a combination of these

II. Hemangiomas

a. Introduction
-True benign neoplasm's
- Comprised of capillaries and venules in superficial and/or deep dermis
-Present during first few weeks of life
-Rapid proliferation and slow involution
-Most resolve completely without major complications

b. Incidence
-Female: male 3:1
-More common in Caucasians than in African Americans
-May be present in 10-20% of premature infants
-Solitary in 80% of patients

c. Location
-Based on percentage of body surface area, they are more common on the face
-By strict numbers, about 30% occur on the face or scalp

d. Precursor Lesions
-Appear prior to the actual proliferation of the hemangioma
-Pale patches
-Telangiectasia
-Macular erythema
-Bluish discoloration
-May be confused with port wine stain or nevus anemicus

e. Proliferative Phase
-Superficial lesions: red, raised, firm, well-demarcated
-Deep lesions; bluish, soft, slowly enlarging
-Wide variation in size -Growth phase: 3-12 months

f. Involution Phase
-Color change from bright to dull red
-Central greying
-Gradual softening
-Eventual resolution *50% by 5 years, 70% by 7 years, 90% by 9 years

g. Alarming Hemangiomas
-Vital/Important structures: eye, larynx, distal extremities
-Cosmetically sensitive regions: nose, lip, ear -Very large trunal resolution

h. Minor Complications
-Bleeding rare in these low flow lesions
-Infection: rare
-Ulceration: rapidly growing lesions and in the diaper area
-Residua: Telangiectasia, atrophy, hypopigmentation

i. Diffuse Neonatal Hemangiomatosis
-Multiple, small, cutaneous lesions
-Dome shaped, uniform in size
-May be associated with visceral lesions
· Liver, GI, CNS
· May be asymptomatic
· High-output cardiac failure, hemorrhage, obstructive jaundice, coagulopathy
-Involution of cutaneous and visceral lesions by age 2 years
-Ultrasound or MRI studies are indicated
-Treat symptomatic patients

j. Kasabach-Merritt Syndrome -hemangioendotheliama or tufted angioma
-extremely is usually involved
-coagulopathy associated with platelet trapping within lesions
-high mortality rate in untreated cases
-treatment
· Surgical excision
· Interferon
· Systemic corticosteroids

k. Associated Syndromes
-less common than with vascular malformations
-PHACE(S) syndrome
· Posterior fossa CNS malformations (Dandy Walker)
· Hemangioma
· arterial anomalies
· cardiac anomalies
· eye anomalies and (sternal defects)
-lumbosacral lesions
· spinal anomalies
· genitourinary anomalies

I. Therapy
-observation
· photography
· regular follow-up visits
· reserve right to initiate therapy at a later date
-systemic corticosteriods
· 2-3 mg/kg/day for 4-6 week and then slowly taper
· younger infants may require a longer or second course
· immunizations: hold until off steroids for 1 month
· side effects: increased appetite, change in sleep patterns, fussiness -intralesional corticosteroids
· 3-5 mg/kg/dose
· systemic absorption is significant
· potential adverse side effects
· soft tissue atrophy
· eyelid necrosis
· perforation of the globe
· retinal artery occlusion
-topical corticosteroids
· high potency
· may be effective in small superficial hemangiomas
-interferon alpha
· antiangiogenic activity
· 3 million units/meter sq/day subcutaneous
· treatment is required for several months
· excellent results in severe or life threatening hemangiomas unresponsive to corticosteroids
· adverse effects
· fever
· neutropenia
· spastic diplegia
· motor delay
-laser
· tunable yellow dye (flash lamp pumped pulse dye)
· very thin or precursor hemangiomas
· ulcerations
· residual telangiectasia
· Nd: YAG
· Bulky facial lesions
· Increased risk of scarring
· Experimental
-surgical excision
· protuberant lesions
· consider surgical consultation when parents are very anxious
· avoid if hemangioma is diffuse -duoderm
· excellent pain control in ulcerated perineal lesions
-cryosurgery
· risk of scarring
· good results are possible with experienced hands

III. Capillary Malformations (Port Wine Stains)

a. Introduction
-vascular malformations limited to dermal blood vessels
-present at birth
-permanent
-associated with other vascular malformations and congenital syndromes

b. Incidence
-0.3% of neonates
-equal sex and racial predilection
-50% of facial PWS restricited to one trigeminal sensory region -remainder involve more than one, cross midline, or are bilateral

c. Appearance
-pink, well-circumscribed patches
-growth is commensurate with growth of the child
-darken and thicken with age

d. Sturge-Weber syndrome -facial port wine stain
· V1 trigeminal sensory region must be involved
-CNS
· Seizures
· Mental retardation
· Railroad track calcifications or cortex
-opthalmologic
· Ipsilateral choroidal angiomatosis
· Glaucoma (can be seen with V2 lesions involving eyelid)

e. Treatment
-tunable dye laser
· Treatment of choice
· Multiple treatments required (average 6.4)
· Very good to excellent results in most patients
· Few side effects
-Anesthesia
· General: infants and children with large lesions
· Topical: older patients with large or small lesions
· None: most adults with small lesions
-psychological evaluation
-neurologic and opthalmologic exam
-other lasers, tattooing, excision, radiation are not indicated

IV. Venous Malformations

a. clinical features
-bluish patch or mass with indistinct borders
-present at birth but may not be evident
-compressible
-phleboliths, thrombosis, hemorrage
-frequently confused with deep hemangiomas

b. treatment
-none
-surgical excision
· Image prior to surgery to determine extent of lesion -sclerotherapy
-elastic stockings

V. Arteriovenous Malformations

a. clinical features
-high flow-may involve bone, muscle, viscera
-often undiagnosed until adulthood
-discoloration or pulsatile mass may be noted

b. treatment -surgical excision -embolization

VI. Lymphatic Malformations

a. localized or diffuse

b. may slowly enlarge over time

c. may be confused with deep hemangiomas

d. superficial lesions may respond to laser therapy

e. incomplete surgical excision can lead to massive overgrowth

f. support garments

VI. Syndromes Associated with Vascular Malformations

a. Klippel-Trenaunay
-definition: soft tissue hypertrophy and bony overgrowth of extremity with PWS
-clinical features
· Usually single lower extremity
· Overgrowth not present at birth
· Significant limb length discrepancy
· Prominent hypertrophy of foot and toes
· No CNS or visceral anomalies
-treatment
· Premature epiphyseal closure of longer leg
· Surgical debulking is usually not feasible

b. Maffucci's syndrome venous malformations -enchondromes -distal extremities

c. Blue Rubber Bleb Nevus syndrome -venous malformations of skin and GI tract -compressible, painful lesions -GI hemorrage is common cause of death

d. Gorham's syndrome -venous and lymphatic malformations involving skin and skeleton -osteolytic bone disease

e. Proteus syndrome: PWS, partial gigantism, macrocephaly, epidermal nevi

f. Wyburn-Mason syndrome: retinal and CNS AVM, facial PWS

g. Riley-Smith syndrome: cutaneous venous malformation, macrocephaly

h. Cobb syndrome: venous malformations of spinal cord, truncal PWS

i. Bannayan-Zonana syndrome: subcutaneous/ visceral venous malformation, lipomas, macrocephaly