Download Vascular Lesions

Vascular Lesions
Annette M. Wagner, MD
Attending Physician, Children’s Memorial Hospital
Department of Pediatrics and Dermatology
Northwestern University’s
Feinberg School of Medicine
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Vascular Lesions
Hemangiomas
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Portwine stains
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How to distinguish vascular lesions
• Portwine stains are present at birth
and stay flat
• Hemangiomas are not present at
birth and grow rapidly
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How to distinguish vascular lesions
Pyogenic granulomas
look just like small
hemangiomas but
occur > 1yr olds
(BLEED!)
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How to distinguish vascular lesions
Spider angiomas are
tiny on face and hands
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Hemangiomas
• 30% faint
macules at birth
• Most appear 1-4
weeks
• 80% solitary,
head/ face most
common site
• F:M =3:1, > in
premies
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Hemangiomas
PHASES
1. Proliferation : 1-10 months
- Most rapid 1-4 mths
- Complications occur in this phase
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Hemangiomas
2. Involution: After 6-10 mths
- Soften, grey surface
- 50% gone by age 5
- 90% gone by age 9
- If fully formed at birth (rare) gone by age 2
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Hemangiomas
LESIONS DO NOT ALWAYS
LEAVE NORMAL SKIN
Residual atrophy, wrinkling, telangiectasias, pallor
and redundant tissue are common
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Complications
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•
•
•
•
•
Ulceration
Alter function
Kasabach-Merritt Syndrome
Congestive heart failure
Posterior fossa defects
Residual skin changes
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Complications
Ulceration
– < 5% of
patients
– Perineum,
central face
– Large lesions
on extremities
and scalp
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Complications
Ulceration
– Bleed and
leave scars
– May get
infected;
PAINFUL
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Treatment
•
•
•
•
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Topical antibiotics
Barriers +/- occlusive dressings
Oral antibiotics; analgesics
Pulsed dye laser
Oral prednisone (2mg/kg)
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Alter Function
VISION
– Obstructive
amblyopia
– Astigmatism,
myopia
– Strabismus
– Proptosis,
pressure on globe
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Complications
RAPIDLY ENLARGING HEMANGIOMAS OF THE
UPPER EYELID ARE MEDICAL EMERGENCIES
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Treatment
• Oral prednisone
• Intralesional steroids
• Pulsed dye laser if early
– Treats superficial component ONLY
• Referral to ophthalmology
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Alter function
LARYNGEAL
–
–
–
–
Respiratory distress
Stridor 6-8 weeks
Post. subglottic area
“Beard” distribution
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Treatment
• MRI if deep
hemangioma in
anterior triangle
of neck
• Pushing of
structures common,
obstruction rare
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Treatment
• Direct
laryngoscopy
for stridor
• Oral prednisone
and CO2 laser
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Kasabach-Merritt Syndrome
Rapid
enlargement
of deep lesion
with tense
Purpuric
surface
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Kasabach-Merritt Syndrome
• Platelet trapping and DIC
• Occurs early (3-6 weeks)
• Trapping in spleen also
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Kasabach-Merritt Syndrome
• NOT true hemangiomas
• Kaposiform hemangioendothelioma
or tufted angiomas
• Acute hemorrhage
• Compression of vital structures
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Kasabach-Merritt Syndrome
• New glut-1 staining available on
tissue can distinguish hemangioma
and other vascular tumors
• If unusual appearance, present
at birth, rapid growth or severe
ulceration refer immediately
to consider biopsy
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Treatment
• CBC, smear, plates, PT, PTT,
coagulation studies
• Culture if suspect infection
• Prednisone, vincristine interferon
• Supportive care
• High mortality
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Congestive Heart Failure
• Usually multiple small
hemangiomas with
liver involvement
• Murmur, anemia,
HSM 2-8 wks
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Congestive Heart Failure
• High mortality (54%)
• Death from CHF,
infection, hemorrhage
• PROBLEMS OCCUR
EARLY
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Treatment
• CBC, platelets, UA,
stool guaiac
• Liver ultrasound, CT
with contrast
• Distinguish from AVM
with angiography
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Treatment
• Cardiology
consult (digoxin)
• Oral prednisone,
vincristine or
interferon-a
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Posterior Fossa Defects
Association with large facial hemangiomas
• Dandy Walker
malformation
• Other
associated
anomalies
• PHACE
syndrome
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Posterior Fossa Defects
PHACE
• Posterior fossa
malformation
• Hemangioma
(segmental)
• Arterial anomalies
• Cardiac anomalies
• Eye defects
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Hemangiomas:
When to worry; whom to refer?
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•
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Facial hemangiomas (around eye, nose, lip)
Lg hemangiomas of face or scalp
Ulcerated hemangiomas
>10 hemangiomas <3 mths
Unusual natural history
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Hemangiomas:
When to worry; whom to refer?
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•
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Any rapidly enlarging deep hemangioma
Hemangioma over LS spine
Bleeding hemangiomas
Presence of thrill or bruit
Uncertain diagnosis
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Hemangiomas:
When to worry; whom to refer?
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Pediatrician Treatments
•
•
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•
•
REASSURANCE
Compression on extremities
Topical antibiotics if ulcerated
Oral antibiotics if infected
Referral when appropriate
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Hemangiomas
NO THERAPY IS STILL
GOOD THERAPY
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Dermatologist Treatments
• Intralesional steroids
• Systemic steroids
– 2-4mg/kg qam
– Life threatening or functionally impairing
hemangiomas only
– Effects in 10 days to 3 weeks
– Can rebound when stopped
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Pediatric Follow-Up
If on systemic steroids
– Follow BP, UA, wt, ht, HC q2wk
– CBC
– Avoid live vaccines only
– Reduce infection risk
– Follow development
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Other Dermatologist Treatments
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•
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Laser
Vincristine
Interferon-α
Surgery
Embolization
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Laser Therapy
• CO2 laser for subglottic hemangiomas
• Pulsed-dye laser (585-595nm) for ulcerated
hemangiomas
• Cosmetic improvement of superficial
hemangiomas
• No Impact on deep lesions
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Interferon ALFA-2A
• Treatment of choice in life threatening
hemangiomas
• Causes Spastic Diplegia in 10-20% of patients:
Close neurologic follow-up essential
• Vincristine is alternate
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Surgery
• For hepatic hemangiomas or in life threatening
circumstances when medical therapy fails
• For cosmetic repair of redundant skin after
involution
• Avoid resection before involution
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Embolization
For life-threatening liver hemangiomas in conjunction
with medical and surgical management
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Nevus Simplex
Most common vascular lesion
in newborns (30-40%)
• Angel’s kiss - glabella
– 20% have;
95% gone by one year
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Nevus Simplex
Most common vascular lesion
in newborns (30-40%)
• Stork bite - nape of neck
– 22% have;
75% resolve
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Management
• No treatment necessary
• Those involving nasal bridge less likely to fade
• Pulsed dye laser can treat if persistent >1-2 yrs
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Portwine Stain
• 0.5% of newborns
• Dilated capillary
vessels
• Present at birth;
grow with child;
do NOT raise
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Portwine Stain
• Darken, thicken and
may develop
angiomatous
papules
• Rare acquired form
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Portwine Stain
Associated Syndromes
-Sturge-Weber syndrome
-Klippel-Trenaunay syndrome
-Cutis marmorata telangiectatica congenita
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Sturge-Weber Syndrome
• Affects 10% with facial
portwine stains
• Patients have staining in the
distribution of the 1st or 2nd
branch of trigeminal nerve
• Vascular malformations
of the ipsilateral brain
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Sturge-Weber Syndrome
• Risk of glaucoma, buphthalmos
– Most common with 2nd branch involvement
• Neurologic manifestations:
– Seizures (89%)
– Mental retardation,progressive (50%)
– Hemiparesis, hemiplegia (6%)
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Management
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Eye exam 6-12 month intervals
MRI optional (non-prognostic)
Close pediatric follow-up
Refer to neurology for any abnormal findings
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Management
• Pulsed dye laser for treatment of stain
• Seizures managed with anticonvulsants, difficult
• Glaucoma responsive to medical therapy only
50% of time, surgery or laser used
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Klippel-Trenauney Syndrome
• Extensive PWS of extremity
with overgrowth of limb
• Vascular or lymphatic
malformation of the skin
• Soft tissue or bone hypertrophy
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CMTC
• Cutis marmorata telangiectatica
congenita
• Mottled stain over extremity
or trunk (may have atrophy)
• May have undergrowth of limb
• Stain improves with time
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Portwine Stains:
When to worry; whom to refer?
• Stains on face
• Stains with limb size discrepancy
• Any stain that parents would like to have treated
with laser
• Any stain with episodes of pain or infection or
bleeding
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Pulsed Dye Laser
• This is treatment of choice
and standard of care
• Nonscarring (<1/100)
• Penetrates < 2mm
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Pulsed Dye Laser
• 94% have marked
improvement
• Average 6-8 treatments
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Pulsed Dye Laser
• Repeat at 2-3 month intervals
• Purpura lasts two weeks
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Pulsed Dye Laser
• Post-op care: lubrication and sun avoidance
• Early treatment may mean fewer sessions
• Use anesthesia
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Pulsed Dye Laser
• Early onset of treatment, small size (<20cm)
good prognosis
• Lateral face responds best
• First 6 treatments most effective
• Selective photothermolysis of small BV causes
cytokine upregulation
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Pulsed Dye Laser
• Recurrence up to 50% 4 years post treatment
• MOST stains do NOT completely resolve
• Dynamic cooling may decrease pain and
allow increased energies
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Summary
• Natural hx and appearance can distinguish
vascular lesions
• Not all hemangiomas are benign
• Facial PWS - Sturge-Weber
• Pulsed dye laser for stains
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