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Tara L. Rosenberg, MD Texas Children’s Hospital
Will this birthmark go away?
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Disclosures
I have no conflicts of interest to disclose. "
Please note this presentation will include photographs and other images of patients. Permission has been obtained to use these images in an educational context.
Nothing shown here should be replayed or distributed outside of this educational context.
Accordingly, videotaping and/or recording of any kind is prohibited.
No Photos Please
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Outline
• Introduction to vascular anomalies • Specific lesions • Conclusions
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Introduction Vascular anomalies • “Vascular birthmarks” • Incidence: approximately 4%
• Occur anywhere but most in head and neck • Many disciplines involved • Therapies can be controversial • Rapidly changing field
60%
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Vascular Anomalies
Tumors" Malformations"
Low Flow" High Flow"
Mulliken & Glowacki. Plast Recon Surg 1982"
ISSVA Classifica2on of Vascular Anomalies ©2014 Interna2onal Society for the Study of Vascular Anomalies
Available at "issva.org/classifica2on" Accessed April 2014
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Similar Presentations Venous Malformation Lymphatic Malformation
Arteriovenous Malformation Hemangioma
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Variable Presentations
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Question 1: What is the diagnosis of this lesion? A. Infantile hemangioma B. Venous malformation C. Lymphatic malformation D. Pyogenic granuloma
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Similar Extent of Disease Venous Malformation Lymphatic Malformation Hemangioma
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Variable Extent of Disease
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Clinical Differences Infantile Hemangioma Vascular Malformation • Small (flat) or absent at birth
• Rapid growth during infancy
• Involution during childhood
• Clinically apparent at young age
• Present at birth
• Never proliferates-Never involute
• Growth proportional to child
• Variable age at presentation
Functional and Cosmetic Impact
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Waner and Suen, 1996
Diagnostic Algorithm
Present at Birth? YES=VM
Size Increase?
NO=Hemang
YES=Hemang
NO=VM
Size Decrease?
YES=Hemang
NO=VM
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Physical Exam
• Discrete borders
• Superficial-subcutaneous
• Mobile
• Not compressible
• Warm
• Borders difficult to delineate
• Superficial and Deep
• Non-mobile
• Compressible
• Variable temperature
Hemangioma/Tumors Vascular Malformations
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Diagnostic Tools: Ultrasound
• Quick
• Inexpensive
• Non-invasive
• Flow characteristics
• First step
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Diagnostic Tools: MRI
Venous Malformation Lymphatic Malformation Hemangioma
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Treatment • Multidisciplinary – Otolaryngology – Interventional radiology – Dermatology – Plastic surgery
• Multimodality • Staged
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Treatment Modalities • Medical – Propranolol
• Interventional radiology – Embolization – Sclerotherapy
• Laser therapy • Surgical
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Infantile Hemangiomas
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Hemangiomas • Most common tumor of childhood • Incidence 4-10% • Female (2-3:1), white, premature, low birth weight, multiple
gestation • Variable appearance • Characteristic growth pattern
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Involution • Historic report – 50% by 5 years – 70% by 7 years – 90% by 9 years
• 10% persist
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Diagnosis • Based largely on clinical presentation
• Ultrasound or MRI in select cases – To validate clinical diagnosis – Evaluate extent of disease
• Biopsy is rare but recommended for atypical clinical or radiological presentation – GLUT-1 (North et al., 2003)
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Hemangioma Classification
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Question 2: What diagnostic study would you obtain on this patient? A. Renal ultrasound B. pH probe test C. Liver ultrasound D. CT head
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Segmental Facial Hemangiomas PHACES • Posterior fossa malformations • Hemangiomas • Arterial anomalies • Cardiac anomalies • Eye abnormalities
• Sternal cleft or supraumbilical raphe syndrome
Frieden et al. Arch Dermatol 1996: 132; 307-311.
Consider the airway!
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Management • Observation • Intralesional steroids • Medical • Surgery • Laser therapy
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Observation vs. Intervention
1. Many hemangiomas persist beyond 9 years 2. Age of self-recognition/concept 4-5 years 3. Large/protruberent hemangiomas leave fibrofatty residuum 4. Scarring/telangiectasias are common 5. Majority occur in aesthetically sensitive areas
Keeping it in perspective…
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Observation
• Superficial lesions in cosmetically and functionally insensitive areas – Away from the head and neck – Small cervical-facial lesions
• Deep hemangiomas without functional or cosmetic impact
• Important to give them a chance
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Observation
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Indications for Intervention • Impact on vital structures
• Active or impending functional impairment
• Possibility of permanent scarring
• Large segmental/ facial hemangiomas
• Ulcerating hemangiomas
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Propranolol Therapy
N Engl J Med. 2008 Jun 12;358(24):2649-51
Propranolol for severe hemangiomas of infancy. Léauté-Labrèze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo JB, Taïeb A.
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PreTherapeutic Recommendations • ECG • Extensive Pulmonary/Cardiac History • Echo if either in question • Cardiology involvement • Frequent pediatric visits for vitals • Reflux precautions/medications
• Consensus Statement
Pediatrics. 2013 Jan;131(1):128-40. doi: 10.1542/peds.2012-1691. Epub 2012 Dec 24.
Initiation and use of propranolol for infantile hemangioma: report of a consensus conference.
Drolet BA, Frommelt PC, Chamlin SL, Haggstrom A, Bauman NM, Chiu YE, Chun RH, Garzon MC, Holland KE, Liberman L, MacLellan-Tobert S, Mancini AJ, Metry D, Puttgen KB, Seefeldt M, Sidbury R, Ward KM, Blei F, Baselga E, Cassidy L, Darrow DH, Joachim S, Kwon EK, Martin K, Perkins J, Siegel
DH, Boucek RJ, Frieden IJ.
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Adjuvant Therapy
>50% Patients: Necessary to improve final outcome 1. Flash Pump Dye Laser 2. Steroid Injection 3. Excision of residuum
Venous Malformations
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Venous Malformations • Congenital ectatic veins
• Progressive growth and disfigurement
• Evident by blue discoloration, low flow, and compressibility
• Frequently present later in life
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Venous Malformations
Presentation • Blue discoloration, swelling
of skin and/or mucosa
• Difficulty breathing when supine
• Obstructive sleep apnea
• May be asymptomatic
• Increase in size with anger/exercise/valsalva
• Progressive increase over time
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Management of Venous Malformations
Richter and Braswell, 2012 "
Nd:YAG Laser
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Nd:YAG Laser • Interstitial laser therapy – Deep Lesions
• Subcutaneous
• Tongue
• Masseter/buccal
• Neck
• Ultrasound-guided • Glass tip Nd:YAG laser fiber
Rosenberg TL and Richter GT. Lasers in the treatment of vascular anomalies. Curr Otorhinolaryngol Rep (2014) 2:265-272.
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Gentle® YAG Laser
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After multiple skin, oral, and airway laser treatments
Combined Gentle® YAG and Nd:YAG Laser Therapy"
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Surgical Excision
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Lymphatic Malformations
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Lymphatic Malformations • Second most common congenital lesion
of the head and neck
• Often noted at birth (<2yr)
• Localized or diffuse
• Associated with CHAOS
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Lymphatic Malformations
Classification • Macrocystic >1cm • Microcystic <1cm • Combined • Mixed Venous-Lymphatic
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Lymphatic Malformations • Mass-variable • Size increases with… – Infection – Hormonal changes – Trauma – Intralesional bleed
• Airway compromise • Temporarily shrink with
steroids and antibiotics
T2 MRI: High Intensity/Fluid Levels
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Treatment Modalities • Observation • Medical – Sirolimus
• Interventional – Sclerotherapy
• Laser therapy • Surgical excision
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CO2 Laser
Palatal Microcystic Lymphatic Malformation
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Lymphatic malformation post primary excision and CO2 laser
Multimodality Treatment
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Multimodality Treatment
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Arteriovenous Malformations
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Arteriovenous Malformations • Often clinically quiescent – Warm – Pulsatile – Dilated draining veins with pulsations – Red – “Throbbing”
• Progressively expanding high flow-vascular lesion • Bleeding, pain, devastating functional and cosmetic consequences
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Focal AVM
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Extensive AVM
Previous multiple embolization and resections
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Treatment Modalities • Medical • Interventional radiology – Embolization
• Laser therapy • Surgical excision
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AVM Following Multimodality Staged Therapy
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Conclusions • Head and neck vascular anomalies are often complex lesions in
functionally/cosmetically concerning areas • Multimodality therapy is often needed for disease control • Laser therapy provides disease control with low morbidity in certain
lesions • Multidisciplinary management is frequently needed