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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