Vascular Lesions of the Vascular Lesions of the Head and NeckHead and Neck

Behrooz A. Torkian MDBehrooz A. Torkian MD

July 29, 2004 July 29, 2004

Submental massSubmental mass

35yo Female with lump in left submental 35yo Female with lump in left submental area as long as she can remember.area as long as she can remember.

No pain, odynophagia, dysphagia, No pain, odynophagia, dysphagia, dysphonia. No f/c/ns/n/v.dysphonia. No f/c/ns/n/v.

Grew to noticeable size in latter stages of Grew to noticeable size in latter stages of pregnancy.pregnancy.

No PMH, no PSH, non-smoker, non-No PMH, no PSH, non-smoker, non-drinker.drinker.

CTCT

MRIMRI

AngiogramAngiogram

Evaluation of MassesEvaluation of Masses

VascularVascularInflammatory / InfectiousInflammatory / InfectiousTraumaticTraumaticAutoimmuneAutoimmuneMetabolicMetabolicIdiopathicIdiopathicNeoplasticNeoplasticCongenitalCongenitalDevelopmentalDevelopmentalEndocrineEndocrine

EvaluationEvaluation

CTCT

MRIMRI

USUS

Angio with/without embolizationAngio with/without embolization

CT AngioCT Angio

Classification of Vascular LesionsClassification of Vascular Lesions

At birthAt birth Vascular malformationsVascular malformations Lymphatic malformationsLymphatic malformations

Shortly after birthShortly after birth HemangiomasHemangiomas

Long after birthLong after birth Further classified: Benign vs. MalignantFurther classified: Benign vs. Malignant

Quick GuideQuick GuidePresent at birth

Rapid growth

Involution

No

No

No

Yes

Yes

Yes

Hemangioma

Hemangioma

Hemangioma

Vasc. Malformation

Vasc. Malformation

Vasc. Malformation

Soft and compressibleNo

Arterial Malformation, Arteriovenous Malformation, or Fistula

Lymphatic, venous, mixed malformations

Grows with infections

Lymphatic malformation

Venous Malformation

Engorges with increased venous pressure

New TerminologyNew Terminology

HemangiomaHemangioma

Most common benign Most common benign tumor of childhoodtumor of childhood

8-12% of children8-12% of children 3:1 female:male 3:1 female:male

preponderancepreponderance

Present around 3-9 Present around 3-9 months with proliferative months with proliferative phasephase

Precursor is a discoloration Precursor is a discoloration or area resembling an or area resembling an ecchymosis apparent at ecchymosis apparent at birthbirth

Rarely fully developed at Rarely fully developed at birthbirth

HemangiomaHemangioma

0.5-5 cm in size and well circumscribed0.5-5 cm in size and well circumscribed

Most are superficial – in the superficial Most are superficial – in the superficial dermisdermis

Involution phase 10% per yearInvolution phase 10% per year 50% completely involute by age 5 years50% completely involute by age 5 years 50% have completely normal skin afterwards50% have completely normal skin afterwards 10-38% have premanent changes10-38% have premanent changes

Skin atrophy, scar, puckering, fibrofatty residuumSkin atrophy, scar, puckering, fibrofatty residuum

ProliferationProliferation

InvolutionInvolution

HemangiomaHemangioma

Complications occur in Complications occur in 20%20%

Ulceration – 10%Ulceration – 10% Airway compromise – Airway compromise –

subglotticsubglottic50% also have a 50% also have a cutaneous lesioncutaneous lesion

Amblyopia or strabismusAmblyopia or strabismus Kasabach-Merritt Kasabach-Merritt

SyndromeSyndromeLarge hemangiomas, or Large hemangiomas, or diffuse hemangiomatosisdiffuse hemangiomatosisLocalized platelet trapping Localized platelet trapping causes thrombocytopenia, causes thrombocytopenia, hemorrhage, consumptive hemorrhage, consumptive coagulopathycoagulopathy

Vascular MalformationsVascular Malformations

Developmental anomaliesDevelopmental anomalies Present at birthPresent at birth No gender predilectionNo gender predilection No proliferating or involution phaseNo proliferating or involution phase

TypesTypes Lymphatic – lymphagnioma: microcystic, macrocysticLymphatic – lymphagnioma: microcystic, macrocystic VenousVenous ArterialArterial CapillaryCapillary MixedMixed

ObservationObservation

Serial PhotographySerial Photography

ReassuranceReassurance

Supportive careSupportive care

SteroidsSteroids

OralOral 2-4 mg/kg prednisone 2-4 mg/kg prednisone

– 6 weeks and taper– 6 weeks and taper Side effectsSide effects Only 30% effectiveOnly 30% effective

IntralesionalIntralesional Combination of Combination of

betamethasone and betamethasone and triamcinolonetriamcinolone

Perioccular, subglotticPerioccular, subglottic

Surgical ResectionSurgical Resection

Pulse Dye Laser 585-600nmPulse Dye Laser 585-600nm

Nd:YAG LaserNd:YAG LaserIoannis Vlachakis,1 Stefanos Gardikis, 1 Ioannis Vlachakis,1 Stefanos Gardikis, 1

Eleni Michailoudi,1 and Eleni Michailoudi,1 and Georgios Charissis1Treatment of Georgios Charissis1Treatment of hemangiomas in children using a Nd:YAG hemangiomas in children using a Nd:YAG laser in conjunction with ice cooling of the laser in conjunction with ice cooling of the epidermis: techniques and results. BMC epidermis: techniques and results. BMC Pediatrics 2003Pediatrics 2003

Capillary MalformationsCapillary Malformations

Port-wine stainsPort-wine stains 1% of newborns1% of newborns Trigeminal nerve distributionTrigeminal nerve distribution Unilateral and do not cross Unilateral and do not cross

midlinemidline Usually isolated, but may be Usually isolated, but may be

associated with other problems associated with other problems such as Sturge-Weber such as Sturge-Weber syndromesyndrome

Treatment – excision and Treatment – excision and grafting or skin expansion, grafting or skin expansion, Pulse-Dye laser.Pulse-Dye laser.

Venous MalformationsVenous Malformations

Present at birth – low flow Present at birth – low flow vascular lesionsvascular lesions

Compressible, engorge Compressible, engorge with dependence, or with dependence, or increased venous increased venous pressurepressure

Treatment includes Treatment includes combination of combination of sclerotherapy and sclerotherapy and surgery, PDL also has a surgery, PDL also has a rolerole

Arteriovenous malformationArteriovenous malformation

The thick vessels, possible smooth muscle The thick vessels, possible smooth muscle in the walls, and lack of angioblastic stroma in the walls, and lack of angioblastic stroma

Lymphatic MalformationsLymphatic Malformations

Present at birth, but may remain Present at birth, but may remain subclinicalsubclinical

Compressible and soft, Compressible and soft, classically enlarge with URI’sclassically enlarge with URI’s

Macrocystic – less infiltrating, Macrocystic – less infiltrating, more easily excisedmore easily excised

Microcystic – cystic hygroma, Microcystic – cystic hygroma, more infiltrative, difficult to more infiltrative, difficult to completely excisecompletely excise

OK-432 – Picibanil: does not OK-432 – Picibanil: does not work for microcystic work for microcystic lymphagiomas. lymphagiomas.

Microcystic LymphagniomaMicrocystic Lymphagnioma

The papillary and reticular dermis are The papillary and reticular dermis are expanded by grossly dilated lymphatic expanded by grossly dilated lymphatic channels, some of which contain blood and channels, some of which contain blood and fibrin thrombi. There is thinning of the fibrin thrombi. There is thinning of the overlying epidermis and down-growth of the overlying epidermis and down-growth of the rete ridges, which appear to enclose some rete ridges, which appear to enclose some of the lymphatic spacesof the lymphatic spaces. .

Arteriovenous MalformationsArteriovenous Malformations

High flow vascular High flow vascular lesions, present at birth, lesions, present at birth, but many are but many are unrecognized until unrecognized until adulthoodadulthoodViolaceous color, Violaceous color, palpable thrill, bruitpalpable thrill, bruitDoppler, MRI, Doppler, MRI, Angiography / Angiography / embolizationembolizationTreatments include Treatments include embolization, surgery or embolization, surgery or surgery following surgery following embolizationembolization

ReferencesReferencesCutaneous vascular anomalies. Part I. Hamartomas, malformations, and Cutaneous vascular anomalies. Part I. Hamartomas, malformations, and dilatation of preexisting vessels, Luis Requena M. Journal of the American dilatation of preexisting vessels, Luis Requena M. Journal of the American Academy of DermatologyAcademy of DermatologyVolume 37 • Number 4 • October 1997Volume 37 • Number 4 • October 1997Copyright © 1997 American Academy of Dermatology, Inc. Copyright © 1997 American Academy of Dermatology, Inc. Benign Cutaneous Vascular Tumors of Infancy: When to Worry, What to Do, Denise Benign Cutaneous Vascular Tumors of Infancy: When to Worry, What to Do, Denise W Metry MD, Adelaide A Herbert MD. Archives of Dermatology 2000; 136:905-914. W Metry MD, Adelaide A Herbert MD. Archives of Dermatology 2000; 136:905-914. Vascular Birthmarks and Other Abnormalities of Blood Vessels and Lymphatics Ilona Frieden, Odile Enjolras and Nancy EsterlyWaner M, Suen JY (1999) A classification of congenital vascular lesions. In: Waner M, Suen JY (1999) A classification of congenital vascular lesions. In: Waner M, Suen JY (eds) Hemangiomas and vascular malformations of the Waner M, Suen JY (eds) Hemangiomas and vascular malformations of the head and neck. Wiley-Liss, New York, p1-12head and neck. Wiley-Liss, New York, p1-12Waner M, Suen JY (1992) Treatment of hemangiomas of the head and Waner M, Suen JY (1992) Treatment of hemangiomas of the head and neck. Laryngoscope 102:1123-1132 neck. Laryngoscope 102:1123-1132 Davies, Derek, Rogers, Maureen, Lam, Albert & Cooke-Yarborough, ClaireDavies, Derek, Rogers, Maureen, Lam, Albert & Cooke-Yarborough, ClaireLocalized Microcystic Lymphatic Malformations Ultrasound Diagnosis.Localized Microcystic Lymphatic Malformations Ultrasound Diagnosis.Pediatric DermatologyPediatric Dermatology 1616 (6), 423-429. (6), 423-429.