Medical Medical Management of Management of HaemangiomasHaemangiomas

Dr Anne HalbertDr Anne Halbert

Department of DermatologyDepartment of Dermatology

Princess Margaret HospitalPrincess Margaret Hospital

HaemangiomaHaemangioma

The most common The most common benign proliferative benign proliferative tumour of infancytumour of infancy

One or more lesions One or more lesions can be found in 10-can be found in 10-12% of infants aged 12% of infants aged 12 months12 months

The vast majority The vast majority require no treatmentrequire no treatment

Potential Complications Potential Complications

UlcerationUlceration The most common The most common

complication (15%)complication (15%) Particularly Particularly

prevalent in the prevalent in the nappy area and on nappy area and on the lipthe lip

PainfulPainful Inevitably heal Inevitably heal

with scarringwith scarring

Ulcerated HaemangiomaUlcerated Haemangioma

Complications of Complications of HaemangiomaHaemangioma

Functional obstructionFunctional obstruction EyeEye

Astigmatic and refractive errorsAstigmatic and refractive errors Amblyopia and blindnessAmblyopia and blindness

NoseNose AirwayAirway

Visual ObstructionVisual Obstruction

Visual ObstructionVisual Obstruction

Airway CompromiseAirway Compromise

Nasal distortionNasal distortion

Airway CompromiseAirway Compromise

Systemic InvolvementSystemic Involvement

Disseminated neonatal Disseminated neonatal haemangiomatosishaemangiomatosis

DNHDNH

DNHDNH

haemangiomas

Thalamic lesion

DNHDNH

Very high mortalityVery high mortality Liver is the most commonly affected Liver is the most commonly affected

organ organ Risk of high output congestive cardiac Risk of high output congestive cardiac

failurefailure Babies with numerous miliary Babies with numerous miliary

haemangiomas need to be screened haemangiomas need to be screened early and often for the development of early and often for the development of visceral lesionsvisceral lesions

Systemic InvolvementSystemic Involvement

Contiguous Contiguous ExtensionExtension

Contiguous ExtensionContiguous Extension

haemangioma

aorta

Spinal cord haemangioma

PHACE SyndromePHACE Syndrome

PP posterior fossa posterior fossa abnormalitiesabnormalities

H H haemangiomahaemangioma

AA arterial arterial abnormalitiesabnormalities

C C cardiac defectscardiac defects

EE eye abnormalities eye abnormalities

Kasabach Merritt Kasabach Merritt SyndromeSyndrome

Usually a rapidly proliferating Usually a rapidly proliferating haemangioendotheliomahaemangioendothelioma

Platelet consumption early in life Platelet consumption early in life Develop disseminated intravascular Develop disseminated intravascular

coagulationcoagulation High mortality rateHigh mortality rate Beware a bruised appearanceBeware a bruised appearance

Kasabach Merritt Kasabach Merritt SyndromeSyndrome

Potentially Permanently Potentially Permanently Disfiguring HaemangiomasDisfiguring Haemangiomas

Large facial haemangiomas which Large facial haemangiomas which may involute leaving altered skin may involute leaving altered skin texture and fibrofatty residuumtexture and fibrofatty residuum

Haemangiomas distorting cartilage Haemangiomas distorting cartilage of nose or earof nose or ear

Post InvolutionPost Involution

TreatmentsTreatments

Pulsed Dye LaserPulsed Dye Laser Treatment of choice for ulcerated Treatment of choice for ulcerated

haemangiomashaemangiomas May help switch off proliferative May help switch off proliferative

phase in very superficial lesionsphase in very superficial lesions Useful after involution, to clear away Useful after involution, to clear away

residual telangiectasiaresidual telangiectasia

TreatmentsTreatments

CorticosteroidsCorticosteroids Potent topical steroidsPotent topical steroids Intralesional steroidsIntralesional steroids

Useful for localized facial lesionsUseful for localized facial lesions 20-40 mg/ml triamcinolone or Celestone 20-40 mg/ml triamcinolone or Celestone

Chronodose repeated 6-8 weeklyChronodose repeated 6-8 weekly Technically difficult – risk of ulcerationTechnically difficult – risk of ulceration Avoid around the eye (central retinal Avoid around the eye (central retinal

artery occlusion)artery occlusion)

TreatmentsTreatments

Systemic CorticosteroidsSystemic Corticosteroids First line treatment for the First line treatment for the

prevention of functional obstruction, prevention of functional obstruction, visceral haemangiomatosis and K-M visceral haemangiomatosis and K-M syndromesyndrome

2 mg/kg/d as a single morning dose2 mg/kg/d as a single morning dose Usually well toleratedUsually well tolerated Treatment lasts 8-12 weeksTreatment lasts 8-12 weeks

Pre-systemic steroids

After 2 wks of steroids

Systemic CorticosteroidsSystemic Corticosteroids

Adverse EffectsAdverse Effects Initial irritability in 75%Initial irritability in 75% RefluxReflux Temporary reduction in growth (no Temporary reduction in growth (no

permanent effect)permanent effect) HPA axis suppressionHPA axis suppression Delay vaccinationsDelay vaccinations

Systemic TreatmentsSystemic Treatments

Interferon AlphaInterferon Alpha Used in conjunction with systemic Used in conjunction with systemic

steroids for life threatening steroids for life threatening complicationscomplications

1 million units/m1 million units/m2 2 /day SC initially/day SC initially Anti-angiogenesis; also speeds Anti-angiogenesis; also speeds

involutioninvolution Adverse effects include neutropenia, Adverse effects include neutropenia,

abnormal LFTs and abnormal LFTs and spastic diplegiaspastic diplegia

Systemic TreatmentsSystemic Treatments

VincristineVincristine

CyclophosphamideCyclophosphamide

Thank youThank you