Hemangiomas and Vascular Malformations

Infantile HemangiomasMost common vascular tumor

of infancy10%

More common in CaucasiansFemalesPremature infantsPlacental abnormalities

Location >50% head and neck25% trunkRest on extremities

TimingSeveral days to weeks after

delivery

Hemangiomas

Infantile HemangiomasDescription

Reddening or bluish discoloration of skin

Bright red nodule or plaque with elevation

TypesSuperficial (epidermal)

“Strawberry” or bright red Well demarcated Elevated Soft compressible Few mm to 5cm

Deep (dermis or subq fat) Bluish hue Indistinct borders Doughy consistency Enlarge when dependent

Mixed Most hemangiomas

Hemangiomas

Infantile HemangiomasCourse

Grow and peak by 6-9 months

Stabilization Involution

10% per yearGraying out of surface

40% with residual skin changesTelangiectasiasFibro-fatty tissue

Hemangiomas

HemangiomatosisMultiple hemangiomasBenign

Limited to the skinNot benign

Numerous small (<2cm), widely dispersed cutaneous lesions

Internal or visceral lesionsLiver

May have AV shunts and precipitate high-output CHF 6-12 weeks of age

GI tract Bleeding

CNS Mass effect

Lungs

Hemangiomas

When to worry?Lower face

Lower lip, chin, preauricular, neck

“beard” distributionAirway involvement

Midline lumbosacralSpinal dysraphism

Hemangiomas

PHACES syndromePosterior fossa

malformationsHemangiomas

Plaque-like segmental hemangioma of the face

May initially be confused with port-wine stain

Often ulcerate and proliferate rapidly

Arterial anomaliesCarotid

Cardiac defectsEye anomaliesSternal clefting

Hemangiomas

ComplicationsPeriorbital and lid lesions

Occlusion of the visual axis

Corneal compressionMust be treated

aggressivelyAmblyopia, strabismus,

astigmatismLips, nose or ears

High potential for disfigurement

High friction areasUlcerationSecondary infectionScarring

Hemangiomas

TreatmentConservative management for mostLesions involving the airway or the eye

SteroidsInterferonSurgical intervention

Hemangiomas

Common benign vascular tumorsOvergrowth of granulation tissue

Following minor trauma Foreign body

Timing Well after the newborn period

Location Usually face or extremity

Description Solitary bright red, soft nodules Pedunculated 5-6mm Friable surface

Treatment Excision Electrodessication of the “feeder”

vessels May recur

Pyogenic Granuloma

Nevus Simplex AKA Salmon Patch or “Stork Bite”Capillary malformationSeen in majority of infants at birthLocation

Nape of neckGlabellaForeheadUpper eyelidsLower back

CourseFade with timeMore apparent when crying or

straining

Vascular Malformations

Nevus FlammeusAKA Port-Wine stainCongenital capillary/venous

malformationDescription

Purple-redLocation

Unilaterally on faceCourse

Do not enlarge or involute

Vascular Malformations

Sturge-Weber SyndromePort wine stain

Distribution of the trigeminal nerve

Vascular malformations of the ipsilateral leptomeninges and cerebral cortex

GlaucomaOther

Seizures, MR, hemiplegia

Klippel-Trenaunay SyndromePort wine stain

Over an extremityHemihypertrophy

Soft tissue and bony overgrowth

Vascular Malformations

A new adolescent patient is seen in your office for a sports physical. He points out a hairless, well-circumscribed, yellowish waxy plaque located on his scalp. He says its been there since birth but has recently become more raised. He wants to know what it is?

A. Epidermal neviB. Congenital

nevomelanocytic neviC. Halo nevusD. Nevus sebaceousE. Ash-leaf spot

Question 9

Nevi

Congenital Nevomelanocytic NeviDescription

Pigmented plaques often associated with dense hair growth

CourseBirth

Tan or light pink with soft vellus hairs

Infancy and childhoodDarkening with small dark

macules or nodules within the plaque and prominent hair

Nevi

Congenital Nevomelanocytic NeviSize

Small <1.5cmMedium 1.5-20cmLarge or Giant >20cm

PrognosisAll have potential for

malignant transformationNew, darker and/or bleeding

nodulesSudden growth1-4% small to medium10-30% Giant

Nevi

Congenital Nevomelanocytic NeviManagement

Small to mediumYearly follow-up with dermExcision if atypical or difficult to monitor

GiantEarly, full thickness excision followed by grafting

ORClose observation every 6 months

Nevi

Acquired Nevomelanocytic NeviTiming

Early childhoodDescription

Small, flat, pigmented macules1-2mm

LocationSun-exposed areas

CourseJunctional nevi

Limited to epidermal-dermal junctionCompound nevi

Papular or pedunculated Proliferation into the dermis

Change slowly over months and only warrant observation

Nevi

Acquired Nevomelanocytic NeviHalo nevus

Hypopigmented or depigmented ring associated with mild local pruritus around a benign nevus

Caused by cytotoxic T-lymphocyte reaction

CourseEventual resolution and

nevus disappears

Nevi

ChildhoodDe novoWithin giant congenital nevus or

other nevusTransplacental transfer

Red FlagsChange in size, shape or outline

Scalloped, irregular bordersChange in surface characteristics

Small, dark, elevated papule or nodule within a flat plaque

Flaking, scaling, ulceration or bleeding

Change in colorDifferent shade or to a mixture of

red, white or blueDevelopment of burning, itching

or tenderness

Melanomas

Blue nevusSmall, firm, blue papuleDeep nevus cells

Traumatic hemorrhageUnder the nails or in mucous

membranesVascular lesions

Pyogenic granuloma or angiokeratoma

Spitz nevusRed and rapidly growing nevusComposed of spindle and

epithelial cellsConfused histologically with

melanoma

Melanoma Differential

Epidermal NeviEpidermal structures onlyTiming

Birth or childhoodDescription

Slightly hyperpigmented papillomatous or verrucous growth

Increases in verrucous changes are common at puberty

Small and localized, linear, dermatomal or generalized

Other associations if extensiveSeizures, MR, ocular and

skeletal defects, hypophosphatemic vitamin D-resistant rickets

Hamartomatous Nevi

Nevus sebaceous of Jadassohn Epidermal proliferation, abortive hair

follicles, sebaceous glands and apocrine structures

TimingPresent at birthMore verrucous, raised and nodular at

puberty Description

Hairless, well-circumscribed, skin-colored or yellowish waxy plaque

Linear or round Location

Scalp, face or neck Course

Routine excision not recommended in childhood Malignancy risk is low

May excise in adolescence or adulthood

Hamartomatous Nevi