Katherine A. Lane
Christopher B. Chambers

BASICS

DESCRIPTION

• Infantile hemangioma (IH) preferred term

• Benign, high flow, vascular hamartoma

• Classified as superficial or deep

• Superficial lesions present as pink, compressible, circumscribed lesions with cutaneous epithelial involvement.

• Deep lesions involve the subcutaneous tissue and orbit with little epithelial involvement. They are darker purple and can cause proptosis.

• Synonyms: Strawberry hemangioma, strawberry nevus, hemangioblastic hemangioma, benign hemangioendothelioma

Pediatric Considerations

• Concern in children for development of amblyopia secondary to strabismus, ptosis, induced astigmatism

• Evaluate for associated systemic disorders such as Kasabach–Merritt and PHACES.

Pregnancy Considerations

Some believe that capillary hemangiomas (CHs) could represent placental “metastasis.”

EPIDEMIOLOGY

Incidence

• The most common benign tumor in pediatric population affect up to 2% of all infants.

– F:M ratio 3:1–2

– Multiple gestations and preterm infants have a higher rate (25%) of cutaneous IH (1)[C].

– Incidence of periocular CH is approximately 1/10 that of systemic IH.

– 60% occur in the head/neck area and 20% have >1 IH.

– Account for 5.6% of all pediatric orbital tumors

– Haik et al. (2)[C] reported a case series of 101 periorbital CHs: 33% had isolated skin lesions, 7% had isolated orbital lesions, the remaining 60% had a combination of both superficial and deep components.

Prevalence

1.1–2.6% of all newborns

RISK FACTORS

• Prematurity

• Multiple gestation

• Advanced maternal age of greater than 30 years

• Low birthweight

• Maternal chorionic villus sampling or amniocentesis

Genetics

• Usually sporadic

• Rarely, heritable infantile CHs have been described.

GENERAL PREVENTION

There are no preventative measures.

PATHOPHYSIOLOGY

• Hamartomatous proliferations of vascular endothelial cells

• Demonstrate a distinct proliferative phase characterized by actively dividing and multiplying endothelial cells, followed by slow involution.

ETIOLOGY

• Unknown, although several theories exist:

– Placenta-to-embryo embolization

– Sequestrations of persistent omnipotent angioblasts

– Abnormal angiogenesis with imbalance angiogenesis and angiostatic factors

COMMONLY ASSOCIATED CONDITIONS

• Subglottic, paratracheal, oral, and nasal IH may coexist with orbital lesions.

– Potential for respiratory distress, asphyxiation

• Amblyopia secondary to induced astigmatism, anisometropia, strabismus, and occlusion (up to 64%)

• Strabismus is seen in 33% of patients.

• Cutaneous changes after involution: dilated capillaries, skin thinning and pigmentation, residual mass

• PHACES syndrome; acronym for Posterior fossa brain malformations, Hemangiomas, Arterial cerebrovascular anomalies, Cardiovascular anomalies, Eye anomalies, Sternal defects, or Supraumbilical abdominal raphe

– 20% of infants with large cervicofacial hemangiomas have another associated anomaly in the PHACES syndrome.

– Eye anomalies in 16% of patients with PHACES (microphthalmos, Horner syndrome, retinal vascular abnormality, optic nerve atrophy, iris hypoplasia, congenital cataracts, sclerocornea, lens coloboma, exophthalmos, strabismus, choroidal hemangioma, congenital 3rd nerve palsy, morning glory deformity, peripapillary staphyloma, and glaucoma)

• Kasabach–Merritt, that is, hemangioma thrombocytopenia syndrome

– Large tumors sequester platelets and consume coagulation factors causing thrombocytopenia

– Lethal in 10–37% of cases

– Treatment includes fresh frozen plasma transfusion, supportive care, and lesion resection

• High-output cardiac failure

– Associated with large visceral IH with abnormal arteriovenous shunts

DIAGNOSIS

HISTORY

• 2% of IHs are present at birth; the majority become evident in the first few postnatal weeks.

• Typically described by the parents as a small pink scratch that enlarges and may become a protruding mass

– This differs from a port-wine stain in Sturge–Weber syndrome, which is present at birth, occurs in the V1 distribution, and does not typically produce mass effect and does not blanch with applied pressure.

• Lesion undergoes rapid expansion after birth, continued expansion up to the first year of life and then begins to involute.

– Usually the quickly expanding lesions will involute more rapidly than its slower growing counterpart

– Most show early partial involution by 6–10 months

– Timing of involution:

30% involute by age 2 years

60% involute by age 4 years

76% involute by age 7 years

PHYSICAL EXAM

• Superficial IHs display classic strawberry red pigmentation that blanch with pressure.

• Deep hemangiomas may have a bluish hue.

• Lesions are soft and compressible.

• May expand when head is held in a dependent position, with crying, or Valsalva

• IH often present at multiple levels.

DIAGNOSTIC TESTS & INTERPRETATION

Imaging

• Gadolinium-enhanced MRI demonstrates extent of orbital lesions.

• Black serpiginous signal voids on T1- and T2-weighted images secondary to high flow rate. T1 lesion is isointense with the brain; T2 lesion is hyperintense.

Diagnostic Procedures/Other

• CT

– Diffuse homogeneous soft tissue mass with marked enhancement with contrast

• B-scan ultrasound—rarely used

– Irregular mass blending with normal orbital structures. Shows variable internal reflectivity depending on the structural composition

• Biopsy

– In cases of rapidly expanding orbital mass

– Immunohistochemical staining is positive for factor VIII.

Pathological Findings

• Nonencapsulated vascular tumor

• Benign appearing endothelial cells with limited basement membrane, assuming a lobular architecture

• High concentration of mast cells, which may be a stimulus for vessel growth

DIFFERENTIAL DIAGNOSIS

• Capillary vascular malformation (port-wine stain)

– Present at birth; flat; enlarge proportionately

• Vascular malformation

– Abnormal aggregates of capillaries, veins, arteries, and lymphatics; no proliferative phase; normal endothelial turnover rate

• Symptoms of rapid orbital growth can mimic:

– Metastatic neuroblastoma

– Rhabdomyosarcoma

– Teratoma

– Lymphangioma

– Cavernous hemangioma

• Color change and lesion enlargement with Valsalva seen in CHs can help differentiate these tumors form other lesions.

TREATMENT

MEDICATION

Treatment Indications

• Decision to treat periocular hemangiomas is directed by four factors:

– Location

– Extent

– Degree of or potential for amblyopia

– Presence of systemic hemangiomas

• Secondary indications for treatment include bleeding, obstruction of nasopharyngeal pathways, and the avoidance of possible cutaneous breakdown.

First Line

• Propranolol, oral (3,4)[C]: 2 mg/kg/day divided in 3 doses. This is a new, evolving treatment for select cases.

– Some advise starting at 0.5 mg/kg/day for 1 week, then 1 mg/kg/day for 1 week if tolerated then maximal dose at 2 to 3 mg/kg/day. If monitoring as inpatient, can increase dose 0.5 mg/kg/day daily to reach maximum dose (3)

– Some advise maintaining the dose until the tumor is resolved, then slowly taper over 3–6 months to prevent rebound.

– Possible mechanisms include vasoconstriction, decreased expression of growth factors, and triggering apoptosis of capillary endothelial cells.

– Do not use during first week of life.

– Monitor at onset of treatment of systemic hypotension, transient bradycardia, bronchospasm, and hypoglycemia.

– Higher risk of adverse events in PHACES–-cardiac evaluation with echocardiogram is recommended.

• Corticosteroids, systemic or intralesional

– 80% show 80% shrinkage in 4–6 weeks

– Systemic oral prednisolone: 1–3 mg/kg/day every morning for 4–6 weeks then taper over 2–4 weeks

Monitor every 2–4 weeks (blood pressure, evaluate for infection)

Complications include lesion rebound (common), steroid side effects (Cushing syndrome, adrenal suppression, irritability, insomnia)

Second Line

• Intralesional steroid (e.g., betamethasone 6 mg/mL and triamcinolone 40 mg/mL)

– Complications: retinal artery occlusion, skin depigmentation, fat atrophy, necrosis, hematoma, eyelid necrosis

• Topical corticosteroids (clobetasol propionate 0.05%)

• Timolol maleate 0.5%, topical ophthalmic solution (5)[C]: 2 drops onto the surface of the hemangioma with a gentle spread twice daily. Most appropriate for small hemangiomas.

– May be effective in very superficial CH

• Now rarely used historical treatments include the following:

– Interferon alpha-2b daily subcutaneous injection: inhibits angiogenesis

– External beam radiation therapy: for superficial lesions. Slows angiogenesis and induces involution. Complications include scarring, cataract formation, and tissue atrophy.

ADDITIONAL TREATMENT

General Measures

• Prospective monitoring without treatment in non–sight-threatening cases

• Spontaneous resolution occurs with time

Issues for Referral

• Ophthalmic:

– Monitoring development of visual acuity

– Amblyopia occurs in up to 60% with periorbital IH.

– Treatment often involves spectacle correction and patching of noninvolved eye.

• Dermatologic:

– Epidermal and subcutaneous hypertrophy may ulcerate, predispose to infection or scarring.

Additional Therapies

• Nd:Yag or argon laser: thermal injury to blood vessels induces involution. Argon absorbed superficially, Nd:Yag penetrates to 5–7 mm.

• May be a useful treatment of residual ecstatic vessels after involution

SURGERY/OTHER PROCEDURES

• Primary excision

– Well-circumscribed IH may be completely excised.

– Large, vision-threatening IH may be debulked.

– May be definitive early treatment and prevent astigmatism and occlusion-related amblyopia

– Intraoperative bleeding and postoperative ecchymosis are common

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

• Ophthalmology every 1–3 months if no signs of amblyopia, anisometropia, astigmatism greater than 1.5 diopters, severe proptosis with exposure keratopathy, or total pupillary occlusion

• Dermatology

Patient Monitoring

• Monitor for development of amblyopia.

• Monitor for response to treatment and treatment-related side effects.

• Monitor at home for development of occlusion, strabismus, or proptosis.

PROGNOSIS

• 70–90% show near-complete resolution.

• Cosmetic and visual recovery is excellent if treatment is instituted at an appropriate time and careful attention is paid to the visual development.

COMPLICATIONS

• Amblyopia (deprivation, anisometropic, astigmatic, or strabismic) can develop in 65%

• Strabismus in 30%

• Orbital bony distortion and/or enlargement

• Skin distention, redundancy, or ulceration leading to dermal scarring

• Residual proptosis

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