Infantile Hemangioma

IHs are not present at birth.
They present in the first few weeks of life with a precursor lesion that can appear as a bluish bruise-like patch, telangiectasias with a rim of pallor, or a red flat stain.
IHs have a characteristic natural history consisting of three phases:
1. The rapid proliferation phase: 3 weeks until 6 to 7 months of age; IHs experience rapid volumetric growth and appear red, firm and have a rubbery texture (Fig. 1.13).
2. The plateau (or late proliferative) phase: variable length, usually 7 to 12 months of age; IHs have a slower rate of growth and color changes to a dull red to gray and begins to break apart and lesions feel soft or spongy.
3. The involution phase: usually starts at 1 year of age and is a gradual process that can last for years; the color continues to fade, some lesions involute completely while others leave fibrofatty residua (Fig. 1.14).
IH can be superficial (Fig. 1.15), deep or mixed (superficial and deep) (Fig. 1.16).
Lesions with a deep component will grow for about month longer than superficial IH.
More recent studies show that most IH growth is complete by 5 months of age and that involution ends at median age 3 years.
Ulceration is the most common complication and is heralded by a gray-white color during the proliferative phase (Fig. 1.17).
IHs in certain locations and of certain morphologies are at higher risk for development of complications and/or associated systemic involvement (see Table 1.2, for a list of high-risk IHs and intervention required). For example, IHs in the perineum are at highest risk for early ulceration, IHs in the central face can lead to permanent cosmetic disfigurement, and large segmental IHs (configuration corresponding to a recognizable and/or significant portion of a developmental segment) on the face should prompt workup for PHACES syndrome.
Early recognition of high-risk IH will allow for more timely intervention.

Diagnosis is clinically based on the typical morphology and characteristic growth pattern.

Differential Diagnosis

Congenital Hemangiomas

Rapidly involuting congenital hemangioma (RICH)—present at birth, undergoes rapid spontaneous involution in the first year of life. Negative for GLUT-1.
Noninvoluting congenital hemangioma (NICH)—present at birth, remains stable in size, does not involute. Negative for GLUT-1.

Vascular Malformations (Capillary, Mixed Capillary-Venous or Venous-Arterial)

Vascular malformations are present at birth and grow proportionately with child over time.

Management

The vast majority of IHs will proliferate and involute with minimal consequence. In these cases, active nonintervention is the treatment of choice and consists of education on the natural history of IH, close monitoring (with or without the use of photography), and reassurance.
It is important to recognize IHs that are at high risk for complications (i.e., ulceration, cosmetic disfigurement, and interference with vital functions) or associated anomalies and intervene quickly with treatment (see below) and proper workup (see Table 1.2).
For IH requiring treatment, the options include topical, local, or systemic therapy.

Topical Treatments

Timolol 0.5% gel-forming solution is a nonselective beta-blocker that is highly effective for ulcerated and superficial hemangiomas.
Timolol 0.5% gel-forming solution is dosed as one drop twice daily on the IH for at least 3 months for best response.
Imiquimod 5% cream has shown some efficacy in treatment of small superficial IH.
PDL therapy (585 to 595 nm) is useful for early superficial lesions, ulcerating lesions (accelerates healing and decreases pain), and the residual erythema and telangiectasias left behind once IH has involuted.
Fractionated CO₂ or Fraxel Lasers—useful for treatment of the residual scarring and telangiectasias.
Mid to superpotent topical corticosteroids (triamcinolone 0.1% ointment or clobetasol 0.05% ointment) applied twice daily to IH can also halt growth and hasten involution.

Intralesional Therapy

Intralesional triamcinolone (Kenalog), 5 to 10 mg/cc, can stabilize growth and decrease the size of IH.
This treatment is most useful for early localized IH on the lip and nasal tip.
Injections should be initiated early and repeated every 3 to 4 weeks for maximal benefit.

Systemic Treatments

Systemic therapy is reserved for larger IH with more aggressive growth characteristics that pose a high threat to a vital function, for cosmetic disfigurement, or for those IH not responding to local therapy.

Propranolol

The nonselective beta-blocker, propranolol, has become the first-line treatment for IH requiring systemic therapy, given its greater efficacy and better side effect profile compared to systemic corticosteroids.
Consensus guidelines on the use of propranolol for IH recommend a baseline cardiopulmonary evaluation prior to initiating propranolol and inpatient initiation if patient is <8 weeks old, or has inadequate social support or comorbidities.
Hemangeol (propranolol hydrochloride 4.28 mg/mL) is a new infant friendly, paraben- and alcohol-free formulation of propranolol that is FDA approved and indicated for the treatment of IHs.

Dosing

Optimal dosing is based on weight with goal therapeutic dose of 2 to 3 mg/kg/day divided and given three times daily with a minimum of 6 hours between doses.
Multiple dosing regimens are used; one commonly used method is:
- Start at 0.33 mg/kg/dose, three times per day, for 3 to 7 days then,
- Increase to 0.5 mg/kg/dose, three times per day, for 3 to 7 days then,
- Increase to goal of 0.66 mg/kg/dose three times per day.
Recommended dose of hemangeol based on the results of a large multicenter randomized trial is 3.4 mg/kg/day divided twice daily for 6 months.
HR and BP should be checked between 1 and 3 hours after initial dose, and after the first dose of a dose increase of >0.5 mg/kg/day.
Propranolol is usually continued through the growth phase of the IH and then tapered off.

Side Effects

Sleep disturbance, intermittent acrocyanosis, gastrointestinal symptoms, and respiratory symptoms.
Serious side effects are rare and include hypotension, bradycardia, and hypoglycemia.

Propranolol should be given during the day with a feeding shortly after administration.
Parents should be educated on the symptoms of hypoglycemia and instructed to ensure regular feeding times, avoid prolonged fasts, and discontinue propranolol during times of decreased oral intake.

Atenolol

Atenolol, a selective 1 receptor antagonist, has demonstrated similar efficacy for problematic infantile hemangiomas.
Commonly used dose is 1 mg/kg/day.

Systemic Corticosteroids

Oral corticosteroids (prednisone or prednisolone) are used to treat IH requiring systemic treatment when there is a contraindication to the use of propranolol.

Dosing

Range is 2 to 4 mg/kg/day divided twice daily.
Treatment is continued until IH growth has stopped and then tapered slowly to avoid rebound growth and adrenal suppression.

Side Effects

Weight gain, increased irritability, stomach upset, hypertension, and immunosuppression.
Interferon 2a or 2b and Vincristine are systemic treatments occasionally used in the treatment of refractory IH requiring treatment.

Treatments for Ulceration

Topical timolol 0.5% gel-forming solution, one drop twice daily, can hasten healing.
Local wound care with topical antibiotic ointments and nonstick dressings, or becaplermin gel.
Pain management with acetaminophen or topical lidocaine gel or cream.
PDL therapy has been shown to accelerate healing and decrease pain.

Surgery

Excisional surgery is sometimes necessary in cases of airway hemangiomas and certain facial lesions.

Points to Remember

Most IHs require no treatment; active nonintervention is all that is needed.
Recognize high-risk hemangiomas and treat early.
Consider a cardiac evaluation in patients who are using topical timolol for ulcerated IH.

Helpful Hints

IH may resemble PWSs early on, but close monitoring in the first month of life will demonstrate the characteristic growth pattern of an IH.
Propranolol is now considered first line for IH that require systemic treatment.
Prior to propranolol initiation for IH, patients should have a baseline cardiopulmonary evaluation.

section name header

Basics ⬇

Pathogenesis ⬆ ⬇

Clinical Manifestations ⬆ ⬇

Diagnosis ⬆