Pharmacotherapy

Treatment for Endangering Complications

Treatment for Ulceration

Local wound treatment is indicated for the 5% of cutaneous hemangiomas that cause skin ulceration. Ulceration is more common on the lips, anogenital region, and parotid region.

Local treatment includes application of hydrated petrolatum and viscous lidocaine, topical antibiotic, or hydrocolloid dressing. Eschars should be debrided and treated with wet-to-dry dressing changes.

Flashlamp pulsed-dye laser may aid healing when two applications are given 4 to 6 weeks apart.

Resection of the ulcerated tumor may be performed when the defect can be closed primarily, and the resulting scar would not be worse than if the lesion were removed during involution. Excision is most commonly performed for small, well-localized lesions on the scalp, chest, or extremity, but not for facial lesions.

10% of hemangiomas cause significant deformity or severe complications. Most complications occur with hemangiomas located in the head and neck. Sublottic hemangioma may obstruct the airway, whereas ulcerated lesions may destroy the eyelid, ear, nose, or lip. Periorbital hemangioma can block the visual axis or distort the cornea causing amblyopia. Gastrointestinal hemangioma may cause bleeding that requires transfusions.

Large hemangiomas, most commonly involving the liver, can cause high-output congestive heart failure or hypothyroidism. Hypothyroidism results from the expression of a deiodinase by the hemangioma that cleaves iodine from thyroid hormone. Consequently, thyroid function should be evaluated in patients with a large hemangioma. Massive intravenous thyroid replacement may be necessary to avoid mental retardation until the hemangioma regresses.

u Systemic pharmacologic intervention may be necessary for endangering, ulcerating, problematic, or life-threatening IHs. Corticosteroids inhibit the vasculogenic potential of hemangioma-derived stem cells, as well as the expression of vascular endothelial growth factor. Prednisone or prednisolone is administered in the morning at 2 to 3 mg/kg/day for 2 weeks. Initial improvement in color and tension is usually evident in the first 1 to 2 weeks. If the IH stabilizes, the dosage is tapered every 2 to 4 weeks with a goal of discontinuation by 10 to 11 months of age. The overall response rate is 80% to 90%. In some cases the tumor may exhibit rebound growth as the corticosteroid is tapered. A return to the initial dose and slower taper will usually suffice. Possible side effects of corticosteroid include Cushingoid facies, irritability, GI reflux, a slowing in the rate of height and weight gain, steroid-induced cardiomyopathy, and hypertension. Nearly all children (88%) return to their pretreatment curves for height and weight within 24 months. Live vaccines (e.g., polio, measles, mumps, rubella, varicella) should not be administered during corticosteroid treatment.

u Intralesional injection of corticosteroid for well-localized tumors of the nasal tip, cheek, lip, or eyelid is used to minimize deformity. There are reports of retinal artery occlusion and eyelid necrosis, presumably from embolization of colloidal particles. Compression at the periphery of the lesion will help minimize this risk. Triamcinolone (25 mg/mL) is injected slowly at a low pressure with a 3-mL syringe and 25-gauge needle. The dosage is 3 to 5 mg/kg per injection. Additional injections can be done at 6- to 8-week intervals; usually, three to five are necessary. Response rates are similar to that of oral corticosteroid.

u Propranolol, a nonselective beta blocker, is being used with increasing frequency for the treatment of IHs. Early reports suggest that propranolol may be as efficacious as corticosteroids for the treatment of problematic IHs. The mechanism of action for propranolol is unknown; theories include vasoconstriction of the tumor vasculature or downregulation of angiogenic proteins. An ongoing, prospective, randomized controlled trial will help elucidate propranolol's safety, efficacy, and tolerability for the treatment of IHs.

u Recombinant interferon-alfa has fallen out of favor as second-line therapy due to the risk of spastic diplegia, which occurs in 5% to 20% of treated infants, particularly those younger than 6 months of age. More suitable second-line agents are chemotherapeutic drugs with antiangiogenic properties such as vincristine. The drugs are administered in a low-dose, high-frequency metronomic regimen.