Hemangiomas

Management Strategy

Cutaneous hemangiomas appear in the first few weeks of life and reach 80% of size by 3 months, with spontaneous involution in most cases by age 10. Early evaluation and treatment are critical to limit the patient’s functional impairment and disfigurement. About half of children with hemangiomas have normal skin after involution, but the rest may have residual changes, including telangiectasias, atrophy, fibrofatty residuum, and scarring.

Differentiating benign common hemangiomas from other vascular anomalies is essential, as the pathophysiology, treatment modalities, and prognoses are significantly different. Vascular malformations and tumors, such as the kaposiform hemangioendothelioma (KE) and tufted angioma (TA), differ from hemangiomas in both clinical and histologic appearance as well as growth rate and involutional tendencies. In particular, KE and TA are associated with Kasabach–Merritt syndrome and the accompanying coagulopathy, whereas common hemangiomas are not. The majority of common hemangiomas occur as solitary lesions but can be a sign of multisystem involvement such as diffuse neonatal hemangiomatosis, LUMBAR syndrome ( l ower body congenital infantile hemangiomas (IHs) and other skin defects, u rogenital anomalies and ulceration, m yelopathy, b ony deformities, a norectal malformations and a rterial anomalies, and r ectal anomalies), and PHACES syndrome ( p osterior fossa malformation, large segmental h emangiomas, a rterial lesions, c ardiac anomalies, e ye abnormalities, and s ternal cleft/ s upraumbilical raphe).

Although the natural course of hemangiomas is self-limited, pharmaceutical intervention is indicated for most hemangiomas due to the potential for rapid growth and potential ulceration and scarring, disfigurement or functional impairment (e.g., compress or obstruct vital structures such as the larynx, eyes, ears, and nose). IHs associated with structural anomalies (e.g., in PHACES or LUMBAR syndromes) also warrant therapy. After identifying an IH, the clinician should facilitate evaluation by a hemangioma specialist within weeks 3–8 of the newborn’s life as the most rapid growth of superficial hemangiomas typically occurs between 5.5 and 7.5 weeks of age (termed the proliferative phase).

Topical and systemic β-blockers have become the preferred first-line therapy in very early or small hemangiomas. Oral propranolol hydrochloride solution was approved by the US Food and Drug Administration (FDA) in 2014 for use in infants with an escalating starting dose of 1.2 mg/kg/day to a target dose of 3.4 mg/kg/day divided into two daily doses. Treatment typically is continued for at least 6 months and often is maintained until 12 months of age or longer. Adverse events are rare but may include the masking of hypoglycemia, hypotension, and bradycardia. Administering the medication after feeding and monitoring the heart rate and blood pressure after dose increases can help reduce these seldom-encountered side effects. Bronchial hyperreactivity is another rare but possible side effect, especially in susceptible individuals. Other β-blockers, such as atenolol and nadolol, have been shown to be effective for the treatment of IHs in small clinical trials, and are probably associated with fewer side effects in comparison to propranolol. Timolol, available as a solution or a gel, has been shown in small randomized clinical trials to be safe and effective for superficial, small, thin non-ulcerated IHs with twice-daily topical application.

Surgical excision, either alone or in combination with corticosteroids or β-blockers, may also be employed in certain cases such as large IHs located periorbitally or on the nasal tip, or for the treatment of residual skin changes after involution. Cyclophosphamide, vincristine, bleomycin, and interferon are considered obsolete therapies since the advent of propranolol.

The diagnosis of a hemangioma is made clinically but the following clinical scenarios warrant imaging: IHs ≥5 cm on the face may need to be imaged with contrast-enhanced MRI and with MR angiography of the head and neck to rule out PHACES syndrome. IHs ≥5 cm on the midline back and perineal region or with segmental morphology in children ≤3 months of age should be imaged with ultrasound of the spine and with color Doppler ultrasound of the abdomen and pelvis to rule out LUMBAR syndrome. Atypical lesions appearing after 6 months of age and ≥5 cutaneous IHs also warrant further imaging. IHs that are segmental, especially if multiple, may raise suspicion for the presence of visceral hemangiomatosis. There is no defined approach for follow-up imaging intervals for superficial IHs; however, liver and visceral involvement might be followed with MR or ultrasound every 2–3 months to assess response to therapy.