What is the approach to managing hemangiomas?

Management of Infantile Hemangiomas

All infantile hemangiomas (IHs) must be risk-stratified immediately upon identification, with high-risk lesions requiring specialist evaluation as soon as possible—ideally by 1 month of age—because the window for optimal intervention closes rapidly as most growth occurs between 1-3 months and is complete by 5 months. 1

Risk Stratification: The Critical First Step

Classify an IH as high-risk if it meets any of these criteria: 1

Life-Threatening IHs

• "Beard-area" distribution (mandibular and lower face): High risk for airway hemangiomas causing obstruction 1
• ≥5 cutaneous hemangiomas: Screen for hepatic involvement with ultrasound; risk of cardiac failure and hypothyroidism 1, 2
• Hepatic hemangiomas: Can cause high-output cardiac failure 1

Functional Impairment

• Periocular IH >1 cm: Risk of astigmatism, anisometropia, proptosis, amblyopia 1, 2
• Lip or oral cavity involvement: Feeding impairment 1

High Ulceration Risk

• Segmental IHs involving: lips, columella, superior helix of ear, gluteal cleft, perineum, perianal skin, or other intertriginous areas (neck, axillae, inguinal region) 1

Associated Structural Anomalies

• Segmental facial or scalp IH: PHACE syndrome (posterior fossa defects, cerebrovascular arterial anomalies, cardiovascular anomalies including coarctation of the aorta, eye anomalies) 1
• Segmental lumbosacral/perineal IH: LUMBAR syndrome (lower body IH with cutaneous defects, urogenital anomalies, myelopathy, bony deformities, anorectal malformations, arterial and renal anomalies) 1

Disfigurement Risk

• Facial IH: Nasal tip or lip (any size), or any facial location ≥2 cm (>1 cm if ≤3 months of age) 1
• Scalp IH >2 cm: Risk of permanent alopecia, especially if thick/bulky; profuse bleeding if ulceration develops 1
• Neck, trunk, or extremity IH >2 cm: Especially during growth phase or with abrupt transition from normal to affected skin ("ledge effect"); thick superficial IH (≥2 mm thickness) 1
• Breast IH in female infants: Permanent changes in breast development or nipple contour 1

Immediate Actions for High-Risk IHs

Facilitate specialist evaluation as soon as possible—this is a strong recommendation. 1 The optimal referral time is 1 month of age, far earlier than traditional practice, because rapid growth accelerates between 5-7 weeks of age. 1 If in-person specialist access is limited, photographic triage or telemedicine consultation is acceptable. 1, 3

Imaging: When and What

Do not perform imaging unless: 1

• Diagnosis of IH is uncertain
• ≥5 cutaneous IHs present (screen for hepatic involvement)
• Associated anatomic abnormalities suspected (PHACE or LUMBAR syndrome)

When imaging is indicated, use ultrasound with Doppler as the initial modality—no sedation required, no radiation exposure. 1, 2 Reserve MRI with contrast for deep facial structures, periorbital/intraorbital extent, or lumbosacral lesions with potential spinal involvement. 2

Treatment Algorithm

Low-Risk IHs: Observation

For small, superficial IHs in non-critical locations that are unlikely to cause disfigurement, observation is appropriate. 1 Monitor periodically to assess growth and potential complications. 2, 3 Educate parents that 90% of IHs involute spontaneously by age 4 years, though residual changes (telangiectasias, redundant skin, scarring) may remain. 2, 3

High-Risk IHs: Active Treatment

First-Line: Oral Propranolol

Propranolol is the drug of choice at 2-3 mg/kg/day divided into three doses. 1, 2 This represents a strong consensus from the American Academy of Pediatrics. 1, 2

Initiation protocol: 2

• Start in a clinical setting with cardiovascular monitoring every hour for the first 2 hours
• Initiate as inpatient if: infant <8 weeks chronological age, postconceptional age <48 weeks, or presence of cardiovascular risk factors
• Continue treatment for at least 6 months, often until 12 months of age (occasionally longer) 1

Efficacy: Rapid reduction in hemangioma size with progressive improvement over at least 3 months; failure rate approximately 1.6%. 2

Second-Line: Systemic Corticosteroids

Use when propranolol cannot be used or is ineffective. 2 Prednisolone or prednisone 2-3 mg/kg/day as a single morning dose, frequently for several months. 2 More effective when started during the proliferative phase. 2

Topical Timolol

May be used for small, thin, superficial IHs. 1, 4 This is particularly useful for uncomplicated superficial lesions or when systemic propranolol carries unacceptable risk. 4

Surgical Management

Generally delay surgical resection until after infancy to allow natural involution. 2, 3 Optimal timing is before age 4 years, as most hemangiomas do not improve significantly after this age. 2 Surgery in infancy carries higher risk of anesthetic morbidity, blood loss, and iatrogenic injury. 2

Consider early surgery only for: 2

• Early hemangioma in a focal location where the surgical scar would be the same if removed after involution
• Specific anatomic situations where waiting is not feasible

Laser Therapy

Pulsed-dye laser (PDL) is the laser of choice for superficial hemangiomas. 2 Nd:YAG laser is preferred for hemangiomas with subcutaneous components. 2 Laser therapy is most useful for treating residual skin changes after involution. 1

Location-Specific Management

Periocular Hemangiomas

Require early evaluation by pediatric ophthalmologist to prevent astigmatism, strabismus, or amblyopia. 2 Propranolol is strongly preferred over intralesional steroids due to risk of retinal artery embolization with the latter. 2

Hepatic Hemangiomas

• Small to medium (<5 cm): Conservative management with observation 2
• Large (>5 cm): Increased monitoring; rupture risk approximately 3.2%, increasing to 5% for lesions >10 cm 2
• Screen infants with ≥5 cutaneous hemangiomas for hepatic lesions with ultrasound 2
• Monitor thyroid function in multifocal or diffuse hemangiomas, as the tumor may inactivate thyroid hormone 2

Vertebral Hemangiomas

Critical distinction: These are NOT infantile hemangiomas. 5 Asymptomatic vertebral hemangiomas discovered incidentally do not require treatment or routine surveillance. 2, 5 They do not involute spontaneously, and propranolol has no role in their management. 5

Common Pitfalls to Avoid

The "wait and see" myth: The outdated belief that all IHs are benign and will disappear without consequence leads to missed treatment windows. 1 By the time damage to dermis, subcutaneous tissues, or anatomic landmarks occurs, it may be permanent. 1

Delayed referral: Waiting until 3-6 months of age is too late for many high-risk IHs, as most significant growth occurs between 1-3 months. 1, 6

Misdiagnosis: Distinguish IHs from congenital hemangiomas (RICH/NICH), vascular malformations, and other entities like kaposiform hemangioendothelioma (which causes Kasabach-Merritt phenomenon, not IHs). 3, 7

Underestimating psychological impact: Visible facial hemangiomas carry significant psychosocial burden; potential for permanent disfigurement is a valid indication for treatment. 8, 4

Assuming all hemangiomas behave identically: Segmental hemangiomas confer higher risk of morbidity and life-threatening complications than localized lesions. 1 IH-MAGs (infantile hemangiomas with minimal or arrested growth) may lack robust proliferation but still cause complications like ulceration or be associated with structural anomalies. 1