What are the treatment options for patients with hemangiomas?

Hemangiomas: Patient Information and Treatment Options

What Are Hemangiomas?

Hemangiomas are benign vascular tumors that are the most common type of birthmark, with infantile hemangiomas (IHs) appearing in early infancy and most (90%) involuting spontaneously by age 4 years without requiring any treatment. 1, 2

• Infantile hemangiomas typically appear within the first few weeks of life, grow rapidly between 5-7 weeks of age, and then slowly shrink over several years 3
• Congenital hemangiomas are present at birth and include rapidly involuting (RICH) and non-involuting (NICH) types 2
• Hepatic (liver) hemangiomas are the most common benign liver tumors, usually found incidentally in adults, and behave differently from infantile skin hemangiomas 3, 4
• Vertebral hemangiomas are distinct vascular lesions in the spine that do not involute spontaneously and are managed completely differently from infantile hemangiomas 5

Natural Course and What to Expect

• After the initial growth phase, hemangiomas gradually change color from bright red to milky-white or gray, flattening and shrinking from the center outward 2
• Even after complete involution, residual changes may remain including telangiectasias (small blood vessels), redundant skin, or scarring in 55-69% of untreated cases 3, 2
• The growth pattern is unpredictable—even on the same patient, one hemangioma may become large and problematic while others barely grow 3

When Treatment Is Necessary

High-Risk Hemangiomas Requiring Immediate Specialist Referral

If your child has a high-risk hemangioma, evaluation by a hemangioma specialist should occur as soon as possible, ideally by 1 month of age, to prevent permanent complications. 3, 1

High-risk features include:

• Life-threatening complications: Heart failure or respiratory difficulty 1
• Functional impairment: Visual obstruction, feeding problems, ptosis, amblyopia, or astigmatism 1
• Periocular (around the eye) location: Can deform the cornea and obstruct vision, requiring early pediatric ophthalmology evaluation 3, 1
• "Beard distribution" on lower face and neck: Higher risk of airway involvement with biphasic stridor and barky cough 3
• Large facial hemangiomas (>4 cm) or those at risk of permanent disfigurement 1, 2
• Segmental facial or scalp hemangiomas: Associated with structural anomalies 1, 2
• Segmental lumbosacral or perineal hemangiomas: Higher risk of ulceration and may be associated with spinal cord abnormalities 3, 1
• Ulceration: A common complication causing pain and bleeding 3, 1

Low-Risk Hemangiomas

• Small lesions on the torso are less likely to be disfiguring and typically do not require active intervention 3
• These should be monitored periodically to assess for unexpected growth or complications 1, 2

Treatment Options

First-Line Medical Treatment: Oral Propranolol

Propranolol (a beta-blocker medication) at 2 mg/kg/day divided into three doses is the first-line treatment for infantile hemangiomas requiring intervention, replacing corticosteroids as the previous gold standard. 3, 1

• Must be started in a clinical setting with cardiovascular monitoring every hour for the first 2 hours 1
• Infants under 8 weeks of age, postconceptional age under 48 weeks, or those with risk factors should be started as inpatients 1
• Results in rapid reduction in hemangioma size with progressive improvement over at least 3 months 1
• Failure rate is only approximately 1.6% 1

Alternative Medical Treatment: Corticosteroids

• Systemic corticosteroids (prednisolone or prednisone 2-3 mg/kg/day as a single morning dose) are an alternative when propranolol cannot be used or is ineffective 1
• More effective when started during the proliferative phase 1
• Treatment frequently requires several months 1

Laser Therapy

Laser treatment may be useful for early non-proliferating superficial lesions, controlling ulceration, and treating persistent telangiectasias after involution. 3

• Pulsed dye laser (PDL) is the laser of choice for superficial hemangiomas 1
• Nd:YAG laser is preferred for hemangiomas with deeper subcutaneous components 1

Surgical Treatment

Surgical resection should generally be delayed until after infancy to allow for natural involution and better outcomes, with the optimal timing before age 4 years. 1

• Surgery in infancy carries higher risk of anesthetic morbidity, blood loss, and iatrogenic injury 1
• Most hemangiomas do not improve significantly after age 4 years 1
• For facial lesions, circular excision with purse-string closure minimizes scar length and distortion of surrounding structures 3
• Lip hemangiomas may require wedge excision for bulkier lesions that cross the vermilion-cutaneous border 3

Special Locations and Considerations

Periocular (Eye Area) Hemangiomas

• Require early evaluation by a pediatric ophthalmologist 3, 1
• Propranolol is strongly preferred over intralesional steroids due to the risk of retinal artery embolization causing blindness 3, 1
• Topical beta-blockers may be useful for intraocular hemangiomas 3

Airway Hemangiomas

• Most commonly subglottic, presenting with biphasic stridor and barky cough often mistaken for croup 3
• Operative endoscopy is generally necessary to identify and assess extent 3
• Treated with propranolol, but patients who do not respond rapidly may require dilation, intralesional corticosteroid injection, or partial resection 3

Hepatic (Liver) Hemangiomas

Small to medium-sized hepatic hemangiomas (<5 cm) can be managed conservatively with observation, while large hemangiomas (>5 cm) require increased monitoring due to higher complication risk. 1, 4, 6

• Infants with cutaneous hemangiomas should be screened for hepatic lesions with ultrasonography 3, 1
• Risk of rupture is approximately 3.2%, increasing to 5% for lesions >10 cm 1, 4
• Surgical resection (preferably enucleation) is reserved for incapacitating pain, diagnostic uncertainty, or compression of adjacent organs 4, 6, 7
• Mortality from spontaneous or traumatic rupture is high (36-39%) 6

Vertebral (Spine) Hemangiomas

Asymptomatic vertebral hemangiomas discovered incidentally do not require treatment or routine surveillance. 1, 5

• These are distinct from infantile cutaneous hemangiomas: they do not involute spontaneously 1, 5
• Propranolol has no role in vertebral hemangioma management 1, 5
• MRI with contrast is indicated only if symptoms of spinal cord compression develop 5

Multifocal Hemangiomas

• Infants with multiple (≥5) cutaneous hemangiomas require screening for hepatic involvement with ultrasonography 1
• Thyroid hormone screening is necessary because the tumor can deactivate thyroid hormone, potentially requiring hormone replacement 3, 1

Imaging and Diagnosis

For Infantile Hemangiomas

• Clinical diagnosis is usually sufficient 1
• Ultrasonography with Doppler is the initial imaging modality of choice when imaging is needed—no sedation required and no radiation exposure 3, 1
• MRI with contrast is reserved for deep facial structures, periorbital/intraorbital extent, or lumbosacral lesions with potential spinal involvement 3, 1
• CT with IV contrast may be useful for optimal airway imaging in supra- or infraglottic hemangiomas 3

For Hepatic Hemangiomas

• Diagnosis is based on a radiologic algorithm according to size and characteristics, with diagnosis possible in 99.6% of cases 4
• Contrast-enhanced ultrasound (CEUS) can provide definitive diagnosis in 77-93% of indeterminate liver lesions 1, 8

Common Pitfalls to Avoid

• Do not adopt a "wait-and-see" approach for high-risk hemangiomas—this can result in a missed window of opportunity to prevent permanent disfigurement or complications 3
• Do not delay referral—the optimal time for treatment initiation is 1 month of age, far earlier than most infants are typically referred 3
• Do not confuse different types of hemangiomas—vertebral hemangiomas behave completely differently from infantile hemangiomas and do not respond to propranolol 1, 5
• Do not perform surgery for psychological reasons alone in hepatic hemangiomas—symptoms often persist postoperatively in these cases 9
• Do not use intralesional steroids for periocular hemangiomas due to risk of blindness from retinal artery embolization 3, 1

Quality of Life Considerations

• Parental and patient quality of life can be adversely affected by visible birthmarks and resultant scarring, particularly in areas that cannot be easily covered with clothing (face, neck, arms, hands) or emotionally sensitive areas (breasts, genitalia) 3
• The psychological impact of visible hemangiomas, especially on the face, should not be underestimated 2
• Parental education about natural history and potential residual changes is essential 2
• Parental preference should be considered in decisions about specialist evaluation and treatment choices 3