What are the current management options for hemangiomas and vascular malformations?

Current Management of Hemangiomas and Vascular Tumors and Vascular Malformations

Critical Distinction: Hemangiomas vs. Vascular Malformations

Infantile hemangiomas (IHs) are vascular neoplasms with abnormal endothelial cell proliferation, while vascular malformations are structural anomalies and inborn errors of vascular morphogenesis—these are fundamentally different entities requiring completely different management approaches. 1

Vascular Malformations Include:

• Capillary malformations (port wine stains) 1
• Venous malformations (most common, 70% of all vascular malformations) 2
• Lymphatic malformations (formerly lymphangiomas or cystic hygromas) 1
• Arteriovenous malformations 1

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Management of Infantile Hemangiomas

Risk Stratification and Timing

Early intervention by 1 month of age is critical because the most rapid IH growth occurs between 1-3 months of age, with growth completed by 5 months in most cases. 1, 3

High-Risk IHs Requiring Urgent Specialist Referral:

• Facial IHs (especially segmental patterns) 1
• Hepatic or airway IHs 1
• Periorbital IHs (risk of functional impairment) 1
• Ulcerated IHs (causing pain or scarring) 1
• Large segmental facial or scalp hemangiomas (associated with PHACE syndrome) 3
• IHs with associated underlying abnormalities 1

Do not reassure families that lesions will simply "go away"—while 90% involute by 4 years, up to 70% leave permanent skin changes including telangiectasia, fibrofatty tissue, redundant skin, atrophy, dyspigmentation, and scarring. 1, 3

First-Line Pharmacologic Treatment

Propranolol is the drug of choice for problematic IHs requiring systemic therapy, dosed at 2-3 mg/kg/day in three divided doses. 1

Treatment Protocol:

• Begin early during proliferative phase 1
• Continue for at least 6 months, often maintained until 12 months of age (occasionally longer) 1
• Target dosage: 2 mg/kg/day in three divided doses 4
• Stop gradually, not abruptly 4

Low-dose propranolol (1 mg/kg/day) has demonstrated excellent response in 35 of 40 treated patients (87.5%), with good response in an additional 4 patients. 5

Second-Line Pharmacologic Treatment

Topical timolol may be used for select small, thin, superficial IHs. 1

• Appropriate for lesions that are small size and not significantly elevated 1
• Risk of local irritation, sleep disturbance, cold extremities, bronchospasm, and bradycardia 1
• More caution needed in preterm infants and those with ulceration 1

For patients unresponsive to propranolol, percutaneous intralesional bleomycin injection (0.3-0.5 mg/kg/dose) demonstrated excellent response in 4 of 5 non-responders and good response in 1 of 5. 5

Surgical and Laser Management

Surgery and laser treatment are most useful for treating residual skin changes after involution, not during the proliferative phase. 1

Timing of Surgical Intervention:

• Defer surgery until 3-5 years of age in most cases 1
• At this age: (1) lesion has resolved significantly, (2) tumor is smaller making operation easier with smaller scars, (3) primarily adipose tissue instead of blood vessels making surgery safer 1
• Most IHs do not improve significantly after 3-4 years of age, so waiting beyond 5 years is unnecessary 1

Early Surgery May Be Indicated When:

• Lesion ulcerates and fails local wound care/pharmacotherapy 1
• Obstruction or deformation of vital structures (airway, orbit) 1
• Well-localized lesions in aesthetically sensitive areas where early surgery simplifies later reconstruction 1

Pulsed dye laser (PDL) has been used for decades but is now less frequently employed with the advent of β-blocker therapy. 1

Specialist Referral

Refer urgently to a hemangioma specialist (pediatric dermatology, plastic surgery, pediatric surgery, or multidisciplinary vascular anomalies center) for growing scalp or facial hemangiomas, ideally by 1 month of age. 3

Qualified Specialists Should:

• Understand time-sensitive nature and accommodate urgent evaluations 1, 3
• Have experience with accurate risk stratification 1, 3
• Provide recommendations for observation, medical therapies, surgical/laser procedures 1, 3
• Maintain thorough knowledge of current and emerging literature 1, 3

If in-person consultation cannot be arranged promptly, use telemedicine for triage and initial evaluation. 3

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Management of Vascular Malformations

Diagnostic Approach

Vascular malformations are present at birth and grow proportionally with body growth, unlike IHs which appear in the first weeks of life and undergo rapid proliferation. 2

Ultrasound with Duplex Doppler is the appropriate first-line imaging modality, distinguishing IHs from venous malformations based on arterial and venous waveforms. 2

MRI/MRA without and with IV contrast is usually appropriate for definitive characterization when ultrasound is inconclusive, defining venous and arterial anatomy. 2

Treatment by Malformation Type

Low-Flow Vascular Malformations (Venous, Lymphatic, Capillary):

Sclerosant therapy is extremely effective, either alone for small lesions or combined with surgical resection or embolization for larger lesions. 6

• Surgical excision alone for lymphovenous malformations has high success rate 6
• 27 patients with lymphovenous malformations treated with surgical excision alone showed high success 6

High-Flow Vascular Malformations (Arteriovenous):

Preoperative embolization combined with surgical excision is the treatment of choice. 6

• Selective angiography used therapeutically when embolization is part of treatment protocol 6
• 16 patients with high-flow malformations treated with this approach 6

Imaging Considerations

For airway hemangiomas (beard-like distribution over face/neck or subglottic location), CT with IV contrast provides improved definition of airway lesion, presence, localization, and complete extent compared to bronchoscopy. 1

Angiography provides valuable diagnostic information about vascular dynamics and extent of lesions, though MRI is now used more frequently for this purpose. 6

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Common Pitfalls to Avoid

• Do not wait to see how large the lesion will become—predicting growth is extremely difficult even for experienced clinicians, and damage may occur during observation 3
• Do not confuse "cavernous hemangiomas" with IHs—these are usually deep IHs or venous malformations requiring different management 1
• Do not attribute Kasabach-Merritt phenomenon to IHs—this consumptive coagulopathy is associated with kaposiform hemangioendothelioma and tufted angioma, not IHs 1
• Do not perform surgery during infancy for most IHs—anesthetic risks are greater, tumor is highly vascular with higher blood loss risk, and outcomes are inferior 1