Infantile Hemangiomas and Vascular Malformations

Infantile Hemangiomas & Vascular Malformations - Intro & Contrast

Infantile Hemangiomas & Vascular Malformations - Strawberry Marks

• Infantile Hemangiomas (IH): Benign vascular tumors. "Strawberry mark" for superficial type.
  • Natural History:
    • Proliferation phase: Rapid growth (first ~6 months).
    • Involution phase: Slow regression (50% by 5 yrs, 90% by 9 yrs).
  • Types: Superficial (raised, bright red), Deep (bluish, subcutaneous), Mixed.
  • When to worry (Indications for treatment):
    • Ulceration (most common complication), bleeding.
    • Obstruction: Vision (periorbital), airway (subglottic).
    • Large facial lesions (disfigurement).
    • Multiple IHs (>5, risk of visceral hemangiomas).
    • Syndromic: PHACE, LUMBAR.
  • Management:
    • Observation for most.
    • Oral Propranolol: First-line for complicated IH.
    • Topical Timolol: Small, superficial lesions.

⭐ Most infantile hemangiomas undergo spontaneous involution; however, propranolol is the mainstay for problematic lesions.

• Vascular Malformations (VM): Present at birth, grow with child, do not involute (e.g., Port-wine stain).

Infantile Hemangiomas & Vascular Malformations - Berry Management

• Observation: Majority of uncomplicated IH; await spontaneous involution.
• Medical Therapy:
  • Oral Propranolol: First-line for high-risk/complicated IH (e.g., periocular, airway, large facial, ulcerated). Dose: 2-3 mg/kg/day. Monitor for hypoglycemia, bradycardia.
  • Topical Timolol: Small, thin, superficial lesions.
  • Systemic Corticosteroids (Prednisolone 2-3 mg/kg/day): Second-line if propranolol fails/contraindicated.
• Interventional:
  • Pulsed Dye Laser (PDL): Ulceration, residual telangiectasias.
  • Surgery: Residual deformity, urgent complications unresponsive to medical therapy.

⭐ Propranolol is the first-line therapy for problematic infantile hemangiomas, significantly reducing the need for corticosteroids or surgery.

Infantile Hemangiomas & Vascular Malformations - Malformation Map

• Congenital lesions; present at birth (may be subtle initially).
• Grow proportionally with child; persist throughout life (no involution).
• Classification (ISSVA based on flow/vessel):
  • Low-Flow:
    • Capillary (CM): e.g., Port-Wine Stain (PWS).
    • Venous (VM): Soft, compressible, blue hue.
    • Lymphatic (LM): e.g., Macrocystic (cystic hygroma).
  • High-Flow:
    • Arteriovenous Malformations (AVM): Often with thrill/bruit.
    • Arteriovenous Fistulas (AVF).
  • Combined-Complex: e.g., Klippel-Trenaunay Syndrome.

⭐ Sturge-Weber Syndrome: Facial PWS (Nevus Flammeus, typically V1 dermatome) + ipsilateral leptomeningeal angiomatosis + glaucoma.

Infantile Hemangiomas & Vascular Malformations - Syndromic Spots

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High‑Yield Points - ⚡ Biggest Takeaways

• Infantile hemangiomas (IH) are GLUT1 positive, show rapid postnatal proliferation then involution.
• Propranolol is the first-line treatment for complicated IH.
• Vascular malformations (VM) are GLUT1 negative, present at birth, and grow proportionally with the child.
• Port-wine stains (PWS), a capillary malformation; consider Sturge-Weber syndrome if in V1 distribution.
• Kasabach-Merritt phenomenon: Severe thrombocytopenia with kaposiform hemangioendothelioma/tufted angioma, not IH.
• PHACES syndrome: Large facial hemangiomas with Posterior fossa, Arterial, Cardiac, Eye, Sternal defects.