What are the different types of hemangiomas and how are they diagnosed and managed?

Hemangioma Types, Diagnosis, and Management

Hemangiomas are classified into distinct types including infantile hemangiomas, congenital hemangiomas (RICH and NICH), and vascular malformations, with diagnosis primarily based on clinical presentation, supported by imaging when necessary, and management tailored to the specific type and associated complications. 1, 2

Types of Hemangiomas

Infantile Hemangiomas (IH)

• Most common vascular tumor of childhood (5% of infants) 1
• Characterized by abnormal proliferation of endothelial cells and aberrant blood vessel architecture
• Typically appear before 4 weeks of age, complete most growth by 5 months
• Female predominance (female:male ratio 1.4:1 to 3:1) 1
• Clinical course:
  • Proliferative phase (up to 12 months)
  • Involution phase (begins between 6-12 months)
  • Majority of regression occurs before 4 years of age 1

Congenital Hemangiomas

• Present and fully formed at birth (unlike IH)
• Do not exhibit postnatal proliferative phase
• Two distinct variants:
  1. Rapidly Involuting Congenital Hemangioma (RICH) - undergoes rapid involution beginning in first year of life
  2. Non-Involuting Congenital Hemangioma (NICH) - remains stable without growth or involution 1
• Unlike IH, do not express glucose transporter protein isoform 1 (GLUT1) 1

Other Vascular Lesions Often Confused with Hemangiomas

Pyogenic Granuloma (Lobular Capillary Hemangioma)

• Reactive proliferative vascular lesion, not a true hemangioma
• Common in infants and children (12% occur in infancy)
• Typically located on head and neck
• Rapidly enlarge to median size of 6.5mm, often with pedunculated base
• Prone to bleeding when eroded 1

Vascular Malformations

• Structural anomalies (not neoplasms)
• Present at birth but may become apparent later
• Do not involute
• Classification based on predominant vessel type:
  • Capillary/venulocapillary malformations (port wine stains)
  • Venous malformations
  • Lymphatic malformations
  • Arteriovenous malformations
  • Mixed malformations 1

Kaposiform Hemangioendothelioma and Tufted Angioma

• Associated with Kasabach-Merritt phenomenon (consumptive coagulopathy)
• Important note: Kasabach-Merritt phenomenon is NOT associated with infantile hemangiomas 1

Diagnosis

Clinical Diagnosis

• Most hemangiomas are diagnosed based on clinical history and physical examination 3
• Key clinical features:
  • Timing of appearance (present at birth vs. appearing in first weeks of life)
  • Growth pattern (rapid growth followed by involution vs. proportional growth with child)
  • Color and texture (bright red superficial vs. bluish deep lesions)
  • Location and distribution

Imaging

• Not routinely required unless:
  • Diagnosis is uncertain
  • Five or more cutaneous IHs are present
  • Associated anatomic abnormalities are suspected 2
• Preferred imaging modalities:
  • Ultrasound with Doppler: initial imaging of choice 2, 4
  • MRI: best for evaluating extent of lesions and relationship to adjacent structures 4

Management

Observation

• Appropriate for most uncomplicated hemangiomas 5
• Regular follow-up every 3-6 months
• Close monitoring during rapid growth phase (5-7 weeks of age) 2

Medical Therapy

• First-line treatment: Oral propranolol

  • Dosage: 2-3 mg/kg/day divided into 2-3 doses
  • Duration: at least 6 months, typically until 9-10 months of age for focal IHs and up to 18 months for segmental IHs 2

• Alternative treatments:

  • Corticosteroids when propranolol is contraindicated (oral prednisolone/prednisone at 2-3 mg/kg/day) 2
  • Topical timolol 0.5% gel for superficial hemangiomas 2
  • Intralesional steroid injections (caution near eyes due to risk of retinal artery embolization) 2

Surgical and Procedural Interventions

• Indications:

  • Failure to respond to medical therapy
  • Significant residual tissue after medical treatment
  • Bleeding, ulceration, or functional impairment
  • Cosmetic disfigurement 2

• Approaches:

  • Surgical excision
  • Laser therapy (particularly pulsed dye laser for superficial components)
  • Often multimodal approach combining laser and surgery 2

Special Considerations for Specific Locations

Hepatic Hemangiomas

• Asymptomatic lesions <5 cm: observation
• Lesions ≥5 cm or symptomatic: surgical options (enucleation, formal liver resection) or bland embolization 2, 6
• Monitoring for high-output cardiac failure
• Screening for thyroid dysfunction 2

Periocular Hemangiomas

• Early referral to ophthalmology
• Can cause astigmatism (20-46% of cases) or anisometropia
• Early intervention (before 9 months) may reverse astigmatism 2

High-Risk Locations

• Immediate referral for hemangiomas in critical locations:
  • Periorbital region
  • Airway
  • Areas prone to ulceration and bleeding 2

Complications and Long-Term Outcomes

• Up to 70% of IHs leave permanent skin changes:

  • Telangiectasia
  • Fibrofatty tissue
  • Redundant skin
  • Atrophy
  • Dyspigmentation
  • Scarring (especially with history of ulceration) 1

• Wound care for ulcerated hemangiomas:

  • Clean with mild antiseptic
  • Apply non-adherent dressing
  • Consider petroleum jelly-impregnated gauze 2

• Prophylactic treatment with propranolol should be considered for high-risk hemangiomas before complications occur 2

When to Refer to Specialists

• Multiple (≥5) cutaneous hemangiomas
• Segmental hemangiomas (higher risk of complications)
• Hemangiomas in critical locations (periorbital, airway)
• Uncontrolled bleeding or hemodynamic instability
• Significant functional impairment or disfigurement 2

The management of hemangiomas has evolved significantly with propranolol becoming the first-line treatment for complicated infantile hemangiomas, replacing older approaches such as systemic corticosteroids and offering better outcomes with fewer side effects 3, 7.