Infantile Hemangioma

Aka: Infantile Hemangioma, Infantile Capillary Hemangioma, Hereditary Capillary Hemangioma, Neonatal Hemangiomatosis, Capillary Hemangioma, Strawberry Hemangioma, Hemangioma, Congenital Hemangioma, Congenital Hemangiomatosis

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II. Background

  1. Infantile Hemangioma (referred to on this page)
    1. Common, benign vascular lesion that appears in the first months of life
    2. Contrast with Congenital Hemangioma (which is prominent at birth) described in differential diagnosis

III. Epidemiology

  1. Prevalence: 1.1 - 2.6% up to 4 to 5% of all live births (up to 10% of infants at one year)
    1. Contrast with the relatively rare Congenital Hemangioma (compared with Infantile Hemangioma)
  2. Gender: Female more common than male
  3. Race: White, non-hispanic

IV. Risk Factors

  1. Premature Infants (or Low Birth Weight Infants)
  2. Multiple Gestation
  3. Maternal factors
    1. Advanced maternal age
    2. Maternal Preeclampsia
    3. Gestational Diabetes
    4. Placental abnormalities

V. Signs

  1. Lesions are NOT present at birth (or barely noticeable pale patch of skin)
    1. Contrast with Congenital Hemangioma (which is prominent at birth)
  2. Characteristics
    1. Erythematous (strawberry red to pale) or purple lobulated lesions
    2. Superficial or Deep (or combination)
    3. Localized, segmental or multiple
  3. Timing (see course below)
    1. Absent at birth (see above)
    2. Rapidly develop at age 1 to 3 months
    3. Proliferation typically ceases by 5 months
    4. Involution begins by 12 months
    5. Lesions disappear by 4 years in 80% of children
  4. Distribution
    1. Midline
    2. Beard region (airway)

VI. Imaging

  1. Abdominal Doppler Ultrasound indications
    1. Five of more Hemangiomas and suspected LUMBAR Syndrome
  2. Consider Imaging for Spinal Dysraphism
    1. See Cutaneous Signs of Dysraphism
    2. Hemangiomas within the perineum, gluteal cleft or lumbosacral region
  3. Head and Neck MRI Indications
    1. Suspected PHACE Syndrome

VII. Differential Diagnosis

  1. Cutaneous Signs of Spinal Dysraphism (e.g. sacral Hemangioma)
  2. Systemic disorders
    1. Thrombocytopenia
    2. Cardiac failure
  3. Congenital Hemangioma
    1. Rare compared with Infantile Hemangioma
    2. Benign vascular tumors present at birth
      1. Contrast with delayed appearance over the first month of life in Infantile Hemangioma
      2. Associated mutations GNAQ, GNA11
    3. Subtypes
      1. Rapidly Involuting (RICH, typically involutes by 6 to 14 months)
      2. Partially Involuting (PICH, decreases in size until 12 to 30 months)
      3. Noninvoluting (NICH)
    4. Complications
      1. Ulceration (esp. in large RICH lesions during involution)
      2. Mild, transient Thrombocytopenia
      3. Congestive Heart Failure (rare)

VIII. Associated Conditions

  1. Kasabach-Merritt Syndrome
  2. PHASEs Syndrome
    1. Posterior fossa malformations (Dandy Walker)
    2. Hemangioma (large, segmental, facial)
      1. Face segment distribution types (central face, V1, V2, V3, periauricular)
      2. Typically >5 cm diameter
    3. Arterial abnormalities
    4. Cardiac anomaly (e.g. coarctation of aorta)
    5. Eye or endocrine disorders
    6. Sternal cleft or supraumbilical raphe
  3. LUMBAR Syndrome
    1. Lower body Hemangiomas (or Cutaneous Signs of Spinal Dysraphism)
    2. Urogenital abnormalities
    3. Myelopathy
    4. Bony abnormalities
    5. Anorectal malformations
    6. Renal anomalies
  4. Tethered Cord with lumbosacral lesions
    1. See Cutaneous Signs of Spinal Dysraphism

IX. Course

  1. Develops in first four weeks after birth
    1. Not typically visible at birth (outside of a faint, subtle, flat lesion)
  2. Proliferates until 9 to 12 months of age
    1. Rapid growth in first 3 months of age
    2. Typical Hemangioma reaches 80% of largest size by 5 months of age
    3. Proliferation after 12 months of age is uncommon
  3. Spontaneous, Gradual Involution (often incomplete)
    1. Findings
      1. Color shifts from bright red to dark red (or violaceous red to gray)
      2. Ulceration may occur following early white discoloration
    2. Involution Timing (10% per year)
      1. Age 5 years: 50% resolution
      2. Age 7 years: 70% resolution
      3. Age 9 years: 90% resolution
    3. Final appearance
      1. Residual atrophy, Hypopigmentation, Telangiectases, or scarring may persist (up to 50% of cases)

X. Management: First-Line

  1. Best treatment efficacy with treatment started in first 10 weeks of life
  2. Indications for treatment relates to complication risk
    1. Disfigurement
    2. Skin Ulceration (16% of cases)
      1. May result in minor bleeding, infection or pain
    3. Functional Impairment (e.g. visual obstruction, overlying joint, sucking and feeding difficulty)
  3. Propranolol
    1. FDA approved for age 5 weeks and older
    2. Well tolerated and very effective at facilitating Hemangioma involution when used in the first year of life
    3. Start in first months of life to prevent proliferation and continue until up to 12 to 18 months of age
    4. Observe for adverse effects (e.g. Hypotension, Sinus Bradycardia, Hypoglycemia)
      1. Adverse effects are higher with propranol than Atenolol, but Propranolol is more effective
      2. Considering observing infants in hospital during Propranolol initiation in higher risk infants
        1. Infants age <5 weeks corrected Gestational age
        2. Difficult social support systems
        3. Cardiopulmonary comorbid conditions
    5. Dosing
      1. Start 1 mg/kg/day divided orally twice daily
      2. May increase to 2 mg/kg/day after the first week
      3. Maximum dose 3 mg/kg/day
      4. Prevent Hypoglycemia by giving after feeding and witholding dose for decreased feeding or Vomiting
    6. References
      1. Léauté-Labrèze (2015) N Engl J Med 372(8):735-46 +PMID: 25693013 [PubMed]
  4. Topical Timolol
    1. Dose 0.5% gel ophthalmic solution 1-2 drops twice daily

XI. Management: Refractory (Interventions by Pediatric Dermatology)

  1. Compression garment or Coban Tape
    1. Hemangiomas on arms or legs
  2. Biosynthetic dressing every 24 hours
  3. Prednisone
    1. Indicated where Hemangioma compresses eye, airway or other vital conditions
    2. Dose: 3 mg/kg daily for 6-12 weeks
    3. Continue until lesion stops growing or size decreases
  4. Interferon alfa (if refractory to Steroids)
  5. Sirolimus
  6. Topical Timolol Maleate
  7. Pulsed-dye laser therapy

XII. Management: Indications for referral or further evaluation

  1. See IHReS link under resources below
  2. Multiple Hemangiomas (>=5 small localized Hemangiomas)
    1. Obtain Ultrasound of the Abdomen to evaluate for gastrointestinal and Liver Lesions
    2. Visceral involvement (esp. liver) is termed diffuse Neonatal Hemangiomatosis
    3. Isolated Hemangiomas without visceral involvement are considered benign Neonatal Hemangiomatosis
  3. Deep Hemangiomas
  4. Large Hemangiomas
    1. Risk of high-output Heart Failure
  5. Sacral Hemangiomas
    1. See Cutaneous Signs of Spinal Dysraphism
  6. Airway Hemangioma (beard distribution, subglottic Hemangioma)
    1. May present with Stridor, Hoarseness or recurrent croup-like episodes
      1. Associated with vascular engorgement and edema with airway obstructive symptoms
      2. Severe cases may require Endotracheal Intubation
    2. Airway Hemangiomas present in first 4 to 6 weeks of life (and enlarge through the first year of life)
    3. May appear on Lateral neck XRay with asymmetric smooth subglottic swelling
    4. Typically responds to Propranolol, and resolves in most cases within 5 to 7 days
    5. Refer to otolaryngology
    6. Rahbar (2004) Laryngoscope 114(11): 1880-91 [PubMed]
  7. Eyelid Hemangioma
    1. May block Vision
  8. PHASEs Syndrome (see above)
    1. Careful physical exam
    2. MRI/MRA of the head and neck
    3. Echocardiogram
    4. Opthalmology exam
  9. LUMBAR Syndrome
    1. Obtain MRI Lumbar Spine

XIII. Resources

  1. Infantile Hemangioma Referral Score Screening Tool (IHReS)
    1. https://www.ihscoring.com/wp-content/uploads/2020/01/PFD-PDF_RESULTATS_US.pdf

XIV. References

  1. Anderson (2024) Mayo Clinic Pediatric Days, lecture attended 1/16/2024
  2. McLaughlin (2008) Am Fam Physician 77: 56-60 [PubMed]
  3. Snyder (2024) Am Fam Physician 109(3): 212-6 [PubMed]

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