Overview
Definition:
Infantile hemangiomas (IH), also known as benign infantile vascular tumors, are the most common benign tumors of infancy, characterized by rapid proliferation followed by slow involution
They are composed of endothelial cells undergoing rapid growth and differentiation.
Epidemiology:
IH occur in approximately 4-10% of infants
They are more common in premature infants (up to 30% in very low birth weight infants) and females
Family history is present in about 10% of cases
Most IH are sporadic.
Clinical Significance:
While most IH are cosmetic concerns, a significant proportion (10-30%) can lead to complications such as ulceration, bleeding, infection, obstruction (e.g., airway, vision), or functional impairment
Timely and appropriate treatment, including propranolol, is crucial to prevent morbidity and improve outcomes.
Clinical Presentation
Symptoms:
Most IH are identified in the first few weeks of life
Early IH may appear as a pale macule or subtle discoloration
Rapid growth phase typically occurs between 1-3 months of age
Complications may manifest as pain (ulceration), breathing difficulty (airway hemangioma), visual impairment (periocular hemangioma), or bleeding.
Signs:
IH present as well-demarcated, erythematous to violaceous, raised lesions
They can be superficial (bright red, strawberry-like), deep (bluish, subcutaneous), or mixed
Localized edema may be present
Large or multifocal IH may be associated with underlying structural abnormalities (e.g., PHACE syndrome).
Diagnostic Criteria:
Diagnosis is primarily clinical, based on characteristic history and physical examination findings
Imaging is usually not required for typical IH
However, for complex cases or suspected complications (e.g., airway involvement), MRI or ultrasound may be indicated.
Diagnostic Approach
History Taking:
Detailed history of onset, growth rate, and any associated symptoms of complication
Inquire about prematurity, birth weight, and family history
For IH in specific locations, assess for functional impairment (e.g., vision, feeding, breathing)
Consider potential for associated syndromes like PHACE (Posterior fossa malformations, Hemangiomas, Arterial anomalies, Coarctation of the aorta, and Eye abnormalities/Esophageal atresia).
Physical Examination:
Thorough examination of all skin surfaces, noting the number, size, location, morphology (superficial vs
deep), and color of hemangiomas
Assess for signs of ulceration, bleeding, or secondary infection
Palpate for depth and consistency
Perform a complete physical exam to rule out systemic involvement or associated syndromes.
Investigations:
Generally not required for uncomplicated IH
Imaging (MRI, USG) may be considered for large, deep, or functionally significant IH to assess extent and involvement of underlying structures, particularly if airway or intracranial involvement is suspected
Echocardiography may be needed if cardiac anomalies are suspected.
Differential Diagnosis:
Vascular malformations (port-wine stains, venous malformations, arteriovenous malformations), pyogenic granuloma, benign neoplasms (e.g., nevus lipomatosus superficialis), malignant tumors (rarely), and congenital nevi.
Management
Initial Management:
Observation is appropriate for small, asymptomatic IH that are not in critical locations
Early intervention with propranolol is indicated for IH with a high risk of functional impairment, cosmetic disfigurement, or complications like ulceration
Treatment decisions should be individualized based on IH characteristics, location, and associated risks.
Medical Management:
Propranolol is the first-line pharmacological treatment for IH requiring intervention
**Initiation:**
- Start at a low dose: 0.5 mg/kg/dose, given orally every 6-8 hours
- Gradual titration is crucial to assess tolerance
- Common initial doses: 1 mg/kg/day total, increasing to 2-3 mg/kg/day divided q6-8h over 1-2 weeks
- Maximum recommended dose: 3 mg/kg/day
- Administer with feed to reduce risk of hypoglycemia and improve absorption
**Monitoring:**
- Initial administration should be in a controlled setting (hospitalization) for at least 24 hours to monitor for bradycardia, hypotension, hypoglycemia, and bronchospasm
- Monitor vital signs (heart rate, blood pressure) regularly
- Assess for signs of adverse effects: lethargy, poor feeding, irritability, sleep disturbances, cold extremities, respiratory distress
- Monitor for growth and involution of the hemangioma
- Treatment duration typically ranges from 6-12 months, with gradual tapering
**Alternative Medical Management:**
- Topical timolol: For superficial, localized IH not responding to or contraindicated for oral propranolol
- Oral corticosteroids: Historically used, but less effective and with more side effects than propranolol
Reserved for severe, rapidly progressing cases unresponsive to propranolol or when propranolol is contraindicated
- Intralesional corticosteroid injection: For focal IH
- Interferon-alfa: For life-threatening IH (e.g., airway), used as a last resort due to significant toxicity.
Surgical Management:
Surgery is generally reserved for:
- Residual skin changes after involution (e.g., telangiectasias, fibrofatty tissue).
- IH that have failed medical management.
- IH in locations where medical therapy is not feasible or effective (e.g., some nasal tip hemangiomas).
- Procedures may include excision, laser therapy (for superficial vascular changes), or debulking.
Supportive Care:
Wound care for ulcerated hemangiomas: gentle cleansing, topical antibiotics if infected, and appropriate dressings
Pain management as needed
Nutritional support, especially if IH affects feeding or growth
Psychological support for parents regarding the cosmetic appearance of the lesion.
Complications
Early Complications:
Ulceration (most common, especially in diaper area, lips, chin)
Bleeding
Infection
Rapid growth leading to disfigurement or obstruction (e.g., stridor from airway hemangioma, visual impairment from periocular hemangioma)
Paradoxical embolism in cases of cardiac hemangiomas.
Late Complications:
Residual fibrofatty tissue, telangiectasias, scarring, or lax skin after involution
Persistent cosmetic deformity
Functional deficits if intervention was delayed (e.g., amblyopia).
Prevention Strategies:
Prompt recognition and initiation of treatment for IH identified as high-risk for complications
Close monitoring for signs of ulceration or obstruction
Careful titration and monitoring during propranolol therapy to prevent adverse effects.
Prognosis
Factors Affecting Prognosis:
Location, size, depth, and presence of complications
Early and appropriate treatment generally leads to excellent outcomes
Large, deep IH or those in critical locations have a poorer prognosis without intervention
Presence of associated syndromes impacts prognosis significantly.
Outcomes:
Most IH (around 50-60%) involute significantly by age 5, and over 90% by age 10
Propranolol therapy promotes faster involution and reduces the risk of complications, leading to better cosmetic and functional outcomes
Untreated IH can result in permanent disfigurement and functional impairment.
Follow Up:
Regular follow-up appointments are necessary to monitor growth, involution, and response to treatment
For IH treated with propranolol, monitoring continues during and after tapering
Long-term follow-up may be needed for residual cosmetic concerns or functional deficits
Discontinue propranolol gradually after 6-12 months of treatment or upon complete involution.
Key Points
Exam Focus:
Propranolol is the first-line treatment for IH requiring intervention
Dosing is critical: start low (0.5 mg/kg/dose) and titrate gradually to 3 mg/kg/day divided q6-8h
Monitor for bradycardia, hypotension, hypoglycemia, and bronchospasm, especially during initial dosing
PHACE syndrome association is a crucial differential for multiple or large facial/cervical IH.
Clinical Pearls:
Always perform vital sign monitoring during initial propranolol administration in a healthcare setting
Educate parents on signs of adverse effects and the importance of consistent dosing with feeds
Consider topical timolol for small, superficial lesions
Imaging is essential for suspected airway or CNS involvement.
Common Mistakes:
Delaying treatment for IH with high risk of functional impairment
Inappropriate dosing or inadequate monitoring of propranolol
Incorrectly identifying IH and treating a vascular malformation with a drug that is not effective
Failing to screen for PHACE syndrome in appropriate cases.