Infantile Hemangiomas

• Core 1 (Pre-clerkship)
  • Integrative Systems
    • Dermatology 
      • Intro to Derm
      • Pediatric derm 
• Core 2 (Clerkship)
  • Pediatrics - Rashes Royale in Fever Didactic 
• Core 3 (Post-clerkship)
  • Electives that may futher knowledge: Pediatric Dermatology

A 3-month-old infant is brought to the pediatric clinic by their parents with concerns about a red, raised lesion on the infant's cheek. The lesion has been present since birth but has grown significantly over the past few weeks. The parents are worried about the appearance and potential complications. 

Onset, location, and progression:  

• Has the lesion been present since birth? 
• If not, when was the lesion first noticed?  
• How many lesions are there? 
• Where is the lesion located? 
• Has it grown, and if so, how rapidly?  

Appearance:  

• Ask about changes in color, texture, or ulceration.  

Symptoms:  

• Is the lesion causing pain, bleeding, or interfering with function (e.g., vision, breathing, feeding)?  

Associated symptoms:  

• Inquire about systemic symptoms or signs suggestive of syndromes like PHACE (Posterior fossa malformations, Hemangiomas, Arterial anomalies, Cardiac defects, Eye abnormalities). 

Maternal/Pregnancy/Birth History:  

• Any maternal infections, medication use, or complications (pre-eclampsia, placenta previa, advanced maternal age, etc.) during pregnancy?  
• Review prenatal screening tests if possible, including anatomy scan and fetal echo is one was done.  
• Was the baby born full term or preterm?  
• Did the baby need to stay in the NICU for any reason? 
• Prematurity and low birth weight are associated with an increased risk of hemangiomas. 

Family history:  

• Any family history of vascular anomalies or similar lesions?  

Source: https://www.ncbi.nlm.nih.gov/books/NBK538249/#:~:text=Prenatal%20and%20perinatal%20history%20are,vitro%20fertilization%2C%20and%20multiple%20gestations.&text=Presenting%20features%20depend%20on%20the,%5B28%5D 

Lesion characteristics: Note the size, location, color, and texture of the lesion.  

• Infantile hemangiomas are typically bright red, raised, and well-demarcated.  

Other lesions: Perform a thorough skin exam and note any other lesions. 

• Most hemangiomas are solitary, but multiple can be seen in up to 20% of infants. 

Growth phase:  

• Infantile hemangiomas often follow a predictable course, with rapid growth during the first few months of life (proliferative phase), followed by stabilization and eventual involution. Notably, the growth rate of a hemangioma is typically faster than the growth rate of the baby, which helps to distinguish this type of tumor from vascular malformations that grow at the same rate as the baby. 

Complications:  

• Look for ulceration, bleeding, or signs of infection.  

Functional impact:  

• Assess for any impairment of vision, breathing, or feeding caused by the lesion.  

Systemic findings:  

• Examine for features of PHACE syndrome, such as facial hemangiomas larger than 5 cm, or other vascular anomalies.  

Source:  https://dermnetnz.org/topics/infantile-haemangioma-complications-and-treatment 

Differential Diagnosis: 

Management of hemangiomas depends on their size, location, and potential complications:  

• Lower-Risk Hemangiomas 
  • Small hemangiomas (less than 2 cm) and located in areas that are not sensitive for cosmetic reasons. 
  • Thin hemangiomas that are also less than 2 cm (or less than 1 cm in infants under 3 months) and found on the outer face in places that are not major cosmetic concerns. 
• Higher-Risk Hemangiomas 
  • Hemangiomas that may cause scarring or disfigurement: 
    • Hemangiomas less than 2 cm (or less than 1 cm in infants under 3 months) on the central face. 
    • Hemangiomas 2 cm or larger in any facial area or on the scalp. 
  • Hemangiomas that could lead to functional issues: 
    • Hemangiomas 1 cm or larger around the eyes. 
    • Hemangiomas involving the lips or mouth. 
  • Ulcerated hemangiomas. 
  • Life-threatening hemangiomas: 
    • Hemangiomas in the "beard area," which may affect breathing. 
    • Five or more skin hemangiomas, which could be linked to liver hemangiomas. These infants require further imaging tests. 
  • Segmental hemangiomas on the face, scalp, or neck that may be associated with PHACE syndrome (which includes brain, blood vessel, heart, and eye anomalies). 
  • Segmental hemangiomas in the lower back, perineal, or anal areas that may be linked to LUMBAR syndrome (which includes lower body hemangiomas and various other defects). 

For lower risk and non-complicated hemangiomas: 

• Observation: Many hemangiomas are small, uncomplicated, and do not require treatment. These lesions often involute spontaneously by 5-7 years of age.  
• Topical beta-blockers: For smaller, superficial infantile hemangiomas, topical timolol gel may be effective. 

For higher risk and complicated hemangiomas:  

• Referral: 
  • Infants with high-risk or complicated hemangiomas should see a pediatric dermatologist or another specialist who knows how to diagnose and treat vascular tumors in children. This referral should happen ideally within the first four to six weeks of the baby’s life for further evaluation and possibly starting treatment. 
  • Multidisciplinary care: Infants with large or segmental hemangiomas, particularly those at risk for PHACE syndrome, may require evaluation by dermatology, cardiology, ophthalmology, and neurology. 
• Systemic therapy:  
  • Oral propranolol, a beta-blocker, is the first-line treatment when there are no structural problems as it promotes regression of the hemangioma. Caregivers should be advised to give propranolol during or after meals and should be informed about possible side effects, such as trouble sleeping and irritation in the airways. 
  • Oral corticosteroids can be used as second-line therapy if propranolol is contraindicated or doesn't work. Triamcinolone or betamethasone injections can treat larger infantile hemangiomas. Surgery or laser treatment can help, especially for skin changes after the hemangiomas shrink, but they may also be used earlier for some cases. 

In summary,  

1. Assess hemangioma characteristics 
   1. Location 
      1. Low-risk areas: Outer face (non-cosmetic zones), trunk, limbs 
      2. High-risk areas: Central face, scalp, periocular, perioral, beard area, perineal/anal region 
   2. Size 
      1. Low-risk: <2 cm (or <1 cm in infants <3 months) 
      2. High-risk: ≥2 cm, or ≥1 cm near functional zones (eye, mouth) 
   3. Type/Features 
      1. Ulcerated? 
      2. Segmental/multiple? 
      3. Associated syndromes suspected (PHACE, LUMBAR)? 
2. Stratify risk level 
   1. Lower risk 
      1. Small (<2 cm), superficial, in low-risk areas 
   2. Higher risk 
      1. Large (≥2 cm), central face/scalp, near eyes/lips, ulcerated, multiple 
   3. Life-threatening 
      1. Beard area (airway risk), ≥5 skin lesions (possible liver involvement), segmental hemangiomas (PHACE/LUMBAR risk) 
3. Determine treatment by risk level 
   1. Lower risk 
      1. Observation (most involute by age 5–7)  
      2. Topical beta-blocker (e.g., timolol) for superficial lesions 
   2. Higher risk 
      1. Refer to pediatric dermatologist (ideally by 4–6 weeks of age) 
      2. Evaluate for complications/syndromes 
   3. Systemic therapy 
      1. Oral propranolol: First-line for complicated or large hemangiomas - Give with/after feeding - Monitor for side effects (sleep issues, bronchospasm) 
   4. If propanolol fails or contra-indicated 
      1. Oral corticosteroids 
      2. Intralesional steroids (triamcinolone, betamethasone) 
      3. Laser or surgery (for residual changes or select cases) 
   5. Multi-disciplinary care 
      1. For segmental or syndrome-associated hemangiomas (PHACE/LUMBAR): involve dermatology, cardiology, ophthalmology, neurology as needed 

• Early identification and referral are key: Hemangiomas in high-risk locations (e.g., near the eyes, airway, or liver) or those associated with syndromes like PHACE require prompt evaluation and management.  
• Propranolol is a safe and effective option for reducing lesion size and preventing complications.  

• Article 
  • AAP Clinical Practice Guideline for the Management of Infantile Hemangiomas 
  • AAP Diagnosis and Management of Infantile Hemangioma 
• Video 
  • Mayo Clinic: Infantile Hemangiomas – aka “Strawberry” Birthmarks 
• Podcast 
  • Pediacast CME: Infantile Hemangiomas 
  • MedBullets Podcast: Infantile Hemangioma 
• Pathways 
  • Connecticut Children’s: Hemangioma Management 

Answers:

Q1: c. Start oral propranolol therapy 

Infantile hemangiomas often undergo a proliferative phase followed by spontaneous involution. However, lesions on the face, especially those that are large, rapidly growing, or located near functional or cosmetic areas (e.g., eyes, lips, nose), may require early intervention to reduce the risk of complications. Propranolol has become the first-line treatment for complicated or high-risk infantile hemangiomas due to its efficacy and favorable safety profile. 

Incorrect Answers:

a. Initiate systemic corticosteroids

Historically used but now rarely first-line due to side effects and the efficacy of propranolol. 

b. Observe and schedule regular follow-up

This may be appropriate for small, non-threatening hemangiomas, but not for rapidly growing facial lesions at risk for disfigurement. 

d. Refer for surgical excision

Surgery is rarely indicated in infancy unless there are complications or failure of medical management. 

Q2: a. Order a brain MRI/MRA and echocardiogram 

This infant has a large segmental facial hemangioma, which is a hallmark feature that raises concern for PHACE syndrome — a neurocutaneous syndrome that includes: 

• Posterior fossa brain malformations (e.g., Dandy-Walker) 
• Hemangioma (large, segmental, typically facial) 
• Arterial anomalies (often cerebrovascular) 
• Cardiac defects (commonly coarctation of the aorta) 
• Eye abnormalities 
  (+ Sternal clefting or supraumbilical raphe in some definitions) 

Given the hemangioma’s distribution and presence of a murmur, this infant should undergo brain MRI/MRA to assess for cerebrovascular and structural anomalies, and echocardiogram to screen for cardiac defects. 

Incorrect Answers:

b. Obtain an EKG and chest x-ray

While useful in some pediatric cases, EKG and chest X-ray are not specific to PHACE syndrome and do not evaluate for brain, vascular, or cardiac abnormalities that are characteristic of this syndrome. They would not be the best diagnostic tests here. An echocardiogram would be preferred for evaluating heart defects, and MRI/MRA is required to rule out cerebral anomalies.

c. Order genetic panel for neurocutaneous syndromes

Genetic testing is not required to diagnose PHACE syndrome. This syndrome is diagnosed clinically based on the presence of large segmental hemangiomas and associated vascular, brain, and cardiac findings. Genetic panels are not part of the diagnostic workup for PHACE.

d. Perform laser therapy on affected area

Laser therapy is reserved for cosmetic treatment after the hemangioma has involuted, or for superficial lesions that are not growing rapidly. Segmental or large hemangiomas require evaluation for systemic involvement, not immediate laser therapy. 

Basic Dermatology: Infantile Hemangiomas and Vascular Malformations (Cases and Questions) (American Academy of Dermatology)