Infantile haemangiomas (including strawberry naevi)

Infantile haemangiomas are rapidly growing, benign proliferations of endothelial cells, appearing within weeks of life, proliferating for approximately 9 months and then involuting, a process which is complete by 10-12 years of age.

This chapter is set out as follows:

• Haemangiomas are the most common of the infantile tumours, most frequently seen in Caucasian female infants and in those born prematurely
• The prevalence of these tumours is about 1%
• They are presumed to be an abnormality of embryonic angioblastic development 
• The capillaries are the most commonly affected vessels 

• About 20% are present at birth, and 90% are seen within a month post-partum   
• Lesions grow rapidly and can bleed or ulcerate 
• Apart from the occasional lesion that is fully developed at birth, 80% of growth occurs within the first 4 months, with lesions reaching a maximum size around 9 months of age. Deep lesions can grow for longer to become very large causing anatomical distortion
• By year 4 there is approximately 80% regression. Ultimately up to 95% will completely / almost completely resolve

Distribution

• Often single but multiple lesions can develop (more than 10 is uncommon)
• 60% of the lesions occur on the head and neck, other common sites include the buttocks and perianal area 

Morphology

​Tumours can be localised or segmental, and superficial or deep - although many have both superficial and deep components and are termed mixed infantile haemangiomas:

• The infantile superficial haemangioma is commonly known as  a strawberry naevus (capillary haemangioma). Clinically they appear as red, relatively soft, and somewhat irregular in outline

• Infantile deep haemangiomas are also called cavernous haemangiomas and are more deeply set in the dermis and subcutis. Clinically they appear as a bluish soft to firm swelling

• Both types of haemangiomas may occur together in mixed infantile haemangiomas when a strawberry naevus overlies a bluish swelling

• Most lesions need no treatment. Regular monitoring in Primary Care may reassure parents. Photographs at 6-12 month intervals are very useful 

• Most of the problematic lesions are segmental proliferative haemangiomas as they occur at a younger age, grow up to ten times larger, and are more likely to have associated congenital anomalies

• Urgent referral (same day) and intervention is required for the following lesions:
• Lesions that are likely to grow into or within an important structure such as the eye, nose/mouth and ear leading to obstruction of the visual axis (amblyopia can occur within 10 days of lesions interfering with vision), airway or external auditory meatus
• Interfering with feeding 
• Lesions under the chin - can have laryngeal and other systemic involvement
• Lesions on the scalp can be associated with intracranial complications 
• Lesions overlying the lumbosacral area - up to 50% of such cases are associated with significant problems such as tethering or compression of the spinal cord, and external genital abnormalities. MRI imaging should be performed in such patients

• Timely referral is required for the following lesions:
  • Lesions that have grown or are likely to grow into or within other important structure such as the genitalia
  • Anywhere on the body if complicated by bleeding, ulceration or causing functional impairment
  • Multiple haemangiomas (5 or more) can be associated with visceral haemangiomas including cardiac lesions 
    
  • Kasabach-Merritt syndrome (consumption coagulopathy) is an uncommon complication, mainly in larger lesions 

• Treatment
  • If treatment is required, first-line therapy is a beta-blocker, usually systemic, although occasionally topically. To be effective treatment must be given early during the proliferative (growth) phase, which usually leads to a rapid and dramatic involution of the haemangioma. Treatment delay can lead to potentially serious outcomes including anatomical distortion  
  • Other treatments sometimes used include laser, and systemic corticosteroids