Infantile proliferative haemangioma

Author: Dr Amanda Oakley, Dermatologist, Hamilton, New Zealand, 1997.

What is infantile haemangioma?

Infantile haemangioma (American spelling ‘hemangioma’) describes a benign (non-cancerous) condition affecting cutaneous blood vessels. It is also known as proliferative haemangioma because it is due to proliferating endothelial cells; these are the cells that line blood vessels.

Infantile haemangiomas are proliferative lesions that usually develop shortly after birth. They are distinct from vascular malformations, which are usually present at birth and are less common.

Over 80% of infantile haemangiomas occur on the head and neck area. They grow to 80% of maximum size in the first three months and most stop growing at about 5 months. However, they may keep growing for up to 18 months.

After that, they undergo regression or involution. This can take as long as 3-10 years. Nearly all flat infantile haemangiomas eventually involute and disappear without treatment. However, regression of bulky haemangiomas tends to be incomplete, and they may leave an irregular atrophic (thin) scar or anetoderma (a dented scar) in at least 50% of cases.

Types of infantile haemangioma

Infantile haemangiomas are classified as superficial, deep or mixed lesions. They may be localised (confined to a small area) or segmental (involving a larger neuroectodermal unit).

Segmental haemangiomas are more serious than localised haemangiomas.

Other haemangiomas

The haemangiomas described below are all very rare conditons.

Verrucous haemangioma
  • Haemangiomas that also show an overgrowth and thickening of skin cells
  • May be a single lesion or group occurring most often on the legs
  • Do not resolve spontaneously and may need to be surgically excised
Eruptive neonatal haemangiomatosis
  • Multiple capillary haemangiomas present at birth or develop with first few weeks of life
  • If only the skin is involved the disorder is called benign eruptive neonatal haemangiomatosis: these usually resolve spontaneously over time
  • If lesions are also present on internal organs of the body (e.g. GI tract, lungs, brain, eyes) this is called disseminated eruptive or diffuse neonatal haemangiomatosis: death generally occurs within the first few months of life
  • A newborn with multiple haemangiomas present must be investigated thoroughly for haemangiomas on internal organs
Ulcero-mutilating haemangiomatosis
  • Rare disorder of multiple haemangiomas that form ulcers that lead to severe tissue damage
Acquired multiple haemangiomatosis
  • Large numbers of haemangiomas appear in childhood or adulthood on the skin and internal organs, particularly the skeleton, brain and liver
  • Lesions persist indefinitely but are usually free of symptoms or complications

The Kasabach-Merritt syndrome is also known as haemangioma-thrombocytopenia syndrome. It is a rare complication of a rapidly growing vascular lesion, but is no longer thought to arise from ordinary infantile haemangiomas.

Which babies get haemangiomas?

Ten percent of babies develop one or more haemangiomas. Localised haemangiomas are more common if the baby has a low birthweight, for example in the following circumstances.

Hypoxia (inadequate oxygen to the skin) is now considered the likely reason for the proliferation of blood vessels. Endothelial progenitor cells (EPCs) circulate in a fetus and cause new blood vessels to form in response to hypoxia. Normally, EPCs have gone by the time a baby is born, but they may still be present in low birthweight or premature babies. As the EPCs disappear later in life, the haemangioma may regress.

Segmental haemangiomas are thought to arise early in gestation (6-8 weeks) as a developmental error.

Investigations in babies with infantile haemangioma

Infantile haemangiomas are usually diagnosed clinically and no investigations are necessary for the majority of superficial lesions.

Deep infantile haemangiomas or segmental haemangiomas are routinely investigated with ultrasound scanning. An ultrasound scan is also often performed when there is uncertainty about the diagnosis or whether underlying tissues are affected. Characteristically, a haemangioma has a firm lobular structure with vessels separating the lobules.

It may also be necessary to perform Magnetic Resonance Imaging (MRI) or angiography to help plan treatment. Children with complex lesions are best assessed by a panel of experts, including paediatrician, dermatologist, radiologist, ophthalmologist, vascular and plastic surgeons.

Haemangiomas arising over the lower part of the spine are sometimes a marker for occult spinal dysraphism (spina bifida), when spinal imaging may be appropriate.

When is treatment necessary for infantile haemangioma?

Because infantile haemangiomas are likely to improve or regress completely with time, there is no need for specific treatment in most cases. Treatment should be considered in the following circumstances.

The baby is best assessed early i.e. during the rapidly growing phase under the age of 3 to 5 months of age. If the lesion obstructs vision, it may prevent the development of normal sight.

Propranolol is the treatment of choice for troublesome haemangiomas. Topical beta blockers such as timolol, available as eye drops or gel, can be used for for small superficial haemangiomas.

Other possible treatments include:

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