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Infantile haemangioma (IH) affects 3-10% of newborn babies and is the most common benign infantile tumour. It arises in the first days or weeks of life, sometimes preceded by a vascular patch, called precursor, with anaemia, telangiectasia or mixed. IH is most common in girls of Caucasian ethnicity and the risk factors, given in Table 1, are known.
There are three clinical forms: superficial IH (50-60% of cases), deep IH (15% of cases) and mixed IH (25-35% of cases). The predominant location is the head and neck area, but the tumour may present anywhere on the skin. Different presentation patterns may be identified depending on the dimensions and distribution: focal or localised, indeterminate, segmental, multifocal. A distinctive morphological variant is straightaway ulcerated IH which presents as a small ulceration present at birth or with early onset. A predominant location is the perineal region; it is more rarely located on the lip.
IH typically presents an early growth phase in the first 3 months of life, which is at its maximum between the 5thand 8thweeks. The segmental variety may have a prolonged growth phase which can reach the 9th-12 months, more rarely up to 24 months. This is followed by a stabilisation phase and an involution or regression phase which in 90% of cases is completed at around 4 years of age. An exception is deep IH which completes regression at around 7-8 years. The clinical sign of regression is the appearance of greyish-white areas on the IH surface. IH resolves spontaneously in 70% of cases with sequelae (Table 2).
Depending on the type of growth, there is also IH with minimal or no growth characterised by a zero or minimal proliferative component that does not exceed 30% of the total area of the IH based on erythema-telangiectasia, which is located predominantly on the lower limbs.
10-15% of cases require treatment due to complications such as: a) life-threatening; b) risk of permanent functional impairment; c) ulceration; d) sequelae with significant dysaesthesia (Table 3).
Neonatal haemangiomatosis is characterised by the presence of over 5 IHs. It may be isolated (benign neonatal haemangiomatosis) or associated with visceral haemangiomas, predominantly affecting the liver (diffuse neonatal haemangiomatosis).
Segmental IHs present the highest risk of complications such as ulceration, cardiac overload, association with deep or visceral IHs (subglottic IH) or complex malformations which mainly affect the central nervous system (CNS) for IH of the face (PHACE syndrome) and spinal bone marrow (dysraphism), and other organs (genital region, urinary tract) for segmental IHs of the perineal and sacral region (SACRAL, PELVIS, LUMBAR syndrome) (Table 4).
Haggstrom AN et al. Patterns of infantile hemangiomas: new clues to hemangioma pathogenesis and embryonic facial development. Pediatrics 2006;117:698-703
In the light of current knowledge, the risk is closely linked to the presence of a deep component, which may become evident in time, usually from the fourth-fifth week of life up until the second or third month, and for IH with “cobblestone” surface or step-edge IH.
In these cases there is a high risk of permanent sequelae, as fibroadipose residue and redundant skin, requiring subsequent corrective surgery. Systemic therapy with propranolol, which is currently the drug of first choice for treating IH, should therefore also be considered for focal IH. The dose is 3 mg/kg/die divided into two doses, for 6 months.
10-15% of infantile haemangiomas are at risk and require systemic treatment; propranolol is currently the drug of first choice.
Thorough follow-up in the first months of life of IHs is necessary when it is not possible to foresee the extent of final growth; some double the initial dimensions although in some cases the growth is greater. In addition the development of the deep component becomes evident after the superficial component, and so diagnostic errors may be made as a superficial IH can become mixed. The diagnostic error may be followed by the decision not to treat, which may result in permanent final sequelae. A sign of initial regression of the haemangioma is the appearance of greyish-white areas on the surface.
IH located on the trunk, and at birth small and superficial. Increase in size and appearance of the deep component at about the fourth month. At 12 months shows signs of involution which occurs with fibroadipose residue clearly evident at the check-up at 27 months.
Step-edge superficial exophytic focal IH of the neck. Spontaneous involution with redundant skin.
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