Signs and symptoms

At birth

1. 30% of the hemangiomas may be visible at birth
2. 70 % are not present at birth.

First weeks of life

1. A blanched macule may be the first sign of an impending hemangioma. In many cases this macule is unnoticed.
2. Some new vessels begin to form within the affected area, which is now surrounded by a halo.
3. After the first few weeks a rapid development is usually evident. The lesion is raised, and the color is bright red. The clinical appearance may change rapidly. The lesion may be tender to palpation and the child may feel pain if the growth rate is high. During this primary growth cycle the initial clinical manifestations of the lesion's characteristics are present.

In general there are two types of hemangiomas:

Focal Hemangiomas: These are the most common and grow as solitary tumors.
They typically grow rapidly in the first few weeks of life and then the growth rate slows.
Most of the growth is done by the age of 6 – 7 months.
Focal hemangiomas grow in specific sites, known as sites of predilection.
They are commonly found in skin or just beneath it. They can also grow in the parotid salivary gland or the breast bud. Focal hemangiomas can be entirely superficial, in which case they are a reddish color, deep in which case the lesion is a bluish color, or compound, with both superficial and deep components.

Segmental Hemangiomas: As their name implies, segmental hemangiomas grow to involve one or more segments of the face and body. This relates to the development of the fetus which takes place segmentally. Their involvement is thus more widespread.

Segmental hemangiomas behave differently in that their growth pattern differs. They also grow rapidly in the first few weeks of life, but they can continue to grow for much longer periods of time.

It is not unusual for a segmental hemangioma to grow for up to 2 years. These hemangiomas ulcerate more frequently and can be locally more destructive.

In about 30% of cases, there are other associated developmental abnormalities. These are collectively known as PHACES syndrome.
Each of the letters in this syndrome stands for a developmental abnormality. It is important to note that it is uncommon for all of these abnormalities to be present in any one child. Most children with this syndrome will have 2 or 3 of these abnormalities. It is however imperative that all children with a segmental hemangioma be investigated for PHACES syndrome. Up to 30% of children with segmental hemangiomas may have PHACES syndrome.

PHACES syndrome is an acronym:

P. Posterior fossa abnormalities
H. Hemangiomas
A. Arterial abnormalities
C. Cardiac abnormalities
E. Eye abnormalities
S. Sternal defect and or supraumbilical raphe
The majority of Hemangiomas are present in or just to the skin.
There are three clinical manifestations of Hemangiomas according to the depth of the skin layer that they involve.

Superficial Hemangiomas are flat and appear reddish in color as a macular or papular mass or a cluster of small vessels. The skin layer involved is the papillary dermis.

Deep Hemangiomas appear bluish in color and expand the overlying skin. They may also be colorless. The skin layer involved is the reticular dermis. The overlying skin layer may be intact.

Compound Hemangiomas usually have clinical elements of both a superficial and a deep hemangioma. The skin layers involved are the papillary and reticular dermis.

signs-symptoms-graph

4-6 months

In some babies another growth phase is commonly seen. The vascular mass may continue growing at a fastest rate comparing to its development during the previous month. The baby may feel uncomfortable due to the tissue distension caused by the lesion. This period of the secondary growth cycle may last for one –two months and then the rate of expansion may be reduced.

9-12 months

The growth phase ceases and the transition from proliferation to involution begins. The lesion will soften and shrink from its center to its periphery. The color changes from bright red to a darker, purplish appearance.

1-12 years

In the majority of Hemangiomas the involution commences. The lesion will definitely stop growing and the first signs of the lesion's shrinkage are evident. The rate of involution is variable; so is the point that the involution is completed.

In 60% of Hemangiomas the involution will be completed by the age of 6 years (rapid regression). Unfortunately this fact is not always synonymous to a complete disappearance of the lesion. Atrophy of the skin, residual telangiectasia and previous ulceration markings may still be evident. It has been estimated that 40% of these children will require some form of corrective intervention.

The remaining 40% of Hemangiomas will shrink by the age of 12 (slow regression). In these cases only 20% will disappear completely. The majority, up to 80%, will require an aesthetic correction as residual elements of the lesion are evident.

All hemangiomas involute. Involution simply means shrink. This process may or may not result in a satisfactory outcome. It is important to realize that involution may take many years and the process may be completed in many cases, by 10 to 12 years of age. If it is likely that the outcome of involution will not leave a satisfactory result or if the process is taking too long, then surgery can be considered. It is always preferable to have the child looking as normal as possible by two and half to three years of age. Early surgery has the advantage of healing with less scarring and this should always be taken into consideration.

At the end of involution, one or more of the following may be left over:

Atrophic scar. This is most frequent. Normal skin, especially that of a young child, has an abundance of collagen and elastin in the dermis. This is what makes a child's skin thick and supple, as compared with that of a mature adult. During the process of aging, we lose some collagen as well as elastin and this is in part, what is responsible for the ageing process of skin. A superficial hemangioma that involves skin, will replace the dermis where the collagen and elastin is contained. As the hemangioma involutes, this layer is not replaced and the end result is thin "aged" or atrophic skin. Often, some of the dilated blood vessels will remain and these will be evident as telangiectasia (broken capillaries).

Residual mass. About half of all focal hemangiomas will leave a residual mass of what is known as fibro-fatty tissue behind at the end of involution. This usually takes the form of a soft doughy mass. If the overlying skin was involved, residual telangiectasia (broken capillaries) and atrophic scarring may be left behind.

Hypertrophic scar. Whenever a hemangioma has ulcerated, it always will leave a residual scar behind. This will either be an atrophic (thin) scar or a hypertrophic (thick scar). It will almost never leave normal skin behind.

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