Angiomas or vascular anomalies

1 - ACKNOWLEDGEMENTS

Chapter written with the help of the EADV, the Fondation René Touraine and the Therapeutics in Dermatology

2 - INTRODUCTION

Angiomas and vascular anomalies include a wide group of entities. Here we discuss infantile hemangioma and vascular malformations.

3 - INFANTILE HEMANGIOMA

3.1 - OVERVIEW

Infantile hemangioma is a benign (not cancerous) overgrowth and it is a type of birthmark. They are the most common benign vascular tumor in infancy and may be present at birth or may appear in the first months after birth. Almost half of the cases disappear by the age of five and the vast majority are gone by age of 10.

3.2 - SIGNS AND SYMPTOMS

The appearance of hemangiomas changes rapidly during early life. A small red mark or swelling may be visible at birth or develop during the first weeks of life. This may increase in size usually most quickly over the first three months. Further change is usually slower and gradual for up to 12 months.

Most hemangiomas do not cause any symptoms. Rarely, the skin over the haemangioma breaks down (ulcerates) and may cause pain. Bleeding of the ulcerated skin may occur but this is rarely severe and usually stops with application of pressure.

3.3 - WHO GETS AND CAUSES

The cause of hemangiomas is unknown. Usually they are not inherited. They are relatively common, affecting about 5% (1 in 20) of babies. They are not contagious. Hemangiomas occur at least three times more often in females than in males and are more common in premature infants with low birth-weight, twin/multiple pregnancies and with increasing age of the mother.

3.4 - DIAGNOSIS AND TREATMENT

The diagnosis can be made by the appearance of the hemangioma and the history of rapid growth followed by slow regression. Sometimes scans help confirm the diagnosis or to get further information about the extent of the hemangioma. For deeper or less typical lesions, a biopsy (skin or tissue sample) may be required to confirm the diagnosis.

Most hemangiomas will gradually shrink in size for up to 10 years. However, some do not grow much and some do not shrink. When a hemangioma shrinks, there may be no visible mark left behind. Some may resolve with residual blood vessels visible, while others may leave a scar, a pale area of skin or an area of loose skin.

Most hemangiomas do not require treatment except if the hemangioma is particularly large or affects areas where resolution may be incomplete such as around the nose, lips or ears; if the hemangioma is ulcerating; or if the hemangioma is interfering with important functions such as feeding or breathing or the development of senses.

The treatment should be individualized, based upon the size of the lesion(s), location, possibility of complications, potential for scarring, the age of the patient, and the rate of growth or involution at the time of evaluation. Treatment can consist of one or a combination of the use of medications. Topical medications include beta blockers, corticosteroids and imiquimod, while oral medications include propranolol, a beta blocker. Laser treatments and surgery may also be considered.

4 - VASCULAR MALFORMATIONS 

4.1 - OVERVIEW

Port wine stains and salmon patches are included in this wide group.

Vascular malformations are also growths of blood vessels. They also are noncancerous and not contagious. They are also called birthmarks and are present at birth. The main differences between vascular malformation and a hemangioma are: vascular malformations are present at birth, enlarge proportionately with the growth of the child, and do not shrink spontaneously.

There are several different types of malformations and are named according to which type of blood vessel is predominantly affected: only veins – venous malformation; only lymph vessels – lymphatic malformations; both veins and lymph vessels – venolymphatic malformations; either in veins or arteries – capillary malformation; arteries connected directly to veins without any capillaries (small blood vessels) in between – arteriovenous malformation.

4.2 - SIGNS AND SYMPTOMS

The most common symptom of vascular malformations is pain. Depending on the location of the vascular malformation, patients may have other symptoms, such as swelling of the limbs. If the patient has a lymphatic malformation, infection may cause complications. In some cases, arteriovenous malformations are stressful on the heart or cause bleeding complications.

4.3 - WHO GETS AND CAUSES

Little is known about the exact commonness of vascular malforma­tions in the general population. The most common types are capillary malformations, then venous malformations. The least common are arteriovenous malformations. Vascular malformations have no gender predilection. They appear to be less frequent in individuals of African and Asian descent.

Vascular malformations may occur in any body part or organ system. They are most easily identified in the skin and mucous membranes, with some of these lesions invading deep into underlying muscles, bones, or joints. Others are found in visceral locations. Vascular mal­formations can appear as localized lesions of varying sizes, occur within a segmental distribution, or be dispersed as multifocal or disseminated lesions.

4.4 - DIAGNOSIS AND TREATMENT

Vascular malformations are often visible. Superficial vascular lesions can be evaluated with ultrasound, but for deeper lesions ultrasound is limited in the information it can provide. Magnetic resonance imaging (MRI) is the most useful diagnostic tool for evaluating vascular malformations and is useful for assessing the success of the treatment during follow-up.

Regarding treatment, conservative treatment is a good option for patients with mild symptoms. Minimally invasive procedures include phlebography and angiography. Contrast material is injected to aid visualization of the vascular malformation. These techniques are usually performed before treatments such as sclerotherapy and embolization. Although surgical removal is a treatment option, it is not always possible and has a high rate of recurrence.

4.5 - TIPS FOR MANAGING

Noticeable hemangiomas and vascular malformations can cause psychological and social issues. Support groups can help you, your child, and your family.

5 - LINK

EADV leaflet published in 2019, produced by the EADV Pediatric Dermatology Task Force:

- Vascular anomalies in infancy