Capillar malformations are commonly known as flat haemangiomas.  As their name implies, they affect the capillars (very thin and small vessels which link arteries and veins through the tissue). They are also called Port-Wine Stains. The port wine stain presents itself as a birthmark of variable size, with a well defined border, where the color varies between light pink and deep purple. It is composed of numerous abnormal capillars. 

The flat angiomes are often confused with Naevus Flammeus or Nauvus de Unna. Over 50% of of the newborns have in fact a small red birthmark at the back of their neck or in the forehead between both eyes. These common anomaly is of no concern and dissapears soon after birth, at most at the age of 2. However a flat haemangioma never dissapears.  

What is the prevalence of flat hemangiomas?  

About 0,3 to 0,5% of caucasians are born with a flat angiome. Male and females are affected in same proportions. It is the most common of all vascular malformations. 

What causes flat hemangiomas?

The root cause is still unknown. They are linked to a wrong development of capillars during pregnancy. Recent research points to a genetic defect on the control of muscles of arterial and capillarie walls. There is so far no known link with the use of any drugs or consumption of food during pregancy having a relation with flat haemangiomas. 

When do flat hemangiomas appear?

They are always present at birth, however the may not be visible until after a some days. They can not be detected by ecography during pregnancy because they are not malformations in relieve.

Where in the body are they localized?  

The face, neck and skull are the main areas, with up to 85% or 90% of the cases. But they may also appear in any other part of the body. If the malformation is capilaire, it is usually unique, however it may be large, sometimes extending over half of the body. 

What is the most common evolution?

At birth, they are normally pink. It will continue growing proportionally with the child. The appearance becomes darker with time. During adolescence they may become dark red. At adulthood, the flat angiome becomes of red wine color.

Evolution depends from one person to another. Not all flat angimes evolve with time and age, however a number of them will degrade if they are not treated. Common problems found are increased skin thickness with its texture also affected. Sometimes these changes in thickness happen when the individual is in their twenties, but more commonly it is after thirty or more years.  

When should I consult with a specialist? 

Most flat angiomes are anomalies that only affect the skin. In some instances however, they may be also a symthom of something more serious. It is threfore recommended to get an examination, which in some cases may require a Doppler Ecography, or an MRI to verify that under the flat angiome there is not a vascular anomaly more serious.

When the flat angiome extends over the forehead or the eyelids, or close to the eyes in general, it is recommended to consult with a specialists as soon as possible to verify if it  could be associated to Sturge-Weber Syndrome. Similarly, if the flat angiome covers the center of the back, an specialist must be consulted to rule out a more complex vascular anomaly underneath.

What are the possible complications with a flat hemangioma?

Apart from the dark red color and thickness of the skin, sometimes it is possible to see inflamation and potential episodes of bleeding. Also, the tissue affected by the flat angiome may overgrow, aggravating the aesthetic implications.   

How are they diagnosed?  

The clinical examination by an specialists – often times a dermatologist – is usually sufficient. This kind of capilar malformation is never hot when touching it, or thrilling. Its surface is flat, unlike on hemangiomes. In general, a flat angiome does not present pain. 

What are the options for treatments? 

The treatment with color pulse laser (vBeam Perfecta) is usually the best choice as it has less risk of leaving scars. Normally multiple sessions are required. In most cases, the flat angiome regrows: therefore the treatment must recommece.  This treatment can be painfull, so it may require anesthesia. For children, some practicies prefer to have the child sleeping, while others only consider local anesthesia. 

Laser treatment has as objective to diminish the coloration of the angiome. Its efficacy varies, as can be seen from statistics published on birthmarks.com:

• 10% to 15% of patients see color becoming normal between 75% and 100%
• 70% to 80% see lighter skin coloring between 25% and 75%
• 10% to 15% see only a small attenuation of color between 0% and 25%

In general, facial areas respond better to treatment than the rest of the body. There are other factors affecting the treatment success, such as skin color, age, and the size and depth of the angiome. Doctors have differing opinions in terms of what age is convenient to start the treatment. There is no consensus yet on the convenience of an early age start. 

Syndromes associated to flat hemangioma 

If in most cases the flat angiome is well localized and stable, sometimes it is only a symthom of a more complex pathology. 

The Sturge-Weber Syndrome presents a capilar malformation affecting the forehead, the upper eyelid, the nose and the existence of an angiome within the brain (affecting the meninges) and at the eye level. It is for this reason that those affected with this syndrome may suffer neurological problems (including epylepsia) or have a high pressure inside the eye (leading to glaucome). Convulsions usually affect the side of the brain opposite to where the angiome is located. Anti-epylepsy drugs can help in these cases. The patient may also suffer a degree of hemiplegia and motricity or cognitive development delays. Children affected by a Sturge Weber Syndrome require a follow up by various diverse specialists: pediatricians, neurologist, oftalmologist and dermatologist. In some cases not all symthoms described are present, sometimes for instance the facial angiome is not visible. The Sturge Weber Syndrome is not hereditary and affects one person in 50,000.

The Klippel-Trenaunay (K-T) Syndrome presents the hypertrophia of a member and a combined and complex vascular malformation. Individuals affected by the K-T syndrome often present scattered capillary malformations, lymphatic malformations which often cause edema and venous anomalies. For 90% of the cases the affected member is in the lower part of the body. In typical cases the extension goes from the hips to the toes.  

The complications of the Klippel-Trenauney Syndrome are notably hemorrhagia and may involve internal organs: for instance rectal or vaginal bleedings. Patients may suffer anemia or see their level of platelets affected, therefore often require iron suplements.  Venous trombosis are frequent, and certain skin infections may happen and become a source of trouble, often time requiring repeated treatment with antibiotics. Finally, the hypertrophy associated to the affected member may limit is functionality and mobility. Pain is another major problem on these patients. Sometimes, using compressing clothing may provide some relief. On some patients sclerotherapy (injection of sclerosant products) is used to obtain venous blood coagulation. However, venous ablation which was a common treatment in the past, is less used now. In any case, any such intervention must be considered in the most prudent way. In cases where hypertrophia is very severe, amputation of the toes or using surgery to reduce the size of the member may be required, even if this is rarely the case.

The Parkes Weber Syndrome involves a diffuse arterio-venous malformation affecting a member with an augmentation of its size in both length and volume. Because a capillar malformation is also present, it is always possible to make a wrong diagnosis. The hypertrophia of the member combined with arterio-venous shuts make this syndrome very serious. When the blood flow drawn by the arterio-venous malformation becomes very high, the heart must perform an extra effort, which increases the risk of cardiac arrest. 

The Cobb Syndrome is characterized by visible skin lesions with underlying spinal angiomas or arteriovenous malformations (AVMs). The intraspinal lesions may be angiomas or AVMs and occur at levels of the spinal cord corresponding to the affected skin dermatomes. They may in turn produce spinal cord dysfunction and weakness or paralysis. Potential complications include: hypertrophia of the members, ulcers, sensorial impediments, and mobility troubles. 

The Proteus Syndrome causes an overgrowth of skin, bones, muscles and other tissues on half of the body. It often has associated vascular anomalies (capillary, venous or lymphatic)Si dans l'immense majorité des cas, l'angiome plan est isolé localisé et stable, il est parfois le marqueur d'une angiomatose complexe.