Comparison of Hemangiomas and Vascular Malformation
Hemangiomas
Vascular Malformations
Midline Venular Malformations
Venular Malformations
Sturge-Weber Syndrome
Venous Malformations
Lymphatic Malformations
Arteriovenous Malformations
Klippel-Trenauney Syndrome (KTS)
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Comparison of Hemangiomas and Vascular Malformations

Hemangiomas

• 30% are visible at birth
• Color varies from red – superficial, to blue/purple if deeper
• Growth – grows intermittently through the first year of life then stops growing and variably involutes (shrinks).
• Incidence – 3-5 time as common in females, also more common in premature babies and twins

Vascular Malformations

• Always present at birth, but may not be apparent until months/years later
• Color depends on type
  • Midline venular malformations – light pink/red and always superficial
  • Venular Malformations (port wine stains) – vary from pink to darker purple and always superficial
  • Venous – purple if superficial, light blue to colorless if deep
  • Arteriovenous – reddish stain frequent of overlying skin with small telangiectasias (superficial blood vessels)
  • Lymphatic malformation – frequently colorless but may have little vesicles which resemble fish eggs, commonly in the mouth/tongue or in area of previous excisions.
• Growth – tends to grow and expand throughout life, lymphatic malformations may swell with infections. Do not involute.
• Incidence – no gender preference

Hemangiomas

It is common to have all vascular anomalies called hemangiomas but they are not. You may hear these lesions referred to as capillary hemangiomas or strawberry birthmarks. There are actually two types of hemangiomas, infantile hemangiomas and congenital hemangiomas.  They are similar in that they don’t tend to grow throughout life but there are some unique properties to each as well.  Infantile hemangiomas are tumors containing small collections of abnormal blood capillaries which have been found to resemble placental tissue.  They are vascular birthmarks that usually appears within the first few weeks of birth, some may even be seen as a small red mark or bruise at birth.   They then grow intermittently, and sometimes quite rapidly, throughout the first 10 -12 months of life.  After a year of age there is usually no more growth and the hemangioma starts its involutional phase.  In this phase, the hemangioma can shrink, and lighten in color or it may not appear to do anything.  Depending on how large the hemangioma has grown, this shrinking is frequently not enough to make the lesion “go away”.  In fact a large percentage of hemangiomas will require some form of intervention to correct the deformity that has been caused by growth of the hemangioma or to correct scarring caused by ulceration.   For this reason, there are several treatment options that are available to us to help control the growth as much as possible. 

The second type of hemangioma is a congenital hemangioma.  These are hemangiomas that are fully formed at birth.  They usually don’t grow any more but they may or may not involute (shrink).  There are two types of congenital hemangioma, the RICH or rapidly involuting congenital hemangioma and the NICH, non involuting congenital hemangioma.  Like each of their names imply, one tends to go away rapidly and the other doesn’t really change at all.

Infantile hemangiomas can be classified by the areas they involve.  Superficial hemangiomas involve skin only.  Deep hemangiomas involve the layers of tissue below the skin and can vary in color from deep purple/blue to skin tones if they are really deep.  Compound hemangiomas involve both the skin and deeper tissues.

There are many treatment options for hemangiomas and there are several things that we consider when we are deciding a correct management for a given patient.  Observation is one form of treatment and is reserved for those hemangiomas that do not affect function, such as vision, or those that remain relatively small.  Certain medicines can also be used to control the growth of hemangiomas including oral steroids, steroid injections and even some chemotherapeutic agents for more aggressive or life threatening situations.  Lasers can be used to treat the superficial portions of the hemangiomas and finally sometimes surgery is required to correct disfigurement and restore normal function to the area affected by the hemangioma.  Each hemangioma is different and a recommendation in each child’s case is dependent on consultation evaluation and not all treatment options are appropriate for every hemangioma.  Our specialists, on the vascular anomalies team, offer all types of treatment for hemangiomas and the recommended treatment will fit the special case that each child presents.

Vascular Malformations

There are many differences between hemangiomas and vascular malformations.  Hemangiomas, made up of capillaries, tend to grow throughout the first year of life and then regress.  Vascular malformations are made up of arteries, veins, or lymphatic channels depending on the type of the malformation.  Vascular malformations also tend to grow throughout life and usually will require some type of intervention.

Midline Venular Malformations

Midline venular malformations are quite common and may be referred to as capillary malformations, stork bites or angel kisses.  They are usually light to medium pink in color and are present on the middle of forehead or on the back of the neck, however they can also appear on the eyelids and between the eyes.  These lesions usually gradually fade over the first couple of years of life and only rarely persist or darken.  If the area is still present into later childhood, laser treatments can be done to help the area fade and go away.

Venular Malformations

These lesions are also known as port wine stains and can occur anywhere on the body.  They are frequently present at birth and start off as a flat pink or red lesion.  They tend to grow with the patient and darken with age.  Some of these port wine stains will eventually form small lumps on the surface called cobblestones.  It is felt that port wine stains occur from a lack or deficiency of the nerves that go to the blood vessels to control flow.  Currently, there is no cure for port wine stains but laser treatment can be a very effective way to lighten the port wine stain and in many cases make is virtually disappear.  The laser treatments frequently need to be continued intermittently into adulthood.

Sturge-Weber Syndrome deserves special mention.  Its characteristics are a port wine stain that involves the skin around the eye and cheek as well as the covering of the brain; seizures; atrophy of the brain tissue; and developmental delay.  Early diagnosis is important to allow control of seizures and monitoring of eye pressures by an ophthalmologist to help preserve vision. 

Venous Malformations

Venous Malformations are made up of malformed veins and can involve both skin as well as deeper structures.  They vary in color from blue to dark purple, depending on how deep the malformation extends and can be very localized or more diffuse.  Common areas to find venous malformations are the cheek, jaw, lips and in the mouth.  They tend to swell with activity/exercise or if the malformation is in the dependent position (ie when lying down).  The mass is usually soft and compressible and then refills when released.  There may be small hard masses palpable in the malformation, these are called phleboliths and are small collections of calcium that have resulted from slow blood flow and blood clots.  There are many treatment options for patients with venous malformations including laser treatment for surface (skin or mucosal) lesions, and sclerotherapy (injecting the area with an irritant to help clot and shrink the area)  and/or surgery for deeper lesions.  Each patient is different and recommendations for treatment will vary depending on the lesion and the patient’s symptoms.

Lymphatic Malformations

There are many names for these lesions including lymphangioma and cystic hygroma but the correct term is lymphatic malformation.  They are made up of abnormal, dilated lymph channels that can be focal or diffuse. They are frequently located in the head and neck area (70-80%) and many of them are present at birth.  Lymphatic malformations can be very large and when present in the neck area, frequently cause airway obstruction and may require tracheostomy.  Lymphatic malformations tend to increase in size with infection such as upper respiratory infections and frequently will require a course of steroids to help them shrink back down.  When malformations involve the inside of the mouth, vesicles (resembling fish eggs) may form and they can be filled with clear fluid or may be filled with blood.  When this occurs, it is common to have pain when eating acidic or salty foods, as well as bad breath and bleeding.    An MRI can be very helpful in confirming the diagnosis of lymphatic malformation as well as define the extent of disease.

Lymphatic malformations are difficult to cure if they are diffuse (affecting more than one small area).  The goal of treatment is to help the patient maintain function such as swallowing and speaking, and also to help look as normal as possible.  Intermittant steroids and antibiotics are used to help control the growth especially when associated with infections.  Lasers can be used to treat the tongue vesicles and help eating become normal. Lasers are also used to help clear malformation from the airway.  In many cases, surgery is required to remove malformation and if the malformation is in one localized area, it can be an effective cure.  If the malformation is diffuse, many surgeries may be required to control the disease, but it can be very successful.  Lymphatic malformations can also be treated in some cases with sclerotherapy usually to help shrink and eliminate large cysts.  Lymphatic malformations present a challenge to both the physician, patient and their family and with a multidisciplinary team approach, we individualize the care that is best for each patient.

Arteriovenous Malformations

Arteriovenous Malformations (AVMs) involve an abnormal connection between arteries and veins that are present from birth but are frequently not noticed until months or years later.  They are also frequently located in the head and neck area but can occur anywhere in the body.  AVMs have a variable growth pattern with some growing very slowly to others that are more aggressive with rapid expansion.  Patients will frequently notice a throbbing pain and growth/thickness of the area involved.  In many cases, the overlying skin has a reddish/pink stain and sometimes patients complain of bleeding from the area of the malformation.  When bleeding occurs it can be quite brisk and may require medical attention.  Also on physical examination, a pulsation or thrill may be felt. Angiogram is the most helpful tool in determining the extent of the arteriovenous malformation and is used frequently in preoperative planning.  Embolization is also a tool which can control areas of bleeding as well as a presurgical tool to decrease the amount of blood loss during surgery.  Surgical removal is usually necessary and can be extensive in some cases.  Lasers can be used to treat the skin portion of the birthmark but will only be effective after the deeper portions of the malformations have been removed.

Klippel-Trenauney Syndrome (KTS)

The characteristics of this syndrome are a mixed venous-lymphatic malformation usually involving the extremities.  There is usually a port wine like stain on the affected limb and there is usually a difference in size between the affected and nonaffected limb, the affected one being larger.  The growth tends to be slowly progressive and can be painful with cramping.  There is currently no cure for KTS, and treatment is centered around preventing progression and controlling symptoms.  Compression garments are important as well as a regimen of physical therapy, with swimming a good activity to exercise the limb and improve circulation while minimizing swelling.  Laser treatments can be done to treat vesicles that sometime form on the skin and surgery is not curative but can be recommended in some cases.

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