What are vascular malformations?

Vascular Malformation is a general term that includes congenital problems of the vascular system, which can include arteries, veins, or lymphatic channels.

They are all present at birth, and become apparent at different ages. They can be part of syndromes, or occur spontaneously. Vascular malformations can cause a variety of symptoms, depending on the location in the body.

Vascular Malformations are mostly benign abnormal growths which can be mostly arterial, venous, lymphatic or a combination of any of these types of vessels. While they can be malignant, most of them are not, and they typically cause symptoms based mostly due to the lesion compressing adjacent structures such as nerves, muscles or other organs. They can also cause symptoms due to them taking blood flow away from an area and redirecting it. If they are large enough, sometimes they can cause destruction of blood components due to them being damaged inside of the malformation, which leads to certain types of anemia due to the body filtering the damaged cells.

Venous malformations (VMs) may cause pain where ever they are located. Venous and lymphatic malformations may cause a lump under the skin. There may be an overlying birthmark on the skin. Bleeding or lymph fluid leaking may occur from skin lesions. Lymphatic malformations tend to become infected, requiring repeated antibiotic treatments. Venous and lymphatic malformations may be associated with a syndrome called Klippel-Trenaunay Syndrome.

picv vas

Arteriovenous malformations (AVMs) may cause pain. They are also more stressful on the heart because of the rapid shunting of blood from arteries to veins. Depending on their location, they may also result in bleeding (for example from the bowels, from the uterus, from the bladder). They can even occur in the lung or brain, causing problems that range from heart failure to hemorrhage.

Hemangioma is another common term used for vascular anomalies. However, this name actually applies to a childhood vascular anomaly that has a rapid growth phase between birth and 3 months of age. These often will resolve completely by age 7. The major reason for us to treat these is for low platelets that do not respond to medical treatment, or in the liver because of massive shunting with a strain on the heart.

What causes vascular malformations?

The vast majority are congenital in nature, and typically occur in two age groups, infants and young adults.  In infants, most cases that are externally visible are diagnosed and treated rapidly given their dramatic external appearance. Most people have seen at some point in their lives an infant with a large vascular malformation.

In adults, they are typically not externally visible and much harder to diagnose due to a wide variety of symptoms, and present later in life.

How do I find out if I have one of these vascular malformations?

The vascular malformations that are present in adulthood have typically been present since birth, but given their lack of external signs, only come to medical attention when they produce symptoms which are typically completely vague due to their dependence on location. For instance, the liver is a very common cause of vascular malformations, but only produces bloating with larger lesions. Lesions in the extremities (such as arms or legs) typically cause symptoms related to nerve compression like numbness and pain. Those in the brain or spine can cause headache, numbness, pain, or even sudden death.

Because of the vague symptoms most vascular malformations are found only after other more common causes of their symptoms are ruled out, and some sort of imaging is obtained, such as an ultrasound, CT scan, or MRI.  Often, they are also discovered incidentally when imaging is obtained for other reasons.

How are these malformations treated?

Some vascular malformations respond to medical management such as hormones etc, but the vast majority are treated by endovascular therapy at this point in time due to the low risk profile and excellent clinical results with minimally invasive surgery. The vast majority of these cases are through a small IV, on an outpatient basis, without general anesthesia and with minimal downtime.  Rarely, endovascular treatment is not successful, and traditional surgery is the only option, however, even if partially successful, typically endovascular decreases the amount of traditional surgery needed for treatment when done beforehand.

What are the risks of treatment?

Endovascular treatment typically has the same risks as any other endovascular procedure, such as blood vessel damage, treatment of vessels that supply normal tissue inadvertently, and rarely, malposition of devices if they are used, however these risks are very low in the appropriate hands. In traditional surgery, additional risks are typically associated with the incision and include higher but still low rate of infection, scarring, and other wound issues. Minimally invasive surgery, since an incision is not made, typically has the lowest risk profile of any surgical intervention.

Medical management risks are exceedingly low, and specific for the medication used.

What are the risks of not treating my problem?

In general, vascular malformations are not life threatening, so delaying therapy does not have critical consequences in most cases. They typically progress, usually slowly with time, however persistence of the specific individual symptoms is typically the only real risk. Some malformations in specific locations, such as the liver, are at spontaneous risk of rupture and can cause fatal bleedingbut this is more rare, and your surgeon should be able to tell you if this is a potential problem in  your case. The one exception is malformations of the central nervous system, meaning spine or brain, where rupture is more common, and even smaller malformations should be quickly treated as these can cause fatal bleeding quickly.

Who decides what treatment I need?

The ideal treatment of vascular malformations requires a full team of specialists, including endovascular surgeons, neurosurgeons, head and neck surgeons, and plastic surgeons, all of which are on staff at NYC Surgical. We decide together what should be done for the ideal care of the patient and ultimately the safest and most effective result. Put your problem in the hands of specialists.

To summarize…

Vascular malformations are very individually unique, can be composed of multiple types of vessels, and typically require staged procedures to treat effectively. The symptoms they cause are very diverse, and typically in adults are related to adjacent structures they compress. Most don’t need urgent treatment, with the exception of specific ones located in the brain, spine or certain organs, and typically the decision to treat should be based on patient symptoms in most cases, which should lead to a very thorough patient understanding about their disease process and treatment options prior to treating. Endovascular treatment is extremely effective in most cases, and has a low risk profile.