Clinic Guides Complex Treatment of Vascular Anomalies
Julie Monteagudo, MD and Debra Watson-Smith, RN give an overview of the conditions treated at the Vascular Anomalies Clinic (VAC) at Hasbro Children’s Hospital. Dr. Monteagudo is medical director of Pediatric Vascular Anomalies Service, and Watson-Smith is the clinic's nurse coordinator.
Stork bites. Salmon patches. Strawberry marks. Port wine stains. These are just a few of the names people give to a very common group of conditions called vascular anomalies.
They can be seen as circumscribed, bright red, raised areas on a baby’s skin, or large, purplish blots in older children and adults. They can present as large soft-tissue lesions in the armpit or the neck, without skin discoloration.
Rarely, vascular anomalies are very large, impairing function – as when an entire leg or arm is involved. Although the vast majority of these lesions have a benign course, their appearance can sometimes cause anxiety or distress – and in very rare cases, they can be life-threatening.
What all these lesions have in common are abnormal blood vessels or lymphatics. The network of blood- and lymph channels is supposed to be composed of tubular structures that branch out as they become ever narrower, ending in capillaries that bring oxygen, nutrients, and immune proteins to all the tissues in our body – and carry carbon dioxide, waste products, and other substances back to the liver, the heart, and the lungs.
Sometimes, there is a glitch. Instead of straight, narrow tubes, a small segment of blood or lymph vessels develops into a cluster of irregular cavities and lakes where blood stagnates or flows turbulently in whirls and eddies.
If these structures are located immediately under the skin, they may appear as rubbery, raised, and red skin lesions. If they are deeper, only an ill-defined, spongy swelling may be noted, sometimes with a bluish hue to it. If they are deeper still, inside an organ, they may not be noticed at all.
In vascular anomalies that are mainly composed of abnormal lymphatic channels rather than blood vessels, there is no skin discoloration, although minor trauma may cause some bleeding into the cavities, resulting in a sudden enlargement and change in color resembling a hematoma.
In the past, these lesions were often grouped into broad categories and went by various names –hemangioma, cystic hygroma, cavernoma, lymphangioma – confusing terminology that did not take into account the etiologies of these lesions. More recently, attempts have been made to classify all vascular and lymphatic anomalies based on their appearance, behavior, and pathology.
A Unified Taxonomy
The International Society for the Study of Vascular Anomalies (ISSVA) has held regular consensus conferences to come up with a unified taxonomy so that clinicians and scientists around the world can compare apples to apples and strawberry marks to strawberry marks.
With a better understanding of their epidemiology, natural history, microscopic appearance, and genetic factors, vascular anomalies can now be categorized as tumors or malformations.
Vascular tumors, which are virtually always benign (especially in children), are lesions that follow a specific growth pattern: they are usually not present at birth but appear in the first few weeks of life. They go through a proliferative stage and can become quite large. Then, around 6 months, they typically stop growing and start a process called involution: little by little, areas within the lesion become paler as microscopic blood vessels become obstructed and blood flow decreases. Over the next months to years, the lesions tend to disappear completely – sometimes leaving little more than a small skin tag. These lesions are called hemangiomas, and they are by far the most common vascular anomalies in young children.
Hemangiomas are most commonly seen on the skin and can present anywhere on the body. They can also occur in deeper structures, such as the liver or the trachea. While they are often solitary, the presence of multiple skin hemangiomas can be an indication of other, deeper lesions.
Because hemangiomas are usually asymptomatic and tend to disappear completely over time, the most common approach is close observation, without the need for more aggressive intervention. However, a small percentage of hemangiomas (particularly those in the liver and other organs) can lead to severe and sometimes life-threatening complications.
Infants with very large liver hemangiomas can be at risk of severe heart failure, bleeding problems, and breathing difficulties as the enlarged organ pushes on the diaphragm and impairs lung expansion. Even small lesions can be dangerous if they are located in critical areas: a small hemangioma in the trachea is at risk of bleeding with minor trauma, threatening the airway.
Importantly, not all vascular anomalies are hemangiomas that disappear spontaneously over time. Some lesions are considered malformations – not tumors that grow bigger, but structural errors that occur during early development, causing blood or lymph vessel anomalies. These lesions are present from birth, although they may not become obvious until later in childhood, or even adulthood. If they are large, or are located in critical areas, they can significantly impair function or cause psychological damage because of their appearance.
It is clear that these very disparate groups of anomalies cannot all be managed the same way – or by one specialist. Decades ago, few options were available for the treatment of vascular anomalies. If observation alone were not enough, only surgical excision could be offered.
For large lesions, this could be a risky undertaking because of the amount of blood loss or the difficulty of removing large sections of an organ – and it often led to unsightly scars or debilitating resections. Today, technological advances, scientific discoveries, and medical progress have paved the way for a much more calculated approach to vascular anomalies, one that can be fully tailored to the patient and the lesion.
Vascular Anomalies Clinic
This approach requires a large multidisciplinary team of specialists who each bring their particular skills and knowledge to the table. Such a resource is the Vascular Anomalies Clinic (VAC), an interdisciplinary team caring for both children and adults at Hasbro Children’s Hospital and Rhode Island Hospital that offers state-of-the-art therapy through a vast array of treatment modalities. Very few children’s hospitals around the country offer this degree of expertise, and fewer still have a “one-stop-shopping” approach that offers all the experts and all the types of treatment under one roof.
The Vascular Anomalies Clinic is a state-of-the-art interdisciplinary consultative program that offers counseling, diagnostic evaluation, and a wide array of treatment modalities to children and adults with complex vascular and lymphatic lesions.
The clinic, which has been active at Hasbro Children’s Hospital for a decade, comprises specialists in pediatric surgery, diagnostic imaging, dermatology, plastic surgery, otolaryngology, hematology, cardiology, and physical therapy. We also have a dedicated nurse coordinator who works directly with patients, their families, their referring doctors – and insurance companies – to make the experience as effective and hassle-free as possible.
Patients are initially seen in the clinical office by all involved specialists. Thorough patient and family history and physical examination can help determine the nature of the lesion, and whether there is a genetic or hereditary cause. The optimal diagnostic plan is drawn up, as a large array of advanced imaging techniques is available, from Doppler ultrasonography to contrast-enhanced magnetic resonance imaging and lymphangiography – as well as a variety of endoscopic procedures.
Our team holds a collaborative monthly conference to discuss patients’ cases and formulate a path forward.
After a consultation at the Vascular Anomalies Clinic, we provide recommendations for treatment and a referral to the appropriate specialist for an in-depth conversation about the suggested therapeutic plan. In addition, the clinic can provide long-term consultative care.
It is important to note that some of these lesions cannot be completely removed – and that, because these are mostly benign conditions, radical interventions would be contraindicated. But while “cure” is not always achievable, the Vascular Anomalies Clinic is there to help patients, families, and providers find the optimal approach to achieve the best functional and cosmetic results.
Thirty years ago, the only treatment modality for vascular anomalies was surgical removal. Today, the possibilities are manifold. Medical treatment, using promising new drugs, can be effective in some cases. If an intervention is warranted, it does not have to be a wide surgical resection: using interventional radiologic techniques, microcatheters can be snaked up the feeding vessels to obliterate the lesion, leaving not even the smallest of surgical scars. Other lesions can be injected with sclerosing agents that will eradicate them, using endoscopes and other minimally invasive techniques. If a larger resection is warranted, working with our plastic surgery specialists can help prevent unsightly scars and deformities.
The Vascular Anomalies Clinic accepts referrals and can be reached at 401-228-0557. Referrals may also be faxed to 401-868-2309. The VAC coordinator, Emily Boudreau, can be reached at [email protected].
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