Coordinated care for children with vascular anomalies and malformations

March 07, 2018

Mayo Clinic goes beyond a multidisciplinary approach to offer coordinated appointments and consultation among specialists for children with vascular anomalies and malformations. At Mayo Clinic's Vascular Malformation Specialty Clinic, patients generally have imaging completed on a Monday, see several specialists as needed on Tuesday morning and receive a plan of care by the end of that day.

"Very few centers have this interdisciplinary approach. Our specialists meet in person to discuss a patient's imaging and evaluations, and to determine a plan of care," says Megha M. Tollefson, M.D., a pediatric dermatologist at Mayo Clinic Children's Center in Rochester, Minnesota, and director of the Vascular Malformation Specialty Clinic. "These vascular disorders are so heterogeneous that they require thorough, comprehensive, holistic care."

Mayo Clinic specialists have experience with a wide range of venous malformations, lymphatic malformations, combined malformations and rare syndromes such as Klippel-Trénaunay's and Sturge-Weber's syndromes.

"Before the visit, based on patients' records and other information they send, we put together a list of which services and physicians they need to see. Usually by noon on Tuesday, the patients have seen all the specialists they need to," Dr. Tollefson says. "Then all of the physicians, including the radiologists, meet to go over the imaging and discuss ideas about how to care for each patient."

Subspecialized expertise in diagnosis and treatment

In addition to specialists in dermatology and interventional radiology, including neurointerventional radiology, Mayo Clinic's Vascular Malformation Specialty Clinic has pediatric orthopedic, vascular and plastic surgeons, as well as neurosurgeons and specialists in ear, nose and throat (ENT) surgery. Other members of the team include specialists in pediatric physical medicine and rehabilitation, pain, lymphedema, and medical genetics. Diagnostic radiologists in the vascular anomalies clinic have subspecialties in neurological, musculoskeletal and ultrasound radiology.

"Depending on the malformation's type and location, it can require entirely different sets of specialists, appointments and procedures," Dr. Tollefson says. "For example, for a patient with a veno-lymphatic malformation of the head and neck, the team might include an interventional radiologist specializing in neuroradiology, an ENT specialist and a dermatologist. For Klippel-Trénaunay's syndrome, the patient might see an interventional radiologist, dermatologist, orthopedic surgeon and specialist in lymphedema."

Pinpointing a diagnosis is crucial to determining treatment. "A lot of patients come to us with a possible diagnosis of an arteriovenous malformation or hemangioma when really they have a venous or a lymphatic malformation," Dr. Tollefson says. "Our imaging expertise allows us to make an accurate diagnosis. Those various diagnoses have very different implications for the patient's prognosis and the type of treatment needed."

At Mayo Clinic, treatment focuses on alleviating symptoms and preventing future complications — while avoiding overtreatment.

"Some providers have a strategy of, 'If it can be treated, it should be treated.' In our experience, that's not necessarily true," Dr. Tollefson says. "Sometimes treatment causes a lot of pain, not just immediately but also later down the road. Overly aggressive sclerotherapy, for example, can injure the skin. That can cause open sores and even damaged nerves. We've seen a lot of that. And some lymphatic malformations that are operated on are likely to recur. The lymphatic vessels underneath the surgical site often ooze and drain for quite some time, which can lead to infections, pain and other complications."

Assessing the risks and benefits of treatment requires experience and expertise. "Every treatment has potential complications," Dr. Tollefson says. "We recommend treatment when we believe there's a very good chance of improving the patient's quality of life. Sometimes the right answer is to treat, but to wait some time to perform that treatment. Every patient and every situation is different, and we take that all into consideration when making our recommendations."

Treatment options might include compression therapy, sclerotherapy or surgery, or a combination of them. "If sclerotherapy or surgery isn't a good option for a patient, our team has been using some cutting-edge laser technology to treat challenging superficial or even internal malformations," Dr. Tollefson says.

Center of excellence for rare disorders

Mayo Clinic pediatric specialists are leaders in treating and researching rare conditions involving vascular anomalies. The Sturge-Weber Foundation has designated Mayo Clinic part of the foundation's Clinical Care Network. The K-T Support Group for people with Klippel-Trénaunay's syndrome holds its biannual meeting at Mayo Clinic's campus in Minnesota.

In addition, Dr. Tollefson has participated in efforts by the Hemangioma Investigator Group to compile treatment guidelines for PHACE syndrome. PHACE refers to posterior fossa anomalies, hemangioma, arterial lesions, cardiac abnormalities/coarctation of the aorta and eye anomalies. As described in the November 2016 Journal of Pediatrics, the guidelines cover immediate and long-term care for patients' neurological, cardiac, ophthalmological and hearing health.

"Vascular malformations can have a profound impact on a child's life," Dr. Tollefson says. "Even if we're not able to offer a cure, our team of specialists is able to offer treatment strategies to improve quality of life."

For more information

Garzon MC, et al. PHACE syndrome: Consensus-derived diagnosis and care recommendations. Journal of Pediatrics. 2016;178:24.