The range of vascular anomalies is extremely broad and variable, and these lesions can show up in any part of the body. For many years, vascular anomalies were a poorly defined entity where diagnoses overlapped and physicians, unsure how to diagnose or specify the lesions, would refer to them in broad terms such as “hemangiomas.” Since the 1990s, there have been efforts to better define these anomalies, characterize them and target their care.
Because of the ambiguity, broad range and scope of vascular anomalies, multiple specialists are often involved in treatment. At my institution, we have a multidisciplinary care clinic that has evolved to encompass specialties from many pediatric departments. These departments work together to create longitudinal care plans, often with interventional radiology as an integral specialty.
Creating a team
When I joined the Children’s Hospital of Philadelphia (CHOP) in 2015, the vascular anomaly service was run by David Low, MD, an accomplished plastic surgeon collaborating with interventional radiologists, (Anne Marie Cahill, MD, FSIR, and myself) to conduct a bidisciplinary clinic service staffed by surgery and IR. However, there were numerous other specialties that could potentially be required for the treatment and management of a patient’s lesion, but were not involved in that clinic. For example, if a patient needed to see an ear, nose and throat (ENT) specialist, they would be referred separately to that physician and undergo treatment outside of the clinic—so while there were collaboration opportunities, it was not truly multidisciplinary.
In 2020, CHOP hired an oncologist, Denise Adams, MD, who specializes in the management of vascular anomalies. Concurrently, there was an initiative to set up a “frontier program”—a special undertaking that utilizes hospital funds to provide start-up services including research and clinical care for a subspecialty service line.
The goal of the vascular anomalies frontier program at CHOP was to establish a multidisciplinary clinic that employed hematologist–oncologists and functioned in collaboration with a wide range of specialists—IR, DR, plastic surgery, ENT, orthopedic surgery, general surgery, occupational therapy, physical therapy, psychology and genetics. The clinic is 1 full day a week, and patients will come from all over—even internationally—to be seen at our center.
Though not all specialists are present in the clinic at one time, this program has allowed streamlined care for our patients.
The need for collaboration
As stated, the scope and variability of vascular anomalies requires that multiple specialties play a role in treatment. For example, if a lymphatic malformation involves the airway, ENT is usually required as a consultant. Some superficial lesions involve the skin, which necessitates the involvement of dermatologists. Oncology has become core to the management of vascular anomalies because we have found that the molecular drivers of these lesions are very similar to those in tumors, due to similar mutations and signal transduction pathway changes. This led to the discovery of the application of oncologic medications to the treatment of vascular anomalies, particularly with very diffuse and morbid lesions. In these cases, standard interventions or surgery will not be feasible for bringing these lesions under control. However, once we find a mutation in the lesion, systemic targeted therapy has shown excellent results in quite a few cases.
The wide range of vascular anomalies means that while one lesion can be very focal, another can be very diffuse and affect numerous areas in the body, causing multiple types of morbidity. Effective management for extensive lesions requires not only interventional specialties, but also physical or occupational therapy. In addition, some of the more severe anomalies can have pervasive psychosocial impacts on patients and families, which is why social work and sometimes child psychologists will be involved as well.
The role of IR in vascular anomalies
At my institution, IR is deeply involved in the vascular anomaly clinic because we are one of the core specialties that manages vascular anomalies. This is because many IR techniques and technologies are well suited for treating vascular lesions. While a lesion on the musculoskeletal system would be the purview of an orthopedic surgeon, and a lesion on the airway would be the domain of an ENT surgeon, IR can be involved in the management of these lesions regardless of location. Although surgery can play a role in the management of these patients, the minimally invasive nature of IR therapies often makes our techniques more attractive.
When we first see a patient in clinic, we assess them by going through the imaging and performing a brief physical examination, make our best judgment on the diagnosis and management, and present it to the multidisciplinary team. We also run a clinical conference each week to discuss patient care and recommendations. Sometimes, based on the lesion and presentation, we may elect not to do any procedure. Sometimes, if the diagnosis is unclear, we will biopsy a lesion.
If the diagnosis and benefits of procedure are clear, we will treat the lesion through several different therapies. Sclerotherapy is used largely for slow-flow vascular anomalies. These are anomalies that predominantly have veins or lymphatic channels in them. For high-flow lesions, we will embolize. Certain vascular anomalies are amenable to cryoablation, which is sometimes more effective than sclerotherapy or embolization.
After performing a standard evaluation for the management of the patient in clinic, we will schedule a date for them to return and undergo the procedure, often with anesthesia. Patients return to our personal clinics for the procedure and follow-up and we make our next recommendations based on the outcome of the initial intervention.
The collaborative spirit of pediatric care
While the treatment of vascular anomalies utilizes guidance from many other specialties, it’s not unusual—especially at a pediatric hospital—for collaborative care to be the standard across many service lines. For example, IR is part of the hepatobiliary service line alongside the liver transplant team, gastroenterology and surgery. Thyroid patients will often be seen by IR, endocrinology and thyroid surgeons. Even our motility service, consisting of gastroenterology subspecialists and surgeons, will send patients to IR for cecostomy and other forms of enteral access. Almost every service line within the pediatric system ends up being multidisciplinary to various degrees.
Perhaps this collaborative spirit is easier to achieve because it is part and parcel of caring for children—following the credo that “it takes a village.” Pediatric specialties are supportive of each other, in my experience. Parents are often very involved in their child’s care plans and become well educated on the various specialties that may be involved, and so they often expect a high level of collaboration. This expectation enables physicians to work together more easily. As a result, we become united in care, right alongside the family of our patients, and the collaborative spirit follows.
