Unique multidisciplinary treatment for vascular malformations at UNC Health Care

Vascular interventional radiologist Joseph Stavas, MD, professor of radiology in the UNC School of Medicine, and clinician in the UNC Center for Heart and Vascular Care, works with UNC physicians from 14 different departments to provide comprehensive care for vascular malformations.

Unique multidisciplinary treatment for vascular malformations at  UNC Health Care click to enlarge Joseph Stavas, MD
Unique multidisciplinary treatment for vascular malformations at  UNC Health Care click to enlarge Darlene Pitcher, patient in the UNC Hemangioma and Vascular Malformations Clinic

Darlene Pitcher of Wilson, NC has been suffering from a congenital vascular malformation since birth.  Her right arm, shoulder, and chest are covered in red and purple welts, causing her chronic pain in both her arms, reduced ability to move, and migraines.  After seeking treatment from physicians in Boston, Colorado, and Minnesota, Darlene had given up hope that she would ever be pain-free.

Enter the exceptional multidisciplinary team that makes up the UNC Hemangioma and Vascular Malformations Clinic.  Vascular interventional radiologist, Joseph Stavas, MD, professor of radiology in the UNC School of Medicine, and clinician in the UNC Center for Heart and Vascular Care, is one of the physicians leading the way on this extraordinary effort to treat patients with vascular malformations.

More than five years ago, Dr. Stavas began working regularly with other physicians at UNC on hard-to-treat cases such as Darlene Pitcher.

“Patients such as Darlene may come to UNC for what we think is only a vascular malformation, but once we begin treatment, we realize that a multidisciplinary approach is crucial to treatment and a successful outcome,” says Dr. Stavas.

The UNC Hemangioma and Vascular Malformations Clinic

The UNC Hemangioma and Vascular Malformations Clinic is unique in that it’s the only clinic in the Southeast to bring together specialists from 14 medical departments—both pediatric and adult—on a regular basis to meet and discuss treatment options for patients with vascular malformations. All of the physicians are located on the UNC Health Care campus in Chapel Hill, allowing for a seamless referral from one specialist to another.

The departments partnering in this endeavor include many pediatric specialties, since vascular malformations are present at birth: Hematology/Oncology, Dermatology, Plastic Surgery, Cardiology, Pulmonary, Gastrointestinal, Neurosurgery, General Surgery, Urology, Head and Neck, Anesthesia, Genetics, and Orthopedics.

Adult specialties in the Clinic include Vascular Surgery as well as Cardiology, Gastrointestinal, Orthopedics, Interventional Neuroradiology and Genetics.  Adult and pediatric Radiology is also an active partner as vascular interventional radiologists and pediatric radiologists provide much of the data needed by the physicians to accurately treat these patients.

What are vascular malformations?

Vascular malformations are abnormalities of the blood vessels: veins, arteries, lymphatics, or some combination. Most are benign conditions present at birth, but some may progress to create problems later in life.

The vascular malformation known to most people are hemangiomas.  Hemangiomas are present at birth, but sometimes are not visible right away.  They are often known as “strawberries” or “stork bites” due to their pinkish tinge.  However, while hemangiomas are a type of vascular malformation, most disappear over time with no medical treatment, and those that don’t disappear, can be treated by physicians in various ways to completely eliminate them.

There are many other vascular malformations with far more serious complications.  Patients with capillary, lymphatic, arterio-venous , venous, or combined vascular malformations may have multiple health problems stemming from this, needing the services of multiple specialists over a variety of medical disciplines.  The malformations may cause blood clots, pain, heaviness, disfigurement, and bleeding.

Capillary malformation (CM) is a common vascular birthmark also known as a port-wine stain because of the pink/red/purple discoloration of the skin. Capillary malformations can often be treated with laser therapy.

Lymphatic malformation (LM) is an abnormality of lymph channels that normally carry clear fats and proteins back to the bloodstream and helps remove bacteria. Malformed lymphatics form large, soft masses, usually in the head and neck, armpit, or groin area.   They may require injection sclerosis or surgical removal.

Arterio-venous malformations (AVM) are large, abnormal connections between arteries and veins. AVMs cause pain, skin breakdown, overgrowth of tissue, and even congestive heart failure, and can be shut down by catheter embolization and surgery.

Venous malformations (VM) consist of enlarged, sponge-like channels in and around the veins.   They may become large over time and grow after puberty, trauma, or an infection.

Combined vascular malformations are complex, involving more than one type malformation. These can be associated with overgrowth of bone and soft tissues, usually in a limb or on the trunk of the body.

Multidisciplinary care at UNC for hemangiomas and vascular malformations

When a vascular malformation is diagnosed at UNC, the physician team of the Hemangioma and Vascular Malformations Clinic work together to identify each area of the body that is affected by the malformation.

Patients often call UNC directly for a referral or, more than likely, they have already started treatment for their hemangioma or malformation in one of the many UNC clinic areas that treat these problems.  If the patient’s physician is not part of the Clinic team, the UNC clinicians know to contact a Malformation specialist so the patient can be fully evaluated.  Once the patient is identified with a hemangioma or malformation, the magic of UNC teamwork begins.

“For example,” says Dr. Stavas. “If a child is seen in the pediatric clinic with a symptomatic vascular anomaly, then Interventional Radiology, Surgery, and Genetics are called, in order to provide additional input about the best treatment option.”

The Clinic team makes sure they accomplish this first consultation on the same day the patient is identified, in order to reduce the number of return visits. The physicians then discuss the patient’s needs, and within a week or less, a treatment plan is devised.

Dr. Stavas points out, “The diagnosis and treatment of malformations is complex and confusing.  Patients often get lost in the complexity of their problems or health systems that try to take care of them. It can be like a tangled net to sort out.”

He adds, “We remove frustrations and begin the education and treatment processes toward a cure.”

(Pictured left to right) Julie Blatt, MD, Cynthia M. Powell, MD, Craig Burkhart, MS, MD, and Joseph Stavas, MD, lead the way in treating vascular malformations as the founding clinicians in the UNC Hemangioma & Vascular Malformations Clinic

Physician photo malformations clinic

UNC has the most comprehensive team of care providers for malformations in the Southeastern United States.  They have physicians, mid-levels, and ancillary staff - physical therapy, occupational therapy, lymphedema specialists, x-ray technologists and nurses – who are aware of the many appearances of vascular anomalies and the multitude of treatment options available.  These providers combine all elements of care in the patient’s treatment plan, including medical/surgical management, research, and teaching.

Recognizing UNC’s expertise, the North Carolina Division of Medical Assistance, which is part of the NC Department of Health and Human Services, asked UNC to become the state referral facility for vascular lesions in 2008.

“Our center is able to diagnosis malformations properly, treat symptoms when appropriate, and keep patients and their families abreast of the latest research developments,” says Dr. Stavas.  “We also provide support groups and genetic counseling when necessary.”

While all treatment plans for patients at UNC are individualized, taking age, symptoms, the type of malformation, and expected future issues into consideration, each discipline within the Hemangioma and Vascular Malformations Clinic uses their expertise and experience in a specific way.

Pediatric Hematology/Oncology determines if any medicines are appropriate to use. They evaluate for clotting problems, work to determine the correct diagnosis, and create a plan to manage any other medical problems.  They also refer to a UNC medical home, if necessary.

Dermatology focuses on determining the type of malformation, and if there is an associated skin lesion, a skin biopsy may be included.  They create an individualized treatment plan for skin-related birth marks, including, as needed, medications, laser therapy, or surgery.

Genetics tests to see if there are inheritable causes for the malformation and if the malformation is part of a syndrome.  They assess the risk of inheritance to other children in the patient’s family.

The Adult and Pediatric Surgical teams, which include General surgery, Plastics, Orthopedics, and Head and Neck, perform surgical removal of the malformation when appropriate and obtain tissue for biopsy.  Any surgical procedure typically works in tandem with other forms of treatment.

Adult and Pediatric Anesthesia assists with special needs for airway control and anesthesia during procedures.

There are some pediatric subspecialties that assist in the management of symptoms related to malformations in children that may affect certain organs. Pulmonary, Gastrointestinal, Cardiology, and Renal specialists are on the team to provide support and treatment when a patient’s individualized plan indicates the need.

Interventional Neuroradiology provides intervention in the neck and brain for arteriovenous malformations.

Vascular Interventional Radiology

VIR Suite Meadowmont

Interventional radiology (IR) at the UNC Center for Heart and Vascular Care performs a wide variety of minimally invasive procedures with the guidance of advanced technologies such as CT (computed tomography), MRI (magnetic resonance imaging), and ultrasound.

A Vascular Interventional Radiology procedure room at the UNC Center for Heart & Vascular Care at Meadowmont

Because certain procedures may require specialty materials available only at major medical centers, UNC is one of the region's leading hospitals for interventional radiology.  The IR division performs over 150 malformation treatments per year on patients of all ages. These are percutaneous, intra-arterial, and intra-venous procedures on all types of malformations. Image guided assistance is used in order to provide precise treatments.

“There is no treatment”

Growing up in New Hampshire as a child, Darlene Pitcher was told that nothing was wrong with her, despite the large red stain on her right arm and shoulder. When she complained of pain, she was punished.  So she simply endured and figured out how to cope with her condition.  She realized that she could ignore the ever-present hurt by staying active, so Darlene played basketball while in junior high and high school.

“When I was young, I was told that I was just being dramatic and overreacting to my ‘birthmark’.  I also had allergies as a kid, so then everyone just told me that I had sensitive skin,” says Darlene.

As a young adult, she moved around a lot, and sought treatment as the pain became unbearable.  In Colorado, Darlene’s physicians started her on a treatment regimen that triggered a life-threatening allergic reaction to sulfur, causing rashes and blisters in her mouth, bleeding, and difficulty breathing.

When she sought treatment at the Mayo Clinic, she spent five days undergoing testing.  They ran the same tests as they had in Colorado with the same results: there is no treatment.

“My previous physicians would tell me, ‘We are learning so much from you during these tests.’  It wasn’t very encouraging to me because I wanted to learn from THEM,” Darlene explains.  “They are the doctors, not me, and no one was providing any help with the pain after all of these tests.”

When Darlene moved to Wilson, NC, she was referred to UNC. After assessment, testing, and consultation by the UNC Hemangioma and Vascular Malformations Clinic physicians, she was diagnosed with CM, a capillary malformation in her right arm, shoulder, and chest.  The physicians also diagnosed chronic pain syndrome in her right arm and reflex sympathetic dystrophy in her left arm.

Craig Burkhart, MS, MD, examines Darlene before treatment for her vascular malformation

Burkhart and Darlene page 4

Craig Burkhart, MS, MD, Assistant Professor of Dermatology, has started Darlene on a three-year laser treatment regimen in her arm to ease the pressure on her constricted veins.  She will continue to see Dr. Stavas for management of any new deep vascular lesions that appear.  Julie Blatt, MD, Professor of Pediatric Hematology & Oncology, will study any new medicines that are developed that could help Darlene. Cynthia M. Powell, MD, Associate Professor of Pediatrics and Genetics , is monitoring genetic discoveries to determine if any are made about her condition.  A team of Anesthesia physicians are providing treatment for Darlene’s pain-related symptoms.

Burkhart and Darlene page 4.2

Dr. Burkhart performs treatment with a pulsed dye laser to ease pressure on constricted veins

Darlene smiles when she talks about her team of physicians at UNC.  “They know what they’re doing.  And the fact that they know what they’re doing is such a relief.”

Her husband, Jimmy, agrees.  “Every time we see them, their bedside manner is so comforting for Darlene.  We are very happy to have found the doctors at UNC.”

And while the physicians at UNC have told Darlene that her vascular malformation cannot be healed, they have shown her that it is treatable, which is something she has never been told before.  Darlene has always hoped for a treatment to ease her pain.  With the treatment she is receiving from the physicians at the UNC Hemangioma and Vascular Malformations Clinic, she is able to spend more of her days tending to her garden in the summer, canning her vegetables in the fall, and trying out new recipes.

With the pain from her malformation still ever-present, she is aware that her treatments build upon each other, with each one providing more and more relief.  But the treatments are allowing her to live her life to the fullest.