Chicago - The concept of too many cooks in the kitchen does not apply to treating vascular anomalies. In fact, experts say, even dermatologists often need to tap into the expertise of multidisciplinary centers that focus on vascular anomalies and other "orphan" skin diseases in order to offer optimum care - especially for their pediatric patients.
- The concept of too many cooks in the kitchen does not apply to treating vascular anomalies. In fact, experts say, even dermatologists often need to tap into the expertise of multidisciplinary centers that focus on vascular anomalies and other "orphan" skin diseases in order to offer optimum care - especially for their pediatric patients.
"This is a concept that is true for many diseases - that when there is a disorder that is fairly unusual that requires expertise that goes outside the field of dermatology, multidisciplinary clinics - or at least groups of individuals who are dedicated and knowledgeable about these diseases - (are) critical to patient care," says Amy S. Paller, M.D., Walter J. Hamlin professor and chairman of dermatology and professor of pediatrics at Northwestern University Feinberg School of Medicine, Chicago.
"There are vascular anomaly centers in many places around the country."
Patients with vascular anomalies fall through the cracks of traditional medical and surgical specialties because their diseases are often not organ-specific, but rather occur in multiple organs or tissue compartments, according to Ilona J. Frieden, M.D., professor of clinical dermatology and pediatrics, University of California, San Francisco (UCSF).
Dr. Frieden, who directs UCSF’s Birthmarks and Vascular Anomalies Conference (founded in 1991), says, "Also depending upon the exact type, vascular anomalies are uncommon to very rare.
"Vascular anomalies centers, particularly those which have been seeing patients for many years, have much more knowledge, expertise and comfort level in diagnosing and managing vascular anomalies than would physicians working in the absence of such a multidisciplinary setting."
The Birthmarks and Vascular Anomalies Center Children’s Hospital of Wisconsin in Milwaukee is a large multidisciplinary center that was founded and is directed by dermatologist Beth Ann Drolet, M.D. It includes otolaryngology, oncology, pathology, surgery, radiology, interventional radiology, plastic surgery and other specialties, according to Paula North, M.D., Ph.D., chief of pediatric pathology at the Medical College of Wisconsin and medical director of pathology and laboratory medicine, Children’s Hospital Wisconsin, Milwaukee.
Dr. North, a board-certified pediatric pathologist and hemangioma researcher, formerly worked at such a center at the Arkansas Children’s Hospital, Little Rock, Ark. Today, she and her Wisconsin-based colleagues are involved with every aspect of this care, including diagnosis, treatment and research into all sorts of hemangiomas, as well as vascular malformations and other rare syndromes involving vascular lesions.
"The diagnosis part is very important, because these lesions that kids get are usually misdiagnosed in the general medical community, and that leads to problems of finding the right therapy and addressing their management over their lifetime," Dr. North says.
When it comes to vascular anomalies, team members at the Birthmarks and Vascular Anomalies Center Children’s Hospital start by properly and accurately diagnosing the type by performing tissue examinations, including biopsies and resections, and radiological imaging, particularly MRI. Specialists also home in on the clinical diagnoses.
"With knowledgeable dermatologists and surgeons, combined with the pathology diagnosis and the radiological diagnosis, we can usually come up with the right answer," Dr. North says.
This and other academic centers devoted to vascular anomalies usually offer the latest in surgical and medical options. And many, including the one in Wisconsin, are immersed in research specific to the needs of these patients.
"We have a full range of research studies that are aimed at these lesions, from clinical studies - for instance, evaluating the effect of one drug versus another - to basic science studies, studying the mechanisms of pathogenesis and various types of vascular anomalies," Dr. North says.
The Vascular Lesion Clinic (VLC) at Children’s Memorial Hospital, directed by Sarah Chamlin, M.D., associate professor of pediatrics and dermatology, Children’s Memorial Hospital and Feinberg School of Medicine, Chicago, includes multiple pediatric specialties.
A core group of pediatric dermatologists, pediatric plastic surgeons, pediatric interventional radiologists, and a pediatric physiatrist evaluate patients at each visit, according to Dr. Chamlin.
The Chicago group commonly treats pediatric patients with complicated vascular malformations including lymphatic, venous, capillary and mixed types.
"Complicated mixed vascular malformations, including patients with Klippel-Trenaunay-Weber syndrome are commonly evaluated in the VLC," Dr. Chamlin says. "Young infants with complicated large infantile hemangiomas are also evaluated and treated in this setting. Many children are referred without a known diagnosis, and often they have been given several inaccurate diagnoses prior to their visit."
Centers such as the VLC act as "gatekeepers" for the care of these children, referring these patients to other specialists and keeping track of their complex medical issues, Dr. Chamlin says.
"Vascular lesion patients often have trouble finding the ‘right specialist’ that will take care of them. This is because that right specialist is really a group of specialists," Dr. Chamlin says.
When to refer
Dr. North says the rule of thumb for dermatologists is that they should refer patients to these centers if they have "complicated or confusing patients" with vascular anomalies.
The earlier the referral to a multidisciplinary center focused on these diseases, the better says Maria C. Garzon, M.D., associate professor of clinical dermatology and clinical pediatrics, and director, division of pediatric dermatology, Morgan Stanley Children’s Hospital of NY-Presbyterian (MSCHONY).
Dr. Garzon co-founded the Columbia University/Morgan Stanley Children’s Hospital of NY-Presbyterian Multispecialty Vascular Anomalies Group, which provides special expertise in treating vascular tumors, including infantile hemangiomas and other rare types of vascular tumors, such as kaposiform hemangioendothelioma and tufted angioma and vascular malformations.
Even if active treatment will not be undertaken early, she says, parents and patients can be counseled and evaluated regarding other aspects of patient management that might impact future quality of life.
An example, she says, would be "monitoring for limb length differences or using custom-fitted support garments in children with combined capillary-venous or capillary-venous-lymphatic malformations (also called Klippel-Trenaunay syndrome) of the legs."
Collaborating from afar
Dermatologists whose patients who are not close enough to these specialty centers to take advantage of them should know that patient collaborations can be done from afar.
"I have an internationally based consultative practice of vascular anomalies, so patients’ samples are sent to me for diagnosis from around the world," Dr. North says. DT