Nancy M. Bauman, MD, an ENT Today editorial board member, is Professor of Otolaryngology-Head and Neck Surgery at Children’s National Medical Center in Washington, DC.
Few medical conditions that otolaryngologists treat possess the breadth of heterogeneity of vascular anomalies. The size of lesions ranges from minute to massive, and the manifestations range from trivial to life-threatening, with the severity of symptoms not always proportional to the size of the lesion. The scope of treatment options extends from observation to intervention, the latter of which includes pharmacologic, radiologic, surgical, or a combination of techniques. The outcome spans the scale of spontaneous involution with little or no residual sequelae, to a lifelong battle of living with active disease and undergoing frequent procedures to maintain as much function of involved structures as possible.
Most important, the expectations of treatment for the patient may be quite disparate from those of the patient’s physician. The former may anticipate complete extirpation of the disease, whereas the latter may reluctantly be forced to accept serial, noncurative treatments that preserve form and function as much as possible.
Most vascular anomalies are first evaluated by pediatricians or family physicians, because most anomalies are apparent at birth or arise during childhood. The ability of a primary care physician to correctly diagnose the lesion and to initiate the most appropriate management or referral is largely dependent on his or her experience in seeing and treating vascular anomalies during residency.
The study of vascular anomalies is often grasped not by textbook reading alone, but rather by learning from an experienced mentor who can share nuances of diagnosis and treatment often not conveyed in literal readings. Although most primary care physicians can readily recognize the distinguishing features of rapid proliferation and gradual involution of hemangiomas, the ability to distinguish such lesions from rare vascular neoplasms such as hemangiopericytomas, or even from unusual appearing solid tumors such as rhabdomyosarcomas, requires an experienced physician. A delay in biopsying malignant lesions can seriously affect patient management, as can failure to obtain imaging studies to assess for other components of syndromes or associations that the vascular anomaly may herald.
Emotional as Well as Physical Impact
The emotional impact of vascular anomalies on patients and their families can be overwhelming. Even prenatal diagnosis cannot adequately prepare parents for their first glimpse of a massively disfiguring lesion of the face in their newborn infant. Although parents assume that modern medical and plastic surgical treatments can correct any deformity, the reality is that our treatment options fall short of miraculous for select anomalies.
The advent of PubMed and parent support groups has served to educate parents and empower them to participate in the decision making process for their children. This awareness is generally advantageous, but it sometimes complicates our job, particularly if the expectations of the family are unrealistic. Authors of publications naturally select photos of their most impressive outcomes, and parents may not recognize differences in the size or stage of their child’s lesions that may prevent the most favorable outcome. What we as surgeons may construe as an excellent outcome-for example, having preserved all branches of the facial nerve entwined in a massive vascular malformation of the face-may be construed as less of a success by a parent who finds the residual mandible and soft tissue hemihypertrophy cosmetically unacceptable. These differences in perception of outcome are expected for the two parties, given their varied backgrounds and expectations.
It is understandable that parents seek the best medical care available to their child. The probing glances, awkward stares, and questions that their child’s anomaly evokes are a part of a family’s daily life that they fervently hope will end with medical intervention. I find that reading Web recollections written by children with facial anomalies who spend time at summer camp with other children with similar conditions are very revealing. They relate the joy they experience when being among a sea of children with similar anomalies allows them to forget about their differences and just enjoy the camp experience as a child should. Their stories give us a glimpse of how their vascular anomaly molds their everyday life.
Our aptitude as physicians treating patients with vascular anomalies extends beyond our surgical and medical armamentarium. I fondly recall being a resident, many years ago, in the clinic of the late Dr. Janusz Bardach, a pediatric plastic and reconstructive surgeon at the University of Iowa, who specialized in cleft lip and palate repair. To Dr. Bardach, every patient was beautiful, and when he told them of their beauty, with his compelling accent, they believed him no matter how far their facial appearance deviated from what society regards as aesthetically pleasing. Instilling confidence about a child’s inner beauty, that beauty found deeper than their skin and muscular facial envelope, is an art that he mastered, and his patients benefited from it just as much as they gained from his surgical successes.
Importance of MVACs
The creation of multidisciplinary vascular anomalies clinics (MVACs) is a great contribution to the care of these patients. MVACs, often modeled after the renowned clinic directed by Drs. John Mulliken and Steven Fishman at Boston Children’s Hospital, afford the opportunity for physicians from different specialties to share their expertise and to create a unified approach to the care of patients. Physicians staffing these clinics have a keen interest in treating vascular anomalies, in educating physicians, and in conducting clinical as well as basic science studies.
Physicians in MVACs manage relatively large volumes of these relatively rare anomalies, which enables them to recognize favorable treatment plans. It also allows them to recognize treatment complications and to challenge the use of certain medications. In the 1990s, interferon (IFN) was advocated to treat symptomatic hemangiomas. A decade later, however, the association between spastic diplegia (SD) and IFN use during early infancy was recognized, and the drug is no longer advocated except in life-threatening circumstances. This illustrates how acceptable treatments can quickly become unacceptable when side effects outweigh their benefit.
The ability to assess long-term outcomes across generations of physicians is another advantage of MVACs. I cared for two women with massive arteriovenous malformations (AVMs) of their faces, whose care had all been provided at a single institution by several generations of physicians across the span of 30 years. Reviewing the yellowed preoperative and postoperative black-and-white photos would make one marvel at the impressive outcome. The cure afforded them a great life for many years, but was of course temporary and, over time, the lesions recurred, tapping into vessels from the internal carotid artery and abolishing the surgeon’s work. I can only hope that future generations of physicians will look at our management and know that we were doing the best we could at the time of our care.
The commitment of a health care institution to foster a MVAC is a tremendous one, as the revenue from such a clinic often pales in comparison to that from multiple single-specialty clinics. I typically see half the number of patients in the MVAC at Children’s National Medical Center that I would see in my otolaryngology clinic, and I see them in conjunction with four other faculty members from various disciplines. The presence of an otolaryngologist on MVACs is of vital importance, however, to preserve our role in treating lesions of the face and neck.
Physicians who have experience in treating vascular anomalies, but who do not have the opportunity to be part of a MVAC, can still benefit by orchestrating a referral of their patients to a MVAC. The goal of MVACs is to improve patient management, and rendering an opinion regarding treatment rather than assuming the care of the patient is in the clinic’s, patient’s, and referring physician’s best interest.
Treating vascular anomalies is a constant challenge, but it is a rewarding one. Fortunately, many are easy to treat, and they keep us in the arena to prepare for those that are not. I am hopeful that our continued efforts in conducting basic science research and outcome-based clinical studies will be fruitful and improve our ability to treat patients with these anomalies.
©2009 The Triological Society