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.
Explore this issue:June 2009
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.