CHAPTER 2 PRINCIPLES OF MANAGEMENT

10.1055/b-0037-145003

CHAPTER 2 PRINCIPLES OF MANAGEMENT

Arin K. Greene

KEY POINTS

Accurate diagnosis of a vascular anomaly must be confirmed before determining the appropriate management.
Treatment of a vascular anomaly may include observation, pharmacotherapy (topical, intralesional, systemic), embolization, sclerotherapy, laser, and/or surgery.
Intervention is based on the type of vascular anomaly, symptoms, and the physician’s preference.
Patients may require interdisciplinary care and are often best managed by physicians who are focused on vascular anomalies.

The field of vascular anomalies is confusing, because lesions look similar and terminology is difficult. Although some vascular anomalies can be treated by a single physician, many require interdisciplinary care (particularly vascular malformations). Patients generally are best managed in a vascular anomalies center by providers who are focused on these lesions. Several types of treatments are used: embolization, laser, pharmacotherapy (topical, intralesional, and systemic), resection, and sclerotherapy. Often a consensus does not exist about the best intervention, because there is a lack of randomized, prospective evidence. Evaluation of treatments is further handicapped by the heterogeneity of the lesions. For example, one arteriovenous malformation can be small and located on the face, whereas another may involve an entire extremity. The intervention chosen to treat a vascular anomaly is frequently based on retrospective evidence and the preference of the physician.

Principles of management include the following:

Patients should be treated by physicians who focus on this field.
A correct diagnosis must be confirmed before choosing management.
Many lesions can be observed and intervention is not mandatory.
The goal of treatment usually is to control rather than to cure the vascular anomaly.
Complete resection rarely is required.
Surgical intervention should not create a worse deformity than the appearance of the lesion.

VARIABLES THAT DETERMINE MANAGEMENT

Three variables determine how a vascular anomaly is managed:

Diagnosis
Morbidity
Physician preference

**Table 2-1** **Major Vascular Anomalies and Treatments**
Tumors			Malformations
Biologic Name	Morbidity	Treatment	Biologic Name	Morbidity	Treatment
Infantile hemangioma	Deformity Obstruction Destruction Ulceration	Observation Topical timolol Triamcinolone (Kenalog) injection Oral prednisolone Oral propranolol Pulsed-dye laser Resection	Capillary malformation	Deformity Tissue overgrowth	Observation Pulsed-dye laser Resection
Congenital hemangioma	Deformity	Observation Resection	Lymphatic malformation	Deformity Bleeding Infection Pain	Observation Sclerotherapy Radiofrequency Ablation Carbon dioxide laser Sirolimus Resection
Kaposiform hemangioendothelioma	Deformity Thrombocytopenia Fibrosis Pain	Vincristine Sirolimus	Venous malformation	Deformity Phlebothrombosis Pain	Observation Sclerotherapy Resection
Pyogenic granuloma	Deformity Bleeding	Cautery Resection	Arteriovenous malformation	Deformity Bleeding Ulceration Pain	Observation Embolization Resection

DIAGNOSIS

The most important determinant of how a vascular anomaly is managed is the patient’s diagnosis. Before a physician treats a vascular anomaly, the lesion must be accurately identified with the correct terminology. At least 90% of vascular anomalies can be diagnosed by history and physical examination; the remainder requires imaging (about 9%) or histopathology (about 1%). Some lesions often do not require treatment (for example, infantile hemangioma, congenital hemangioma, and capillary malformation), whereas others almost always necessitate intervention (for example, kaposiform hemangioendothelioma and pyogenic granuloma). Because most vascular anomalies are asymptomatic at the time of initial diagnosis, treatment is frequently based on the natural history of the lesion. For example, a macrocystic lymphatic malformation is usually treated prophylactically with sclerotherapy, because it is at risk for enlargement, bleeding, and infection. In contrast, an arteriovenous malformation most commonly is managed by observation; embolization and/or resection typically are performed only if the lesion becomes symptomatic.

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