Stephen A. Goldstein

Vascular lesions of the head and neck region are relatively common when all groups are added together. They can be classified by several systems, but two broad categories of classification predominate and are easiest to remember:

1. Congenital versus acquired

2. Benign versus malignant

Congenital

Congenital lesions have also been grouped in many ways over the years, but utilizing history and physical exam they can more readily be separated into hemangiomas or vascular malformations. At birth, 40% of hemangiomas are present, whereas 99% of vascular malformations are noted.

Hemangiomas

Hemangiomas are the most common benign vascular lesion of the head and neck. Hemangiomas can rarely be present at birth but are most notorious for their development after birth and rapid proliferation starting within the first 2 weeks of life. This rapid phase is followed by eventual involution, usually in the second year of life. These lesions may be divided into two subcategories depending on their location and histology.

Capillary hemangiomas: “Strawberry hemangiomas “are superficial in the papillary dermis and noted to be red. They have a 3:1 female predominance. The proliferation of the endothelial cells results in either flat or nodular lesions with ill-defined pattern yet delineated appearance.

Cavernous hemangiomas: “Deep hemangiomas “involve the reticular dermis and subcutaneous tissues. In the head and neck region mucosal surfaces are also at risk for involvement. These may appear as a pale, skin-colored blue or red mass. These too are ill defined in shape but well delineated. Hyperhidrosis over the area is common. Recurrent episodes of thrombophlebitis around the lesion can occur; however, most are asymptomatic.

Kasabach-Merritt syndrome: A rare yet feared complication of large hemangiomas. The platelets and clotting factors are locally consumed within the hematoma, causing a consumptive coagulopathy. The patient will often present in disseminated intravascular coagulation (DIC), or rapid enlargement of the lesion or petechia, pallor, bruising, and prolonged bleeding from minor abrasions. It may also occur in adults.

Vascular malformations are almost always present at birth (99%). Unlike hemangiomas, these grow in proportion to children as they age. They result from a genetic error in vascular morphogenesis. The most recognized is probably the “stork bite” or “salmon patch” typically located in the nape of the neck but may occur anywhere on the face. They present as red macular patches with irregular shapes. They are superficial within the dermis. On the face they will fade over the first year of life, whereas those on the neck often remain for life.

Nevus flammeus or port wine stains are another congenital malformation often involving the face and neck. These start as smooth, flat, red-purple patches in infants but with age will darken in color. Also with age these lesions tend to become papular. By the fifth decade of life, two thirds become nodular or hypertrophic. There are several syndromes with nevus flammeus that have a neurocutaneous component. Sturge-Weber syndrome is the most familiar, with its distribution following the dermatomes of the trigeminal nerve. These patients are often diagnosed at birth. Other syndromes that become evident in childhood include OslerWeber-Rendu disease, Cobb syndrome, and von Hippel-Lindau disease.

Lymphangiomas