Congenital Hemangiomas

Nevus flammeus (56,57) is most commonly referred to as port-wine stain (Fig. 16). It is characterized clinically by a vascular, dull red, or bluish-red plaque that usually affects the lateral and medial face. Lesions tend to thicken and become nodular with time. They do not show spontaneous regression. Histological examination shows abundant, mature, dilated capillaries lined by a single layer of endothelial cells. The Sturge-Weber syndrome (58) is the association of a port-wine stain of the face with leptomeningeal and ocular angiomatosis. Approximately 80% of patients have epilepsy and cerebral calcifications. In the Klippel-Trenaunay syndrome, there is a nevus flammeus on a limb with hypertrophy of the soft tissues and bones of the same extremity.

The flashlamp-pumped pulsed dye laser is the treatment of choice (59,60). This laser system emits yellow light with a wavelength of 585 nm and pulse duration

Figure 16 An extensive port-wine stain of the right chest, shoulder, and arm. Initially, the lesion is bright-red and flat but it becomes thicker and purple over time.

of 450 ms. Results are excellent and there is minimal risk of scarring. Treatment with this laser requires multiple sessions given 6-8 weeks apart until resolution. The major disadvantage is immediate purpura in the treated sites lasting 10-12 days.

Capillary hemangiomas are rapidly growing, benign vascular tumors appearing shortly after birth. Lesions are sharply circumscribed, dome-shaped, bright red or purple, and with a smooth or lobulated surface. The clinical evolution is characteristic. The hemagiomas appear within the first months of life, grow rapidly until a significant size is reached, and then slowly involute leaving an atrophic scar. Occasionally, the tumors become ulcerated and bleed. Large lesions may obstruct the airway, interfere with feeding, or with binocular vision. In rare cases a large hemangioma sequestrates and destroys platelets, leading to marked thrombocytope-nia and consumption coagulopathy, the so-called Kasabach-Merritt syndrome. On histopathological examination, there is considerable proliferation of endothelial cells and numerous capillary lumina.

Treatment is not necessary for uncomplicated lesions located on the trunk or other noncosmetic areas because lesions will resolve spontaneously. Therapy is indicated for hemangiomas located on functional or cosmetic areas of the face, distal extremities, and genitalia; ulcerated and bleeding lesions; and lesions occurring as part of the Kasabach-Merritt syndrome.

Effective therapies include oral prednisone (2-4mg/kg/day for 2 weeks), surgical excision, or flashlamp-pumped pulsed dye laser. In cases of Kasabach-Merritt syndrome, platelets and blood transfusions are indicated.

Cavernous hemangiomas are true vascular malformations and therefore do not resolve spontaneously. They are deep, subcutaneous, compressible nodules of various sizes that can occur at birth, in infancy, or adulthood. Histological examination shows dermal and subcutaneous vascular spaces lined by endothelial cells and containing blood. The blue rubber-bleb syndrome consists of multiple congenital cavernous hemangiomas associated with intestinal and visceral angiomas. Patients experience chronic gastrointestinal bleeding and anemia or acute bleeding episodes. Maffucci's syndrome is the association of cavernous hemangiomas with dyschondro-plasia (defective calcification and deformities of bone) and osteochondromas. The latter may transform into chondrosarcomas.

Vascular and magnetic resonance imaging (MRI) studies are indicated prior to surgical excision. Lasers are not effective treatment for such large and deep structures.

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How To Reduce Acne Scarring

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