INTRODUCTION Also known as a benign hemangioendothelioma or strawberry nevus, this common vascular lesion occurs in 1% to 2% of infants and is the most common orbital tumor found in children. It is felt to represent a vascular hamartoma derived from endothelial rests. Periorbital heman-giomas may present as a superficial cutaneous lesion (strawberry hemangioma), subcutaneous lesion, deep orbital tumor, or can occur in a combination of these different locations. Approximately one-third of lesions are clinically noticed at birth, with virtually all-remaining lesions becoming apparent by six months of age. Typically an initial phase of rapid growth of the lesion occurs within 6 to 12 months of diagnosis. This is usually followed by a period of dormancy and then subsequent spontaneous involution over the course of several years. It is estimated that approximately 74% to 90% will regress to some extent by seven to nine years of age. Girls are more commonly affected than boys, with a ratio of 3:2. The most significant ocular complication is amblyopia, which may result from occlusion of the visual axis or from ani-sometropia due to pressure-induced astigmatism.
CLINICAL PRESENTATION Superficial capillary hemangiomas initially present as a flat red lesion with telangiectatic surface vessels. As they enlarge they typically become a red, elevated, domed mass with a soft consistency. The lesion is compressible and blanches easily with gentle pressure. Subcutaneous lesions present as a bluish-purple mass seen through the skin. Like its superficial counterpart it has a soft, spongy consistency. With the Valsalva maneuver or
when the child is crying, capillary hemangiomas will show a transient enlargement and darkening in color as they fill with blood. Involuted lesions develop a pale pink or white mottled appearance with fibrosis and inability to blanch. Ultrasound, computed tomography, and magnetic resonance imaging may aid in diagnosis and in determining the extent of orbital involvement.
HISTOPATHOLOGY Capillary hemangiomas are composed of closely packed, thin-walled capillaries.
Lobules are separated by thin fibrous septa. The capillaries may be lined by flattened endothelium and contain erythrocytes, or they may have plump endothelial cells making the vascular lumen inconspicuous. The tumors are usually well circumscribed by fibrous connective tissue.
DIFFERENTIAL DIAGNOSIS The differential diagnosis includes other vascular lesions such as nevus flammeus, cavernous hemangioma, and lymphangioma, as well as other lesions including dermoid cyst, encephalocele, rhabdomyosarcoma, neuroblastoma, and inflammatory masses.
TREATMENT Since most capillary hemangiomas will undergo some degree of spontaneous regression conservative observation is appropriate. Intervention should be reserved for those patients who develop functional limitations of the eyelid, amblyopia, or astigmatism. Intralesional corticosteroid injection or systemic corticosteroids typically result in dramatic reduction in the size of the lesion, but treatment may have to be repeated. However, prolonged adrenal suppression and failure to thrive has been documented following such treatment, and steroid injection has been associated with visual loss form embolization of ocular arterial flow by retrograde pressure. A topical fluorinated corticosteroid, clobetasol propionate, may produce a measurable reduction in the size of these lesions with no obvious systemic manifestations. Limited experience with injections of interferon-alpha has shown arrest in lesion progression and a possible accelerated rate of regression. Radiotherapy has also been advocated, but carries a risk of secondary orbital tumors. Laser treatment and surgery should be considered for selected localized lesions, or for those that fail to respond to corticosteroids. In some cases residual tumor may have to be left behind to avoid injury to important eyelid structures.
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