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Lymphangioma Simplex

Figure 1.140. Milroy's disease is an inherited autosomal dominant condition which presents with the typical bilateral lymphedema in the lower extremities. The condition occurs as a result of absence of lymphatics and is always confined to the legs and feet.

Figure 1.141. Lymphangioma simplex of the anterior chest present at birth in an infant. There are four major forms of lymphangiomas: lymphangioma simplex, lymphangioma circumscriptum, cavernous lym-phangioma, and cystic hygroma. Lymphangioma simplex is a solitary, well-circumscribed, flesh-colored dermal or subcutaneous tumor. It may occur anywhere on the subcutaneous or mucosal surface and is seen most commonly on the neck, upper trunk, proximal extremities and tongue. The surface is generally smooth and it may remain stable or grow quickly.

Figure 1.142. On removal, this mass attached by a stalk to the right side of this infant's face was confirmed to be a typical simple lymphangioma. The small preauricular tag was not associated with any other pathology.

Figure 1.143. Circumscribed lymphangioma of the left lower extremity in a neonate. Lymphangioma circumscriptum is the most common form of lymphangioma. It is characterized by groups of deep-seated, thick-walled vesicles that have the appearance of "frog spawn" or "grape clusters." The common sites of involvement are the proximal limbs, shoulders, neck, axilla and adjacent chest wall, perineum, inguinal folds, tongue, and mucous membranes.

Figure 1.144. A large cavernous lymphangioma of the left axilla and anterior chest present at birth in an infant. Cavernous lymphangioma consists of diffuse soft tissue masses of large cystic dilatations of lymphatic vessels in the dermis and subcutaneous tissue, and may involve the intermuscular septa. The lesions are ill-defined and frequently involve large areas of the face, trunk, and extremities. They may occur in the tongue, resulting in macroglossia. Lymphangiomas frequently have a hemangiomatous component (hemangiolymphoma) so that some of the vesicles are filled with fresh or altered blood. Treatment is surgical but recurrences are common.

Figure 1.142. On removal, this mass attached by a stalk to the right side of this infant's face was confirmed to be a typical simple lymphangioma. The small preauricular tag was not associated with any other pathology.

Lymphangioma Lower Extremity Photos

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