Lymphangioma circumscriptum

Two children with lymphangioma circumscriptum (LC), a rare cutaneous lymphangioma, are described. One was diagnosed antenatally as having a right axillary cystic hygroma; in addition, postnatally LC was noted on the right forearm. A few years later a mediastinal lymphangioma was diagnosed. This combination of LC associated with additional malformations of the lymphatic system has not been reported previously, and may point to a generalized developmental disorder of the lymphatic system. The pathophysiology of the lesion is discussed with a review of the pertinent literature. It appears that LC primarily affects the subcutaneous tissue in the form of cystic dilatation of lymphatic channels without systemic lymphatic communication. The skin lesions are probably secondary to the increased intraluminal pressure. The typical clinical appearance is manifested by multiple skin vesicles and lymphorrhea. The main treatment modality is surgical excision. Recurrences are not unusual and require re-excision.