Splenic lymphangiomatosis associated with skeletal involvement (Gorham's disease): a new case and review of literature

Abstract

Lymphangiomatosis is a well-recognized congenital benign tumour, frequently seen in infancy and childhood, characterized by the presence of multiple lymphangiomas. Diffuse lymphangiomatosis also involving bony tissue is called Gorham's disease. This condition generally affects somatic soft tissue, where lymphatics are normally found. A predilection of this affection for bone, thoracic and neck involvement is well known, while involvement of abdominal viscera is very unusual. In bone this non malignant proliferation of lymphatic channels results in destruction and resorption of the osseous matrix. We report on a child in whom lymphangiomatosis involved both the bone and the spleen. A review of 166 cases is also reported.