Massive cavernous lymphangiomatosis associated with a large lipoma and hemangiomas : a new syndrome?

Abstract

Eur J Plast Surg (1993) 16 : 42M4 European ~r'~l ~e Journal ot nn--'n~m-a~ Summary. A 3-year-old girl with massive cavernous lymphangiomatosis, a huge lipoma, and several types of hemangiomata is presented. A deep lipoma on the back was removed. The left margin of the lipoma intertwined with several cysts of cavernous lymphangiomas, resulting in continuous fluid accumulation postoperatively. At the first operation, a partial excision of cavernous lymphangiomas of the forearm was also performed. The forearm healed uneventfully, except for a moderate degree of hypertrophic scarring. As a result of a literature review, it can be stated that the case presented does not conform to any well-defined entity and would appear to represent a new syndrome to be categorized under congenital hamartomatous disorders. Everyone is interested in the fate of massive lymphangiomatoses such as this, and in the effects on several tissues or organs, such as breasts, muscles and bones.