The next thing I want to talk about is lymphatic malformations. You're all familiar with cystic hygroma. This was originally described in 1843 and subsequently classified by Wegner. McClure, from an embryologic study, said that this is an abnormal development of lymphatics, that they develop independently from the veins and then communications are established. John Mullikan, who is at Boston Children's Hospital and an expert in vascular malformations, has described histologic endothelial activity within the abnormal lymphatics and assigned it to the vascular malformation category of illness.
I think we need to think of cystic hygroma as a failed communication between the existing lymphatics and veins. Lymphatic malformations represent something different; this is really failed morphogenesis. Lymphangioma circumscriptum is that disease that involves primarily skin and subcutaneous tissue and when these patients recur after surgery that is how they present, at the dermal and subcutaneous level very much like lymphangioma circumscriptum.
Presentation is usually at birth. It can be localized or extensive. Head and neck, axilla and proximal limbs are involved. Sometimes there are vesicles and dilated subcutaneous lymphatics. There can be bleeding secondary to hemorrhage in the skin giving them a purple color and, as we know, recurrence after surgery is common.
Symptoms are those of the mass, which can cause pressure and obstruction, especially in the head and neck area, sometimes pain. Patients can have lymphatic fluid leaking from these, drainage and occasionally bleeding. Some patients will experience infection, sometimes multiple episodes of infection, which results in fibrosis and hardening of this making it very difficult. They also cause a cosmetic problem because of the deformity associated with it.