Chapter: Lymphatic malformations
Article: 8 of 14
Update: Mar 15, 2021
Author(s): Meyer, Lutz
Some cystic lymphatic malformations (hereafter “LM”) can be cured. For circumscribed lymphatic malformations of the skin and subcutis, the entire lesion can be removed by surgery. For cystic LMs that are located laterally on the neck and predominantly subcutaneous without involvement of deeper structures, either everything can be removed by sclerotherapy or surgical excision, or sclerotherapy can be done to the extent that no recurrences are likely in later life.
In the case of more extensive and deeper lymphatic malformations, complete removal and healing is not possible without damage to important surrounding structures. Other therapeutic goals apply here. In the case of lymphatic malformations in the head and neck region, the aim is to reduce tumor masses, eliminate functional impairments such as dyspnea or dysphagia, and prevent or reduce long-term damage such as dysplasia of the facial skull. For intrathoracic or retroperitoneal lymphatic malformations, the focus is on reducing functional limitations. We describe the treatment goal to patients as “control” of the disease in the sense that they can live as unimpaired a life as possible.
Any center for vascular anomalies that treats high enough numbers of patients will unfortunately be familiar with the problem of patients who can neither be cured nor have their disease completely controlled. These are usually patients with extensive lymphatic malformations in the head and neck, chest and retroperitoneum, primary lymphedema or generalized lymphatic anomaly (GLA). For example, despite numerous surgical resections, sclerotherapy or laser therapies, tracheostomy and/or gastrostomy removal is not possible until adulthood. These are hence patients in whom systemic drug therapy is attempted.
In general, macrocystic lymphatic malformations are easier to treat and heal more often in the long term. Microcystic lymphatic malformations, especially with skin involvement and lymphorrhea, are more difficult to treat and tend to recur.