General Considerations

Abstract

Over the last 20 years, insight into lymphatic structural biology and function has undergone an unparalleled renaissance. There has been a consequent growth in comprehension of lymphatic function in health and in disease. The diagnosis of lymphatic edema needs a differential diagnostic approach that may require distinct imaging approaches and that will, by inference, lead to precise utilization of treatment resources, including physical modalities, surgical interventions, and, increasingly, pharmacology. These are all intended to improve lymph flow and macromolecular clearance from the affected edematous regions.

• Failure of fluid homeostasis, immune traffic, and/or lipid uptake and transport are the hallmarks of the entire spectrum of lymphatic disease, including both primary and acquired forms of lymphedema, lymphatic inflammatory, lymphatic maldevelopment, and cancer metastasis.

• Insights into normal function, and its aberration in disease, derive from the active investigation of lymphatic vascular development and postnatal remodeling and repair.

• Edema occurs if lymphatic load is abnormally increased, if the transport capacity is reduced (obstructive lymphedema), or if there is any combination of these two.

• Primary lymphedema can be sporadic, hereditary, or syndrome-associated. Secondary lymphedema is acquired and can be further classified as benign (on the basis of trauma, infection, or iatrogenic causes) or malignant (the result of direct neoplastic invasion). Mixed lymphaticovenous edemas are also common.

• Lymphedema is not usually life-threatening, but the presence of lymphatic disease typically has a very deleterious effect on quality of life (7 Chap. 37), based upon loss of function, restriction of movement, loss of body image and self-esteem, impaired psychosocial adjustment, and the risk of infection.

• The clinical diagnosis of lymphedema relies most heavily upon observations made at the bedside. Discrimination from other non-lymphatic forms of edema requires recognition of the unique cutaneous sequelae of lymphedema. The diagnosis can be confirmed through a variety of imaging modalities, including duplex ultrasonography, lymphoscintigraphy, computed tomography, magnetic resonance imaging, positron emission tomography, near-infrared fluorescent lymphography, and oil-based lymphangiography.

• Treatment of lymphedema is mandatory at the earliest detectable time point in the evolution of the disease. Decongestive physiotherapies been shown to have a very beneficial impact upon edema volume and patient symptomatology.

• Pharmacologic approaches include aggressive control of infection through antibiotic therapy. Molecular modifications, including growth factor-based and cellular therapies (7 Chap. 36), continue to hold great future promise.

• Surgical therapies for lymphedema are enjoying increasing utilization as these approaches continue to be refined. The surgical approach to the lymphedema patient may include both reconstructive (Part VIII) and excisional (Part IX) techniques.

• Surgical therapies for lymphedema are enjoying ever-increasing utilization as these approaches continue to be refined. The surgical approach to the lymphedema patient can incorporate both reconstructive and excisional techniques.