Medically necessary positions for treatment of lymphedema at body sites other than the limbs are based on clinical input. Additional details from clinical input are detailed in the Appendix. Individuals who fail to respond to an initial trial of a nonprogrammable pump may benefit from programmable pumps with pulsatile features that can be tailored to address individual lymphatic flow dysfunction patterns. Clinical input supports the use of non-pneumatic compression pumps on the basis of the evidence and clinical experience, emphasizing the importance of compliance with treatment. Clinical input was mixed on the use of compression pumps for the treatment of head and neck lymphedema. Ongoing evidence generation in head and neck cancer populations is expected to elucidate clinical benefit.

Lymphedema

Lymphedema is an accumulation of fluid due to disruption of lymphatic drainage. It is characterized by nonpitting swelling of an extremity or trunk, and is associated with wound healing impairment, recurrent skin infections, pain, and decreased quality of life. Lymphedema can be caused by congenital or inherited abnormalities in the lymphatic system (primary lymphedema) but is most often caused by acquired damage to the lymphatic system (secondary lymphedema). Breast cancer treatment (surgical removal of lymph nodes and radiotherapy) is one of the most common causes of secondary lymphedema. In a systematic review of 72 studies (N=29,612 women), DiSipio et al (2013) reported that nearly 20% of breast cancer survivors will develop arm lymphedema. The risk factors with robust evidence for the development of lymphedema included extensive surgical procedures (such as axillary lymph node dissection, a higher number of lymph nodes removed, and mastectomy) as well as being overweight or obese.

Diagnosis and Staging

A diagnosis of secondary lymphedema is based on history (e.g., cancer treatment, trauma) and physical examination (localized, progressive edema and asymmetric limb measurements) when other causes of edema can be excluded. Imaging, such as MRI, computed tomography, ultrasound, or lymphoscintigraphy, may be used to differentiate lymphedema from other causes of edema in diagnostically challenging cases.

Table 1 lists International Society of Lymphology guidance for staging lymphedema (2023) based on "softness" or "firmness" of the limb and the changes with an elevation of the limb.

Table 1. Recommendations for Staging Lymphedema

Management and Treatment

Lymphedema is treated using elevation, compression, and exercise. Conservative therapy may consist of several features depending on the severity of the lymphedema. Individuals are educated on the importance of self-care including hygiene practices to prevent infection, maintaining ideal body weight through diet and exercise, and limb elevation. Compression therapy consists of repeatedly applying padding and bandages or compression garments. Manual lymphatic drainage is a light pressure massage performed by trained physical therapists or by affected individuals designed to move fluid from obstructed areas into functioning lymph vessels and lymph nodes. Complete decongestive therapy is a multiphase treatment program involving all of the previously mentioned conservative treatment components at different intensities. Pneumatic compression pumps may also be considered as an adjunct to conservative therapy or as an alternative to self-manual lymphatic drainage in individuals who have difficulty performing self-manual lymphatic drainage. In individuals with more advanced lymphedema after fat deposition and tissue fibrosis has occurred, palliative surgery using reductive techniques such as liposuction may be performed.

Venous Ulcers

Venous ulcers, which occur most commonly on the medial distal leg, can develop in patients with chronic venous insufficiency when leg veins become blocked. Standard treatment for venous ulcers includes compression bandages or hosiery supplemented by conservative measures such as leg elevation.

Compression Pumps

Pneumatic compression pumps (PCPs) may be used in lymphedema or wound care clinics, purchased, or rented for home use; home use is addressed herein. PCPs consist of pneumatic cuffs connected to a pump. These pumps use compressed air to apply pressure to the affected limb. The intention is to force excess lymph fluid out of the limb and into central body compartments in which lymphatic drainage should be preserved. Many PCPs are available, with varying materials, designs, degrees of pressure, and complexity. There are 3 primary types of pumps. Single chamber nonprogrammable pumps are the simplest pumps, consisting of a single chamber that is inflated at 1 time to apply uniform pressure. Multichamber nonprogrammable pumps have multiple chambers ranging from 2 to 12 or more. The chambers are inflated sequentially and have a fixed pressure in each compartment. They can either have the same pressure in each compartment or a pressure gradient, but they do not include the ability to adjust the pressure manually in individual compartments. Single- or multi-chamber programmable pumps are similar to the pumps described above except that it is possible to adjust the pressure manually in the individual compartments and/or the length and frequency of the inflation cycles. In some situations, including patients with scarring, contractures, or highly sensitive skin, programmable pumps are generally considered the preferred option. PCPs are also proposed to supplement standard care for patients with venous ulcers. Recently, non-pneumatic, wearable compression pumps have become available. These garments can be programmed to provide graduated sequential compression therapy while providing patients with a functional range of motion and mobility.