131: Lymphedema

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CHAPTER 131

Lymphedema

Mabel Caban, MD, MS; Lori Hall, PT, MS, CLT-LANA

Synonyms

Primary lymphedema

Secondary lymphedema

Post-mastectomy lymphedema

Nonne-Milroy-Meige syndrome

Familial lymphedema

ICD-9 Codes

457.0   Postmastectomy lymphedema syndrome

457.1   Lymphedema

  Acquired

  Praecox

  Secondary

757.0   Hereditary edema of legs

  Chronic hereditary

  Congenital

997.99  Surgical

ICD-10 Codes

I97.2   Postmastectomy lymphedema syndrome

I89.0   Lymphedema, not elsewhere classified, praecox and secondary

Q82.0  Hereditary lymphedema

Definition

Between 3 and 5 million people in the United States suffer from lymphedema, which in a significant number develops from cancer and its treatment [1]. The incidence of post-mastectomy lymphedema ranges from 6% to 48%, depending on whether the patient received axillary radiation and surgery; if only lumpectomy is performed, the incidence drops to only 6%. This increases to 23% with use of sentinel node biopsy and radiation therapy; but if axillary lymph node dissection and radiation therapy are used, the incidence is even higher at about 35% to 48% [1].

About 80% of lymphedema cases involve the lower limbs and relate to peripheral vascular disease [1]. Cervical cancer, melanoma, and pelvic cancers increase the frequency of secondary lymphedema of the lower extremity. An incidence of 21% to 49% is reported after cervical cancer surgery and radiation therapy. In women with endometrial cancer, 11% developed lower limb lymphedema after surgery and radiation therapy. Around the world, infection in the form of filariasis is the most common cause of lymphedema. An estimated 15 million people present with lower extremity lymphedema in filariasis-endemic regions in the world [2].

Lymphedema is a condition of an abnormal enlargement of part of the body associated with lymphostasis from dysfunction of the lymphatic system (Fig. 131.1). The dysfunction is due to the abnormal morphology of the lymphatic system, high production of lymph, or blockage in drainage [3,4]. The lymphatic system consists of vessels with lymph traveling through lymph nodes. The lymphatic vessels transport fluid, plasma proteins, and other substances from the tissue back into the circulatory system. Lymphedema results from the inability to drain the lymphatic fluid and the accumulation of it in the limb. Chronic edema initiates a cascade of events that cause transformation of the tissue with skin thickening, fibrosis, deposition of fat, and unhealthy skin changes after months to years. A classification of lymphedema includes primary (heritable) and secondary (acquired) causes [5]. Phlebolymphedema pertains to dysfunction of the lymphatic system in association with chronic venous insufficiency [6]. Chronic venous insufficiency is a manifestation of deep venous thrombosis, angiodysplasia, lack of valves in the venous system, or varicosities.

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FIGURE 131.1 Abnormal skin changes in the patient with lymphedema.

The radical excision of lymph nodes for malignant disease does not always cause lymphedema [7]. Lymphedema could occur as a late complication of surgery for malignant disease because of gradual failure, making the distal lymphatics pump harder through the most proximal damaged ducts over time. The body compensates by regenerating the transected lymphatics to some extent. With irradiation, fibrous scarring forms, increasing the risk of lymphedema. The diagnosis depends on the history and characteristic changes of the skin on clinical examination (Fig. 131.1).

Symptoms

The hallmark of lymphedema is painless swelling. Although many people believe that pain is a major component of lymphedema, this is not typically present, especially in the early stages. One of the first symptoms of lymphedema is tightness of the cutis and subcutis [8]. Chronic indolent swelling of a limb is the most common presentation of lymphedema. Patients may complain that the arm feels heavy or that rings feel tight. Hypertrophy of fatty tissue occurs and eventually fibrosis too. In true lower extremity lymphedema, the feet are involved. The skin condition changes from being soft initially to becoming harder over time. Early or postsurgical lymphedema can spread proximally, an unusual finding after the first year, and lasts about 3 months after surgery [7].

Physical Examination

The patient with limb edema requires a thorough physical examination before the initiation of therapy. Swelling of a limb with or without pain may be the presentation of an infection, deep venous thrombosis, or obstruction caused by a tumor at the level of the lymph nodes. Limb temperature and discoloration should be noted. In early stages, the edema is pitting, but as lymphedema progresses, the edema becomes nonpitting. In the absence of treatment, distal enlargement of the wrist or ankle occurs. A discrepancy of 2 cm in circumference or a difference of 200 mL in volume from side to side has been accepted for the diagnosis of lymphedema. A multitude of skin changes occur over time (Table 131.1) [9]. Signs of lymphedema progression include a cool limb, skin thickening, limited range of motion, increased presence of papillomas, mycosis, and bacterial infection. Infection, such as cellulitis, erysipelas, and lymphangitis, must be recognized to prevent systemic malaise and worsening of the swelling [3].

Staging of the condition helps assess progression and evaluate better treatment options. Stages of lymphedema are as follows:

Stage 0 Preclinical: there is no swelling, and it can lasts months or years.

Stage I Reversible stage: swelling is soft and pitting.

Stage II Spontaneously irreversible stage: increased swelling and change in tissues with fibrosis formed.

Stage III Lymphostatic elephantiasis: limb becomes extremely swollen with thickened skin.

Lymphedema does not cause neurologic deficits, but paresthesias and numbness may occur in the same distribution of the edema. Explanations include coexistence of neurologic damage from node dissection, peripheral nerve injury, plexopathy, and chemotherapy-induced polyneuropathy. The possibility of cancer recurrence must be ruled out with imaging, such as computed tomography or magnetic resonance imaging with contrast enhancement.

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