CHAPTER 43 Lymphedema Complications and Their Treatment

10.1055/b-0037-143499

CHAPTER 43 Lymphedema Complications and Their Treatment

Arin K. Greene, Reid A. Maclellan

KEY POINTS

Besides swelling, lymphedema can cause numerous health problems.
Cardiovascular complications, although rare, can occur.
Genitourinary complications can accompany penoscrotal or vulval edema. Sexual function per se is not compromised, although it may be practically.
Infection is one of the major and most common morbidities caused by lymphedema.
Malignant transformation is extremely rare but is associated with a very poor prognosis.
Massive localized lymphedema is not uncommonly associated with morbid obesity. In general, the obesity must be treated first.
Orthopedic issues can develop related to the weight of the lymphedematous tissue and the strain it puts on the skeletal framework.
Skin changes are very commonly associated with lymphedema and may precipitate soft tissue infections.
Psychosocial issues are very common.

Lymphedema is a chronic and incurable condition. Nevertheless, there are certain issues that we know and recognize about lymphedema. For example, patients who are compliant with interventions tend to have fewer problems than less compliant patients, although a few patients have severe morbidity. ¹ Active individuals have less complications than patients with a sedentary lifestyle. Muscle contraction aids the movement of lymph fluid through the body. Exercise slows disease progression by enhancing lymphatic flow and stabilizing the body mass index of patients with lymphedema. It is well documented that lymphatic function is negatively affected by obesity. ² , ³ We also know that individuals born with an anomalous lymphatic system have less difficulties from the disease than patients who develop lymphedema from an injury to their lymphatic vessels. ¹ Perhaps individuals with primary lymphedema counteract the debilitated lymphatic system better than patients with secondary disease. Morbidity from lymphedema is not insignificant (Box 43-1). The conditions discussed in this chapter are the most commonly seen complications from lymphedema.

BOX 43-1 Morbidity From Lymphedema

Cellulitis
Discomfort
Hyperkeratosis
Massive localized lymphedema
Lymphangiosarcoma
Lymphatic vesicles
Problems with the extremity
Progression of the disease
Psychosocial distress
Wearing clothing

Complications Affecting Specific Body Systems

COMPLICATIONS OF THE CARDIOVASCULAR SYSTEM

Lymphedema does not affect the arteries or veins; therefore patients rarely develop congestive heart failure secondary to their disease. Nevertheless, there are exceptions (see Chapter 10). We have one patient with massive disease who had cardiac morbidity from lymphedema (Fig. 43-1). He presented with the most severe expression of primary lymphedema in our experience. The disease began in his right lower extremity, and over time the disease progressed to the point that he became nonambulatory. High-output congestive heart failure manifested as a result of excessive blood shunting to his right lower extremity and buttock. His congestive heart failure resolved after 45 pounds of skin and subcutis were excised from the diseased area.

**FIG. 43-1** This 32-year-old man had congestive heart failure resulting from primary lymphedema of his right lower extremity and buttock. There was massive shunting of blood through the diseased area.

COMPLICATIONS OF THE GENITOURINARY SYSTEM

Psychosocial morbidity is the main issue for patients with penile/scrotal lymphedema, because individuals feel that the affected area is unattractive. They may worry about their appearance in clothing or the difficulty of fitting into clothing because of the bulk of the disease. Vesicles can also form that leak and cause infection (Fig. 43-2). Dysuria or phimosis may occur; however, genital lymphedema does not influence sexual function or cause sterility per se. It is recommended that patients wear tight-fitting exercise shorts to increase compression to the diseased area. Leaking lymphatic vesicles can be sclerosed or treated with a carbon dioxide laser. Resection of the skin and subcutaneous tissue can alleviate the psychosocial morbidity by improving the appearance of the genitals.

**FIG. 43-2** This 24-year-old man with primary lymphedema had low self-esteem because of the appearance of his genitals. He also had leaking lymphatic vesicles.

COMPLICATIONS OF THE MUSCULOSKELETAL SYSTEM

Lymphedema is primarily a disease of the skin and subcutaneous tissue. The bone is not directly affected; therefore children are not at risk for vertical limb overgrowth and do not need monitoring for a leg-length discrepancy. Patients may have a reduced ability to use an extremity because of muscle weakness and/or heaviness from overgrowth of the disease. Joint mobility may also be compromised by operative or radiation fibrosis. Compression therapy is prescribed for early lymphedema to decrease the size of the affected area and improve the ability to perform activities of daily living.

As the disease progresses, lymph accumulation in the superficial interstitial space leads to subcutaneous adipose deposition. ⁴ Patients initially increase muscle strength in the diseased extremitybecause of the additional weight. Over time, the addition of fibroadipose deposition and muscle hypertrophy cause a decrease in function. Individuals may have difficulty dressing themselves, because they can no longer raise their arm over their head. The weight of lower extremity disease may impede ambulation (Fig. 43-3).

**FIG. 43-3** Lymphedema can decrease the ability to perform activities of daily living. This 40-year-old woman with primary lymphedema has difficulty walking because of the weight of her left extremity.

Orthopedic morbidities are caused by severe disease exacerbated by fibroadipose deposition. Conservative interventions (for example, compression garments, pneumatic compression devices, and massage) do not address this problem. More aggressive treatments that decrease the weight of an extremity by removing excess skin and subcutaneous tissue help alleviate symptoms. Suction-assisted lipectomy or staged skin or subcutaneous tissue excision can reduce the excess weight of the extremity.

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