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CHAPTER FIVE: Lymphedema

Marisa Perdomo, PT, DPT, CLT-Foldi, CES; Rose L. Hamm, PT, DPT, CWS, FACCWS

CHAPTER OBJECTIVES

At the end of this chapter, the learner will be able to:

  1. Differentiate between the components of the lymphatic and the venous system.

  2. Relate the function of each lymph system component to the formation of lymphedema.

  3. Diagnose lymphedema according to cause, pathophysiology, and stage.

  4. Differentiate between lymphedema and chronic venous insufficiency.

  5. Define the components of Starling’s Law and describe their role in lymphatic flow.

  6. Select the appropriate compression therapy for a patient with peripheral lymphedema.

  7. Design an exercise program for a patient with lymphedema.

  8. Discuss the principles of manual lymphatic mobili-zation, as well as the indications and contraindications.

  9. Educate patients on skin care and strategies to prevent recurrent lymphedema complications.

INTRODUCTION

Lymphedema is a chronic inflammatory condition that develops as a result of lymphatic insufficiency. Lymphatic insufficiency occurs from a decrease in reabsorption or a decrease in transport capacity of the lymphatic system. It can be primary malformation of the lymph system or an acquired condition due to obstruction or damage to the system. (TABLE 5-1)

Table 5-1

Primary and Secondary Lymphedema: Causes, Onset, and Characteristics

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Table 5-1

Primary and Secondary Lymphedema: Causes, Onset, and Characteristics
Primary Lymphedema Causes Onset Secondary Lymphedema Causes Onset

  • Inherited genetic mutations resulting in abnormal development of lymph vessels:

  • ▪ Hypoplasia

  • ▪ Hyperplasia

  • ▪ Aplasia

  • ▪ Kinmonth syndrome (inguinal lymph node fibrosis)

  • Genetic mutations with autosomal dominant pattern:

  • ▪ Chromosome 5q34-q35

  • ▪ FLT4

  • ▪ FOXC2

  • ▪ VEGFR3

  • ▪ SOX18

 Variable age onset depending upon the gene involved∗

  • Damage to lymph nodes and/or lymph vessels that results in decreased lymphatic reabsorption and transportation (mechanical insufficiency)

  • Trauma

  • Radiation therapy

  • Tumor obstruction

  • Infection

  • CVI

  • Any time after damage to lymph nodes and vessels:

  • Either soon after lymph node dissection or months, years, or decades later

  • Depends on patient medical history, co-morbidities (number of lymph nodes removed, radiation therapy, chemotherapy, venous pathologies, and obesity), and general health.

  • Milroy’s disease (congenital lymphedema)

  • FOXC2

  • VEGEFR3

  • SOX18∗

 Onset during infancy∗

  • Surgery

  • Trauma

  • Radiation therapy

  • Tumor obstruction (cancer)

  • Infection

  • CVI

 Depends on patient medical history, co-morbidities, and general health.

  • Meige’s disease (lymphedema praecox)

  • MIM153200∗

 Onset in childhood or around puberty; may begin in early 20’s or 30’s
Lymphedema tarda Onset at 35 yrs or later

∗Primary and secondary lymphedema differ in causes and onset, which are used to make a differential diagnosis; however, the characteristics are very similar—an edematous limb, skin changes, loss of joint spaces. There are some characteristics of hereditary lymphedema, however, that distinguish it from secondary lymphedema. These include but are not limited to hypoparathyroidism, microcephaly, intestinal lymphangiectasia, ptosis, yellow nails, pleural effusions, cerebral arteriovenous anomalies, distichiasis, congenital heart defects, and webbing of the neck.

Data from Connell F, Brice G, Jeffery S, Keeley V, Mortimer P, Mansour S. Clini Genet. 2010;77:438–452; Levinson KL, et al. J Pediatr. 2003;142:704–708; Rizzo ...

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