Chapter 72

### PATIENT STORY

A 58-year-old morbidly obese woman presents for a second opinion regarding leg swelling. She describes bilateral lower extremity swelling and discoloration that has progressed over the past 4 years. Examination is remarkable for a body mass index (BMI) of 47 and severe nonpitting bilateral leg swelling involving the dorsal feet and toes with extension to the distal thighs. Atrophy is identified within hyperpigmented distal calves, which are diffusely indurated (Figure 72-1). Verrucous-appearing papules with deep creases overlie the distal calves. Although venous duplex ultrasonography does not indicate deep venous thrombosis (DVT), incompetent deep and superficial veins are identified bilaterally. The patient is ultimately diagnosed with a combination of chronic venous insufficiency (CVI) and lymphedema or phlebolymphedema.

###### FIGURE 72-1

Classic example of secondary phlebolymphedema in a morbidly obese patient. Note the marked bilateral calf, foot and toe swelling consistent with lymphedema, yet associated relative distal calf atrophy and hyperpigmentation consistent with chronic stasis lipodermatosclerosis. The distal calf papulonodular and verrucous appearance is consistent with elephantiasis.

### EPIDEMIOLOGY

• Because of the vast unawareness and underdiagnosis of phlebolymphedema1,2 and the potentially confusing overlap in manifestations of CVI and lymphedema, there is little data in regard to the prevalence of this entity.

• CVI is estimated in up to 10% to 20% of the population, and up to 20% to 30% of patients with CVI will have associated lymphatic dysfunction.

• While the gender difference in phlebolymphedema is not reported, both CVI and lymphedema are more common among women. However, venous insufficiency is generally more severe in men.

• The emergence of venous insufficiency generally begins in the fourth or fifth decade and becomes more prevalent with advancing age.

### ETIOLOGY AND PATHOPHYSIOLOGY

• Phlebolymphedema is defined as combined insufficiency of both the venous and lymphatic systems.

• Although a preponderance of phlebolymphedema is secondary to severe CVI of various causes, primary cases in association with congenital lymphovenous defects exist. Klippel-Trenaunay syndrome is the classic example of primary phlebolymphedema (Figure 72-2).

• CVI is due to incompetent venous valves, venous obstruction (compression from an external mass or luminal obstruction from a venous thrombus), or both.

• CVI causes venous hypertension with subsequent excessive capillary filtration of fluid into the interstitial space. Lymphatic transport initially increases to compensate for this excess filtrate. If venous hypertension remains unabated, the lymphatic system is overwhelmed, eventuating in secondary lymphedema. Chronic stasis-associated inflammation can lead to lymphangiothrombosis, which further impairs the lymphatic system.

• As in primary and secondary lymphedemas, the interstitial fluid in phlebolymphedema ultimately becomes protein rich, as opposed to the protein-poor fluid of uncomplicated venous insufficiency.

• The presence of high-protein lymphatic fluid in the interstitium stimulates fibroblasts, keratinocytes, and leukocytes, leading to the deposition of collagen and destruction of elastic fibers eventually yielding cutaneous and subcutaneous fibrosis.

• In severe cases, ...

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