Chronic Venous Disease and Lymphedema

Chapter 293 | Part 6: Cardiovascular Disorders

KEY CLINICAL POINTS

• Chronic venous disease encompasses telangiectasias, varicose veins, and chronic venous insufficiency, with varicose veins affecting ~30% of women and 15% of men in the U.S.
• CEAP classification (clinical, etiologic, anatomic, pathophysiologic) is the standard framework for staging chronic venous disease.
• Lymphedema is classified as primary (genetic, congenital, or idiopathic) or secondary (acquired due to infection, surgery, or obstruction).
• Management includes graded compression therapy (20–40 mmHg), decongestive physiotherapy, and surgical interventions for severe cases.
• Lymphangiosarcoma is a rare but lethal complication of chronic lymphedema, particularly in patients with filariasis or post-mastectomy lymphedema.

1. DEFINITION & OVERVIEW

Chronic venous disease (CVD) and lymphedema are disorders of venous and lymphatic systems, respectively. CVD includes varicose veins, chronic venous insufficiency, and complications like ulcers. Lymphedema is characterized by lymphatic dysfunction and limb swelling.

Table 293-1: CEAP Classification

Clinical Classification	Etiologic Classification	Anatomic Classification	Pathophysiologic Classification
C0 (No visible signs)	E Primary (p)	A Superficial (s)	P Reflux (r)
C1 (Telangiectasias)	E Secondary (s)	A Perforator (p)	P Obstruction (o)
C2 (Varicose veins)	E Secondary – intravenous (si)	A Deep (d)	P Reflux and obstruction (r,o)
C2r (Recurrent varicose veins)	E Secondary – extravenous (se)	A No venous anatomic location identified (n)	P No pathophysiology identified (n)

1.1 Erythromelalgia

A burning pain and erythema of extremities, more common in males. Primary form linked to SCN9A mutations; secondary causes include myeloproliferative disorders, drugs, and connective tissue diseases.

1.2 Frostbite

Tissue damage from cold exposure. Superficial frostbite affects skin and subcutaneous tissue; deep frostbite involves muscle, nerves, and blood vessels.

1.3 Venous Anatomy

Superficial veins (great/small saphenous) and deep veins (popliteal, femoral) form the venous system. Perforating veins connect superficial and deep systems.

1.4 Varicose Veins

Dilated, tortuous superficial veins ≥ 3 mm. Primary varicose veins result from valve dysfunction; secondary from deep venous insufficiency or obstruction.

2. EPIDEMIOLOGY

Varicose veins affect ~15% of men and 30% of women in the U.S., increasing with age. Chronic venous insufficiency with edema affects ~7.5% of men and 5% of women. Lymphedema prevalence is ~1.15 per 100,000, with females more commonly affected.

2.1 Risk Factors

Age, pregnancy, hormonal therapy, obesity, prolonged standing, and genetic predisposition. Secondary causes include deep vein thrombosis, tumors, and iatrogenic injury.

3. ETIOLOGY & PATHOPHYSIOLOGY

Primary CVD results from valve dysfunction and vein wall weakness. Secondary causes include deep venous obstruction, perforating vein incompetence, and chronic venous hypertension. Lymphedema arises from lymphatic obstruction, reflux, or congenital defects.

3.1 Genetic Causes

Milroy’s disease (VEGFR3/VEGF-C mutations), lymphedema-distichiasis (FOXC2), and hypotrichosis-lymphedema-telangiectasia (SOX18).

3.2 Acquired Causes

Infections (filariasis, bacterial lymphangitis), surgery (lymph node dissection), radiation, and chronic venous insufficiency.

4. CLINICAL FEATURES

Symptoms include leg heaviness, swelling, skin changes (hyperpigmentation, lipodermatosclerosis), and ulcers. Lymphedema presents as painless swelling with progressive induration and fibrosis.

4.1 Complications

Venous ulcers, lymphangiosarcoma, cellulitis, and chronic skin changes (peau d’orange, acanthosis).

5. DIFFERENTIAL DIAGNOSIS

Differentiate from acute DVT (softer edema, stasis dermatitis), lymphedema (nonpitting, fibrotic), and other causes like heart failure or drug-induced edema.

5.1 Key Differentiators

Chronic venous insufficiency shows pitting edema with elevation; lymphedema is nonpitting with fibrotic changes. Lymphedema may coexist with CVD.

6. INVESTIGATIONS & DIAGNOSIS

Venous duplex ultrasound for reflux and obstruction. Lymphoscintigraphy or MRI for lymphatic imaging. CEAP classification guides staging.

6.1 Diagnostic Tests

Venous duplex ultrasound, MRI (honeycomb pattern), and lymphoscintigraphy to confirm primary/secondary lymphedema.

7. MANAGEMENT & TREATMENT

Conservative measures (elevation, compression), pharmacologic (aspirin, flavonoids), and surgical (ablation, stripping) for varicose veins. Lymphedema managed with decongestive therapy, compression, and liposuction.

7.1 Compression Therapy

Graduated compression stockings (20–40 mmHg) for CVD; 30–40 mmHg for ulcers. Multilayer bandages for advanced stages.

7.2 Surgical Options

Endovenous ablation (laser/radiofrequency), sclerotherapy, and phlebectomy. Lymphatic surgery for severe cases.

8. PROGNOSIS & COMPLICATIONS

Venous ulcers risk ~20% in CVD; lymphangiosarcoma risk ~0.1% in chronic lymphedema. Complications include recurrent infections, progressive fibrosis, and limb loss.

8.1 Lymphangiosarcoma

Rare but lethal complication of chronic lymphedema, particularly in filariasis or post-mastectomy cases.

9. SPECIAL CONSIDERATIONS

Pregnancy: increased risk of varicose veins; compression therapy advised. Elderly: higher risk of complications; careful monitoring. Phlebolymphedema: overlap between chronic venous insufficiency and lymphedema.

9.1 Iatrogenic Causes

Surgical/irradiation of lymph nodes (breast, prostate cancer) leads to secondary lymphedema. Post-operative phlebolymphedema is increasingly recognized.

10. KEY POINTS & CLINICAL PEARLS

1. Use CEAP classification for CVD staging. 2. Graded compression (20–40 mmHg) is first-line for CVD and ulcers. 3. Lymphedema management includes decongestive therapy and compression. 4. Avoid diuretics in lymphedema. 5. Lymphangiosarcoma is a rare but fatal complication of chronic lymphedema.