Lymphoedema – causes

Primary – usually the result of inadequate lymphatics, such as hypoplasia (55%), varicose dilatation (24%), or aplasia (14%):

• congenital lymphoedema – i.e. present at birth
  • Milroy’s disease – chronic hereditary lymphoedema
• lymphoedema praecox – manifests in teens/twenties, female, unilateral or bilateral
• lymphoedema tarda – manifest > 35 years old

Secondary – usually the result of absent or inadequate nodes:

• malignant infiltration
  • secondary carcinoma
  • primary reticulocytosis
• surgical excision
• radiation
• infection
  • filariasis
  • tuberculous
  • Granuloma Inguinale
• inflammation

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Pathophysiology of lymphoedema:

lymphatic obstruction → increased pressure / dilatation lymphatic channels → secondary incompetence of valves → reduced amount proteinaceous fluid drained → increased interstitial oncotic pressure → increased water content (compensatory) of interstitium

Complications (chronic):

• thickened hyperkeratotic skin
• recurrent cellulitis / lymphangitis
  • fibrosis (non-pitting)
• lymphangiosarcoma – lymphatic endothelium

Immunohistologic and electron microscopic examinations proved that this tumor originates in the vascular endotheliocytes, even though they are clinically chronic lesions apparently derived from the lymphatic vessels.¹

Diagnosis:

• firm, rubbery → non-pitting
• overnight elevation → little reduction in swelling
• natural history: progressive swelling

Investigations:

• phlebogram
• lymphangiogram
• lymphoscintigraphy

Management:

1. control oedema early
   1. foot elevation
   2. elastic stockings
   3. reduced salt intake + diuretics (acutely)
   4. skin hygiene / care to prevent recurrent cellulitis
      1. otherwise, long-term prophylactic antibiotics
2. prevent recurrent infection – treat before fibrosis sets in
3. surgery – indicated for the ~10% of patients with pain, impaired function of limb (due to size and weight), lymphangiosarcoma, cosmetic
   1. excision of skin and lymphedematous subcutaneous tissue + skin grafting
   2. transfer lymphatics with skin / omental flaps
   3. microvascular surgery

Although not the sole basis of decision making, cosmesis is an important factor as most patients are young women.

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References

1. Tomita K, Yokogawa A, Oda Y, Terahata S. Lymphangiosarcoma in postmastectomy lymphedema (Stewart-Treves syndrome): ultrastructural and immunohistologic characteristics. J Surg Oncol. 1988 Aug;38(4):275-82. doi: 10.1002/jso.2930380415. PMID: 2842548.