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Clinical Review ABC of arterial and venous disease

Swollen lower limb—2: Lymphoedema

BMJ 2000; 320 doi: (Published 03 June 2000) Cite this as: BMJ 2000;320:1527
  1. Peter S Mortimer

    Lymph conducting pathways may become reduced in number, obliterated, obstructed, or dysfunctional (because of failure of contractility or valve incompetence). A lack of sensitive methods for investigation makes it difficult to distinguish between these mechanisms. A defect in the lymph conducting pathways leads to primary lymphoedema; in practice this means no identifiable outside cause can be found. Secondary lymphoedema is due to factors originating outside the lymphatic system.

    Classification of primary lymphoedema

    Primary lymphoedema

    Congenital lymphoedema presenting at or soon after birth is rare. A family history suggests Milroy's disease. Swelling invariably affects both lower limbs, but the upper limbs and face may also swell.

    Limb swelling may be the presenting and major manifestation of congenital lymphatic malformations either in a pure form—for example, diffuse lymphangioma—or in combination with a congenital vascular syndrome—for example, Klippel-Trenaunay syndrome (varicose veins, excessive long bone growth, and vascular birthmark).

    Primary lymphoedema with bilateral below knee swelling due to hypoplasia of peripheral lymphatic vessels

    Most forms of primary lymphoedema present after puberty with foot and ankle swelling. Women are more often affected, and the condition may be familial—for example, Meige's disease. Lymph reflux due to lymphatic vessel hypertrophy or megalymphatics is clinically distinguishable.

    Secondary lymphoedema

    Lymphoedema manifesting with sudden onset of swelling of one whole leg suggests proximal obstruction. Pelvic causes of venous or lymphatic …

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