Lymphoedema Evidence Library
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Definitions and staging

Definitions, classification and staging

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Evidence in one minute

  • Lymphoedema is low-output failure of lymph transport — primary (developmental) or secondary (surgery, radiotherapy, infection, filariasis, trauma); ~85% worldwide is secondary. High-output oedema (heart, liver, kidney, venous) is different, but long-standing high-output states can damage lymphatics and become mixed. Executive Committee of the International Society of Lymphology 2023
  • Chronic oedema = swelling present ≥3 months; Wounds Canada 2025 treats all chronic oedema as lymphatic dysfunction — a deliberately broad definition. Keast 2025 · Charles 2013
  • ISL stages: 0 latent (measurable only — BIS, TDC); I pitting, reduces with elevation; II fat and fibrosis, elevation rarely helps; III lymphostatic elephantiasis. A limb can carry more than one stage.
  • Severity (unilateral): minimal/mild >5 to <20% excess volume; moderate 20–40%; severe >40%.
  • Primary lymphoedema by age: congenital (<2 y), praecox (2–35 y), tarda (>35 y); familial forms warrant genetic testing (FOXC2, FLT4/VEGFR-3, VEGFC, SOX18, GJC2, PIEZO1, GATA2 and others).
  • Lipoedema is not lymphoedema (fat disorder sparing hands and feet; Stemmer negative) but can progress to lipo-lymphoedema. Lipoedema
  • SA usage: the LAOSA statement adopts ISL staging and the same primary/secondary split. Davey 2018

Certainty: definitions and staging are consensus (LoE 5), internationally agreed; severity bands are conventional rather than outcome-validated.

Burden figures (all estimates)

  • >200 million worldwide; 1–2% of population quoted by SA authors (extrapolated, no SA data) → 530 000–1.06 million South Africans. Davey 2014
  • Primary lymphoedema ~1 in 6000 births (Keast; other sources 1.15 per 100 000 under 20 — figures conflict); lymphatic filariasis >120 million infected. Keast’s “80% of the morbidly obese have lymphoedema” is uncited (revised 2026-10-09). Keast 2014
  • Chronic oedema 1.33 per 1000, ~1 per 200 over 65 (Moffatt 2003, via Charles 2013).

Gaps

No South African prevalence study exists in this library.

How the evidence points

HighMod.LowV. lowNone
Positive00000
Null or negative00000

Sources on this page with a comparison, by the result of their primary outcome and the GRADE certainty of their main finding (Mod. = Moderate, V. low = Very low, None = not graded). Context sources are not counted. How direction is decided.

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