Lymphoedema Evidence Library › Topics › Definitions and staging
Definitions and staging
Definitions, classification and staging
33 sources
Evidence in one minute
- Lymphoedema is low-output failure of lymph transport: primary (developmental) or secondary (surgery, radiotherapy, infection, filariasis, trauma); about 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 is swelling present for ≥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, by BIS or 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 or mild >5 to <20% excess volume; moderate 20–40%; severe >40%.
- Primary lymphoedema by age at onset: congenital (<2 years), praecox (2–35), tarda (>35); familial forms warrant genetic testing (FOXC2, FLT4/VEGFR-3, VEGFC, SOX18, GJC2, PIEZO1, GATA2 and others).
- Lipoedema is not lymphoedema (a fat disorder sparing the 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 international consensus (level 5); the severity bands are conventional, not validated against outcomes.
How the ISL consensus evolved
- Adelaide 1985 working groups → first consensus 1995 → 2001 draft → 2003 (three stages written down; stage 0 recognised) → 2009 → 2013 → 2016 → 2020 → 2023. None has a systematic search or evidence grading. Casley-Smith 1985 · International Society of Lymphology Executive Committee 1995 · International Society of Lymphology 2003 · International Society of Lymphology 2009 · International Society of Lymphology 2013 · Executive Committee of the International Society of Lymphology 2016 · Executive Committee of the International Society of Lymphology 2020
- Positions that changed: liposuction "discouraged" (1995) → accepted for fat-dominant lymphoedema (2003 on); arm "precautions" questioned from 1998 (Witte editorial) and in the text from 2009; weight loss "has been shown to help" (2016) → "very limited evidence" (2020); lipoedema added to the differential in 2020.
- Other guidance: Dutch CBO 2002 defines lymphoedema as a ≥10% volume rise over the pre-operative baseline; Japan 2011 grades no treatment above C and drugs E; ICF core sets 2015. Damstra 2007 · Japan Lymphoedema Study Group 2011 · Viehoff 2015
Natural history
- Untreated lymphoedema grows with duration, arms faster than legs. Casley-Smith 1995
- Most rodent models resolve spontaneously within weeks, unlike human lymphoedema, so treat animal-based treatment claims with caution. Hsu 2022
Burden figures (all estimates)
- More than 200 million people worldwide; SA authors quote 1–2% of the population (extrapolated, with no SA data), giving 530 000–1.06 million South Africans. Davey 2014
- Primary lymphoedema about 1 in 6 000 births (Keast; other sources give 1.15 per 100 000 under 20 — the 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 1 000, about 1 in 200 over 65 (Moffatt 2003, cited in Charles 2013).
For the therapist
- Do: record ISL stage, severity (excess volume) and the ICF-based functional impact; add pitting, Stemmer sign and skin changes.
- Remember: stage correlates poorly with quality of life and symptoms, so record both.
- Certainty: consensus.
Gaps and warnings
- No South African prevalence study exists in this library.
How the evidence points
| High | Mod. | Low | V. low | None | |
|---|---|---|---|---|---|
| Positive | 0 | 0 | 0 | 0 | 0 |
| Null or negative | 0 | 0 | 0 | 0 | 0 |
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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