Lymphoedema

Last reviewed: November 2018

Last updated: April 2018

Summary

Chronic, progressive swelling of tissue with protein-rich fluid as a consequence of developmental (primary lymphoedema) or acquired (secondary lymphoedema) disruption of the lymphatic system. Extremities are most commonly affected, followed by genitalia.

Most cases are secondary to nematode infection (filariasis), malignancy, or cancer-related treatment.

Typically presents with painless unilateral limb swelling; pitting oedema is present in early disease, whereas non-pitting oedema is a sensitive but non-specific finding in advanced disease.

Diagnosis is usually made on clinical grounds and confirmed by lymphoscintigraphy.

First-line treatment involves compression, ranging from static garments to complex massage and pneumatic compression devices. Surgical procedures are reserved for patients refractory to conservative measures and/or with significant morbidity.

There is no cure. Successful care requires a long-term, collaborative approach between patient and providers.

Definition

The progressive swelling of a body part, usually an extremity, following developmental (primary lymphoedema) or acquired (secondary lymphoedema) disruption of the lymphatic system resulting in lymph (a protein-rich fluid) accumulating in the interstitial space. The extremities are most commonly involved, followed by the genitalia.

History and exam

Key diagnostic factors

hx of malignancy
hx of travel to endemic filariasis area
hx of previous surgery
hx of radiotherapy
painless unilateral swelling of extremity or genitalia
involvement of distal extremity
positive Stemmer's sign

Full details

Other diagnostic factors

hx of penetrating trauma to the axilla or groin
limb heaviness and/or weakness
non-pitting oedema
skin changes
FHx of lymphoedema

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Risk factors

breast cancer treatment
nematode infection (filariasis)
surgery near axillary or inguinal lymph nodes
advanced tumour, nodes, and metastasis (TNM) stage
radiotherapy
trauma
curvilinear scars
FHx of lymphoedema
genetic syndrome
obesity

Full details

Diagnostic investigations

1st investigations to order

lymphoscintigram

Full details

Investigations to consider

MRI scan of affected extremity
CT scan of affected extremity
ultrasound of affected area
lymphangiography
blood smear for filariasis
genetic testing

Full details

Treatment algorithm

ONGOING

all patients

Contributors

Authors VIEW ALL 

Reid A. Maclellan, MD, MMSc

Department of Plastic and Reconstructive Surgery

Instructor of Surgery

Children’s Hospital Boston

Harvard Medical School

Boston

Disclosures

RAM is an author of a number of references cited in this monograph.

Arin K. Greene, MD, MMSc

Department of Plastic and Reconstructive Surgery

Children's Hospital Boston

Co-director

Lymphedema Clinic

Associate Professor

Harvard Medical School

Boston

Disclosures

AKG is an author of a number of references cited in this monograph.

Acknowledgements

Dr Reid A. Maclellan and Dr Arin K. Greene would like to gratefully acknowledge Dr Sumner A. Slavin and Dr Carolyn C. Schook, previous contributors to this monograph. SAS and CCS are authors of a number of references cited in this monograph.

Peer reviewers VIEW ALL 

Hakan Brorson, MD, PhD

Senior Consultant Plastic Surgeon

Department of Plastic and Reconstructive Surgery

Lund University

Malmoe University Hospital

Malmoe

Sweden

Disclosures

HB is an author of a number of references cited in this monograph.

Neil R. Feins, MD

Professor

Harvard Medical School

Senior Surgeon

Children's Hospital Boston

Boston

Disclosures

NRF declares that he has no competing interests.

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Lymphoedema

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic investigations

1st investigations to order

Investigations to consider

Treatment algorithm

all patients

Contributors

Authors VIEW ALL 

Reid A. Maclellan, MD, MMSc

Disclosures

Arin K. Greene, MD, MMSc

Disclosures

Acknowledgements

Peer reviewers VIEW ALL 

Hakan Brorson, MD, PhD

Disclosures

Neil R. Feins, MD

Disclosures

Differentials

Guidelines

Patient leaflets

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