Clinical features and diagnosis of peripheral lymphedema
- Emile R Mohler III, MD
Emile R Mohler III, MD
- Section Editor — Vascular Medicine
- Professor of Medicine
- University of Pennsylvania School of Medicine
- Babak Mehrara, MD
Babak Mehrara, MD
- Associate Professor of Surgery
- Cornell Weill Medical Center
- Section Editors
- John F Eidt, MD
John F Eidt, MD
- Section Editor — Vascular and Endovascular Surgery
- Professor of Surgery, Texas A&M Health Science Center
- Vice Chair of Vascular Surgical Services, Baylor Heart and Vascular Hospital at Dallas
- Joseph L Mills, Sr, MD
Joseph L Mills, Sr, MD
- Section Editor — Vascular and Endovascular Surgery
- Professor and Chief
- Division of Vascular Surgery and Endovascular Therapy
- Baylor College of Medicine
- Harold Burstein, MD, PhD
Harold Burstein, MD, PhD
- Section Editor — Breast Cancer
- Associate Professor of Medicine
- Harvard Medical School
Lymphedema is defined as the abnormal accumulation of interstitial fluid and fibroadipose tissues resulting from injury, infection, or congenital abnormality of the lymphatic system. Lymphedema is classified as primary or secondary depending on etiology and presentation.
The etiology, risk factors, clinical manifestations, classification, diagnosis, and differential diagnosis of lymphedema will be reviewed here. The prevention and treatment of lymphedema, the operative management of primary and secondary lymphedema, and an overview of the pathophysiology and etiology of generalized edema are presented separately. (See "Clinical staging and conservative management of peripheral lymphedema" and "Surgical treatment of primary and secondary lymphedema" and "Pathophysiology and etiology of edema in adults" and "Pathophysiology and etiology of edema in children".)
NORMAL LYMPHATIC SYSTEM AND LYMPHEDEMA
Lymph flow takes place in a low-pressure system. Uptake of interstitial fluid begins in lymphatic capillaries and precollectors that abut the interstitial space. The process is facilitated by local arterial pulsation, skeletal muscle contraction, and unidirectional valves that prevent retrograde flow. The initial lymphatics merge into vessels of increasing caliber partially surrounded by a specialized smooth muscle layer that, upon contraction, promotes peristaltic movement of the lymph fluid toward the systemic circulation.
The lymph draining from both lower extremities, the gastrointestinal tract, and the left upper body (upper extremity, chest wall, upper back, shoulder, and breast) enter the venous circulation through the thoracic duct, which opens into the venous angle between the left subclavian vein and left internal jugular vein (figure 1) . The lymph draining from the right upper body drains into the right lymphatic duct via a similar right-sided venous angle.
In the extremities, the superficial lymphatic system drains the skin and subcutaneous tissue and drains into the deep system that then drains into the lymph nodes in the axilla or pelvis [1,2].
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- NORMAL LYMPHATIC SYSTEM AND LYMPHEDEMA
- - Primary
- - Secondary
- EPIDEMIOLOGY AND RISK FACTORS
- Cancer and cancer treatment
- - Lymphadenectomy
- - Radiation therapy
- Inflammatory disorders
- Hereditary syndromes
- CLINICAL FEATURES
- History and physical
- Extremity measurements
- - Limb circumference
- - Limb volume
- Clinical severity
- Differential diagnosis
- FURTHER EVALUATION
- Imaging the lymphatic system
- Genetic testing
- INFORMATION FOR PATIENTS