2009.42.2.concensus.pdf

Purpose & Patient Population

To review the evidence regarding evaluation and management of patients with peripheral lymphedema

Type of Resource/Evidence-Based Process

This International Society of Lymphology (ISL) Consensus Document is the current revision of the 1995 Document for the evaluation and management of peripheral lymphedema. It is based on modifications that were

Suggested and published following the 1997 XVI International Congress of Lymphology (ICL) in Madrid, Spain, which were discussed at the 1999 XVII ICL in Chennai, India, and considered and confirmed at the 2000 (ISL) Executive Committee meeting in Hinterzarten, Germany.
Derived from integration of discussions and written comments obtained during and following the 2001 XVIII ICL in Genoa, Italy, as modified at the 2003 ISL Executive Committee meeting in Cordoba, Argentina.
From suggestions, comments, criticisms, and rebuttals as published in the December 2004 issue of Lymphology.
Suggested from discussions from both the 2005 XX ICL in Salvador, Brazil, and the 2007 XXI ICL in Shanghai, China, as modified at the 2008 Executive Committee Meeting in Naples, Italy.

Search strategy was not provided.

Results Provided in the Reference

The consensus included the following components.

General considerations: Because lymphedema is a chronic, generally incurable ailment, it generally requires, as do other chronic disorders, lifelong care and attention along with psychosocial support. The compliance and commitment of the patient also is essential to an improved outcome.
Staging of lymphedema: The current lymphedema stages (Stage O, Stage I, Stage II, and Stage III) only refer to the physical condition of the extremities. A more detailed and inclusive classification needs to be formulated in accordance with improved understanding of the pathogenetic mechanisms of lymphedema (e.g., nature and degree of lymphangiodysplasia, lymph flow perturbations and nodal dysfunction as defined by anatomic features and physiologic imaging and testing) and underlying genetic disturbances. Recent publications incorporating both physical (phenotypic) findings with functional imaging into a combined staging may be forecasting the future changes in staging.
Diagnosis: The diagnosis of lymphedema can be readily determined from the clinical history and physical examination. A thorough medical evaluation is indispensable before embarking on lymphedema treatment.
- Imaging: If the diagnosis of lymphedema is unclear or in need of better definition for prognostic considerations, consultation with a clinical lymphologist or referral to a lymphologic center if accessible is recommended. The diagnostic tool of isotope lymphography (also termed lymphoscintigraphy or lymphangioscintigraphy) has proved extremely useful for depicting the specific lymphatic abnormality.
- Genetic testing is almost becoming practical to define a limited number of specific hereditary syndromes with discrete gene mutations such as lymphedemadistichiasis (FOXC2), some forms of Milroy disease (VEGFR-3), and hypotrichosislymphedema-telangiectasis (SOX18).
- Biopsy: Caution should be exercised before removing enlarged regional lymph nodes in the setting of longstanding peripheral lymphedema as the histologic information is seldom helpful and such excision may aggravate distal swelling.
Treatment: Therapy of peripheral lymphedema is divided into conservative (nonoperative) and operative methods.
- Meticulous skin hygiene and care (e.g., cleansing, low pH lotions, emollients) is of importance to the success of virtually all treatment approaches.
- Basic range of motion exercise of the extremities combined with external limb compression and limb elevation are also helpful to virtually all patients undergoing treatment. As previously stated, even widely used methods have yet to undergo sufficient meta-analysis of multiple studies, which have been rigorous, well-controlled, and with sufficient follow-up.

Guidelines & Recommendations

The following Research Agenda has been proposed.

Ongoing epidemiologic studies on the incidence and prevalence of lymphedema regionally and worldwide need to be conducted.
Assessment of lymphedema risk and steps for lymphedema prevention in different groups of at-risk patients need to be determined. Studies might include research on minimizing or preventing secondary lymphedema through altered operative or sampling techniques (e.g., sentinel node biopsy, precise anatomical knowledge of derivative pathways), vector control (as demonstrated in China) and prophylactic drugs for filariasis, identification of patients with heritable genetic defects for lymphangiodysplasia (lymphedema), and use of massage or compression where lymphatic drainage is subclinically impaired as documented by imaging techniques.
Research in molecular lymphology, including lymphatic system genomics and proteomics, should be encouraged. With the cellular and molecular basis of lymphedema-associated syndromes better defined, an array of specificbiologically based treatments, including modulators of lymphatic growth and function, should become available.
Improved imaging techniques and physiological testing need to be devised to allow more precise noninvasive methods to measure lymph flow dynamics and lymphangion activity. Continuous improvement is needed in imaging techniques as well as in the development of new technologies (e.g., near infrared) to visualize the superficial and deep lymphatic system.
As knowledge accrues, the current crude classification of lymphedema should be revisited and modified to include a more encompassing clinical description based on genetic, anatomic, and functional disability.
Accordingly, treatment, whether by designer drugs, gene or stem cell therapy, tissue engineering, physical methods, or new operative approaches, should be directed at preventing, reversing, or ameliorating the specific lymphatic defect and restoring function and quality of life.

Nursing Implications

Lymphedema may be simple or complex but should not be neglected. Accurate diagnosis and effective therapy is now available, and lymphology itself is now recognized as an important specialty in which clinicians are carefully trained in the intricacies of the lymphatic system, lymph circulation, and related disorders. The emerging era of molecular lymphology should result in improved understanding, evaluation, and treatment in clinical lymphology. Limited evidence exists regarding treatment. Basic recommendations are meticulous skin hygiene and care and range of motion exercise with compression. This document provides extensive information on the state of knowledge and areas for future research in lymphedema prevention and management.