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The Diagnosis and Treatment of Peripheral Lymphedema: 2016 Consensus Document of the International Society of Lymphology

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The Diagnosis and Treatment of Peripheral Lymphedema: 2016 Consensus Document of the International Society of Lymphology 170 Lymphology 49 (2016) 170-184 THE DIAGNOSIS AND TREATMENT OF PERIPHERAL LYMPHEDEMA: 2016 CONSENSUS DOCUMENT OF THE INTERNATIONAL SOCIETY OF LYMPHOLOGY This International Society of Lymphology “Consensus” of the international community (ISL) Consensus Document is the latest based on various levels of evidence. The revision of the 1995 Document for the document is not meant to override individual evaluation and management of peripheral clinical considerations for complex patients lymphedema (1). It is based upon modifica- nor to stifle progress. It is also not meant to tions: [A] suggested and published following be a legal formulation from which variations the 1997 XVI International Congress of define medical malpractice. The Society Lymphology (ICL) in Madrid, Spain (2), understands that in some clinics the method discussed at the 1999 XVII ICL in Chennai, of treatment derives from national standards India (3), and considered/ confirmed at the while in others access to medical equipment 2000 (ISL) Executive Committee meeting in and supplies is limited; therefore the suggested Hinterzarten, Germany (4); [B] derived from treatments might be impractical. Adaptability integration of discussions and written and inclusiveness does come at the price that comments obtained during and following the members can rightly be critical of what they 2001 XVIII ICL in Genoa, Italy as modified see as vagueness or imprecision in definitions, at the 2003 ISL Executive Committee meeting qualifiers in the choice of words (e.g., the use in Cordoba, Argentina (5); [C] suggested of “may... perhaps... unclear”, etc.) and from comments, criticisms, and rebuttals as mentions (albeit without endorsement) of published in the December 2004 issue of treatment options supported by limited hard Lymphology (6); [D] discussed in both the data. Most members are frustrated by the 2005 XX ICL in Salvador, Brazil and the reality that NO treatment method has really 2007 XXI ICL in Shanghai, China and undergone a satisfactory meta-analysis modified at the 2008 Executive Committee (let alone rigorous, randomized, stratified, meeting in Naples, Italy (7,8);[E] modified long-term, controlled study). With this under- from discussions and written comments from standing, the absence of definitive answers the 2009 XXII ICL in Sydney, Australia, the and optimally conducted clinical trials, 2011 XXIII ICL in Malmö, Sweden, the 2012 and with emerging technologies and new Executive Committee Meetings (9),and [F] approaches and discoveries on the horizon, from discussions at the 2013 XXIV ICL in some degree of uncertainty, ambiguity, and Rome, Italy, and the 2015 XXV ICL in San flexibility along with dissatisfaction with Francisco, USA, as well as multiple written current lymphedema evaluation and manage- comments and feedback from Executive ment is appropriate and to be expected. Committee and other ISL members during We continue to struggle to keep the document the 2016 drafting. concise while balancing the need for depth The document attempts to amalgamate and details. With these considerations in the broad spectrum of protocols and practices mind, we believe that this 2016 version advocated worldwide for the diagnosis and presents a Consensus that embraces the entire treatment of peripheral lymphedema into a ISL membership, rises above national coordinated proclamation representing a standards, identifies and stimulates promising Permission granted for single print for individual use. Reproduction not permitted without permission of Journal LYMPHOLOGY. 171 areas for future research, and represents the giosclerosis (secondary lymphedema) or as best judgment of the ISL membership on how a consequence of functional deficiency to approach patients with peripheral lymph- (e.g., lymphangiospasm, stasis, and valvular edema in the light of currently available insufficiency in primary or secondary evidence. Therefore, the document has been, lymphedema. Nonetheless, the common and should continue to be, challenged and denominator is that the lymphatic system debated in the pages of Lymphology (e.g., as (whether vessels, nodes, interstitium, etc, Letters to the Editor) and ideally will remain a or combinations) transport has fallen below continued focal point for robust discussion at the capacity needed to handle the presented local, national and international conferences load of microvascular filtrate including in lymphology and related disciplines. We plasma protein and cells that normally leak further anticipate as experience evolves and from the bloodstream into the interstitium. new ideas and technologies emerge that Swelling is produced by accumulation in the this “living document” will undergo further extracellular space of excess water, filtered/ periodic revision and refinement as the diffused plasma proteins, extravascular blood practice and conceptual foundations of cells and parenchymal/stromal cell products. medicine and specifically lymphology change This process culminates in proliferation of and advance. parenchymal and stromal elements with excessive deposition of extracellular matrix Keywords: Consensus, lymphedema, substances and adipose tissue. High output diagnosis, treatment, ISL, International failure (dynamic insufficiency) of the lymph Society of Lymphology circulation, on the other hand, occurs when a normal or increased transport capacity of I. GENERAL CONSIDERATIONS intact lymphatics is overwhelmed by an excessive burden of blood capillary filtrate. As a fundamental starting point, Examples include hepatic cirrhosis (ascites), lymphedema is an external (and/or internal) hypoalbuminemia associated with nephrotic manifestation of lymphatic system syndrome (anasarca), right heart failure, insufficiency and deranged lymph transport and deep venous insufficiency of the leg Some members prefer to define peripheral (peripheral edema). Although the final lymphedema as a symptom or sign resulting pathway is the manifestation of tissue edema from underlying lymphatic disease. It is whenever lymph formation exceeds lymph defined as an illness by the International absorption, the latter entities should properly Classification of Diseases from the World be distinguished from lymphedema, which is Health Organization. Lymphedema may be characterized by decreased lymphatic trans- an isolated phenomenon or associated with a port. In some syndromes where high output multitude of other disabling local sequelae or lymphatic transport failure is longstanding, even life-threatening systemic syndromes. Its a gradual functional deterioration of the nature may be acute, transitory, or chronic. draining lymphatics may supervene and In its purest form, the central disturbance is a thereby reduce overall transport capacity. low output failure (mechanical insufficiency) A reduced lymphatic circulatory capacity of the lymphvascular system; that is, overall then develops in the face of increased blood lymphatic transport is reduced. This capillary filtration. Examples include derangement arises either from congenital recurring infection, thermal burns, and lymphatic dysplasia (primary lymphedema) repeated allergic reactions. These latter or anatomical obliteration, such as after conditions are associated with “safety valve radical operative dissection (e g., axillary or insufficiency” of the lymphatic system where retroperitoneal nodal sampling), irradiation, the lymph load increases over “normal” flow or from repeated lymphangitis with lymphan- to eventually become overwhelmed and this Permission granted for single print for individual use. Reproduction not permitted without permission of Journal LYMPHOLOGY. 172 can be considered a mixed form of edema/ patient condition (ambulation, portability, lymphedema and as such are particularly fragility, etc.) can all impact individualized troublesome to treat. patient care and treatment. Peripheral lymphedema associated with chylous and non-chylous reflux syndromes Prevention/Early Identification and Treatment is an infrequent but complex condition that requires specific diagnostic measures and The recent promulgation of lists of risk treatment methods. There are other factors for secondary lymphedema has complicating diagnoses (e.g., genetic with become a highlighted issue due to publica- Turner or Noonan syndromes or arterial/ tions of “do’s and don’ts”. These are largely venous malformations) which require anecdotal and not sufficiently investigated. additional considerations. While some precautions rest on solid In the treatment of “classical” lymph- physiological principles (e.g., avoiding edema of the limbs (that is, peripheral excessive heat on an “at risk” limb, not lymphedema), improvement in swelling can having chemotherapy administered into the usually be achieved by non-operative therapy. limb unless medically necessary, or trying to Because lymphedema most often becomes a avoid infections), others are less supported. chronic, generally incurable condition, it Consistently, a BMI >25, axillary node generally requires, as do other chronic dissection, radiation to the axilla, and disorders, lifelong care and attention along appearance of cellulitis following operation with psychosocial support. The continued are more firmly supported as true risks. It need for therapy does not mean a priori that must be noted that most published studies on treatment is unsatisfactory, although often it incidence of secondary lymphedema of the is less than optimal. For example, patients extremities report less than 50% chance of with diabetes
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