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Lymphedema arises due to a malfunction of the lymphatic system and can lead to massive tissue swelling. Complete decongestive therapy (CDT), consisting of manual lymphatic drainage (MLD) and compression bandaging, is aimed at mobilizing fluid and reducing volume in affected extremities. Lymphatic dysfunction has previously been associated with chronic inflammation processes. We investigated plasma ADMA as an indicator of endothelial function/inflammation before-, during- and after-CDT. Also assessed were vascular function parameters such as carotid-femoral pulse wave velocity (PWVcf), flow-mediated dilatation (FMD) and retinal microvasculature analysis. 13 patients (3 males and 10 females, 57 ± 8 years old (mean ± SD), 167.2 ± 8.3 cm height, 91.0 ± 23.5 kg weight), with lower limb lymphedema were included. Vascular function parameters were assessed on day 1, 2, 7, 14 and 21 of CDT, pre- and post-MLD. ADMA was significantly lower post-MLD (p=0.0064) and tended to reduce over three weeks of therapy (p=0.0506). PWVcf weakly correlated with FMD (r=0.361, p=0.010). PWVcf, FMD and retinal microvasculature analysis did not show changes due to physical therapy. The novel results from this study indicate that lymphe - dema does not affect endothelial function and lymphedema patients may therefore not have a higher risk of cardiovascular diseases. Our results further suggest that manual lymphatic drainage with or without full CDT could have potentially beneficial effects on endothelial function in lymphedema patients (by reducing ADMA levels), which has not been reported previously. [ABSTRACT FROM AUTHOR]

Acupuncture is a potential therapy for breast cancer-related lymphedema (BCRL). Despite a recent meta-analysis on efficacy, data on acupuncture safety in BCRL are lacking. Current clinical guidelines recommend avoiding needling in the upper extremity affected by lymph node dissection. We undertook a systematic review focusing on acupuncture safety and treatment protocols in clinical trials for BCRL. Literature searches were conducted in PubMed, Ovid, CINAHL, and Cochrane library. Eight clinical trials on acupuncture for BCRL were analyzed. The Standards of Acupuncture intervention (STRICTA 2010) and Cochrane risk of bias (RoB2 2019) were applied to assess methods for acupuncture interventions within Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) framework. Quantity and severity of adverse events (AE) were reviewed. A total of 189 subjects participated in 8 clinical trials with 2965 acupuncture treatments. No serious adverse events (SAE) were reported regardless of treatment laterality or protocol, with only a single grade 2 skin infection in 2,965 total treatments (0.034%), including 1,165 bilateral and 225 ipsilateral treatments. Our comprehensive review of clinical trials of acupuncture for BCRL demonstrated no significant adverse events in 2,965 treatments, including 1,390 in the affected limb. An approach for routine integration of acupuncture into BCRL maintenance therapy is proposed., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

Functional compressive bandaging (FCB) is a therapeutic resource used to control lymphedema resulting from the treatment of breast cancer. However, the reliability of the technique is unknown. We evaluated intra- and inter-rater reliability of the spiral technique in the four-layer FCB of the arm and forearm in breast cancer survivors with lymphedema. Forty-five breast cancer survivors with a mean age of 64.88±10.01 years participated in the study. Evaluations were performed by two examiners at different times analyzing the pressure exerted (mmHg) by the spiral FCB in the arm and forearm of the upper limb affected by lymphedema. The intraclass correlation coefficient (ICC2,1) was used to determine intraand inter-examiner reliability, with a 95% confidence interval, minimum detectable change, and standard error of the measurement. Intrarater reliability was considered low to high in the arm and forearm region. Inter-rater reliability in the arm region was considered low and in the forearm region low to moderate. Our results indicate that spiral FCB has low to moderate intra-examiner and inter-examiner reliability., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

Although cancer survivors are recommended to exercise, they may lack confidence (self-efficacy) to be active. This research aimed to measure exercise barriers and related selfefficacy in individuals with cancer-related lymphedema as well as examine relationships between self-efficacy and participant characteristics. A cross-sectional survey was undertaken in individuals with cancer-related lymphedema using a validated 14-item Likert scale assessing self-efficacy to overcome general and lymphedema-specific exercise barriers (0%=not at all confident, 100%=extremely confident). Demographic, medical and lymphedema data were also collected. Of 109 participants (52% response), 79% (n=86) had breast cancer-related lymphedema. Participants were found to be moderately confident to exercise when facing general (48% [95% CI: 44, 52]) and lymphedema- specific exercise barriers (51% [95% CI: 47, 55]). Participants who were female, sedentary (p<0.05), had lymphedema for ≥2 years, and reported greater symptom burden (p<0.05) recorded lower general exercise barriers selfefficacy. Lower lymphedema-specific exercise barriers self-efficacy was reported by individuals who were sedentary, had cancers other than breast, and higher symptom burden. These findings suggest general and lymphedema- specific barriers challenge exercise confidence in those with cancer-related lymphedema, and strategies tailored to improve confidence in overcoming exercise barriers are warranted. Supporting individuals to be sufficiently active during and following cancer treatment should consider behavior change strategies tailored to the unique needs faced by individuals with lymphedema., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

Worldwide, lymphedema can present as a significant health issue. Left untreated, it can have long-term medical and psychological consequences for patients. Cryotherapy is a new physical therapy modality used for many purposes including reduction of pain, inflammation, and edema. It is thought to decrease interstitial fluid volume through many mechanisms. Therefore, it is reasonable to think that cryotherapy might have a positive effect in treatment of lymphedema. The goal of this study was to investigate how local cryotherapy in combination with standard therapy affects patient outcomes. Forty post-mastectomy female patients aged 40-60 years old with lymphedema were referred to the outpatient clinics of the Faculty of Physical Therapy at South Valley University for medical treatment and follow-up by the vascular surgery department. Patients were randomly divided into two groups of equal size. Traditional physical therapy programs (manual lymphatic drainage, pneumatic compression, bandaging, breathing exercises, circulatory exercises, shoulder mobilizations, and ROM exercises) were combined with pulsed local cryotherapy three times per week for 12 weeks in Group (A). For 12 weeks, Group (B) received only traditional physical therapy three times per week. Patients were evaluated using circumferential measurement with tape at the wrist, below the elbow, and above the elbow level, as well as ultrasonography to assess skin thickness before the start of physical therapy, 6 weeks later, and at the end of the treatment (after 12 weeks). Results indicate that cryotherapy is an effective adjunct modality for the treatment of secondary lymphedema and should be added to physical therapy protocols for lymphedema rehabilitation., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

Lymphedema is a debilitating disease characterized by abnormal lymphatic drainage, either due to primary maldevelopment of the lymphatic system or to secondary injury. The clinical features of primary and secondary lymphedema differ, with primary lymphedema more often involving progressive bilateral lower extremity disease as compared to secondary lymphedema characteristically having more localized symptoms related to the origin of injury. This case presentation describes a patient who presented with bilateral lower extremity swelling, left greater than the right, with imaging results to support the diagnosis of lymphedema. During the time he was followed in our clinic, our team witnessed rapid progression of his lymphedema despite compliance with conservative management. We believe that the primary mechanism of systemic damage to our patient's lymphatic system is the lenalidomide and bortezomib therapy prescribed to treat multiple myeloma. This review explores the relationship between lenalidomide, bortezomib, and lymphedema in efforts of understanding this unique pathology of iatrogenic lymphedema mimicking primary nature., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

We designed a study to compare effects of complete decongestive therapy (CDT) and kinesiology taping (KT) (with exercise and skin care) on limb circumference, lymphedema volume, grip strength, functional status, and quality of life in patients with unilateral breast cancer-related lymphedema (BCRL). Forty patients with unilateral stage 2 BCRL were randomized to either the CDT group (n=20) or the KT group (n=20). Patients in the CDT group underwent 30-min manual lymphatic drainage (MLD) and multi-layer, short-stretch bandaging once a week for four weeks. Patients in the KT group underwent taping once a week for four weeks. In addition, all patients were informed about skin care and given an exercise program throughout the treatment. Upper extremity circumference and volume differences as primary outcomes and grip strength, Quick-Disabilities of the Arm, Shoulder and Hand (Q-DASH), and Functional Assessment of Cancer Therapy-Breast (FACT-B) scores as secondary outcomes were assessed initially, after treatment (4 weeks), and at the 1st month follow-up. Limb circumference and volume differences were significantly reduced in the CDT group after the 4-week treatment compared with the KT group (p=0.012 and p=0.015, respectively), but there was no difference between the groups in the 1st month follow-up (p>0.05). There was no difference between the groups in terms of grip strength, Q-DASH, and FACT-B scores after treatment and at the 1st month follow-up (p>0.05). Our results show that both KT and CDT were found to significantly reduce limb volume and circumference individually at 4-weeks and the one-month follow-up in patients with BCRL and that CDT significantly reduced both limb volume and circumference compared to KT at the 4- week time point, but not at the follow-up. Further randomized controlled trials with patients at different stages of BCRL are needed to confirm and expand these results., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

Lymphedema is a common condition with global impact and a multitude of complications, however, only a few professionals specialize in its management. A retrospective analysis of 105 subjects with unilateral lymphedema upper or lower limb was performed to investigate whether the duration of lymphedema constitutes an important factor associated with the efficacy of complete decongestive therapy (CDT). Subjects were classified into two groups according to the duration of lymphedema, prior to CDT: group A (≤1 year) and group B (>1 year). Both groups were treated daily according to the same CDT protocol for four weeks. The CDT efficacy was determined based on the percent reduction of excess volume (PREV) measurements. Lymphedema was significantly reduced in both groups of subjects, but significantly more in group A (p<0.001). In subjects with upper limb lymphedema, median value of PREV was 80.8% (interquartile range, 79.1-105.0%) in group A and 62.0% (interquartile range, 56.7-66.5%) in group B (p<0.001). In subjects with lower limb lymphedema PREV was 80.7% (interquartile range, 74.9-85.2%) and 64.5% (interquartile range, 56.0-68.1%) for groups A and B, respectively (p<0.001). Duration of lymphedema was found to be a strong predictive factor that may significantly impact CDT efficacy. Therapeutic effects were increased in subjects who were detected and treated earlier for lymphedema., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

Instagram® is one of the most active social media platforms with over a billion users worldwide. Since the importance of education on lymphedema has been established due to the chronic nature of the disease, seeking knowledge attracts much attention not only clinically but also on social platforms such as Instagram ®. Our aim was to examine content by analyzing posts tagged with hashtags on Instagram ® related to lymphedema. Nine predefined hashtags related to lymphedema were used to search posts uploaded to Instagram® via the Apify tool. Retrieved public posts were classified and analyzed by four researchers for their content and post-type. We found that the vast majority of sharing on Instagram® in the context of lymphedema and its related aspects have relatively low scores for both relevancy and accuracy with a 77% irrelevancy rate. The best posts were those determined to be educational, which were found 57% relevant and correct. Medical professionals should consider that disseminating true guidance and therapy carries importance for patients with lymphedema and treatment success. The ability for patients to reach knowledge via social media might also be an important aspect in reliving suffering due to lymphedema. However, our results demonstrate that Instagram® might not be a good platform for patients to discover reliable information about lymphedema., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

Secondary lower extremity lymphedema is a common complication of treatment for gynecological cancers. Conservative therapy plays an important role in the treatment of patients with secondary lower extremity lymphedema; in particular, complex decongestive therapy (CDT) has been recognized as an effective nonoperative technique for these patients. But CDT therapy for secondary lower extremity lymphedema remains a problem in China because this technique and its effectiveness have not achieved widespread use and popularity. Our goal was to assess effects of CDT in patients with secondary lower limb lymphedema after treatment for gynecological cancers. The retrospective study consisted of 60 patients who were treated with 20 sessions of CDT. Assessments included objective changes in limb circumference, degree of LE, imaging features, and incidence of erysipelas before and after CDT treatment. We found that CDT can effectively improve lymph stasis and promote backflow, and decrease circumference, interstitial fluid content, and incidence of erysipelas of lymphedematous lower limb. Our results demonstrate that CDT is an effective treatment method for patients with secondary lower limb lymphedema following treatment for gynecologic cancers. This technique should be more widely utilized and popularized in China to improve the quality of life of millions of patients with secondary lower limb lymphedema., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

This International Society of Lymphology (ISL) Consensus Document is the latest revision of the 1995 Document for the evaluation and management of peripheral lymphedema (1). It is based upon modifications: [A] suggested and published following the 1997 XVI International Congress of Lymphology (ICL) in Madrid, Spain (2), discussed at the 1999 XVII ICL in Chennai, India (3), and considered confirmed at the 2000 (ISL) Executive Committee meeting in Hinterzarten, Germany (4); [B] 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 (5); [C] suggested from comments, criticisms, and rebuttals as published in the December 2004 issue of Lymphology (6); [D] discussed in both the 2005 XX ICL in Salvador, Brazil and the 2007 XXI ICL in Shanghai, China and modified at the 2008 Executive Committee meeting in Naples, Italy (7,8); [E] modified from discussions and written comments from the 2009 XXII ICL in Sydney, Australia, the 2011 XXIII ICL in Malmö, Sweden, the 2012 Executive Committee Meetings (9); [F] discussions at the 2013 XXIV ICL in Rome, Italy, and the 2015 XXV ICL in San Francisco, USA, as well as multiple written comments and feedback from Executive Committee and other ISL members during the 2016 drafting (10); informal discussions at the XXVI ICL in Barcelona, Spain; and [G] discussions at a dedicated, focused Post-Congress session at the XXVII ICL in Iguazú, Argentina (2019) followed by additional written comments from the Executive Committee and others. The document attempts to amalgamate the broad spectrum of protocols and practices advocated worldwide for the diagnosis and treatment of peripheral lymphedema into a coordinated proclamation representing a "Consensus" of the international community based on various levels of evidence. The document is not meant to override individual clinical considerations for complex patients nor to stifle progress. It is also not meant to be a legal formulation from which variations define medical malpractice. The Society understands that in some clinics the method of treatment derives from national standards while in others access to medical equipment, technical expertise, and supplies is limited; therefore, the suggested treatments might be impractical. Adaptability and inclusiveness does come at the price that members can rightly be critical of what they see as vagueness or imprecision in definitions, qualifiers in the choice of words (e.g., the use of "may... perhaps... unclear", etc.) and mentions (albeit without endorsement) of treatment options supported by limited hard data. Most members are frustrated by the reality that NO treatment method has really undergone a satisfactory meta-analysis (let alone rigorous, randomized, stratified, long-term, controlled study). With this understanding, the absence of definitive answers and optimally conducted clinical trials, and with emerging technologies and new approaches and discoveries on the horizon, some degree of uncertainty, ambiguity, and flexibility along with dissatisfaction with current lymphedema evaluation and management is appropriate and to be expected. We continue to struggle to keep the document concise while balancing the need for depth and details. With these considerations in mind, we believe that this 2020 version presents a Consensus that embraces the entire ISL membership, rises above national standards, identifies and stimulates promising areas for future research, and represents the best judgment of the ISL membership on how to approach patients with peripheral lymphedema in the light of currently available evidence. Therefore, the document has been and should continue to be challenged and debated in the pages of Lymphology (e.g., as Letters to the Editor) and ideally will remain a continued focal point for robust discussion at local, national and international conferences in lymphology and related disciplines. We further anticipate as experience evolves and new ideas and technologies emerge that this "living document" will undergo further periodic revision and refinement as the practice and conceptual foundations of medicine and specifically lymphology change and advance., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

Surgical treatment of lymphedema with liposuction typically requires subsequent compression therapy. Here we describe an approach where secondary arm lymphedemas are initially treated by autologous lymphatic grafting to bypass the axilla and restore lymphatic flow. In the presence of excess adipose tissue, liposuction is then performed in a second procedure. To assess outcomes, the authors evaluated 28 consecutive adult patients who had undergone secondary liposuction following lymphatic grafting. Arm volumes were measured prior to lymphatic grafting and after the secondary liposuction. The necessity for additional treatment by compression garment and manual lymphatic drainage was assessed prior to lymphatic grafting and after the secondary liposuction following the direct postoperative regimen. The mean arm volumes were reduced significantly (p<0.001) from a mean of 3417± 171 (SEM) cm3 prior to lymphatic grafting to 3020±125 cm3 after reconstruction of the lymphatic vascular system and finally to 2516±104 cm3 after the secondary liposuction (SLS). All 28 adult patients underwent continuous compression and manual lymph-drainage (MLD) prior to the reconstructive surgery. All 28 patients were evaluated regarding necessity of any additional therapy more than 6 months after SLS with a median follow up period of 37 months (range, 7-160 months). 18 of 28 patients did not require any supportive therapy beyond 6 months after SLS to maintain the results. Three patients continued to utilize manual lymphatic drainage, 4 used a combination of MLD and compression therapy and 3 used elastic compression therapy (one patient only while at work). These results indicate that microsurgical restoration of lymphatic outflow followed by SLS eliminates the need for additional treatment in more than two thirds of patients., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

In the absence of guidance from scientific evidence, a range of lymphedema prevention and management, guidelines were developed by relevant organizations around the world. These became publicly available, promoted and endorsed, particularly to women with breast cancer. The recommendations advised avoidance of any activity that could overload or restrict the lymphatic system and need for caution when participating in specific physical activities. However, over the past 20 years evidence has accumulated which has significantly challenged the safety of these recommendations, in particular for those with cancer. There now exists consistent and compelling evidence in support of exercise following a diagnosis of cancer. Participating in exercise during and following cancer treatment improves function and quality of life, reduces treatment-related morbidity, and may improve survival. Further, exercise, including resistance exercise at moderate or high load, is considered safe for those at risk- or with lymphedema. That is, exercise has not been shown to cause or worsen cancer-related lymphedema. This article provides a historical account of the advice given to patients in the prevention and management of lymphedema and how this advice has evolved., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

Lymphedema of the lower limbs often contributes to the mobility impairment of morbidly obese patients. Defining novel costeffective protocols is important for reducing treatment costs. The study aimed to assess if Capacitive and Resistive Energy Transfer (TECAR) can reduce edema and the minimum number of sessions needed to observe volume reduction. Forty-eight severely obese subjects (age range: 46-78 years; BMI >40 kg/m2) with bilateral lower limb lymphedema were divided into three groups undergoing either manual lymphatic drainage, pressure therapy, or TECAR, in addition to a multidisciplinary rehabilitation program. They were compared to a control group composed by 12 women (age: 67.4 ± 8.9 years, BMI: 44.6 ± 4.1 Kg/m2) undergoing only the rehabilitation program. A handheld laser scanner 3D system was used for volume measurements. In addition, patients were evaluated with a Timed Up and Go (TUG) test and pain/heaviness of the lower limbs with a Visual Analog Scale (VAS). A significant volume reduction was observed after 6 sessions of TECAR: specifically, in the whole limb (PRE: 9.7+2.8 dm3; POST: 9.4+2.8 dm3; p<0.05) and in the thigh (PRE: 3.5+1.3 dm3; POST: 3.3+1.2 dm3; p<0.05). The TUG and VAS for pain showed a significant improvement in all groups. Our preliminary results suggest that TECAR can provide a relatively early reduction of lower limb edema with improvement of patients' function and pain., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

Advanced lymphedema is associated with a number of adverse skin changes including color, thickening of the epidermis, dryness, and hyperkeratosis. These changes are related to prolonged lymph stasis and contribute to an increased risk of infection. Similarly, lipedema is associated with skin thickening and appearance of nodular adipose deposition. Skin care is essential in both conditions. We examined whether inclusion of targeted skin products for 2 weeks to an established pre-surgical conservative treatment program was associated with beneficial effects on the skin condition in 150 patients with lymphedema and lipedema. Patients were randomly assigned to control or one of two treatment groups. All three groups (and for both lymphedema and lipedema) demonstrated a significant reduction in softness. Dimpling/ redness was significantly reduced in the targeted skin product groups for both patients with lymphedema or lipedema. Only patients with lipedema demonstrated a significant reduction in dryness/ hyperkeratosis following targeted skin product treatment. This study demonstrates that short-term use of targeted skin products in both patients with lymphedema and lipedema can be of benefit and further studies are needed to replicate these results and explore possible mechanisms., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

This is a randomized pilot study evaluating the effectiveness of customized compression garments (CG) in reducing the risk of lower limb lymphedema (LLL) in gynecological cancer patients. Patients who completed pelvic node dissection or radiation were routinely educated on reducing the risk of LLL by good skin care and manual lymphatic massage. After baseline lower limb volume perometry and clinical assessment, they were randomized to customized compression garment (CG) for 6 weeks (26 patients) or observation (30 patients). Both groups were followed up for 2 years and the primary outcome was the development of LLL. LLL incidence in the control group was 13.3% (4 of 30 patients) compared to 7.7% (2 of 26 patients) in the CG group. However the difference was not statistically significant (P=0.496). In the control group, 10.7% (3/28) who underwent node dissection developed LLL vs 7.7% (2/26) in the CG group. Among patients with node dissection plus radiation, LLL incidence was 14.3% (1/7) in the control group vs 12.5% (1/8) in the CG group. The mean onset of LLL was 12 months; compliance to CG wearing was high and QOL scores were similar in both groups. Customized low-compression CG worn for 6 weeks may have a possible benefit in reducing the risk of LLL when added to patient education on risk reduction although statistic significance was not achieved in this small pilot study. A larger multi-center study would be justified to expand these findings., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

We present an integrated therapeutic approach performed on a 37 year old female with giant lymphedema (Grade IV) due to lymphatic filariasis of 27 years duration. Our therapeutic approaches consisted of a basic foot care program for two weeks, followed by a course of oral penicillin for a week including conservative treatment with complete decongestive therapy (CDT) together with respiratory physiotherapy and walking exercises. In addition, advanced surgical techniques with supra-fascial excision of alternate lumps in three stages over an interval of ten days followed by a nodo-venal shunt resulted in reversing the stage IV lymphedema condition. Over a ten year follow-up, the patient remains essentially unchanged confirming the success of the treatment without any recurrence or complications. Finally, by combining multimodal treatment, we were able to achieve a near normal limb in Stage III and Stage IV lymphedema of the lower limb in lymphatic filariasis., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

This study evaluated how different levels of pressure under inelastic multi-layer bandages affect the reduction of secondary arm lymphedema and patient comfort throughout therapy. Ninety-six women with lymphedema after breast cancer treatment were randomized into three groups of 32 patients depending on level of applied pressure in compression therapy: group I (control) at 21-30 mmHg; study groups II A at 31-40 mmHg and group II B at 41-60 mmHg. All patients were treated with complex decongestive therapy (CDT) and intermittent pneumatic compression (IPC) before compression. Fixed points of bilateral arms were measured at the start (first visit), after 24 h, 7 days, and 14 days of therapy. Edema severity was measured by summary calculation. Results were presented as a relative metric coefficient of arm lymphedema (RMCAL) which is the difference between sums of circumferences of the edematous arm and the contralateral side expressed in percents. In order to evaluate the patient comfort after finishing a two-week therapy all patients assessed the level of accompanying pain using the numeric pain rating scale (NRS). At the start of therapy median arm circumference difference (RMCAL) was 18.60%, 18.51%, and 19.05% in groups I-II B, respectively. After 24 h the median RMCAL was reduced to 14.49%, 12.13%, and 12.64%. This was further reduced to 10.77%, 6.98%, and 8.48% at one week and 10.28%, 5.75%, and 7.20% in each group, respectively. There was no statistically significant difference between RMCAL values in group II A and II B throughout the therapy. In group II A (NRS = 2), applied bandages were better tolerated than in both II B (NRS = 5) and control groups (NRS = 8). These results demonstrate that inelastic multi-layer bandages applied in groups II A and II B (41-60 mmHg) led to the same reduction of swollen arm circumference with group II being better tolerated. The lowest compression (control at 21-30 mmHg) produced the smallest reduction. In addition, since the greatest reduction was seen in the first week of therapy while the second week served to maintain the reduction, compression garments may be able to be ordered after one week of therapy for more efficient patient care., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

During treatment of lymphedema, routine use of patient-reported outcomes measures (PROMs) is recommended to monitor patient progress; however, the validity functional status (FS) PROMs in these patients is unknown. Our aims were to examine construct validity of the shoulder computerized adaptive testing (CAT) and the foot-and-ankle CAT, as a measure of FS for patients selecting the shoulder or the foot-and-ankle as their main body part affected by their lymphedema. We assessed the ability of the FS scores to distinguish between patient groups in clinically expected ways at admission and discharge from physical therapy. At admission (n=1600), patients who were younger and had more acute symptoms, less severe lymphedema, less co-morbidities, no relevant surgical history, did not use medications for chronic conditions, and exercised regularly, had higher FS. At discharge (n=611), patients who were younger and had less advanced lymphedema, fewer co-morbidities, no relevant surgical history, did not use medications for chronic conditions, exercised regularly, and had more acute symptoms had higher FS change, after controlling for their baseline FS score. Low participation rates in FS outcomes data collection could have biased results. Overall, the CAT-based FS PROMs used in this study discriminated between patient groups in clinically logical ways both at intake and at discharge from lymphedema treatment., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

Lymphatic diseases, especially lymphedema, represent a serious problem in the health community. We investigated strategies and methods for physiotherapeutic rehabilitation of lymphedema by carrying out a comprehensive search of the Medline and Embase databases from 1990 to 2016 to identify relevant published studies, articles, and reviews. Approaches for conservative management of lymphedema include the following: manual lymphatic drainage, lymphedema rehabilitation exercises, compression therapy, skin care, pneumatic compression, elevation of the extremities, thermal therapy, complete decongestive physiotherapy (CDT), taping, and aqua lymphatic therapy. Treatment of lymphedema with CDT, which is a combination of four methods (manual lymphatic drainage, lymphedema rehabilitation exercises, compression therapy, skin care), can achieve a 45-70% reduction in lymphedema volume. Prerequisites for successful physiotherapy are the availability of physicians, nurses and therapists who are specifically trained, educated, and experienced in each method. CDT is the most effective treatment as it reduces the symptoms of lymphedema and improves patients' functionality, mobility, and quality of life. Although other therapeutic techniques have demonstrated positive results, these surveys are limited and more studies are needed to confirm findings., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright by International Society of Lymphology.)

This International Society of Lymphology (ISL) Consensus Document is the latest revision of the 1995 Document for the evaluation and management of peripheral lymphedema (1). It is based upon modifications: [A] suggested and published following the 1997 XVI International Congress of Lymphology (ICL) in Madrid, Spain (2), discussed at the 1999 XVII ICL in Chennai, India (3), and considered/ confirmed at the 2000 (ISL) Executive Committee meeting in Hinterzarten, Germany (4); [B] 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 (5); [C] suggested from comments, criticisms, and rebuttals as published in the December 2004 issue of Lymphology (6); [D] discussed in both the 2005 XX ICL in Salvador, Brazil and the 2007 XXI ICL in Shanghai, China and modified at the 2008 Executive Committee meeting in Naples, Italy (7,8);[E] modified from discussions and written comments from the 2009 XXII ICL in Sydney, Australia, the 2011 XXIII ICL in Malmö, Sweden, the 2012 Executive Committee Meetings (9),and [F] from discussions at the 2013 XXIV ICL in Rome, Italy, and the 2015 XXV ICL in San Francisco, USA, as well as multiple written comments and feedback from Executive Committee and other ISL members during the 2016 drafting. The document attempts to amalgamate the broad spectrum of protocols and practices advocated worldwide for the diagnosis and treatment of peripheral lymphedema into a coordinated proclamation representing a “Consensus” of the international community based on various levels of evidence. The document is not meant to override individual clinical considerations for complex patients nor to stifle progress. It is also not meant to be a legal formulation from which variations define medical malpractice. The Society understands that in some clinics the method of treatment derives from national standards while in others access to medical equipment and supplies is limited; therefore the suggested treatments might be impractical. Adaptability and inclusiveness does come at the price that members can rightly be critical of what they see as vagueness or imprecision in definitions, qualifiers in the choice of words (e.g., the use of “may... perhaps... unclear”, etc.) and mentions (albeit without endorsement) of treatment options supported by limited hard data. Most members are frustrated by the reality that NO treatment method has really undergone a satisfactory meta-analysis (let alone rigorous, randomized, stratified, long-term, controlled study). With this understanding, the absence of definitive answers and optimally conducted clinical trials, and with emerging technologies and new approaches and discoveries on the horizon, some degree of uncertainty, ambiguity, and flexibility along with dissatisfaction with current lymphedema evaluation and management is appropriate and to be expected. We continue to struggle to keep the document concise while balancing the need for depth and details. With these considerations in mind, we believe that this 2016 version presents a Consensus that embraces the entire ISL membership, rises above national standards, identifies and stimulates promising areas for future research, and represents the best judgment of the ISL membership on how to approach patients with peripheral lymphedema in the light of currently available evidence. Therefore, the document has been, and should continue to be, challenged and debated in the pages of Lymphology (e.g., as Letters to the Editor) and ideally will remain a continued focal point for robust discussion at local, national and international conferences in lymphology and related disciplines. We further anticipate as experience evolves and new ideas and technologies emerge that this “living document” will undergo further periodic revision and refinement as the practice and conceptual foundations of medicine and specifically lymphology change and advance.

Soft tissue pitting is the occurrence of a temporary indentation on the body surface after the release of sustained thumb or finger pressure. In the management of lymphedema, presence or absence of pitting can contribute to clinical reasoning and guide healthcare management. However, the pitting test and its application has not been described consistently nor is it a standardized part of assessment. Therefore investigations are needed to assess the outcome measures of pitting identification and characterization of lymphedematous tissue. To determine valid testing parameters for a future study, we evaluated six therapists of varying lymphedema experience who assessed a range of locations on six patients with lymphedema representing the breadth of pitting assessment techniques used clinically. The consequence of an unstandardized assessment is demonstrated by the large variation in techniques observed for test duration (1.1 to 76.0 seconds), pressure (1.3 to 14.4 N/cm2) and contact area (0.2 to 6.8 cm2). Experienced therapists performed the pitting test with a significantly different technique from their inexperienced counterparts, involving a longer duration (p < 0.001), higher pressure (p < 0.001) and a larger contact area (p < 0.001). The results of this pilot study support the need for evaluation of the underlying tissue response to sustained pressure and release, in order to inform the development of a standardized procedure.

The incorporation of resistance exercises into the lifestyle of patients with lymphedema is understudied and an emerging interest. We investigated the effectiveness and results of adding a moderate intensity resistance exercise program for 8 weeks in conjunction with intensive CDT for 1 or 2 weeks (depending on severity) on arm volume, arm function, QOL, and muscular strength in patients with breast cancer-related lymphedema. This prospective, pilot trial included forty-four patients with a history of breast cancer who were beginning complex decongestive therapy for lymphedema. They were assigned to either the intervention (n = 22) or control (n = 22). groups. The intervention comprised of resis- tance band exercises 5 times a week for 8 weeks. These were initially supervised during the intensive lymphedema treatment, but performed independently during the study period. Limb volume, muscular strength, and the European Organization for Research and Treatment of Cancer QOL Questionnaire C30 (EORTC QLQ-C30), EORTC-Breast Cancer-Specific QOL Questionnaire (EORTC QLQ-BR23), and Disabilities of Arm, Shoulder, and Hand (DASH) questionnaires were assessed at baseline and at 8 weeks. After 8 weeks, the intervention group demonstrated statistically significant differences (p < 0.05) in the DASH score and muscular strength compared to the control group. Our results indicate that upper body resistance exercise demonstrates a positive effect on arm function and muscular strength without increasing arm volume in breast cancer related lymphedema during and shortly post intensive CDT lymphedema treatment.

Intermittent negative pressure devices were initially developed by NASA to enhance blood perfusion and combat a reduction in orthostatic tolerance. Investigational studies have demonstrated that the pressure differential produces changes in the blood and cardiac systems and also documented changes in weight and cellulite in obese patients. Although the mechanisms are not known, previous investigation has also reported changes in lymphedematous limbs. These initial results suggested to us that the inclusion of intermittent negative pressure into a lymphedema treatment protocol would be beneficial. We subsequently undertook a study of 50 patients with lymphedema adding intermittent negative pressure to our CLyFT protocol and compared them to the CLyFT protocol without intermittent negative pressure. We found a significant difference between the groups with an additional 7% reduction in lymphedema volume (p = 0.008). Our study results indicate that the inclusion of intermittent negative pressure therapy into the CLyFT protocol was beneficial and further incorporation into other protocols should be investigated.

Most studies evaluating lymphedema treatment effect focus on objective reductions in limb volume, with little attention given to subjective treatment outcomes. The objective of this work was to describe the range of lymphedema symptoms experienced by patients and the importance of symptom improvement following treatment. The second aim was to explore lymphedema treatment use and the effect of individual treatments on symptoms, from the patient's perspective. Australian adults with lymphedema (n = 421) completed a self-administered questionnaire. Information was collected about patients' symptoms, the importance of symptom improvement following treatment, as well as treatment types used and perceived effectiveness of each treatment. In addition to swelling, the vast majority of participants experienced heaviness and tightness in the affected region. Overall, symptoms of lymphedema varied between individuals but the majority considered subjective symptom improvement to be an important outcome of treatment. The most commonly used treatments were compression garments, self- massage, prescribed exercises, and manual lymph drainage, and the majority (95%) of participants had used multiple treatments to manage their lymphedema. The impact of treatments on subjective symptoms varies widely between treatments. Consequently, in addition to objective measures of swelling, it is important to include patient-reported outcomes in future prospective lymphedema treatment studies.

The use of compression garments during exercise is recommended for women with breast cancer-related lymphedema, but the evidence behind this clinical recommendation is unclear. The aim of this randomized, cross-over trial was to compare the acute effects of wearing versus not wearing compression during a single bout of moderate-load resistance exercise on lymphedema status and its associated symptoms in women with breast cancer-related lymphedema (BCRL). Twenty-five women with clinically diagnosed, stable unilateral breast cancer-related lymphedema completed two resistance exercise sessions, one with compression and one without, in a randomized order separated by a minimum 6 day wash-out period. The resistance exercise session consisted of six upper-body exercises, with each exercise performed for three sets at a moderate-load (10-12 repetition maximum). Primary outcome was lymphedema, assessed using bioimpedance spectroscopy (L-Dex score). Secondary outcomes were lymphedema as assessed by arm circumferences (percent inter-limb difference and sum-of-circumferences), and symptom severity for pain, heaviness and tightness, measured using visual analogue scales. Measurements were taken pre-, immediately post- and 24 hours post-exercise. There was no difference in lymphedema status (i.e., L-Dex scores) pre- and post-exercise sessions or between the compression and non-compression condition [Mean (SD) for compression pre-, immediately post- and 24 hours post-exercise: 17.7 (21.5), 12.7 (16.2) and 14.1 (16.7), respectively; no compression: 15.3 (18.3), 15.3 (17.8), and 13.4 (16.1), respectively]. Circumference values and symptom severity were stable across time and treatment condition. An acute bout of moderate-load, upper-body resistance exercise performed in the absence of compression does not exacerbate lymphedema in women with BCRL.

There are few studies showing that physical exercise can improve secondary lymphedema. We hypothesized that water exercise would be more effective than land exercise in reducing limb volume. Secondary objectives were joint movement, BMI, daily function, well-being, and body image. Limb volume was measured with circumference or was volumetric. Well-being and body image were measured with a study-specific questionnaire and daily function with DASH and HOOS questionnaires. Eighty-eight eligible patients with secondary lymphedema after breast or gynecological cancer participated in this controlled clinical intervention study. There was a higher proportion of women who participated in water exercises who reduced their secondary arm limb volume (p = 0.029), and there were also significant differences for BMI (p = 0.047) and self-reported frequency of swelling (p = 0.031) in the water exercise group after intervention. Women with arm lymphedema in the land exercise group improved DASH scores (p = 0.047) and outer rotation in the shoulder (p = 0.001). Our results suggest that to reduce objective and self-reported swelling, lymphedema patients may be offered water exercise training while to improve daily shoulder function, land exercises are preferred. To guide female cancer survivors with lymphedema to effective exercise resulting in reduced limb volume and improved function, adequate evidenced-based programs are needed.

The incidence of lymphedema (LE) related to treatment of women's cancer (breast and gynecologic) is as high as 40%. Treatment of LE varies around the world but was decades ago initially based on programs including manual lymph drainage (MLD), compression, skin care and easy exercise. With accumulating evidence and experience, it is time to consider if altering these treatment principles is needed. Based on accumulating evidence, we suggest less emphasis on manual lymph drainage and more on early diagnosis, compression, weight control and exercise for improvement of strength and circulation.

Once clinically manifested as a swollen limb, lymphedema can be difficult to manage. Our focus thus must shift from reactive treatment to proactive management and prevention. On the basis of strong evidence in the literature, lymphedema specialists now encourage exercise as it can improve lymphatic drainage through muscle pump action. However, exercise may increase the lymph load on the vulnerable limb. We aimed to examine whether low level sporting compression is a reasonable recommendation for those with early stage lymphedema by measuring whether sporting compression (SC) tights decrease limb extracellular fluid as measured by Bio-impedance Spectroscopy (BIS) and Perometry in legs following exercise in both healthy controls and those with early stage lymphedema. A group of normal subjects (n=10) and a group of Stage 1 (ISL) lymphedema patients (n=9) were enrolled. Efforts were made to match participants in each group. For those with unilateral lymphedema, the non-affected leg was used as a control. All were measured using BIS, Perometry and Indurometry before and after exercise both with and without sporting compression clothing. The exercise regime was standardized and involved treadmill walking at increasing rates within each person's activity limitation. SC tights were shown to significantly decrease the fluid build up caused by exercise in lymphedema-affected limbs as measured with BIS (p=0.0302). Perometry measurements showed that SC caused a significant decrease in limb volume post exercise of the whole cohort (p=0.0081) and of the control Group B (p=0.0348). Our findings support the notion that SC may provide a socially acceptable and effective means of lymphedema control during exercise for early lymphedema management.

Our aim was to compare the responses of physical treatment with or without manual lymphatic drainage (MLD) in lymphedema after breast cancer treatment in a Brazilian population. This was a controlled clinical trial with lymphedema secondary to breast cancer treatment patients that were randomized into either: Group 1 consisting of MLD, skin care, bandaging and remedial exercises; or Group 2 using skin care, bandaging and remedial exercises. Sixty-six patients were randomized and 9 were excluded during the first phase, resulting in a total of 57 patients eligible for analyzes with 28 in Group 1 and 29 in Group 2. The first phase of treatment had an average duration of 24 days (+/- 12.38) and final volume excess average (VE) between limbs was 494.51 ml, corresponding to 29.18% of the initial volume. Volume reduction was highly significant, independent of the intervention (p < 0.001), and both treatments led to an average of percentage volume excess reduction (PVER) of 15.02%. Patients with incomplete range of motion and lymphatic-related fibrotic tissues showed a statistically significant reduction in the percentage of volume excess (p = 0.010; p = 0.009). The presence of arm paresthesia was associated with the lowest therapeutic response (p = 0.024). Both treatment groups demonstrated absolute and relative reductions of excess limb volume, and the addition of MLD did not significantly increase the therapeutic response in women with lymphedema after breast cancer.

It is well documented that resistance exercise can be performed by patients with breast cancer-related arm lymphedema. The aim of this pilot study was to evaluate the feasibility and safety of a 12-week self-administered weight lifting program for arm and shoulder, and its influence on arm lymphedema status, upper extremity muscle strength, and disability. Twenty-three patients with breast cancer-related arm lymphedema performed the program 3 times/week. The weight resistance levels were individually adjusted for shoulder flexion and adduction, and elbow extension and flexion corresponding to a repetition range of 8-12 repetition maximum. A log book was used to evaluate adherence to the program, wearing of compression sleeve and perceived exertion. Measurements were performed before a 2-week control period without intervention, and before and after intervention, and with arm volume measurements every fortnight to check for adverse events. Results revealed no significant changes during the control period. Adherence to the intervention program was excellent, and two adverse events were registered during the first weeks. After intervention, an increase of shoulder and arm strength (measured by an isometric muscle strength device) was found in all exercises (p = 0.001-0.003). A reduction of excess volume was shown, in ml (p = 0.03) and percentage (p = 0.005), measured by water displacement method. A tendency towards reduction (p = 0.07) of fat tissue in the upper arm (n = 10) in both arms was found measured by MRI. In this pilot study, we concluded that a home-based weight-lifting program performed by patients with breast cancer-related arm lymphedema is feasible and safe providing that the program includes regular follow-up for safety.

Currently, a limited number of studies have been conducted that examine sources of information and knowledge level in individuals with lymphedema. This study aimed (1) to examine self-reported information sources and perceived lymphedema knowledge among individuals with lymphedema; and (2) to examine differences in self-reported information sources and perceived lymphedema knowledge among individuals with primary or secondary lymphedema; and with upper or lower extremity lymphedema. The National Lymphedema Network (NLN) conducted a survey to collect self-report data from March 2006 to January 2010. Overall, participants preferred a variety of sources of information. Participants reported low levels of knowledge about the types of lymphedema, treatment approaches and methods, and self-administrated therapies. In comparison to participants with secondary or upper extremity lymphedema, participants with primary or lower extremity lymphedema reported lower knowledge level regarding causes of lymphedema, risks for and complications of lymphedema, treatment approaches and methods for lymphedema, and self-administered therapies. Opportunities exist to expand lymphedema information sources. Healthcare professionals should focus on delivering high quality information about treatment and self-care management to individuals with lymphedema.

Post-mastectomy chronic lymphedema as a complication of breast cancer treatment is primarily managed with Complete Decongestive Therapy (CDT). We examined various factors for correlating with results of Phase I CDT treatment in controlling the upper extremity lymphedema. Study population consisted of patients with lymphedema referred to the Lymphedema Clinic of the Iranian Breast Cancer Research Center for control of arm edema. After obtaining the demographic and clinical data, patients were treated with CDT for 2 - 3 weeks. One hundred and thirty seven patients (mean age +/- SD; 53.5 +/- 10 years) were studied. In 48.7% of patients, the affected arm was the dominant limb. Fifty percent of patients experienced lymphedema during the first year after surgery, and mean duration of lymphedema was 35 +/- 43 months. Mean volume reduction was 43% +/- 14.87% (p = 0.03). There was a significant relationship between the percent of volume reduction and initial lymphedema volume (p=0.003) as well as duration of lymphedema (p=0.002). Our results demonstrate that Phase I CDT treatment is very effective for post mastectomy lymphedema, and particularly if it is provided in earlier stages of disease. In addition, CDT also has an important role in reducing clinical symptoms and improving limb function. In the appropriate setting, Phase I CDT has been an effective method of controlling post mastectomy lymphedema in this Iranian population.

A project of the American Lymphedema Framework Project (ALFP), this review seeks to examine the policy and economic impact of caring for patients with lymphedema, a common side effect of cancer treatment. This review is the first of its kind undertaken to investigate, coordinate, and streamline lymphedema policy initiatives in the United States with potential applicability worldwide. As part of a large scale literature review aiming to systematically evaluate the level of evidence of contemporary peer-reviewed lymphedema literature (2004 to 2011), publications on care delivery models, health policy, and economic impact were retrieved, summarized, and evaluated by a team of investigators and clinical experts. The review substantiates lymphedema education models and clinical models implemented at the community, health care provider, and individual level that improve delivery of care. The review exposes the lack of economic analysis related to lymphedema. Despite a dearth of evidence, efforts towards policy initiatives at the federal and state level are underway. These initiatives and the evidence to support them are examined and recommendations for translating these findings into clinical practice are made. Medical and community-based disease management interventions, taking on a public approach, are effective delivery models for lymphedema care and demonstrate great potential to improve cancer survivorship care. Efforts to create policy at the federal, state, and local level should target implementation of these models. More research is needed to identify costs associated with the treatment of lymphedema and to model the cost outlays and potential cost savings associated with comprehensive management of chronic lymphedema.

This International Society of Lymphology (ISL) Consensus Document is the current revision of the 1995 Document for the evaluation and management of peripheral lymphedema (1) for discussion at the XXIV International Congress of Lymphology. It is based upon modifications: [A] suggested and published following the 1997 XVI International Congress of Lymphology (ICL) in Madrid, Spain (2) discussed at the 1999 XVII ICL in Chennai, India (3) and considered/ confirmed at the 2000 (ISL) Executive Committee meeting in Hinterzarten, Germany (4); [B] 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 (5); [C] suggested from comments, criticisms, and rebuttals as published in the December 2004 issue of Lymphology (6); [D] discussed in both the 2005 XX ICL in Salvador, Brazil and the 2007 XXI ICL in Shanghai, China and modified at the 2008 Executive Committee Meeting in Naples, Italy (7,8); and [E] modified from discussions and written comments from the 2009 XXII ICL in Sydney, Australia, the 2011 XXIII ICL in Malmo, Sweden and 2012 Executive Committee Meetings. The document attempts to amalgamate the broad spectrum of protocols advocated worldwide for the diagnosis and treatment of peripheral lymphedema into a coordinated proclamation representing a "Consensus" of the international community. The document is not meant to override individual clinical considerations for problematic patients nor to stifle progress. It is also not meant to be a legal formulation from which variations define medical malpractice. The Society understands that in some clinics the method of treatment derives from national standards while in others access to medical equipment and supplies is limited, and therefore the suggested treatments are impractical. Adaptability and inclusiveness does come at the price that members can rightly be critical of what they see as vagueness or imprecision in definitions, qualifiers in the choice of words (e.g., the use of "may... perhaps... unclear", etc.) and mention (albeit without endorsement) of treatment options supported by limited hard data. Most members are frustrated by the reality that NO treatment method has really undergone a satisfactory meta-analysis (let alone rigorous, randomized, stratified, long-term, controlled study). With this understanding, the absence of definitive answers and optimally conducted clinical trials, and with emerging technologies and new approaches and discoveries on the horizon, some degree of uncertainty, ambiguity, and flexibility along with dissatisfaction with current lymphedema evaluation and management is appropriate and to be expected. We continue to struggle to keep the document concise while balancing the need for depth and details. With these considerations in mind, we believe that this latest version presents a Consensus that embraces the entire ISL membership, rises above national standards, identifies and stimulates promising areas for future research and represents the best judgment of the ISL membership on how to approach patients with peripheral lymphedema as of 2013. Therefore the document has been, and should continue to be, challenged and debated in the pages of Lymphology (e.g., as Letters to the Editor), and ideally will remain a continued focal point for robust discussion at local, national and international conferences in lymphology and related disciplines. We further anticipate as experience evolves and new ideas and technologies emerge that this "living document" will undergo further periodic revision and refinement as the practice and theories of medicine and specifically lymphology change and advance.

Intermittent pneumatic compression (IPC) therapy is an effective modality to reduce the volume of the lymphedematous limbs alone or in conjunction with other modalities of therapy such as decongestive therapy. However, there is no consensus on the frequency or treatment parameters for IPC devices. We undertook a systematic review of contemporary peer-reviewed literature (2004-2011) to evaluate the evidence for use of IPC in the treatment of lymphedema. In select patients, IPC use may provide an acceptable home-based treatment modality in addition to wearing compression garments.

Crohn's disease is an inflammatory intestinal disease that primarily causes abdominal pain and diarrhea. We report a male patient who presented with penile and scrotal lymphedema and inguinal fistulas as the first manifestations of Crohn's disease. Extraintestinal or metastatic Crohn's disease initially presenting as genital lymphedema with fistula formation is rare. Skin lesions in extraintestinal Crohn's disease typically show non-caseating, sarcoidal granulomas with numerous foreign body- and Langhans-type multinucleated giant cells, which are separated from intestinal involvement by normal skin. Treatment options are limited and include multi-immunosuppressant medications.

Lipedema is a disproportional obesity featuring light pressure-induced or spontaneous pain. On the basis of our clinical observations, lymphedema therapy, as practiced in our clinic, reduces the perception of pain beyond leg volume reduction. We therefore aimed to measure pain intensity prior and subsequent to treatment. 38 women with lipedema were enrolled in the study with 19 patients undergoing treatment and 19 serving as the control group using exclusively moisturizers. Treatment consisted of once daily manual lymph drainage (MLD), intermittent pneumatic compression (IPC), and multilayered short-stretch bandaging performed throughout a 5-day-course. Pain was evaluated with a 10-item questionnaire, a pain rating scale (PRS), and the Wong-Baker Faces scale. Treatment resulted in a significant reduction of pain with a decrease in mean scores of all three measures. In the control group, only PRS showed significant decrease. Our study results indicate that this treatment regimen not only reduces leg volume and capillary fragility, but also improves pain intensity in patients with lipedema.

Questions remain on the use of sequential pneumatic compression including where does the fluid flow to and whether fluid can be moved to the non-swollen tissues of the hypogastrium and gluteal region? During pneumatic massage of the limb, we studied pathways of lymph and mobile tissue fluid flow using lymphoscintigraphy: a) from the calf and thigh across the inguinal region to the healthy non-swollen tissues of the hypogastrium and b) in the hypogastrium to the lateral and upper abdominal quadrants. To examine if there was effective fluid flow during pneumatic massage, plethysmographic flow measurements were also carried out. We demonstrated that: (i) pneumatic compression moved isotope in lymph remaining in functioning lymphatics and in tissue fluid in the interstitial space toward the inguinal region and femoral channel, (ii) there was no isotope crossing the inguinal crease or moving to the gluteal area, and (iii) isotope injected intradermally in the hypogastrium did not spread during manual massage to the upper and contralateral abdominal quadrants. In conclusion, intermittent pneumatic compression is effective in pushing mobile tissue fluid and relocating large fluid volumes toward the groin. However, the question that still remains is how to facilitate further flow toward the non-swollen tissues and thereby increase local absorption of fluid.

Mutation of the transcription factor FOXC2 gene has been identified as the cause of lymphedema-distichiasis syndrome (LD). Subjects with LD usually present with lower extremity lymphedema and distichiasis--an additional row of eyelashes. Typically, lymphscintigrams of patients with LD show good transport of the radiotracer from the feet to the inguinal lymph nodes accompanied by reflux of tracer to the skin of the lower extremities ("dermal backflow"). We have examined two patients with LD syndrome and were able to demonstrate two different distinct lymphscintigraphic patterns: lymphatic hyperplasia with reflux and obstructive.

Cutaneous metastasis from pancreatic cancer is relatively rare as an initial symptom, and it is generally localized on the periumbilical area that is known as Sister Mary Joseph's nodule. We report a rare case of a 49-year-old female who developed cutaneous metastasis of pancreatic cancer as an initial symptom. The patient was referred to our department for treatment of lymphedema due to surgical treatment of cervical cancer and underwent combined physiotherapy and, 2 months later, a lymph venous anastomosis (LVA) for treatment of the lymphedema. Two months after the operation, she developed erythema on her right leg which spread from the leg to the groin in series. This pattern corresponded to the direction of lymph drainage, which may have been enhanced by the conservative physiotherapy and LVA treatments. These facts suggest a possible relationship between cutaneous metastasis of carcinoma and treatment for lymphedema. Alternatively, the lymphedematous limb may be a privileged site for cancer growth, and metastatic seeding could have taken place from pre-existing hematogenous spread at the time of operation.

There is no cure for breast cancer related lymphedema. This study was conducted to compare two treatment methods for postmastectomy lymphedema: Complex Decongestive Therapy (CDT) and Modified CDT (MCDT) combined with Intermittent Pneumatic Compression (IPC). One hundred and twelve patients referred to the Lymphedema Clinic of the Iranian Center for Breast Cancer in 2008, were included in a randomized clinical trial. They were randomly allocated into two equal groups receiving daily CDT alone or in combination with IPC. The volume reduction of the upper limb was measured by water displacement volumetry. No statistically significant differences in demographic and clinical variables between the two groups were observed. During the intensive phase (phase I) of treatment, CDT alone yielded a significantly higher mean volume reduction than the combination modality (43.1% vs. 37.5%; p = 0.036). Limb volume measured three months following treatment, showed 16.9% volume reduction by CDT alone, and 7.5% reduction by MCDT plus IPC. This study demonstrated that the use of CDT alone, or in combination with IPC significantly reduced limb volume in patients with post mastectomy lymphedema. CDT alone provided better results in both treatment phases. Further studies will help to define the role of multidisciplinary approaches in the management of postmastectomy lymphedema.

The application of intermittent pneumatic compression (IPC) as a part of complex decongestive physiotherapy (CDP) remains controversial. The aim of this study was to investigate whether the combination of IPC with manual lymph drainage (MLD) could improve CDP treatment outcomes in women with secondary lymphedema after breast cancer treatment. A randomized study was undertaken with 13 subjects receiving MLD (60 min) and 14 receiving MLD (30 min) plus IPC (30 min) followed by standardized components of CDP including multilayered compression bandaging, physical exercise, and skin care 10 times in a 2-week-period. Efficacy of treatment was evaluated by limb volume reduction and a subjective symptom questionnaire at end of the treatment, and one and two months after beginning treatment. The two groups had similar demographic and clinical characteristics. Mean reductions in limb volumes for each group at the end of therapy, and at one and two months were 7.93% and 3.06%, 9.02% and 2.9%, and 9.62% and 3.6%, respectively (p < 0.05 from baseline for each group and also between groups at each measurement). Although a significant decrease in the subjective symptom survey was found for both groups compared to baseline, no significant difference between the groups was found at any time point. The application of IPC with MLD provides a synergistic enhancement of the effect of CDP in arm volume reduction.