Physical therapies in the decongestive treatment of lymphedema: A randomized, non-inferiority controlled study.

OBJECTIVE: To assess whether the treatment with intermittent pneumatic compression plus multilayer bandages is not inferior to classical trimodal therapy with manual lymphatic drainage in the decongestive lymphedema treatment. STUDY DESIGN: Randomized, non-inferiority, controlled study to compare the efficacy of three physical therapies' regimens in the Decongestive Lymphatic Therapy. PARTICIPANTS: 194 lymphedema patients, stage II-III with excess volume > 10% were stratified within upper and lower limb and then randomized to one of the three treatment groups. Baseline characteristics were comparable between the groups. INTERVENTION: all patients were prescribed 20 sessions of the following regimens: Group A (control group): manual lymphatic drainage + Intermittent Pneumatic Compression + Bandages; Group B: pneumatic lymphatic drainage + Intermittent Pneumatic Compression + Bandages; and Group C: only Intermittent Pneumatic Compression + Bandages. END-POINT: Percentage reduction in excess volume (PREV). RESULTS: All patients improved after treatment. Global mean of PREV was 63.9%, without significant differences between the groups. The lower confidence interval of the mean difference in PREV between group B and group A, and between group C and group A were below 15%, thus meeting the non-inferiority criterion. Most frequent adverse events were discomfort and lymphangitis, without differences between groups. A greater baseline edema, an upper-limb lymphedema and a history of dermatolymphangitis were independent predictive factors of worse response in the multivariate analysis. CONCLUSIONS: Decongestive lymphatic therapy performed only with intermittent pneumatic compression plus bandages is not inferior to the traditional trimodal therapy with manual lymphatic drainage. This approach did not increase adverse events.

Manual lymphatic drainage with or without fluoroscopy guidance did not substantially improve the effect of decongestive lymphatic therapy in people with breast cancer-related lymphoedema (EFforT-BCRL trial): a multicentre randomised trial.

QUESTIONS: When added to decongestive lymphatic therapy (DLT), what is the effect of fluoroscopy-guided manual lymphatic drainage (MLD) versus traditional MLD or placebo MLD for the treatment of breast cancer-related lymphoedema (BCRL)? DESIGN: Multicentre, three-arm, randomised controlled trial with concealed allocation, intention-to-treat analysis and blinding of assessors and participants. PARTICIPANTS: At five hospitals in Belgium, 194 participants with unilateral chronic BCRL were recruited. INTERVENTION: All participants received standard DLT (education, skin care, compression therapy and exercises). Participants were randomised to also receive fluoroscopy-guided MLD (n = 65), traditional MLD (n = 64) or placebo MLD (n = 65). Participants received 14 sessions of physiotherapy during the 3-week intensive phase and 17 sessions during the 6-month maintenance phase. Participants performed self-management on the other days. OUTCOME MEASURES: All outcomes were measured: at baseline; after the intensive phase; after 1, 3 and 6 months of maintenance phase; and after 6 months of follow-up. The primary outcomes were reduction in excess volume of the arm/hand and accumulation of excess volume at the shoulder/trunk, with the end of the intensive phase as the primary endpoint. Secondary outcomes included daily functioning, quality of life, erysipelas and satisfaction. RESULTS: Excess lymphoedema volume decreased after 3 weeks of intensive treatment in each group: 5.3 percentage points of percent excessive volume (representing a relative reduction of 23.3%) in the fluoroscopy-guided MLD group, 5.2% (relative reduction 20.9%) in the traditional MLD group and 5.4% (relative reduction 24.8%) in the placebo MLD group. The effect of fluoroscopy-guided MLD was very similar to traditional MLD (between-group difference 0.0 percentage points, 95% CI -2.0 to 2.1) and placebo MLD (-0.2 percentage points, 95% CI -2.1 to 1.8). Fluid accumulated at the shoulder/trunk in all groups. The average accumulation with fluoroscopy-guided MLD was negligibly less than with traditional MLD (-3.6 percentage points, 95% CI -6.4 to -0.8) and placebo MLD (-2.4 percentage points, 95% CI -5.2 to 0.4). The secondary outcomes also showed no clinically important between-group differences. CONCLUSION: In patients with chronic BCRL, MLD did not provide clinically important additional benefit when added to other components of DLT. REGISTRATION: NCT02609724.

Intermittent Pneumatic Compression for the Treatment of Lower Limb Lymphedema: A Pilot Trial of Sequencing to Mimic Manual Lymphatic Drainage Versus Traditional Graduated Sequential Compression.

Background: Recent advances in technology have allowed intermittent pneumatic compression (IPC) devices to develop so that their function mimics the process and principles of manual lymphatic drainage (MLD); however, research into the effectiveness of such devices is lacking. This study aimed to investigate the effectiveness of a patented IPC technique designed to mimic MLD (the LymphAssist), compared with a typical sequential IPC regimen. Methods and Results: Forty patients with a confirmed diagnosis of lower limb ISL (International Society of Lymphology) stage II or III lymphedema were recruited into this three-phased study. A bilateral leg volume assessment and quality-of-life assessment were completed at four clinic visits across the course of the study. The LymphAssist IPC regimen was significantly more effective in reducing distal leg volume than the sequential mode (mean volume reduction: 230 ± 135 mL vs. 140 ± 84 mL, respectively, p = 0.01). Improvements in leg volume were transient as both groups demonstrated a rebound or increase in volume during the washout period (LymphAssist: 238 ± 168 mL, sequential: 276 ± 158 mL, p = 0.3). Overall, IPC was effective in improving quality-of-life scores (mean reduction: 10 ± 11, p < 0.001). Conclusion: IPC is effective in reducing limb volume and improving quality of life for patients with lower limb lymphedema. IPC that mimics the MLD process has been shown to be more effective in reducing leg volume compared with traditional sequential IPC in the distal aspect of the leg. The increase in leg volume observed after discontinuation of IPC suggests that regular treatment is required to maintain its associated effects. Clinical Trial Registration Number: NTC 03856281.

Various Clinical Scenarios in Secondary Malignant Lymphedema.

Diagnosing malignant lymphedema is a challenge in daily clinical practice. Clinically, patients may show clear signs of malignancy, but this is not always the case, and at times the diagnosis is not straightforward. In some patients, pain, hardness of the tissues, joint stiffness, proximal involvement, collateral circulation, or an acute onset will provide the clue to determining malignancy. Our aim is to describe several diverse scenarios of Secondary Malignant Lymphedema (SML) with the etiopathogenesis. One possible cause is lymphatic obstruction due to extrinsic compression of lymphatic vessels and/or nodes by either the primary tumor or metastatic masses. Lymphatic obstruction can also be caused by tumoral infiltration. This infiltration can affect both deep and regional nodes as well as cutaneous and subcutaneous vessels and is commonly known as lymphangitis carcinomatosa. Malignant lymphedema can also be secondary to obstruction of the venous flow due to tumoral venous thromboembolism or to extrinsic compression of the veins by tumors or adenopathic masses. Nevertheless, the most frequent cause of this illness is a mixed mechanism of compression of the lymphatic and venous systems. Frequently, SML is the first manifestation of relapse. When lymphedema appears abruptly, is progressive, with intense pain, associated with collateral circulation, or with hard and infiltrated skin or joint stiffness, SML must be ruled out with an urgent referral to the oncologist and an imaging evaluation.

Quality of Life and Costs Within Decongestive Lymphatic Therapy in Patients with Leg Lymphedema: A Multicountry, Open-Label, Prospective Study.

Background: Little is known of the impact in terms of health-related quality of life (HRQoL) and cost-effectiveness with decongestive therapy. Objectives: To examine changes in limb volume, quality of life (QoL), and treatment cost of methods of decongestive lymphatic therapy (DLT). Methods: Patients with chronic edema/lymphedema of the leg were invited to participate in a study of DLT in four countries (United Kingdom, France, Germany, and Canada). In each country two sites were selected. One site used their standard method of DLT in their service, including compression with multilayer bandaging with inelastic material. The other site used a system that included 3M™ Coban 2™ as the bandage treatment alongside other standard components of DLT. Patients were followed for either 2 or 4 weeks depending on the local protocols. At entry, at 2 weeks, and at 4 weeks, patients were assessed by using a health index (EQ-5D), a disease-specific HRQoL tool (LYMQOL) and resource usage was recorded over the treatment period. Results: Of the 165 patients with cost data, 90 were treated with Coban 2 and 75 with standard care compression bandaging. There was good evidence of an improvement in EQ-5D of 0.077 (p < 0.001) in all patients. LYMQOL showed significant improvements (p < 0.001) with lower scores. There were no major differences between the two arms of the study with respect to HRQoL. The number of treatment episodes was higher in those treated with standard care (8.15 vs. 6.37), but the overall treatment cost was higher with Coban 2 (£890.7) compared with standard care (£723.0). Conclusion: QoL improved in the standard care and Coban 2 group bandages, and there was no demonstrable difference between the care systems. Further work is required to examine the role of the individual parts of DLT that provide the greatest benefit to patients and the health systems that support them.

Factors Predicting Limb Volume Reduction Using Compression Bandaging Within Decongestive Lymphatic Therapy in Lymphedema: A Multicountry Prospective Study.

Objectives: To identify predictive factors associated with limb volume reduction using different decongestive lymphatic therapy (DLT) systems in patients with lymphoedema, over a period of up to 28 days. Methods: A multicountry (Canada, France, Germany, the United Kingdom) prospective cohort study using (DLT): skin care, exercise, compression bandaging, and manual lymphatic drainage for up to 4 weeks. Reduction in limb volume comparing DLT with (1) standard multilayer bandaging with inelastic material, and with (2) multilayer bandaging with Coban2, together with the identification of factors associated with limb volume changes. Results: Out of 264 patients with upper or lower limb lymphedema, 133 used Coban2 and 131 used standard care. Following DLT, mean limb volume reduction was 941 mL using Coban2 compared with 814 mL using standard care. A difference of 127 mL was found (95% confidence interval -275 to 529 mL, p = 0.53). Of the 176 patients with leg swelling, 166 (94.3%) had a limb volume measurement after 28 days and were included in the risk factor analysis. Of these, 132 (79.5%) were female, with overall mean age of 60.1 years (standard deviation = 14.7), with secondary lymphedema in 102/163 (62.6%). Duration of lymphedema was >10 years in 75/161 (46.6%) and 99/166 (59.7%) were International Society of Lymphology late-stage II/III, indicating longstanding and/or a high frequency of patients with advanced stages of lymphedema. Ninety-one (54.8%) received Coban2 and 75 (45.2%) had standard care. Multivariable factors for a greater leg volume reduction were large initial leg volume (p < 0.001), DLT treatment duration of 4 weeks compared with 2 weeks (p = 0.01), and peripheral arterial disease (p = 0.015). Conclusion: Limb volume changes were found to be similar between groups. Lack of standardization of DLT makes interpretation of effectiveness problematic. There is an urgent need for randomized-controlled trials. Despite this, severe lymphedema with a large limb volume responded well to DLT in this study.

Debulking surgery for elephantiasis nostras with large ectatic podoplanin-negative lymphatic vessels in patients with lipo-lymphedema.

OBJECTIVE: Elephantiasis nostras is a rare complication in advanced lipo-lymphedema. While lipedema can be treated by liposuction and lymphedema by decongestive lymphatic therapy, elephantiasis nostras may need debulking surgery. METHODS: We present 2 cases of advanced lipo-lymphedema complicated by elephantiasis nostras. After tumescent microcannular laser-assisted liposuction both patients underwent a debulking surgery with a modification of Auchincloss-Kim's technique. Histologic examination of the tissue specimen was performed. RESULTS: The surgical treatment was well tolerated and primary healing was uneventful. After primary wound healing and ambulation of the patients, a delayed ulceration with lymphorrhea developed. It was treated by surgical necrectomy and vacuum-assisted closure leading to complete healing. Mobility of the leg was much improved. Histologic examination revealed massive ectatic lymphatic vessels nonreactive for podoplanin. CONCLUSIONS: Debulking surgery can be an adjuvant technique for elephantiasis nostras in advanced lipo-lymphedema. Although delayed postoperative wound healing problems were observed, necrectomy and vacuum-assisted closure achieved a complete healing. Histologic data suggest that the ectatic lymphatic vessels in these patients resemble finding in podoplanin knockout mice. The findings would explain the limitations of decongestive lymphatic therapy and tumescent liposuction in such patients and their predisposition to relapsing erysipelas.

Lipedema: an overview of its clinical manifestations, diagnosis and treatment of the disproportional fatty deposition syndrome - systematic review.

Lipedema is a disproportionate, symmetrical fatty swelling characterized by pain and bruising existing almost exclusively among women. We undertook a systematic review of the available literature about lipedema, given the lack of knowledge and little evidence about this disorder especially among obesity experts. Diagnosis of lipedema is usually based on clinical features. Symmetrical edema in the lower limbs with fatty deposits located to hips and thighs usually appears at puberty and often affects several members of the same family. Main disorders considered for differential diagnosis are lymphedema, obesity, lipohypertrophy and phlebedema. Treatment protocols comprise conservative (decongestive lymphatic therapy) and surgical (liposuction) approaches. Early diagnosis and treatment are mandatory for this disorder otherwise gradual enlargement of fatty deposition causes impaired mobility and further comorbidities like arthrosis and lymphatic insufficiency.