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Introduction: Given the projected rise in the incidence of cancer treatment-related conditions, such as lymphoedema, and the limited research on lymphoedema in cancers with poor prognoses, there is a need for a better understanding of cancer-related lymphoedema incidence and associated risk factors across all types of cancers. The objectives of this review are (1) to produce a resource on an open-access platform that facilitates continuous update of incidence estimates and risk factors as evidence emerges, (2) to provide the most up-to-date estimate of the incidence of cancer-related lymphoedema and (3) to evaluate the strength and consistency of the association between lymphoedema and cancer treatment and non-treatment-related risk factors., Methods and Analysis: A living systematic and grey literature search will be conducted to identify studies reporting the incidence, prevalence of lymphoedema or associated risk factors in individuals who have undergone treatment for any type of cancer. Two investigators will independently extract data and assess the risk of bias using the Cochrane Risk of Bias Tool Version 2.0, the Risk of Bias in Non-randomised Studies - of Interventions or the National Institutes of Health Heart, Lung and Blood Institute Study Quality Assessment Tools, depending on study design. The overall strength of evidence will be appraised with the Grading of Recommendations, Assessment, Development and Evaluations tool. Random effect models will be used to produce pooled overall lymphoedema incidence estimates. Subgroup analyses that explore relationships between lymphoedema incidence and lymphoedema measurement method, time since cancer diagnosis and treatment and diagnosis, treatment and behavioural characteristics will be conducted dependent on available data., Ethics and Dissemination: This living systematic review enables clinicians and researchers to consult a contemporary, comprehensive overview of the incidence of cancer-related lymphoedema and the association between lymphoedema and treatment and non-treatment-related risk factors., Prospero Registration Number: CRD42022333291., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: In female patients diagnosed with gynaecological cancer, the main priority is treating cancer itself. However, a significant number of these patients develop lower limb lymphoedema (LLL), with its incidence strongly influenced by the diagnostic cut-off (a difference in leg circumference between both legs of 5% or 10%). LLL significantly impacts patients' quality of life (QoL) due to functional impairment, the daily time investment required for treatment, financial costs and affected body image. Additionally, it increases the risk of cellulitis, often leading to hospitalisation for intravenous antibiotics. Timely recognition and acknowledgement of symptoms are crucial first steps in improving the QoL for these women. LLL is a common and irreversible complication following treatment for gynaecological cancer. Despite its clinical significance, there are limited prospective studies investigating LLL incidence, risk factors, early detection and clinical course in this patient population. Therefore, the objective of this observational cohort study is to investigate the incidence rate of stage 0-1 or stage 2-3 LLL based on the International Society of Lymphology criteria in the first 2 years after gynaecological cancer treatment. The embedded randomised control study (RCT) examines the added value of prophylactic compression garments (class II) to standard care in preventing irreversible lymphoedema after gynaeco-oncological therapy., Methods and Analysis: In June 2022, two university hospitals in Belgium initiated a multicentre observational cohort study, which also includes an embedded RCT. The study aims to enrol 400 patients before they begin cancer treatment or within 14 days of starting chemotherapy, radiotherapy or surgery. This cohort will be followed for up to 2 years. The embedded RCT will focus on patients who develop clinical lymphoedema stage 0-1 within the first 12 months following their initial cancer treatment. A total of 196 patients will be randomised into two groups: the intervention group (98 patients), receiving usual care plus prophylactic compression garments (compression class 2 (CCL2): 23-32 mm Hg), and the control group (98 patients), receiving only usual care. The RCT aims to assess the impact of adding prophylactic compression garments to standard care on further deterioration. Assessments will be conducted at baseline, and at 3, 12 and 24 months after initial cancer treatment. An additional assessment (T-visit) will be provided if there is a transition to LLL stage 0-1 or from stage 0-1 to stage 2-3 LLL, based on follow-up findings or patient initiative, using the predictive value of a validated self-report lower extremity questionnaire. This questionnaire is part of a lymph diary app provided to all patients. The primary outcome of the observational study is to determine the incidence rate of stage 0-1 or stage 2-3 LLL in the first 2 years after gynaecological cancer treatment. The primary outcome of the RCT is to evaluate the effect of wearing CCL2 preventive garments to avoid progression to higher-stage lymphoedema in patients who develop LLL within 1 year of treatment. Key secondary outcomes of the observational study include the timing of the development of LLL, risk factors for developing LLL and the impact of LLL on QoL and sexuality, as well as the evaluation of screening and diagnostic tools. The secondary outcomes of the RCT include tolerance and compliance with wearing the preventive compressive garments, the impact of the garments on limb volume and the patient's time and financial investment., Ethics and Dissemination: The study was approved by the Ethics Committee of the two academic hospitals: the University Hospital of Ghent in September 2021 and the University Hospital of Leuven in December 2021. Approval has been granted for the study protocol, informed consent forms and other related documents by the main Ethics Committee of Ghent (BC-09915) and the local Ethics Committee of Leuven (S65724). All patients will provide written informed consent before participating in the trial. The results will be shared through peer-reviewed journals and presentations., Trial Registration Number: NCT05469945., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Patients undergoing treatment for prostate cancer may develop lymphoedema of the midline region. This has a substantial impact on a patient's quality of life and its diagnosis is often delayed or missed. Therefore, the purpose of this study is to compare the characteristics of patients with leg and midline lymphoedema to patients with only leg lymphoedema., Methods: We retrospectively collected patient-, cancer-, lymphoedema- and lymphoedema treatment-related data of 109 men with lymphoedema after treatment for prostate cancer. First, 42 characteristics were compared between both groups. Second, factors predicting presence of midline lymphoedema were explored by multivariable analyses., Results: The mean age of the patients with lymphoedema was 68 ( ±7) years and mean BMI is 28 (±4) kg/m 2 . Median duration of lymphoedema before the first consultation was 27 (9;55) months. Based on univariable analyses, patients with leg and midline lymphoedema had more frequently upper leg lymphoedema (89% (31/35) vs. 69% (51/74), p = 0.026), skin fibrosis (34% (12/35) vs. 16% (12/74), p = 0.034) and lymphatic reconstructive surgery (9% (3/35) vs. 0% (0/71), p = 0.020) than patients with only leg lymphoedema. Additionally, patients with leg and midline lymphoedema reported less frequently lower leg lymphoedema (77% (27/35) vs. 95% (70/74), p = 0.007). Based on the multivariable analysis, not having lower leg lymphoedema, skin fibrosis, performing self-bandaging and self-manual lymphatic drainage appear to be predictors for having midline lymphoedema., Conclusions: If patients with lymphoedema after prostate cancer do not have lower leg lymphoedema, have skin fibrosis, perform self-bandaging or self-manual lymphatic drainage, they possibly have midline lymphoedema., (© 2024. The Author(s).)

Background: Lymphoedema is a chronic and progressive disease characterised by excessive accumulation of lymph in the interstitial compartment, leading to tissue swelling and fibroadipose deposition. Lymphangiogenesis is partly regulated by ketone body oxidation, and a ketogenic diet (KD) has shown therapeutic efficacy in a preclinical mouse tail lymphoedema model. Therefore, we aimed to investigate the potential therapeutic effect of a KD in patients with secondary lymphoedema., Methods: Nine patients with unilateral stage 2 lymphoedema secondary to lymphadenectomy were included in this quasi-experimental exploratory study consisting of a short run-in phase to gradually induce ketosis, followed by a classic KD (CKD) and modified Atkins diet (MAD) phase during which patients consumed a CKD and MAD, respectively. Lymphatic function and oedema volume, the primary outcomes, were assessed at baseline and at the end of both the CKD and MAD phase. Secondary outcomes included health-related and lymphedema-specific quality of life (QoL)., Results: Seven out of nine patients completed the study protocol. Lymphatic function was improved upon consumption of both a CKD (dermal backflow score [mean ± SD]: 7.29 ± 2.98 vs. 10.86 ± 2.19 at baseline; p = 0.03) and MAD (6.71 ± 2.06; p = 0.02), whereas oedema volume did not decrease during the course of the study (excess limb volume [mean ± SD]: 20.13 ± 10.25% at end of CKD and 24.07 ± 17.77% at end of MAD vs. 20.79 ± 12.96% at baseline; p > 0.99 and p > 0.30, respectively). No changes were observed in health-related, nor lymphoedema-specific QoL at the end of CKD and MAD., Conclusions: The consumption of a KD improved lymphatic function and was associated with a clinically meaningful reduction in oedema volume in some patients (3/7 at end of CKD, 2/7 at end of MAD) with unilateral stage 2 secondary lymphoedema. These results highlight the potential of a KD to improve lymphatic function in patients with lymphoedema. However, further studies are required to substantiate our findings., (© 2024 The British Dietetic Association Ltd.)

Purpose: This study investigated the accuracy of activity trackers in chronic lower limb lymphoedema (LLL) patients and in comparison to matched controls., Materials and Methods: Seventeen LLL patients and 35 healthy subjects wore an activity tracker at the hip (Fitbit Zip/Inspire; hip-AT) and one at the wrist (Fitbit Alta/Inspire; wrist-AT) combined with a reference activity monitor (Dynaport Movemonitor; DAM), for 14 consecutive days. To analyze accuracy and agreement, mean daily step count from both AT's were compared to DAM. To evaluate the accuracy as coaching tool, day-by-day differences were calculated. The Kendall correlation coefficient was used to test consistency of ranking daily steps between the AT's and the DAM., Results: The wrist-AT significantly overestimated daily step count compared to DAM in the LLL group (+1221 ± 1754 steps per day, p = 0.011) while the hip-AT underestimated the step count, although not significantly. Similar results were found in the healthy control group. As a coaching tool, both wrist-AT and hip-AT showed a moderate correlation with the DAM (r = 0.507 and 0.622, respectively) in the LLL group regarding consistency of ranking from most to least active days., Conclusion: Wrist-AT's significantly overestimate daily step count in a LLL population. As a coaching tool, both trackers show moderate validity, indicating applicability to improve physical activity., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 Blondeel et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Introduction: Lymphoedema is a chronic condition caused by lymphatic insufficiency. It leads to swelling of the limb/midline region and an increased risk of infection. Lymphoedema is often associated with mental and physical problems limiting quality of life. The first choice of treatment is a conservative treatment, consisting of exercises, skin care, lymph drainage and compression. Reconstructive lymphatic surgery is also often performed, that is, lymphovenous anastomoses, lymph node transfer or a combination. However, robust evidence on the effectiveness of reconstructive lymphatic surgery is missing. Therefore, the objective of this trial is to investigate the added value of reconstructive lymphatic surgery to the conservative treatment in patients with lymphoedema., Methods and Analysis: A multicentre randomised controlled and pragmatic trial was started in March 2022 in three Belgian university hospitals. 90 patients with arm lymphoedema and 90 patients with leg lymphoedema will be included. All patients are randomised between conservative treatment alone (control group) or conservative treatment with reconstructive lymphatic surgery (intervention group). Assessments are performed at baseline and at 1, 3, 6, 12, 18, 24 and 36 months. The primary outcome is lymphoedema-specific quality of life at 18 months. Key secondary outcomes are limb volume and duration of wearing the compression garment at 18 months. The approach of reconstructive lymphatic surgery is based on presurgical investigations including clinical examination, lymphofluoroscopy, lymphoscintigraphy, lymph MRI or CT angiography (if needed). All patients receive conservative treatment during 36 months, which is applied by the patient's own physical therapist and by the patient self. From months 7 to 12, the hours a day of wearing the compression garment are gradually decreased., Ethics and Dissemination: The study has been approved by the ethical committees of University Hospitals Leuven, Ghent University Hospital and CHU UCL Namur. Results will be disseminated via peer-reviewed journals and presentations., Trial Registration Number: NCT05064176., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Lymphoedema is caused by an imbalance between fluid production and transport by the lymphatic system. This imbalance can be either caused by reduced transport capacity of the lymphatic system or too much fluid production and leads to swelling associated with tissue changes (skin thickening, fat deposition). Its main common complication is the increased risk of developing cellulitis/erysipelas in the affected area, which can worsen the lymphatic function and can be the cause of raised morbidity of the patient if not treated correctly/urgently. The term primary lymphoedema covers a group of rare conditions caused by abnormal functioning and/or development of the lymphatic system. It covers a highly heterogeneous group of conditions. An accurate diagnosis of primary lymphoedema is crucial for the implementation of an optimal treatment plan and management, as well as to reduce the risk of worsening. Patient care is diverse across Europe, and national specialised centres and networks are not available everywhere. The European Reference Network on Rare Multisystemic Vascular Diseases (VASCERN) gathers the best expertise in Europe and provide accessible cross-border healthcare to patients with rare vascular diseases. There are six different working groups in VASCERN, which focus on arterial diseases, hereditary haemorrhagic telangiectasia, neurovascular diseases, lymphoedema and vascular anomalies. The working group Paediatric and Primary Lymphedema (PPL WG) gathers and shares knowledge and expertise in the diagnosis and management of adults and children with primary and paediatric lymphoedema. The members of PPL WG have worked together to produce this opinion statement reflecting strategies on how to approach patients with primary and paediatric lymphoedema. The objective of this patient pathway is to improve patient care by reducing the time to diagnosis, define the best management and follow-up strategies and avoid overuse of resources. Therefore, the patient pathway describes the clinical evaluation and investigations that lead to a clinical diagnosis, the genetic testing, differential diagnosis, the management and treatment options and the patient follow up at expert and local centres. Also, the importance of the patient group participation in the PPL WG is discussed., (Copyright © 2023 The Authors. Published by Elsevier Masson SAS.. All rights reserved.)

Objective: The purpose of this study was to investigate the effectiveness of fluoroscopy-guided manual lymph drainage (MLD) versus that of traditional and placebo MLD, when added to decongestive lymphatic therapy (DLT) for the treatment of breast cancer-related lymphedema (BCRL) (EFforT-BCRL trial), on the suprafascial accumulation of lymphatic fluid and skin elasticity., Methods: In this multicenter, 3-arm, double-blind, randomized controlled trial (EFforT-BCRL trial), 194 participants (mean age = 61 [SD = 10] years) with unilateral BCRL were recruited. All participants received standardized DLT (education, skin care, compression therapy, exercises) and were randomized to fluoroscopy-guided, traditional, or placebo MLD. Participants received 60 min/d of treatment during the 3-week intensive phase and 18 sessions of 30 minutes during the 6-month maintenance phase. During this phase, participants were instructed to wear a compression garment, to perform exercises, and to perform a self-MLD procedure once daily. This study comprises secondary analyses of the EFforT-BCRL trial. Outcomes were the amount of fluid accumulation in the suprafascial tissues (local tissue water, extracellular fluid, and thickness of the skin and subcutaneous tissue) and skin elasticity at the level of the arm and trunk. Measurements were performed at baseline; after intensive treatment; after 1, 3, and 6 months of maintenance treatment; and after 6 months of follow-up., Results: At the level of the arm, there was a significant improvement over time in the 3 groups for most of the outcomes. At the level of the trunk, no remarkable improvement was noted within the individual groups. No significant interaction effects (between-group differences) were present. Only skin elasticity at the level of the arm, evaluated through palpation, showed a significant interaction effect., Conclusion: All 3 groups showed similar improvements in response to DLT regardless of the type of MLD that was added. The effect of the addition of MLD to other components of DLT for reducing local tissue water and extracellular fluid or skin thickness and for improving skin elasticity and fibrosis in participants with chronic BCRL was limited., Impact: Although MLD has been applied all over the world for many years, evidence regarding its added value in reducing arm volume in patients with BCRL is lacking. These results show that adding MLD to other components of DLT has limited value in reducing local tissue water and extracellular fluid or skin thickness and in improving skin elasticity and fibrosis in patients with chronic BCRL. To date, there is no clinical indication to continue including time-consuming MLD in physical therapist sessions for patients with chronic BCRL., (© 2022 American Physical Therapy Association. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Little is known about the overall prevalence of lymphoedema in children and the types of paediatric lymphoedema seen by specialist centres. Therefore, this study was aimed to provide a profile of children with primary or secondary lymphoedema seen by the expert centres of the paediatric and primary lymphoedema working group (PPL-WG) of VASCERN and to compare the profile between the different countries. A retrospective review of all children (aged up to 18 years) seen for the first time by the expert centres over one year (2019) was carried out. Lymphoedema-, patient- and genetics-related data was collected and described for the whole group and compared between the different European countries/UK. In 2019, a total of 181 new children were seen by eight expert centres. For primary lymphoedema, the phenotype was based on the St George's classification of lymphatic anomalies. The percentages diagnosed according to each category were: 7.2% for syndromic lymphoedema, 2.8% for systemic/visceral involvement, 30.4% for congenital, 35.9% for late-onset lymphoedema and 19.3% for vascular/lymphatic malformations. 4.4% had secondary lymphoedema. Nearly 10% of all children had had at least one episode of cellulitis. The median delay from onset of symptoms to being seen by an expert centre was 2.4 years. In 44.4% of the children with primary lymphoedema a genetic test was performed, of which 35.8% resulted in a molecular diagnosis. Across the different centres, there was a wide variety in distribution of the different categories of paediatric lymphoedema diagnosed and the frequency of genetic testing. In conclusion, this paper has demonstrated that there is a large delay between the onset of paediatric lymphoedema and the first visit in the expert centres and that an episode of cellulitis is a relatively common complication. Diagnostic variation across the centres may reflect different referral criteria. Access to genetic testing was limited in some centres. It is recommended that these issues are addressed in the future work of the PPL-WG to improve the referral to the expert centres and the consistency in service provision for paediatric lymphoedema in Europe., (Copyright © 2022. Published by Elsevier Masson SAS.)

Purpose: It is not clear to what extent signs and symptoms other than arm swelling, including pain, altered sensory function, and body perception disturbances, differ between women with measurable and non-measurable breast cancer-related lymphedema (BCRL). A case-control study was performed to compare these signs and symptoms between (1) women with self-reported BCRL with objectively measurable swelling; (2) women with self-reported BCRL without objective confirmation; and (3) a control group with no self-reported BCRL., Methods: The three groups were compared for (1) the severity of self-reported signs and symptoms of BCRL, (2) problems in functioning related to BCRL, (3) pain-related outcomes, (4) sensory functions, and (5) body perception., Results: All self-reported outcomes related to signs and symptoms of BCRL and problems in functioning were significantly different between the control group and the other two groups with and without measurable self-reported BCRL (p < 0.001-0.003). Except for "skin texture" (p = 0.01), "hand swelling" (p=0.301) and 'difficulty writing' (p=0.676), no differences were found between groups. For pain-related outcomes, sensory function, and body perception, significant differences were only found for the mechanical detection threshold (p < 0.01) and self-reported disturbances in body perception (p < 0.001) between the self-reported BCRL groups and control group., Conclusions: Diverse signs and symptoms related to BCRL, sensory function, and perception were different among women with self-reported BCRL compared to controls. No differences between women with and without measurable self-reported BCRL were found., Implications for Cancer Survivors: The presence of self-reported BCRL, with or without measurable swelling, is a first indication for the need of further diagnostic evaluation., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Of the 1.38 million women who are diagnosed every year with breast cancer worldwide, 21% will develop arm lymphedema. Near-infrared fluorescence lymphatic imaging (NIRFLI) is an effective method for real-time evaluation of the lymphatic system. Reliability studies of the scoring of this NIRFLI are lacking. The aim of this study is to investigate if evaluation of the superficial lymphatic architecture and transport in patients with breast cancer-related lymphedema through NIRFLI can be performed in a reliable way. Methods and Results: The outcome parameters used to assess agreement were the presence of lymphatic transport from the injection sites, of dermal backflow patterns, of efferent lymphatic vessels, and of lymph nodes. The NIRFLI evaluations were scored before and after a break separately by two assessors. Twenty patients with lymphedema of the arm and/or hand were included. After the injection, there was weak to very strong agreement regarding the presence of transport from the injection sites ( K  = 0.459-1.000). The interpretation of the type of dermal backflow pattern varied from weak (WK = 0.452) to very strong agreement (WK = 1.000) between the two assessors. Agreement in the visualization of efferent lymphatic vessels was weak before and after the break ( K  = 0.490 and K  = 0.571) and agreement regarding the presence of lymph nodes was very strong ( K  = 1.000). Conclusion: Overall, there was moderate to strong agreement between the assessors when evaluating the lymphatic architecture and transport through NIRFLI. The study has been registered at clinicaltrials.gov (NCT02609724).

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., (Copyright © 2022 Australian Physiotherapy Association. Published by Elsevier B.V. All rights reserved.)

Background: Compression therapy is an important part of the treatment of patients with lymphedema or chronic venous disease. However, there is no validated questionnaire evaluating the effect of compression and its acceptance by the patient. Therefore, the aims of this study were to construct a questionnaire evaluating the effect of compression and its acceptance by the patient, that is, the Dutch ICC Compression Questionnaire (ICC-CQ), to investigate its reliability and validity, and to translate it into English. Methods and Results: Eleven experts in applying compression and 51 Dutch patients with experience of using compression were involved in the construction process. One part of the ICC-CQ has to be completed by the patient and evaluates seven domains. The other part has to be completed by the health care provider and comprises three domains. Reliability and validity of the final version was investigated in a new group of 79 Dutch-speaking patients with lymphedema or chronic venous disease, wearing compression garments ( N  = 52) or bandages ( N  = 27). Except for one domain, the Intraclass Correlation Coefficients for test-rest/interrater reliability ranged from 0.55 to 0.93. Cronbach's alpha for internal consistency ranged from 0.71 to 0.97. Eighty-nine percent of the patients fully understood the questionnaire indicating good face validity, and 87% found it complete indicating good content validity. Construct validity was considered good since 10 out of 11 hypotheses were accepted. Conclusion: The ICC-CQ is the first reliable and valid questionnaire evaluating different kinds of compression and the experience by patients with lymphedema or chronic venous disease.

Introduction: Lymphedema of the upper extremity is one of the most feared complications following breast cancer treatment. Lymphofluoroscopy is a sensitive instrument for detection of lymphedema and visualization of superficial lymphatic transport, thus suitable for early detection. Early detection of lymphedema is important as it can prevent lymphedema to progress into more severe stages and minimalize impact on quality of life and medical costs., Objective: To determine agreement between the presence of early disturbance of the lymphatic transport and outcome of clinical measurement tools evaluating the development of lymphedema., Methods: A prospective study was conducted in 128 breast cancer patients scheduled for breast cancer surgery. Patients were evaluated before surgery and 1, 3-, 6-, 9-, and 12-months' post-surgery. Cohen's Kappa was used to determine agreement between presence of early disturbance in lymphatic transport and presence of pitting/increased skinfold thickness/increased Percentage Water Content ratio (PWC)/increased arm-hand volume (circumference measures and water displacement)., Results: For pitting status (Kappa 0.23), for skinfold thickness (Kappa 0.29) and the PWC ratio (Kappa 0.21) a minimal agreement was found. The circumference measurement had a minimal agreement for 5% volume difference (Kappa 0.22) and no agreement for 3% volume difference (Kappa 0.19). Sensitivity was weak for all clinical assessments. The specificity was excellent for pitting status, skinfold thickness, PWC ratio, and for 5% volume difference. For 3% a high specificity was found., Conclusion: The clinical tools assessed in this study were not able to predict an early disturbance of the lymphatic transport seen on lymphofluoroscopy., (Copyright © 2021. Published by Elsevier Inc.)

Introduction: Breast cancer survivors with secondary upper limb lymphoedema (ULL) may report a wide range of self-reported symptoms. At the moment, no overview of ULL-specific symptoms is available. The first aim, therefore, was to compare the prevalence rates of self-reported signs and symptoms in people with and without secondary ULL due to breast cancer treatment. The second aim was to determine whether symptoms of lymphoedema could be predictive for the development of ULL. The third aim was to describe the association between the presence/severity of symptoms and the presence/severity of ULL., Methods: A systematic search was conducted in Medline, Scopus, CINAHL and EMBASE databases, with key words related to breast cancer, symptoms and ULL., Results: Twenty-nine articles were eligible. The most frequently reported signs and symptoms were swelling (80.9%) and heaviness (66.7%) in the ULL group and tenderness (37%) and numbness (27%) in the non-ULL group. Perceived larger arm size, as well as feelings of arm tightness, stiffness, puffiness, pain, sensory disturbances and functional changes were predictive for the development of ULL. Moderate correlations were found between the presence of swelling, firmness in the past year and tightness now and severity of ULL. There was also moderate correlation between the presence of swelling and heaviness now and the presence of ULL., Conclusions: Swelling and heaviness are the most commonly reported symptoms in patients with ULL. The presences of these two symptoms are moderately correlated with the presence and/or severity of ULL. Although limited information regarding the predictive self-reported symptoms for the development of ULL was found. Further research with standardised definitions of ULL and validated questionnaires for self-reported signs and symptoms are needed to confirm which signs and symptoms are related to ULL and which to other upper limb morbidities., (© 2021 John Wiley & Sons Ltd.)

Purpose: Upper limb lymphedema is a vexing morbidity that can occur after the treatment for breast cancer. The Lymphedema Functioning, Disability and Health Questionnaire for Upper Limb Lymphedema (Lymph-ICF-UL) is a valid and reliable tool assessing problems in functioning in patients with breast cancer-related lymphedema. Until now, a French-language version was lacking. The aim of this study was to perform a cross-cultural validation of the French version of the Lymph-ICF-UL questionnaire., Methods: A forward-backward translation process between the original language (Dutch) and the target language (French) was performed. Psychometric properties of this final French version were examined in 50 participants., Results: Intraclass correlation coefficients for test-retest reliability ranged from 0.66 to 0.95. Cronbach's alpha coefficients for internal consistency were higher than 0.77. Face and content validity were very good because the scoring system was clear for all participants (100%), questions were understandable (100%), and all complaints due to BCRL were mentioned by 78% of the participants. Construct validity was moderate. Convergent validity was established since 3 out of 5 expected domains of the Lymph-ICF-UL showed a moderate correlation with expected domains of the 36-item Short-Form Health Survey. There was satisfactory divergent validity as 6 out of 9 hypotheses assessing divergent validity were accepted., Conclusion: The French version of the Lymph-ICF-UL is a reliable and valid questionnaire and ready for use in clinical as well as in scientific practice.Implications for rehabilitationSince the introduction of more effective treatment modalities increasing the number of breast cancer survivors, the amount of patients dealing with lymphedema is rising likewise up to a pooled incidence rate of more than 16% of the women treated for breast cancer.The French version of the Lymph-ICF-UL is a reliable and valid questionnaire for assessing problems in functioning of patients with breast cancer-related lymphedema of the arm and/or hand.As the questionnaire provides patient information in the different domains of the International Classification of Functioning, Disability and Health, it facilitates evaluating the impact of breast cancer-related lymphedema on daily functioning.Based on the outcomes of the Lymph-ICF-UL treatment goals can be set, where after the questionnaire can be used to monitor long-term results of this treatment and self-care.

Objective: To investigate the reliability, time efficiency and clinical feasibility of five commonly used methods for assessing excessive arm volume in patients with breast cancer-related lymphoedema (BCRL)., Design: Cross-sectional study., Setting: University Hospitals Leuven, Belgium., Subjects: 30 participants with unilateral BCRL., Methods: Excessive arm volume was determined by five different methods: traditional volumetry with overflow, volumetry without overflow, inverse volumetry, optoelectronic volumetry and calculated volume based on circumference measurements. To investigate intra- and inter-rater reliability, measurements were performed twice by the same assessor and once by a different assessor. Intraclass correlation coefficients (ICCs), standard errors of the measurement (SEMs) and systematic changes between the means were calculated. To determine time efficiency, the mean setup time, execution time and total time were examined for each method. Furthermore, 12 limitations regarding clinical feasibility were listed and scored for each method. Finally, an overall ranking score was determined between the methods., Results: Mean age was 65 (±8) years and mean body mass index was 28 (±4) kg/m 2 . Intra- and inter-rater reliability ranged between strong and very strong. Calculated arm volume based on circumferences (mean excessive arm volume: assessor A: 477 (±367) mL; assessor B: 470 (±367) mL; assessor A (second time): 493 (±362) mL) showed the highest intra- and inter-rater ICCs of .987 and .984, respectively. Optoelectronic volumetry was the fastest method, representing a mean total time of 1 minute and 43 (±26) seconds for performing a bilateral measurement. The least limitations were reported on the calculated volume based on the circumference method (3 out of 12 limitations)., Conclusion: Calculated volume based on arm circumferences is the best measurement method for evaluating excessive arm volume over time in terms of reliability, low error rate, low cost, few limitations and the time spent.

Background: Lymphedema is associated with significant physical and psychosocial problems. The Lymphedema Functioning, Disability and Health questionnaire for upper limb lymphedema is a valid and reliable tool quantifying the amount of problems in functioning in patients with breast cancer-related lymphedema. Patients suggested a revision of the scoring system to facilitate completion of the questionnaire. Therefore, adjustment of the questionnaire was carried out by implementing a numeric rating scale instead of the existing visual analog scale. Purpose of this study was to investigate reliability and validity of the revised Lymph-ICF, called the Lymph-ICF-UL. Methods and Results: Reliability and validity of the Lymph-ICF-UL were examined in 56 participants with upper limb lymphedema. Intraclass correlation coefficients for test-retest reliability ranged from 0.79 to 0.95. Cronbach's alpha coefficients for internal consistency were higher than 0.80. Face and content validity were very good because the scoring system was clear for all participants (100%), questions were understandable for all participants (100%), and all complaints due to arm lymphedema were mentioned by 98% of the participants. Construct validity was good. Convergent validity was established since four out of five expected domains of the Lymph-ICF-UL showed a moderate correlation with expected domains of the 36-Item Short-Form Health Survey questionnaire. There was good divergent validity because seven out of nine hypotheses assessing divergent validity were accepted. Conclusion: The Lymph-ICF-UL is a reliable and valid questionnaire using a simplified and clearer scoring procedure to assess impairments in function, activity limitations, and participation restrictions of patients with breast cancer-related arm lymphedema.

Question: What are the short-term and long-term preventive effects of manual lymph drainage (MLD), when used in addition to information and exercise therapy, on the development of lymphoedema after axillary dissection for breast cancer?, Design: Randomised controlled trial with concealed allocation, blinded assessors and intention-to-treat analysis., Participants: Adults undergoing unilateral dissection for breast cancer were recruited, with 79 allocated to the experimental group and 81 to the control group., Intervention: The experimental group received guidelines about prevention of lymphoedema, exercise therapy and MLD. The control group received the same guidelines and exercise therapy, but no MLD. The interventions in both groups were delivered for 6 months., Outcome Measures: The primary outcome was cumulative incidence of arm lymphoedema defined in four ways (≥200ml,≥2cm,≥5%, and≥10% increase), which represent the difference in arm volume or circumference between the affected and healthy sides compared with the difference before surgery. Secondary outcomes included point prevalence of lymphoedema, change in arm volume difference, shoulder range of movement, quality of life and function., Results: Incidence rates were comparable between experimental and control groups at all follow-up measurements. Sixty months after surgery, the cumulative incidence rate for the≥200ml definition was 35% for the experimental group versus 29% for the control group (RR 0.89, 95% CI 0.51 to 1.54, p=0.45); for the≥2cm definition 35% versus 38% (RR 0.93, 95% CI 0.59 to 1.45, p=0.73); for the≥5% definition 68% versus 53% (RR 1.28, 95% CI 0.97 to 1.69, p=0.08) and for the≥10% definition 28% versus 24% (RR 1.18, 95% CI 0.66 to 2.10, p=0.57). The secondary outcomes were comparable between the groups at most assessment points., Conclusion: Manual lymph drainage may not have a preventive effect on the development of breast cancer-related lymphoedema in the short and long term., Trial Registration: Netherlands Trial Register NTR 1055. [Devoogdt N, Geraerts I, Van Kampen M, De Vrieze T, Vos L, Neven P, Vergote I, Christiaens M-R, Thomis S, De Groef A (2018) Manual lymph drainage may not have a preventive effect on the development of breast cancer-related lymphoedema in the long term: a randomised trial. Journal of Physiotherapy 64: 245-254]., (Copyright © 2018 Australian Physiotherapy Association. Published by Elsevier B.V. All rights reserved.)

Objectives: Lymphoedema is a dreadful complication following breast cancer therapy. According to the International Society of Lymphology, the consensus treatment for breast cancer-related lymphoedema (BCRL) is the decongestive lymphatic therapy. This is a two-phase treatment and combines different treatment modalities including skin care, manual lymphatic drainage (MLD), compression therapy and exercise. However, the additional effect of MLD is debated since pooled data only demonstrated a limited non-significant additional value. A possible explanation is that in previous studies MLD has been applied blind, without knowledge of patient-specific lymphatic routes of transport. In addition, the MLD hand manoeuvres used by the therapists in previous studies, possibly did not optimally stimulate lymphatic transport. Recently, near-infrared fluorescence imaging has been introduced to visualise the superficial lymphatic network which allows MLD at the most needed location. The aim of the present study is to determine the effectiveness of the fluoroscopy-guided MLD, additional to the other parts of the decongestive lymphatic therapy and compared to the traditional or a placebo MLD, in the treatment of BCRL., Study Design: A three-arm double-blinded randomised controlled trial will be conducted in different university hospitals in Belgium. Based on a sample size calculation, 201 participants with chronic BCRL stage 1 or 2 of the arm or hand, with at least 5% difference between both sides (corrected for hand dominance) need to be recruited. All participants receive the standard treatment: skin care, compression therapy and exercises. The intervention group additionally receives fluoroscopy-guided MLD. One control group additionally receives the traditional 'blind' MLD and a second control group receives a placebo MLD. All subjects receive 3 weeks of daily intensive treatments and 6 months of maintenance treatment. Follow-up period is 6 months. The primary outcomes are the reduction in lymphoedema volume of the arm/hand and the change in stagnation of lymph fluid at level of the shoulder/trunk., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Patients may develop primary (congenital) or secondary (acquired) lymphedema, causing significant physical and psychosocial problems. To plan treatment for lymphedema and monitor a patient's progress, swelling, and problems in functioning associated with lymphedema development should be assessed at baseline and follow-up., Objective: The purpose of this study was to investigate the reliability (test-retest, internal consistency, and measurement variability) and validity (content and construct) of data obtained with the Lymphoedema Functioning, Disability and Health Questionnaire for Lower Limb Lymphoedema (Lymph-ICF-LL)., Design: This was a multicenter, cross-sectional study., Methods: The Lymph-ICF-LL is a descriptive, evaluative tool containing 28 questions about impairments in function, activity limitations, and participation restrictions in patients with lower limb lymphedema. The questionnaire has 5 domains: physical function, mental function, general tasks/household activities, mobility activities, and life domains/social life. The reliability and validity of the Lymph-ICF-LL were examined in 30 participants with objective lower limb lymphedema., Results: Intraclass correlation coefficients for test-retest reliability ranged from .69 to .94, and Cronbach alpha coefficients for internal consistency ranged from .82 to .97. Measurement variability was acceptable (standard error of measurement=5.9-12.6). Content validity was good because all questions were understandable for 93% of participants, the scoring system (visual analog scale) was clear, and the questionnaire was comprehensive for 90% of participants. Construct validity was good. All hypotheses for assessing convergent validity and divergent validity were accepted., Limitations: The known-groups validity and responsiveness of the Dutch Lymph-ICF-LL and the cross-cultural validity of the English version of the Lymph-ICF-LL were not investigated., Conclusions: The Lymph-ICF-LL is a Dutch questionnaire with evidence of reliability and validity for assessing impairments in function, activity limitations, and participation restrictions in people with primary or secondary lower limb lymphedema.

Background: Compared to volume/circumference measurements to diagnose lymphedema, thickening and disturbance of echogenicity of cutis and subcutis associated with the development of lymphedema is measured directly by ultrasonography. The aim of the present study was to investigate the evolution of thickness and echogenicity of cutis and subcutis of the lower and upper arm after axillary dissection for breast cancer and compare patients with and without arm lymphedema., Methods and Results: Immediately after the surgery and at 6 and 12 months post-surgery, 42 patients received an ultrasonography of both arms, at the wrist, ventral and dorsal side of the lower arm, and biceps and triceps side of the upper arm. Thickness and echogenicity of cutis and subcutis were determined. Lymphedema was defined as 5.0% or more increase compare to the preoperative value. Eighteen patients had developed lymphedema of the lower arm, and 24 patients of the upper arm. Thickness of the cutis decreased significantly during the postoperative period on the healthy and affected side as well. The difference of cutis thickness between affected and healthy side increased postoperatively in patients with lymphedema and decreased in patients without lymphedema at the wrist and dorsal side of the lower arm. Patients with lymphedema had more frequent disturbance of echogenicity of the cutis at all measurement points. In exception of the triceps, thickness and echogenicity of the subcutis is less obvious disturbed. Sensitivity to detect arm lymphedema by ultrasonography was acceptable for increased subcutis thickness at the ventral lower arm and triceps and disturbed cutis echogenicity at the wrist (67%-100%). Sensitivity for the other measurements was bad. Specificity was for all measurements acceptable (59%-100%)., Conclusion: Ultrasonography may be useful to diagnose lymphedema developing after axillary dissection for breast cancer. However, it cannot be used as a separate diagnostic test for lymphedema.

Objective: To determine the preventive effect of manual lymph drainage on the development of lymphoedema related to breast cancer., Design: Randomised single blinded controlled trial., Setting: University Hospitals Leuven, Leuven, Belgium., Participants: 160 consecutive patients with breast cancer and unilateral axillary lymph node dissection. The randomisation was stratified for body mass index (BMI) and axillary irradiation and treatment allocation was concealed. Randomisation was done independently from recruitment and treatment. Baseline characteristics were comparable between the groups., Intervention: For six months the intervention group (n = 79) performed a treatment programme consisting of guidelines about the prevention of lymphoedema, exercise therapy, and manual lymph drainage. The control group (n = 81) performed the same programme without manual lymph drainage., Main Outcome Measures: Cumulative incidence of arm lymphoedema and time to develop arm lymphoedema, defined as an increase in arm volume of 200 mL or more in the value before surgery., Results: Four patients in the intervention group and two in the control group were lost to follow-up. At 12 months after surgery, the cumulative incidence rate for arm lymphoedema was comparable between the intervention group (24%) and control group (19%) (odds ratio 1.3, 95% confidence interval 0.6 to 2.9; P = 0.45). The time to develop arm lymphoedema was comparable between the two group during the first year after surgery (hazard ratio 1.3, 0.6 to 2.5; P = 0.49). The sample size calculation was based on a presumed odds ratio of 0.3, which is not included in the 95% confidence interval. This odds ratio was calculated as (presumed cumulative incidence of lymphoedema in intervention group/presumed cumulative incidence of no lymphoedema in intervention group)×(presumed cumulative incidence of no lymphoedema in control group/presumed cumulative incidence of lymphoedema in control group) or (10/90)×(70/30)., Conclusion: Manual lymph drainage in addition to guidelines and exercise therapy after axillary lymph node dissection for breast cancer is unlikely to have a medium to large effect in reducing the incidence of arm lymphoedema in the short term. Trial registration Netherlands Trial Register No NTR 1055.

Background: Lymphedema occurs frequently after axillary dissection for breast cancer and causes significant physical and psychosocial problems. To plan the treatment for lymphedema and monitor the patient's progress, arm swelling and arm function need to be assessed., Objective: The purpose of this study was to investigate the reliability (test-retest, internal consistency, measurement variability) and validity (content and construct) of data obtained with the Lymphoedema Functioning, Disability and Health questionnaire (Lymph-ICF)., Methods: The Lymph-ICF is a descriptive and evaluative tool and consists of 29 questions about impairments in function, activity limitations, and participation restrictions of patients with breast cancer and arm lymphedema. The questionnaire is divided into 5 domains: physical function, mental function, household activities, mobility activities, and life and social activities. Reliability and validity were examined on 60 patients with lymphedema and 30 patients without lymphedema., Results: Intraclass correlation coefficients for test-retest reliability ranged from .65 to .93. Cronbach alpha coefficients for internal consistency were higher than .70. There were no systematic changes from the first test to the second test, and measurement variability was acceptable (standard errors of measurement=4.8-12.5). Content validity was good because all questions were understandable for all participants, the scoring system (visual analog scale) was clear for 88% of the participants, and all complaints due to arm lymphedema were mentioned by 85% of the participants. Construct validity was good. There was good convergent validity because 5 expected domains of the Lymph-ICF had the strongest correlation with 5 expected domains of the 36-Item Short-Form Health Survey questionnaire (SF-36). Thus, the 5 hypotheses assessing convergent validity were accepted. There was acceptable divergent validity because 3 of 5 hypotheses assessing divergent validity were accepted. There was good known-groups validity because patients with lymphedema had a higher total score on the Lymph-ICF and had a higher score on each domain and on each question (except one) compared with patients without lymphedema., Conclusion: The Lymph-ICF is a reliable and valid questionnaire to assess impairments in function, activity limitations, and participation restrictions of patients with arm lymphedema after axillary dissection for breast cancer.

Aim: The aim of this paper was to examine the reliability and validity of a new measurement device that counters the disadvantages of the traditional method of arm circumference measurements., Methods: We measured the arm on the non-operated side of breast cancer patients. Sixty-four patients were measured twice by the same assessor and 48 patients were measured twice by two different assessors. The arm circumferences were measured at the olecranon and each 4 cm proximal and distal of the olecranon. The measurements were performed with a self-developed device consisting of a stainless steel bar on which a tapeline was fixed at every 4 cm distance. The arm volume was calculated from the circumference measurements with the frustrum formula and was also measured directly with the water displacement method., Results: For the circumference measurements, intrarater and interrater ICCs ranged between 0.942 and 0.998. ICCs for the calculated arm volume were also very high. No systematic changes between the first to the second assessment could be found. The standard error of measurement for the circumference measurements as well as for the calculated arm volume was low (between 0.8% and 2.0%). An increase of 1.0 cm or more of the arm circumference at any measurement side and of 55 ml or more of the calculated arm volume was clinically significant., Conclusion: Arm circumferences and also the calculated arm volume can be measured quickly and accurately with a simple and inexpensive device.

The purpose of this article is to provide a systematic review of Combined Physical Therapy, Intermittent Pneumatic Compression and arm elevation for the treatment of lymphoedema secondary to an axillary dissection for breast cancer. Combined Physical Therapy starts with an intensive phase consisting of skin care, Manual Lymphatic Drainage, exercises and bandaging and continues with a maintenance phase consisting of skin care, exercises, wearing a compression sleeve and Manual Lymphatic Drainage if needed. We have searched the following databases: PubMed/MEDLINE, CINAHL, EMBASE, PEDro and Cochrane. Only (pseudo-) randomised controlled trials and non-randomised experimental trials investigating the effectiveness of Combined Physical Therapy and its different parts, of Intermittent Pneumatic Compression and of arm elevation were included. These physical treatments had to be applied to patients with arm lymphoedema which developed after axillary dissection for breast cancer. Ten randomised controlled trials, one pseudo-randomised controlled trial and four non-randomised experimental trials were found and analysed. Combined Physical Therapy can be considered as an effective treatment modality for lymphoedema. Bandaging the arm is effective, whether its effectiveness is investigated on a heterogeneous group consisting of patients with upper and lower limb lymphoedema from different causes. There is no consensus on the effectiveness of Manual Lymphatic Drainage. The effectiveness of skin care, exercises, wearing a compression sleeve and arm elevation is not investigated by a controlled trial. Intermittent Pneumatic Compression is effective, but once the treatment is interrupted, the lymphoedema volume increases. In conclusion, Combined Physical Therapy is an effective therapy for lymphoedema. However, the effectiveness of its different components remains uncertain. Furthermore, high-quality studies are warranted. The long-term effect of Intermittent Pneumatic Compression and the effect of elevation on lymphoedema are not yet proven., (Copyright 2009 Elsevier Ireland Ltd. All rights reserved.)

DEVOOGDT N., VAN KAMPEN M., CHRISTIAENS M.R., TROOSTERS T., PIOT W., BEETS N., NYS S. & GOSSELINK R. (2011) European Journal of Cancer Care, 77-86 All breast cancer patients, suspected with lymph node invasion, need an axillary lymph node dissection. This study investigated the short- and long-term effects of the treatment for breast cancer on shoulder mobility, development of lymphoedema, pain and activities of daily living. Patients who had a modified radical mastectomy (33%) or a breast-conserving procedure (67%) in combination with axillary lymph node dissection were included. Shoulder mobility, lymphoedema, pain and activities of daily living were evaluated at 3 months and at 3.4 years after surgery. At long term, 31% of the patients experienced impaired shoulder mobility, 18% developed lymphoedema, 79% had pain and 51% mentioned impaired daily activities. Between 3 months and 3.4 years after surgery, impaired shoulder mobility decreased from 57% to 31%. The incidence of lymphoedema increased from 4% to 18%. Patients experienced an equal amount of pain but fewer problems with daily activities. At 3.4 years, no significant differences between mastectomy and breast-conserving procedure were found. In conclusion, at long term, significant number of breast cancer survivors still had impaired shoulder mobility, developed lymphoedema, had pain and experienced difficulties during daily activities. Shoulder mobility, pain and daily activities evolved positively, while the incidence of lymphoedema increased. [ABSTRACT FROM AUTHOR]