An overview of manual lymphatic drainage.

Manual lymphatic drainage is an advanced therapy that enhances the movement of lymph via a gentle form of massage. In this article, Sarah Jane Palmer provides an overview of manual lymphatic drainage as a treatment for lymphoedema, and summarises the latest research in the area.

Podoconiosis in Uganda: prevalence, geographical distribution and risk factors.

BACKGROUND: Podoconiosis is a neglected debilitating yet preventable disease. Despite its public health significance, podoconiosis is often misdiagnosed and confused with lymphatic filariasis. No appropriate diagnostic tests exist, contributing to underestimation and the absence of control interventions. METHODS: A population-based cross-sectional survey was conducted in seven districts with suspected or reported cases of podoconiosis or an altitude of 1200 m above sea level. Conducted from 30 January to 19 March 2023, the survey employed multilevel stratified sampling to reach eligible household members. RESULTS: Of the 10 023 participants sampled, 187 (confidence interval 1.25 to 2.78) had clinical features of podoconiosis. The highest prevalence was recorded in Nakapiripirit (7.2% [58/809]) and Sironko (2.8 [44/1564]) and the lowest in Kasese (0.3% [5/1537]), but ranged from 1.1 to 1.8% in Zombo, Rukungiri, Gomba and Hoima districts. The duration of podoconiosis was reported to range from 1 to 57 y. Factors associated with podoconiosis occurrence included advanced age, tungiasis, household cleanliness and personal hygiene. Sleeping on a bed, bathing daily, use of soap and use of footwear in at least moderate condition were protective against podoconiosis. CONCLUSIONS: Podoconiosis occurred in all the sampled districts and was linked to personal hygiene. Long-standing cases suggest an absence of treatment. There is potential for early intervention using a holistic care model in managing this condition. Urgent action and stakeholder engagement are essential for effective podoconiosis management.

Localised Objective Characterisation Assessment of Lymphoedema (LOCAL): Using High-Frequency Ultrasound, Bioelectrical Impedance Spectroscopy and Volume to Evaluate Superficial Tissue Composition.

Lymphoedema tissue is characterised by excess free fluid and structural changes to the extracellular matrix (ECM) in the form of fibrotic and fatty deposition. These tissue characteristics are integral to the assessment of lymphoedema progression; however, clinicians and researchers often focus on changes in the free fluid, volume and function of lymphatic vasculature to inform practice. Subsequently, little is known about the effect of clinical interventions on lymphoedema tissue composition. This article presents a novel approach to classify lymphoedema tissue. The Localised Objective Characterisation Assessment of Lymphoedema (LOCAL) classification combines diagnostic and clinically meaningful objective assessment thresholds to infer lymphoedema pathophysiological changes in tissue layers. The LOCAL classification method was verified using data from fifteen women with unilateral breast cancer-related lymphoedema who were evaluated at three sites on each arm using high-frequency ultrasound (HFUS), bio-electrical impedance spectroscopy (BIS) and volume measurements. Participants exhibited an uneven distribution of volume between the proximal and distal segments of the arm (p = 0.023), with multiple tissue compositional categories observed across sites on the same limb (p < 0.001). The LOCAL method demonstrated utility in categorising a diverse range of lymphoedema tissue layer changes beyond what can be ascertained from whole-limb measures.

Pitting Is Not Only a Measure of Oedema Presence: Using High-Frequency Ultrasound to Guide Pitting Test Standardisation for Assessment of Lymphoedema.

The pitting qualities of lymphoedema tissue change with disease progression. However, little is known about the underlying tissue response to the pitting test or the tissue characteristics that enhance or resist indentation. The pitting test is currently unstandardised, and the influence of test technique on pitting outcomes is unknown. Understanding how tissue reacts to applied pressure will build evidence for the standardisation of the pitting test. Ninety pitting test sites from fifteen women with unilateral breast cancer-related lymphoedema were evaluated using high-frequency ultrasound (HFUS), bioelectrical impedance spectroscopy (BIS), and limb volume measures. Three sites on each lymphoedema and non-lymphoedema arm were subject to a 60-s (s) staged pitting test, with changes in tissue features captured with ultrasound imaging before, throughout, and after the pitting test. Pitting qualities of tissues varied greatly, with lymphoedema sites pitting more frequently (p < 0.001) with greater depth (p < 0.001) and requiring a longer recovery time (p = 0.002) than contralateral unaffected tissue. Pitting is not solely attributable to oedema volume. Non-structural and structural characteristics of dermal and subcutaneous layers also influence tissue responses to sustained pressure. To enhance the validity and reliability of pitting assessment, a 60 s staged pitting test with an observation of tissue recovery is recommended for lymphoedema presentations.

Evidence for the clinical effectiveness of decongestive lymphoedema treatment for breast cancer-related arm lymphoedema, a systematic review.

PURPOSE: Early treatment is advised for breast cancer-related arm lymphoedema (BCRL), a common sequelae of breast cancer treatment. Expert guidance recommends two-phase decongestive lymphoedema treatment (DLT), although evidence is lacking for current treatment protocols and UK women are routinely offered self-treatment with hosiery. This systematic review considered evidence regarding treatment of early BCRL, that is, within 12 months of developing BCRL. METHODS: A systematic review of evidence for clinical effectiveness of DLT for women with less than 12-month BCRL duration (early BCRL) was undertaken using the Joanna Briggs Institute (JBI) method. Studies included women with < 12-month or mean < 9-month BCRL duration; some studies reported only one eligible group. The original search was conducted in 2016 and updated in 2018 and 2022. Methodological quality of identified studies was assessed using JBI critical appraisal instruments. Outcomes of interest were extracted with eligible results displayed in narrative and tabular format. Strength of evidence was rated using the GRADE system. RESULTS: Seven trials and three descriptive studies provided weak evidence (grade B) for effectiveness of DLT for early BCRL. Heterogeneous protocols limited comparison of findings. There was no evidence for the most effective treatment or treatment combination or optimal frequency or duration of treatment. CONCLUSION: There is no evidence to justify change in current lymphoedema treatment, whether self-treatment with hosiery (UK) or two-phase DLT (other countries). Further research for the early BCRL population is required. IMPLICATIONS FOR CANCER SURVIVORS: Women with early BCRL require early and effective treatment although this updated review shows there is still no evidence for what that treatment should be.

Addressing the Syndemic Relationship between Lymphatic Filariasis and Mental Distress in Malawi: The Potential of Enhanced Self-Care.

Lymphatic filariasis (LF) causes disfiguring and disabling lymphoedema, which can lead to mental distress and requires life-long self-care treatment. This study applies syndemic theory to understand the biosocial relationship between LF and mental distress in Malawi. Using in-depth qualitative methods, we critically evaluate experiences of mental distress and LF through 21 life-history interviews, to narrate experiences from the perspective of persons affected by LF, and to understand how enhanced self-care (ESC) for lymphoedema management disrupts the syndemic relationship. Complementary key informant interviews with Ministry of Health LF programme staff were conducted to further understand intervention and health system delivery. All interviews were recorded, transcribed, and translated, and then subject to thematic analysis. Our findings suggest that for persons affected by LF in Malawi, before being trained in ESC, absent referral pathways, inequalities in healthcare provision or available treatment, and limited knowledge of the condition (LF) drove the syndemic of LF and mental distress. Distress was often exacerbated by stigma and social exclusion, and shaped by intersections of gender, generation, poverty, and extreme climate conditions. We argue that addressing the syndemic suffering associated with LF and mental distress through interventions which center the needs of persons affected is critical in effective and equitable LF care delivery.

Quality of Life in Lymphedema Patients Treated by Microsurgical Lymphatic Vessel Transplantation-A Long-Term Follow-Up.

INTRODUCTION: Lymphedema is a chronic condition characterized by the accumulation of lymph fluid in the upper or lower extremities, leading to swelling, discomfort, and disability in everyday life. While various treatment modalities exist, microsurgical lymphatic vessel transplantation (LVT) has emerged as a promising option. However, there is little to no long-term follow-up data regarding patients' improvement in quality of life for this surgical technique. The present study conducts an investigation of the long-term health-related quality of life (HRQoL) over more than 20 years in patients with lymphedema treated with LVT and accomplishes this by utilizing an adapted SF-12 survey. PATIENTS AND METHODS: A retrospective analysis was conducted on patients who underwent LVT between 1 January 1983 and 1 October 2010 at LMU Clinic Munich (n = 35). Quality of life scores were assessed preoperatively and today in terms of physiological conditions, psychological conditions, and burden of therapy using a SF-12 survey adapted to the symptoms and impairments that chronic lymphedemas are known to cause. RESULTS: Our findings demonstrate a significant improvement in HRQoL following LVT, with notable enhancements in physiological and psychological conditions such as burden of therapy. Physiological conditions showed a significant positive change of 3.2648 (p < 0.01). Psychological conditions improved significantly by a factor of 2.0882 (p < 0.01). Additionally, the burden of therapy improved significantly by 1.5883 points (p < 0.01). CONCLUSION: Previous studies have already shown a significant improvement of HRQoL within the first postoperative years for patients treated by LVT. This study also demonstrates significant long-term improvement after LVT, thus underlining the effectiveness of using LVT to improve the quality of live for patients with both primary and secondary lymphedema long-term.

The effect of exercise and educational programs for breast cancer patients on the development of breast cancer-related lymphoedema: secondary endpoint from a randomized controlled trial in the Setouchi Breast Project-10.

BACKGROUND: Although the association between higher physical activity and preventive effect on breast-cancer-related lymphoedema (BCRL) has been reported, it is unclear what intervention is optimal. We aimed to investigate the effect of exercise and educational programs on BCRL development. METHODS: This study was a secondary endpoint analysis from a prospective randomized controlled trial. We enrolled patients with stage 0-III breast cancer from March 2016 to March 2020 and randomly assigned them to the control (n = 111), education (n = 115), or exercise (n = 104) group. As secondary endpoint, we assessed the incidence of and preventive effect on BCRL at 12 months post-intervention. RESULTS: There were no significant differences in the incidence of BCRL at 12 months post-intervention between the exercise and control groups (9.8% and 10.8%, P = 0.83) and the education and control groups (11.6% and 10.8%, P = 1.00). There were no significant differences in time to BCRL onset from the day of surgery between the exercise and control groups (event rate at 12 months: 20.7% and 17.2%, log-rank, P = 0.54) and the education and control groups (18.8% and 17.2%, log-rank, P = 0.57). The multivariable analyses indicated that axillary dissection and obesity significantly increased the risk of BCRL [hazard ratio (HR): 2.36, 95% confidence interval (CI) 1.52-3.67 and HR: 1.68, 95% CI 1.07-2.63, respectively]. CONCLUSIONS: The intervention did not decrease the risk of BCRL, and axillary dissection and obesity were the risk factors of BCRL. TRIAL REGISTRATION NUMBER: UMIN000020595 at UMIN Clinical Trial Registry.

Obesity-related pubis lymphedema enclosing genitalia: An atypical case of genital reconstruction.

Massive localized lymphoedema (MLL) is a rare complication of morbid obesity and has been scarcely reported in the literature, especially in the pubic area and genitalia. It is associated to BMI more than 40 kg/m2. We report the case of a 37-year-old patient known for morbid obesity with 68.8 kg/m2 BMI and hypogonadism-obesity syndrome presenting an unusually voluminous scrotal MLL mass. Malignancy was ruled out before surgery. In total a 7.5 kg scrotal mass was resected. Surgery was performed with minor complications without requiring additional surgery.

Feasibility of implementing a remote system for lymphoedema conservative therapy: a case study.

BACKGROUND: Poor access to lymphoedema specialists and communication between them and patients prevents appropriate lymphoedema management. Therefore, development and dissemination of remote systems is necessary to improve care in rural areas with limited medical personnel or access to medical coordination. AIMS: The authors evaluated the elements required for providing patient education on conservative therapy for lymphoedema, to determine the feasibility of remote management. METHODS: The study involved connecting a health professional in a local clinic (point A) treating a patient with lymphoedema, who was present alongside the clinician, with a specialist certified lymphoedema therapist (CLT) located remotely in a university (point B). FINDINGS: The CLT was able to greet, interview and provide guidance to the patient on conservative therapy. Direct contact with the patient was not possible, which limited visualisation, palpation, leg circumference measurement, and lymphatic drainage management. CONCLUSION: The findings suggest that remote a lymphoedema management approach involving conservative therapy benefits both patients and health professionals, particularly in rural regions. Future studies are needed to confirm the effectiveness of this approach to confirm adequate treatment.

Potential therapeutic effect of a ketogenic diet for the treatment of lymphoedema: Results of an exploratory study.

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.

The lymphedema patient experience within the healthcare system: a cross-sectional epidemiologic assessment.

Lymphedema is a progressive lymphatic disease that potentiates physical and psychosocial distress. Despite its impact, patients reportedly encounter lymphatic ignorance throughout the healthcare system. This cross-sectional study aims to summarize clinical characteristics and interactions of lymphedema patients within the healthcare system. Two lymphedema patient cohorts were included: The Global Registry Analysis Cohort included lymphedema patients who contributed to an international digital lymphatic registry and the Interactions Cohort included patients who initiated a questionnaire about interactions with the medical system. The global registry was used to obtain demographic and clinical characteristics from affiliated lymphedema patients. A 23-item online questionnaire on healthcare experiences and satisfaction with lymphatic healthcare was then distributed to the Interactions Cohort. Complete responses were obtained from 2474 participants. Participants were a mean age of 57.5 ± 16.1 years and 51.4% had a cancer history. Participants reported substantial delays in diagnosis and treatment. Cancer-related and non-cancer-related lymphedema patients reported similar levels of perceived physician disinterest in their lymphedema; however, non-cancer-related lymphedema patients reported more care dissatisfaction. Ultimately, patients continue to face delays in lymphedema diagnosis and treatment. We developed an evidence-based model highlighting areas of reform needed to improve lymphatic education and healthcare.

Panniculectomy for a Rare End-Stage Complication of Severe Obesity and Increasing Surgical Phenomenon.

Panniculus morbidus (PM) is a presentation of severe chronic abdominal lymphoedema associated with obesity resulting in oedema and chronic fibrosis. It is a multifaceted condition with significant clinical and psychosocial implications. A 29-year-old female weighing 260 kg with a body mass index of 95 kg/m2 had recurrent infections and sepsis associated with an abdominal pannus extending to her knees and an area of ulceration. The pannus was indurated with extensive fibrosis that significantly affected her quality of life (QOL) requiring assistance for all activities of daily living (ADLs). A panniculectomy was performed with a negative pressure skin dressing over the skin wound. She was discharged after two days. Two months postoperatively, she reported significant improvement in QOL and can now mobilise and perform ADLs independently with no recurrent admissions. The global prevalence of obesity is reaching pandemic proportions and so will its complications. It can be functionally debilitating and worsen obesity. Surgical resection is indicated to restore mobility and function, prevent recurrent infections, improve QOL, and reduce economic burden. Patients report high satisfaction rates following surgery. Panniculectomy is an effective treatment to alleviate morbidity in severe obesity and should be considered in patients with recurrent infections and a significant impact on QOL.

Reducing the burden: managing lymphoedema and its complications.

Lymphoedema effects approximately 200 000 people in UK. It is a chronic condition without a cure but there is much that can be done to ease and manage these complications. This article looks at the eight most common complications, providing advise on how to manage them, derived from the literature and the author's own experience. It also presents advise on how nurses can promote self-management of these complications, promoting patients' self-care.

BE-WEL trial (breast: evaluation of weight and exercise for lymphoedema) testing weight control and exercise programmes for women with breast cancer related lymphoedema: a feasibility trial.

PURPOSE: A combined body weight loss and upper body/arm exercise programme is a potential strategy for managing Breast cancer related lymphoedema (BCRL), but there is limited data on the best method for delivery or its potential efficacy. METHODS: Fifty-seven women with overweight/obesity and BCRL were randomised to a 12 week supervised (n = 12) or home-based combined weight loss and upper body/arm exercise programme (n = 16), a home-based upper-body arm exercise only programme (n = 17) or standard care (n = 12). Primary outcomes were uptake, retention and changes in weight and change in Relative Arm Volume Increase (RAVI) using analysis of covariance (ANCOVA). RESULTS: Sixteen percent of women invited joined the study and 49 completed the trial (85% retention). Reductions in weight occurred in the supervised and home-based weight control and exercise programmes; Mean (95% CI) change compared to standard care - 1.68 (- 4.36 to - 1.00), - 2.47(- 4.99 to - 0.04) Kg. Reductions in perometer assessed RAVI were seen in the supervised and home-based combined weight control and arm exercise groups and the weight stable home-based arm exercise only group: mean (95% CI) change compared to standard care - 2.4 (- 5.0 to + 0.4),- 1.8 (- 4.3 to + 0.7), - 2.5(- 4.9 to - 0.05)%. CONCLUSION: Women with BCRL and overweight and obesity engaged in diet and exercise weight loss programmes. Both weight loss/arm exercise programmes led to modest changes in weight and BCRL. Comparable reductions in BCRL were reported in the weight stable group undertaking arm exercise only. The independent and combined effects of weight loss and exercise on BCRL need further study. TRIAL REGISTRATION: ISRCTN86789850 https://doi.org/10.1186/ISRCTN86789850 , registered 2011.

FLT4 causes developmental disorders of the cardiovascular and lymphovascular systems via pleiotropic molecular mechanisms.

AIMS: Rare, deleterious genetic variants in FLT4 are associated with Tetralogy of Fallot (TOF), the most common cyanotic congenital heart disease. The distinct genetic variants in FLT4 are also an established cause of Milroy disease, the most prevalent form of primary hereditary lymphoedema. The phenotypic features of these two conditions are non-overlapping, implying pleiotropic cellular mechanisms during development. METHODS AND RESULTS: In this study, we show that FLT4 variants identified in patients with TOF, when expressed in primary human endothelial cells, cause aggregation of FLT4 protein in the perinuclear endoplasmic reticulum, activating proteostatic and metabolic signalling, whereas lymphoedema-associated FLT4 variants and wild-type (WT) FLT4 do not. FLT4 TOF variants display characteristic gene expression profiles in key developmental signalling pathways, revealing a role for FLT4 in cardiogenesis distinct from its role in lymphatic development. Inhibition of proteostatic signalling abrogates these effects, identifying potential avenues for therapeutic intervention. Depletion of flt4 in zebrafish caused cardiac phenotypes of reduced heart size and altered heart looping. These phenotypes were rescued with coinjection of WT human FLT4 mRNA, but incompletely or not at all by mRNA harbouring FLT4 TOF variants. CONCLUSION: Taken together, we identify a pathogenic mechanism for FLT4 variants predisposing to TOF that is distinct from the known dominant negative mechanism of Milroy-causative variants. FLT4 variants give rise to conditions of the two circulatory subdivisions of the vascular system via distinct developmental pleiotropic molecular mechanisms.

Limb Volume Measurements: A Comparison of Circumferential Techniques and Optoelectronic Systems against Water Displacement.

BACKGROUND: Accurate measurements of limb volumes are important for clinical reasons. We aimed to assess the reliability and validity of two centimetric and two optoelectronic techniques for limb volume measurements against water volumetry, defined as the gold standard. METHODS: Five different measurement methods were executed on the same day for each participant, namely water displacement, fixed-height (circumferences measured every 5 (10) cm for the upper (lower limb) centimetric technique, segmental centimetric technique (circumferences measured according to proportional height), optoelectronic plethysmography (OEP, based on a motion analysis system), and IGOODI Gate body scanner technology (which creates an accurate 3D avatar). RESULTS: A population of 22 (15 lower limbs, 11 upper limbs, 8 unilateral upper limb lymphoedema, and 6 unilateral lower limb lymphoedema) participants was selected. Compared to water displacement, the fixed-height centimetric method, the segmental centimetric method, the OEP, and the IGOODI technique resulted in mean errors of 1.2, 0.86, -16.0, and 0.71%, respectively. The corresponding slopes (and regression coefficients) of the linear regression lines were 1.0002 (0.98), 1.0047 (0.99), 0.874 (0.94) and 0.9966 (0.99). CONCLUSION: The centimetric methods and the IGOODI system are accurate in measuring limb volume with an error of <2%. It is important to evaluate new objective and reliable techniques to improve diagnostic and follow-up possibilities.

The Role of Inflammation in Lymphedema: A Narrative Review of Pathogenesis and Opportunities for Therapeutic Intervention.

Lymphedema is a chronic and progressive disease of the lymphatic system characterized by inflammation, increased adipose deposition, and tissue fibrosis. Despite early hypotheses identifying lymphedema as a disease of mechanical lymphatic disruption alone, the progressive inflammatory nature underlying this condition is now well-established. In this review, we provide an overview of the various inflammatory mechanisms that characterize lymphedema development and progression. These mechanisms contribute to the acute and chronic phases of lymphedema, which manifest clinically as inflammation, fibrosis, and adiposity. Furthermore, we highlight the interplay between current therapeutic modalities and the underlying inflammatory microenvironment, as well as opportunities for future therapeutic development.

Management of Lymphoedema and Lymphorrhoea with Wrap around Compression in Breast Secondary to Carcinoma Breast: A Case Report.

Lymphoedema secondary to breast cancer is one of the most unfortunate outcomes, and lymphoedema secondary to breast cancer in the ipsilateral upper extremity and its management, has been described in the literature. We are reporting a case of lymphoedema and lymphorrhoea in the breast itself, managed by compression bandaging with limited resources and the use of active pharmacological agents in the palliative care unit of a tertiary care hospital. Lymphoedema and lymphorrhoea in the breast are not very common to see in clinics, but management of this case even though no protocol available, was done by modification of bandaging techniques available for limb lymphoedema management.

Bilateral axillary web syndrome in a patient with primary lymphoedema of upper limbs and non-Hodgkin lymphoma.

Primary lymphoedema, axillary web syndrome (AWS) and yellow nail syndrome may be related. Mr B is a 66-year-old gentleman with genital lymphoedema and lymphoedema of all four extremities. In 2023, he was diagnosed with non-Hodgkin lymphoma and also underwent cardiac surgery. In November 2023, he completed an inpatient rehabilitation at the Földi clinic in Germany, where he received intensive treatment for his lymphoedema and was also diagnosed with bilateral AWS. The presence of AWS in a patient with primary lymphoedema and no history of axillary surgery is unique. Although AWS typically presents after axillary surgery, this case highlights that it can also occur in patients without lymph node surgery. While the precise cause of this presentation of AWS is not known, it may be connected to yellow nail syndrome or potentially the recent chemotherapy treatment. This article will describe the clinical case, highlighting the need for further research on AWS present in primary lymphoedema.