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Lymphedema is the chronic, progressive swelling of tissue due to inadequate lymphatic function. Over time, protein-rich fluid accumulates in the tissue causing it to enlarge. Lymphedema is a specific disease and should not be used as a generic term for an enlarged extremity. The diagnosis is made by history and physical examination, and confirmed with lymphoscintigraphy. Intervention includes patient education, compression, and rarely, surgery. Patients are advised to exercise, maintain a normal body mass index, and moisturize / protect the diseased limb from incidental trauma. Conservative management consists of compression regimens. Operative interventions either attempt to address the underlying lymphatic anomaly or the excess tissue. Lymphatic-venous anastomosis and lymph node transfer attempt to create new lymphatic connections to improve lymph flow. Suction-assisted lipectomy and cutaneous excision reduce the size of the area by removing fibroadipose hypertrophy.

PURPOSE: Lipedema is a chronic, common but underdiagnosed disease masquerading obesity, with female predominance, characterized by disproportional abnormal adipose tissue distribution of the lower and also upper extremities. The present study was designed to determine whether lipedema is associated with three-dimensional (3D) speckle-tracking echocardiography (3DSTE)-derived left ventricular (LV) deformation abnormalities, and to assess the effects of 1-hour use of medical compression stockings (MCS). METHODS: The present study comprised 19 female patients with lipedema (mean age: 42.2 ± 12.4 years), compared to 28 age-matched healthy female controls (mean age: 42.0 ± 9.8 years). RESULTS: Lipedema patients showed larger left atrial and LV dimensions and greater LV ejection fraction than controls, without significant difference in other echocardiography variables. Lipedema patients had greater 3DSTE-derived global and mean segmental LV circumferential and area strains than controls. Following 1-hour use of wearing MCS, neither global and nor mean segmental LV strains showed significant impairment or improvement. CONCLUSIONS: Increased LV strains could be compensatory effects maintaining LV pumping function in lipedema. Short-term wearing of MCS has no global effect on LV strains.

Lipedema is a lymphedema-masquerading symmetrical, bilateral and disproportional obesity. Its conservative maintenance treatment comprises the use of flat-knitted compression pantyhoses. Lipedema is known to be associated with left ventricular morphological and functional alterations. The present study aimed to assess the effects of graduated compression stockings on left ventricular (LV) rotational mechanics measured by three-dimensional speckle-tracking echocardiography (3DSTE) in lipedema patients. The present study comprised twenty lipedema patients (mean age: 45.8 ± 11.0 years, all females) undergoing 3DSTE who were also compared to 51 age- and gender-matched healthy controls (mean age: 39.8 ± 14.1 years, all females). 3DSTE analysis was performed at rest, and subsequent to 1 hour application of compression class 2 made-to-measure flat-knitted pantyhose. Six lipedema patients showed significant LV rotational abnormalities. Of the remaining fourteen lipedema patients LV basal rotation rotation showed significant reduction, while LV apical rotation showed significant increase with unchanged LV twist after a 60-minute use of compression garment. Significant changes in LV rotational mechanics could be detected among 14 women with lipedema after the use of compression garment however six probands have special LV rotational abnormalities at baseline and/or after compression.

Lipoedema is a chronic progressive disorder of adipose tissue leading to an enlargement of lower extremities. It is considered to be rare; however, the prevalence of the disease is underestimated because it is commonly misdiagnosed as obesity or lymphedema and the general awareness is poor. The etiology of the disorder is considered to be multifarious, including genetic inheritance, hormonal imbalance and microcirculation alterations. Diagnosis is mainly based on medical history and physical examination. Management of lipoedema is focused on reducing the symptoms, improving the quality of life and preventing further progression of the disease. The aim of this paper is to raise the awareness of the disease and provide appropriate clinical guidance for the assessment of lipoedema. We searched through the PubMed/MEDLINE database and took into consideration all of the results available as of 6September, 2020 and outlined the current evidence regarding lipoedema epidemiology, etiology, clinical presentation, differential diagnosis, and management. Better understanding of lipoedema is crucial for establishing an early diagnosis and a proper treatment, which in turn will reduce the psychological and physical implications associated with the disease.

Background/objectives Patients with obesity and lipedema commonly are misdiagnosed as having lymphedema. The conditions share phenotypic overlap and can influence each other. The purpose of this study was to delineate obesity-induced lymphedema, obesity without lymphedema, and lipedema in order to improve their diagnosis and treatment. Subjects/methods Our Lymphedema Center database of 700 patients was searched for patients with obesity-induced lymphedema (OIL), obesity without lymphedema (OWL), and lipedema. Patient age, sex, diagnosis, cellulitis history, body mass index (BMI), and treatment were recorded. Only subjects with lymphoscintigraphic documentation of their lymphatic function were included. Results Ninety-eight patients met inclusion criteria. Subjects with abnormal lymphatic function (n = 46) had a greater BMI (65 ± 12) and cellulitis history (n = 30, 65%) compared to individuals with normal lymphatic function [(BMI 42 ± 10); (cellulitis n = 8, 15%)] (p < 0.001). Seventeen patients had a history of lipedema and two exhibited abnormal lymphatic function (BMI 45, 54). The risk of having lower extremity lymphedema was predicted by BMI: BMI < 40 (0%), 40–49 (17%), 50–59 (63%), 60–69 (86%), 70–79 (91%), ≥80 (100%). Five patients with OIL (11%) underwent resection of massive localized lymphedema (MLL) or suction-assisted lipectomy. Three individuals (18%) with lipedema were treated with suction-assisted lipectomy. Conclusions The risk of lymphedema in patients with obesity and lipedema can be predicted by BMI; confirmation requires lymphoscintigraphy. Individuals with OIL are at risk for cellulitis and MLL. Patients with a BMI > 40 are first managed with weight loss. Excisional procedures can further reduce extremity size once BMI has been lowered.

ICD-10 Coordination and Maintenance Committee Meeting September 8-9, 2020 Lipedema and Lipolymphedema Lipedema, initially described at the Mayo clinic in 1940, is a loose, connective-tissue (fat) disease (lipomatosis) with a pathological deposition of fibrotic fatty tissue on the limbs of women sparing the trunk, hands and feet, resulting in a disproportionate body habitus. There is no specific ICD-10-CM code for lipedema. Deposition of lipedema fat increases with stage and body mass index (BMI) and likely involves sex hormones during times when weight is gained (puberty, pregnancy and menopause). Lipedema is inherited in 60% of women likely through genes affecting microvessels resulting in excess fluid bound to glycosaminoglycans in the interstitial space. Unique to lipedema is fat that is highly resistant to loss by diet, exercise, or bariatric surgery. Lipedema is often confused with secondary obesity or lymphedema. Women with lipedema and/or obesity can develop lymphedema called lipolymphedema, for which there is no ICD-10-CM code. There is no cure for lipedema, but treatments aimed at reducing the lymphedema component of lipedema such as manual decongestive therapy, wrapping, exercise, compression garments and pumps, and some medical foods and medications are helpful. Expertly performed suction assisted lipectomy is the treatment of choice for suitable lipedema patients with an inadequate response to conservative and supportive measures. Lipedema is thought to affect 11% of the female population. Lymphedema is a chronic and progressive swelling caused by a low output failure of the lymphatic system, resulting in the development of a high-protein edema in the tissues. Lymphedema is a lifelong condition for which no cure exists. Lymphedema can be either primary (hereditary) or secondary. Secondary lymphedema is the most common cause of the disease and affects approximately 1 in 1000 Americans. Complications of lymphedema include recurrent bouts of cellulitis and/or lymphangitis, bacterial and fungal infections, lymphangio-adenitis, deep venous thrombosis, poor wound healing, leg ulcers, severe functional impairment, disability, and necessary amputation. Patients with chronic lymphedema for 10 years have a 10% risk of developing lymphangiosarcoma. Praecox lymphedema is currently captured in ICD-10-CM as a secondary lymphedema; it is more accurately classified under code Q82.0: Hereditary lymphedema. With support from the American Vein & Lymphatic Society (AVLS), the requestor is submitting the following modifications to identify and track lipedema and lipolymphedema patients.

AIM: To assess imaging findings and characteristics of the lymphatic system in patients affected by lipedema and lipolymphedema of the lower extremities on Non-Contrast MR Lymphography (NCMRL). MATERIALS AND METHODS: 44 lower extremities in 11 consecutive female patients affected by lipedema, and 11 patients with lipolymphedema were examined by NCMRL. MR imaging was performed on 1.5-T system MR equipment. The examination consisted of one 3D short-tau inversion recovery (STIR) and one heavily T2-weighted 3D-Turbo Spin Echo (TSE) sequence. RESULTS: All patients showed symmetrical enlargement of the lower extremities with increased subcutaneous fat tissue. The fat tissue was homogeneous, without any signs of edema in pure lipedema patients. In all the extremities with lipolymphedema, high signal intensity areas in the epifascial region could be detected on the 3D-TSE sequence (p < .001) with evidence of mild epifascial fluid collections (p < .001). No sign of honeycomb pattern fat appearance was observed. The appearance of the iliac lymphatic trunks was normal in both lipedema and lipolymphedema patients. Dilated peripheral lymphatics were observed in 2 patients affected by lipedema, indicating a subclinical status of lymphedema, and in 10 patients with lipolymphedema (p = .001). Signs of vascular stasis were observed in both groups, without statistically significant difference (p = .665). CONCLUSION: NCMRL is a non-invasive imaging technique that is suitable for the evaluation of patients affected by lipedema and lipolymphedema, helping in the differential diagnosis.

BACKGROUND: Lower extremity lymphedema is frequently encountered in the vascular clinic. Established dogma purports that cancer is the most common cause of lower extremity lymphedema in Western countries, whereas chronic venous insufficiency (CVI) is often overlooked as a potential cause. Moreover, lymphedema is typically ascribed to a single cause, yet multiple causes can coexist. METHODS: A 3-year retrospective analysis was conducted of demographic and clinical characteristics of 440 eligible patients with lower extremity lymphedema who presented for lymphatic physiotherapy to a university medical center's cancer-based physical therapy department. RESULTS: The four most common causes of lower extremity lymphedema were CVI (phlebolymphedema; 41.8%), cancer-related lymphedema (33.9%), primary lymphedema (12.5%), and lipedema with secondary lymphedema (11.8%). The collective cohort was more likely to be female (71.1%; P < .0001), to be white (78.9%; P < .0001), to demonstrate bilateral distribution (74.5%; P < .0001), and to have involvement of the left leg (bilateral, 69.1% [P < .0001]; unilateral, 58.9% [P = .0588]). Morbid obesity was pervasive (mean weight and body mass index, 115.8 kg and 40.2 kg/m(2), respectively) and significantly correlated with a higher International Society of Lymphology lymphedema stage (stage III mean weight and body mass index, 169.2 kg and 57.3 kg/m(2), respectively, vs stage II, 107.8 kg and 37.5 kg/m(2), respectively; P < .0001). Approximately one in three (35.7%) of the population sustained one or more episodes of cellulitis, but patients with stage III lymphedema had roughly twice the rate of soft tissue infection as patients with stage II, 61.7% vs 31.8%, respectively (P < .001). Multifactorial lymphedema was present in 25%. Approximately half of the patients with lipedema with secondary lymphedema (48.1%) or primary lymphedema (45.5%) had a superimposed cause of swelling that was usually CVI. Total knee arthroplasty was the most common cause of noncancer surgery-mediated worsening of pre-existing lymphedema. CONCLUSIONS: In a large cohort of patients treated in a cancer-affiliated physical therapy department, CVI (phlebolymphedema), not cancer, was the predominant cause of lower extremity lymphedema. One in four patients had more than one cause of lymphedema. Notable clinical characteristics included a proclivity for female patients, bilateral distribution, left limb, cellulitis, and nearly universal morbid obesity.

BACKGROUND: Lipedema is a common adipose tissue disorder affecting women, characterized by a symmetric subcutaneous adipose tissue deposition, particularly of the lower extremities. Lipedema is usually underdiagnosed, thus remaining an undertreated disease. Importantly, no histopathologic or molecular hallmarks exist to clearly diagnose the disease, which is often misinterpreted as obesity or lymphedema. MATERIALS AND METHODS: The aim of the present study is to characterize in detail morphologic and molecular alterations in the adipose tissue composition of lipedema patients compared with healthy controls. Detailed histopathologic and molecular characterization was performed using lipid and cytokine quantification as well as gene expression arrays. The analysis was conducted on anatomically matched skin and fat tissue biopsies as well as fasting serum probes obtained from 10 lipedema and 11 gender and body mass index-matched control patients. RESULTS: Histologic evaluation of the adipose tissue showed increased intercellular fibrosis and adipocyte hypertrophy. Serum analysis showed an aberrant lipid metabolism without changes in the circulating adipokines. In an adipogenesis gene array, a distinct gene expression profile associated with macrophages was observed. Histologic assessment of the immune cell infiltrate confirmed the increased presence of macrophages, without changes in the T-cell compartment. CONCLUSIONS: Lipedema presents a distinguishable disease with typical tissue architecture and aberrant lipid metabolism, different to obesity or lymphedema. The differentially expressed genes and immune cell infiltration profile in lipedema patients further support these findings.

BACKGROUND: Lipedema is a condition of painful increase in subcutaneous fat affecting almost exclusively women. Several studies have examined the effectiveness of liposuction in the treatment of lipedema, but none has focused on water-jet-assisted liposuction technique. METHODS: A standardized treatment protocol for liposuction in lipedema, which was established over the course of 15 years, is presented. Patients received questionnaires preoperatively and after operative treatment assessing characteristics and symptom severity on visual analog scales in a prospective manner. RESULTS: Pre- and postoperative questionnaires were available for 63 patients. Median age was 35 years and mean (body mass index) BMI 28.4 ± 0.6, all patients had stages I or II lipedema diagnosed by two separate specialists. After a mean follow-up of 22 months after operative treatment, all assessed symptom had decreased significantly in severity. All patients wore compression garments and/or received manual lymphatic drainage preoperatively; this could be reduced to only 44% of patients needing any conservative treatment postoperatively. CONCLUSION: Liposuction in water-jet-assisted technique using the presented treatment protocol is an efficient method of operative treatment of early-stage lipedema patients leading to a marked decrease in symptom severity and need for conservative treatment.

Lipedema is an often underdiagnosed chronic disorder that affects subcutaneous adipose tissue almost exclusively in women, which leads to disproportionate fat accumulation in the lower and upper body extremities. Common comorbidities include anxiety, depression, and pain. The correlation between mood disorder and subcutaneous fat deposition suggests the involvement of steroids metabolism and neurohormones signaling, however no clear association has been established so far. In this study, we report on a family with three patients affected by sex-limited autosomal dominant nonsyndromic lipedema. They had been screened by whole exome sequencing (WES) which led to the discovery of a missense variant p.(Leu213Gln) in AKR1C1, the gene encoding for an aldo-keto reductase catalyzing the reduction of progesterone to its inactive form, 20-α-hydroxyprogesterone. Comparative molecular dynamics simulations of the wild-type vs. variant enzyme, corroborated by a thorough structural and functional bioinformatic analysis, suggest a partial loss-of-function of the variant. This would result in a slower and less efficient reduction of progesterone to hydroxyprogesterone and an increased subcutaneous fat deposition in variant carriers. Overall, our results suggest that AKR1C1 is the first candidate gene associated with nonsyndromic lipedema.

Lipoedema is associated with widespread adipose tissue expansion, particularly in the proximal extremities. The mechanisms that drive the development of lipoedema are unclear. In this Perspective article, we propose a new model for the pathophysiology of lipoedema. We suggest that lipoedema is an oestrogen-dependent disorder of adipose tissue, which is triggered by a dysfunction of caveolin 1 (CAV1) and subsequent uncoupling of feedback mechanisms between CAV1, the matrix metalloproteinase MMP14 and oestrogen receptors. In addition, reduced CAV1 activity also leads to the activation of ERα and impaired regulation of the lymphatic system through the transcription factor prospero homeobox 1 (PROX1). The resulting upregulation of these factors could effectively explain the main known features of lipoedema, such as adipose hypertrophy, dysfunction of blood and lymphatic vessels, the overall oestrogen dependence and the associated sexual dimorphism, and the mechanical compliance of adipose tissue.

Liposuction is one of the most common procedures undertaken in plastic surgery with a steadily increasing trend over the years. Although usually performed as an aesthetic procedure for body contouring, it can also be utilized in specific patient groups for disease symptom reduction. One such disease entity is lipedema. The goal of this video to present the authors' technique in the surgical treatment of lipedema, and to offer the viewer a better understanding of the differences between an aesthetic liposuction and a functional liposuction as performed on a lipedema patient. Between July 2009 and July 2019, 106 lipedema patients have been treated in the authors' specialized lipedema clinic, with a total of 298 liposuction procedures and a median follow-up of 20 months. The mean amount of lipoaspirate was 6354.73 ml (± 2796.72 ml). The patients reported a significant reduction in lipedema-associated complaints and improvement in quality of life. The need for conservative therapy was significantly reduced. No serious complications were reported. The authors also present before and after photographs of three patients. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

The COVID-19 pandemic poses a challenge to the management of non-COVID pathologies such as lymphatic diseases and lipoedema. The use of telemedicine can prevent the spread of the disease. A system is needed to help determine the clinical priority and selection of face-to-face or telemedicine options for each patient and how to carry them out during the pandemic. The Spanish Lymphology Group has drafted a consensus document with recommendations based on the literature and clinical experience, as clinical practice guidelines for the management of lymphatic abnormalities and lipoedema during the COVID-19 pandemic. These recommendations must be adapted to the characteristics of each patient, the local conditions of the centres, and the decisions of health care professionals. The document contains minimum criteria, subject to modifications according to the evolution of the pandemic, scientific knowledge and instructions from health authorities.

BACKGROUND: The aim was to quantify and to compare the associations between longitudinal changes in pain and depression in different chronic pain conditions. METHODS: Data were retrieved from 6 observational cohort studies. From baseline to the 6-month follow-up, the score changes on the Short Form (36) Health Survey (SF-36) bodily pain (pain) and the SF-36 mental health (depression) scales (0=worst, 100=best) were quantified, using partial correlations obtained by multiple regression. Adjustment was performed by age, living alone/with partner, education level, number of comorbidities, baseline pain and baseline depression. RESULTS: Stronger associations were found between changes in levels of pain and depression for neck pain after whiplash (n = 103, mean baseline pain=21.4, mean baseline depression=52.5, adjusted correlation r = 0.515), knee osteoarthritis (n = 177, 25.4, 64.2, r = 0.502), low back pain (n = 134, 19.0, 49.4, r = 0.495), and fibromyalgia (n = 125, 16.8, 43.2, r = 0.467) than for lower limb lipedema (n = 68, 40.2, 62.6, r = 0.452) and shoulder arthroplasty (n = 153, 35.0, 76.4, r = 0.292). Those correlations were somewhat correlated to baseline pain (rank r=-0.429) and baseline depression (rank r=-0.314). LIMITATIONS: The construct of the full range of depressive symptoms is not explicitly covered by the SF-36. CONCLUSIONS: Moderate associations between changes in pain and depression levels were demonstrated across 5 of 6 different chronic pain conditions. The worse the pain and depression scores at baseline, the stronger those associations tended to be. Both findings indicate a certain dose-response relationship - an important characteristic of causal interference. Relieving pain by treatment may lead to the relief of depression and vice versa.

PURPOSE: The aim of this narrative review of the literature was to evaluate and summarize the current literature regarding the effect of lipedema on health-related quality of life (HRQOL) and psychological status. METHODS: The authors collected articles through a search into Medline, Embase, Scopus, Web of Science, Physiotherapy Evidence Database (PEDro), and the Cochrane Review. Search terms used included "Lipoedema," "Lipedema," "psychological status," "Quality of life," "Health related quality of life," and "HRQOL." RESULTS: A total of four observational studies were evaluated. The included studies were moderate-quality according to the Newcastle-Ottawa Scale. Three of the included studies demonstrated deterioration of HRQOL and psychological status in patients with lipedema. These studies also identify that pain and tenderness are a more common and dominant characteristic. CONCLUSION: Future studies should establish a specific approach to treat and manage lipedema symptoms. Based on this narrative review of the literature findings, we recommended for the health care provider to pay more attention to HRQOL and psychological status. Moreover, validated and adapted measures of HRQOL and psychological status for patients with lipedema are required. LEVEL OF EVIDENCE: Level V, narrative review.

PURPOSE: Breast cancer treatment-related lymphedema (BCRL) evaluation is frequently performed using portable measures of limb volume and bioimpedance asymmetry. Here quantitative magnetic resonance imaging (MRI) is applied to evaluate deep and superficial tissue impairment, in both surgical and contralateral quadrants, to test the hypothesis that BCRL impairment is frequently bilateral and extends beyond regions commonly evaluated with portable external devices. METHODS: 3-T MRI was applied to investigate BCRL topographical impairment. Female BCRL (n = 33; age = 54.1 ± 11.2 years; stage = 1.5 ± 0.8) and healthy (n = 33; age = 49.4 ± 11.0 years) participants underwent quantitative upper limb MRI relaxometry (T2), bioimpedance asymmetry, arm volume asymmetry, and physical evaluation. Parametric tests were applied to evaluate study measurements (i) between BCRL and healthy participants, (ii) between surgical and contralateral limbs, and (iii) in relation to clinical indicators of disease severity. Two-sided p-value < 0.05 was required for significance. RESULTS: Bioimpedance asymmetry was significantly correlated with MRI-measured water relaxation (T2) in superficial tissue. Deep muscle (T2 = 37.6 ± 3.5 ms) and superficial tissue (T2 = 49.8 ± 13.2 ms) relaxation times were symmetric in healthy participants. In the surgical limbs of BCRL participants, deep muscle (T2 = 40.5 ± 4.9 ms) and superficial tissue (T2 = 56.0 ± 14.8 ms) relaxation times were elevated compared to healthy participants, consistent with an edematous micro-environment. This elevation was also observed in contralateral limbs of BCRL participants (deep muscle T2 = 40.3 ± 5.7 ms; superficial T2 = 56.6 ± 13.8 ms). CONCLUSIONS: Regional MRI measures substantiate a growing literature speculating that superficial and deep tissue, in surgical and contralateral quadrants, is affected in BCRL. The implications of these findings in the context of titrating treatment regimens and understanding malignancy recurrence are discussed.

BACKGROUND: Literature on the validity of outcome measurement in lymphedema and lipedema is very sparse. This study aimed to examine the convergent, divergent and discriminant validity of a set of 5 instruments in both conditions. METHODS: Cross-sectional outcome was measured by the generic Short Form 36 (SF-36), the lymphedema-specific Freiburg Quality of Life Assessment for lymphatic disorders, Short Version (FLQA-lk), the knee-specific Knee Outcome Survey Activities of Daily Living Scale (KOS-ADL), the Symptom Checklist-90-revised (SCL-90R), and the Six-Minute Walk Test (6 MWT). Construct convergent/divergent validity was quantified by bivariate correlations and multivariate factor analysis, and discriminant validity by standardized mean differences (SMDs). RESULTS: Health was consistently better in lymphedema (n = 107) than in lipedema (n = 96). The highest construct convergence was found for physical health between the SF-36 and KOS-ADL (bivariate correlations up to 0.78, factor loads up to 0.85, explained variance up to 56.8%). The second most important factor was mental health (bivariate correlations up to 0.79, factor loads up to 0.86, explained variance up to 13.3%). Discriminant validity was greatest for the FLQA-lk Physical complaints (adjusted SMD = 0.93) followed by the SF-36 Bodily pain (adjusted SMD = 0.83), KOS-ADL Function (adjusted SMD = 0.47) and SF-36 Vitality (adjusted SMD = 0.39). CONCLUSIONS: All five instruments have specific strengths and can be implemented according to the scope and aim of the outcome examination. A minimum measurement set should comprise: the SF-36 Bodily pain, SF-36 Vitality, FLQA-lk Physical complaints, FLQA-lk Social life, FLQA-lk Emotional well-being, FLQA-lk Health state, KOS-ADL Symptoms, KOS-ADL Function, and the SCL-90R Interpersonal sensitivity.