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Background: Lymphedema results from inadequate lymphatic function due to failure of lymphatic development or injury to a functioning lymphatic system. Patients suffer enlargement of the affected area, psychosocial morbidity, infection, and functional disability. The purpose of this study was to characterize the disease in a cohort of patients referred to a specialized center. Methods and Results: Our Lymphedema Program database was reviewed for all referrals between 2009 and 2019. Diagnosis was determined based on history, physical examination, and lymphoscintigraphy. Lymphedema type (primary, secondary, and obesity-induced), location of swelling, morbidity, previous management, accuracy of referral diagnosis, the geographic origin of the patients, and treatment in our center were analyzed. Seven hundred patients were referred with a diagnosis of "lymphedema"; 71% were female and 38% were children. Lymphedema was confirmed in 71% of the cohort: primary (62%), secondary (22%), and obesity-induced (16%). Twenty-nine percent of individuals labeled with "lymphedema" had another condition. One-half of patients had not received treatment, and 36% resided outside of our local referral area. One-third of subjects with lymphedema had an infection and 30% had >1 visit to the center. Patients with confirmed lymphedema were managed with compression stockings (100%), pneumatic compression (69%), and/or an excisional procedure (6%). Conclusions: Patients with lymphedema typically are adequately managed with conservative compression therapies and rarely require excisional operations. Diagnostic confusion is common and individuals with possible lymphedema are best managed by physicians focused on the disease.

Lipedema is a chronic and progressive disease characterized by a symmetrical and bilateral swelling of the lower extremities. In general, the feet are not involved. Lipedema is believed to affect nearly 1 in 9 adult women worldwide. Despite this relatively common disease, lipedema is often confused with primary lymphedema or obesity. In clinically advanced lipedema stages, fat continues to build up and may block the lymphatic vessels causing a secondary lymphedema (Lipo-Lymphedema). We consecutively evaluated 54 women with a clinical diagnosis of lower limbs lipedema. Two doses of 99mTc-nanocolloid were injected intradermally at the first intermetatarsal space and in the lateral malleolar area. Two static planar scans were taken at rest immediately following the intradermal injection. Subsequently, all patients were asked to perform an isotonic muscular exercise (stepping) for 2 min. Then, post exercise scans were performed to monitor the tracer pathway. Subsequently, the patient was asked to take a 30-40 min walk (prolonged exercise) and delayed scans were acquired. In early clinical stages, the lymphatic flow is usually preserved and the rest/stress intradermal lymphoscintigraphy may visualize a normal lymphatic drainage with a frequent pattern (tortuous course) of the leg lymphatic pathway. In clinically advanced stages, lymph stagnation areas were observed. Unlike obesity, lipedema fat storage is resistant to dietary regimen, bariatric surgery, and physical activity. Surgical treatment (tumescent liposuction and reductive surgery) is the most effective treatment to remove adipose tissue. Complex decongestive therapies are helpful in reducing the lymph stagnation, especially in patients with advanced lipolymphedema.

Lipedema (LI) is a common yet misdiagnosed condition, often misconstrued with obesity. LI affects women almost exclusively, and its painful and life-changing symptoms have long been thought to be resistant to the lifestyle interventions such as diet and exercise. In this paper, we discuss possible mechanisms by which patients adopting a ketogenic diet (KD) can alleviate many of the unwanted clinical features of LI. This paper is also an effort to provide evidence for the hypothesis of the potency of this dietary intervention for addressing the symptoms of LI. Specifically, we examine the scientific evidence of effectiveness of adopting a KD by patients to alleviate clinical features associated with LI, including excessive and disproportionate lower body adipose tissue (AT) deposition, pain, and reduction in quality of life (QoL). We also explore several clinical features of LI currently under debate, including the potential existence and nature of edema, metabolic and hormonal dysfunction, inflammation, and fibrosis. The effectiveness of a KD on addressing clinical features of LI has been demonstrated in human studies, and shows promise as an intervention for LI. We hope this paper leads to an improved understanding of optimal nutritional management for patients with LI and stimulates future research in this area of study.

Multiple associated comorbidities have been described for lipedema patients. Disease diagnosis still remains challenging in many cases and is frequently delayed. The purpose of this study was to determine the most common comorbidities in lipedema patients and the impact of surgical treatment onto disease progression. A retrospective assessment of disease-related epidemiologic data was performed for patients who underwent liposuction between July 2009 and July 2019 in a specialized clinic for lipedema surgery. All patients received a standardized questionnaire regarding the clinical history and changes of lipedema-associated symptoms and comorbidities after surgery. 106 patients who underwent a total of 298 liposuction procedures were included in this study after returning the questionnaire fully filled-in. Multiple comorbidities were observed in the assessed collective. The prevalence for obesity, hypothyroidism, migraine, and depression were markedly increased in relation to comparable nonlipedema populations. Despite a median body mass index (BMI) of 31.6 kg/m2 (IQR 26.4-38.8), unexpected low prevalence of diabetes (5%) and dyslipidemia (7%) was found. Diagnosis and initiation of guideline-appropriate treatment were delayed by years in many patients. After surgical treatment (medium follow-up 20 months, IQR 11-42), a significant reduction of lipedema-associated symptoms was demonstrated. Lipedema occurs with a diversity of associated comorbidities. Therefore, on the basis of available data, the authors suggest the necessity of a multimodal therapy concept for a comprehensive and holistic treatment. Despite a commonly increased BMI, lipedema patients appear to have an advantageous metabolic risk profile.

<b><i>Introduction:</i></b> Lipoedema is characterized as subcutaneous lipohypertrophy in association with soft-tissue pain affecting female patients. Recently, the disease has undergone a paradigm shift departing from historic reiterations of defining lipoedema in terms of classic edema paired with the notion of weight loss-resistant leg volume towards an evidence-based, patient-centered approach. Although lipoedema is strongly associated with obesity, the effect of bariatric surgery on thigh volume and weight loss has not been explored. <b><i>Material and Methods:</i></b> In a retrospective cohort study, thigh volume and weight loss of 31 patients with lipoedema were analyzed before and 10–18 and ≥19 months after sleeve gastrectomy (SG) or Roux-en-Y gastric bypass (RYGB). Fourteen patients, with distal leg lymphoedema (i.e., with healthy thighs), who had undergone bariatric surgery served as controls. Statistical analysis was performed using a linear mixed-effects model adjusted for patient age and initial BMI. <b><i>Results:</i></b> Adjusted initial thigh volume in patients with lipoedema was 23,785.4 mL (95% confidence interval [CI] 22,316.6–25,254.1). Thigh volumes decreased significantly in lipoedema and control patients (baseline vs. 1st follow-up, <i>p</i> &#x3c; 0.0001 and <i>p</i> = 0.0001; baseline vs. 2nd follow-up, <i>p</i> &#x3c; 0.0001 and <i>p</i> = 0.0013). Adjusted thigh volume reduction amounted to 33.4 and 37.0% in the lipoedema and control groups at the 1st follow-up, and 30.4 and 34.7% at the 2nd follow-up, respectively (lipoedema vs. control <i>p</i> &#x3e; 0.999 for both). SG and RYGB led to an equal reduction in leg volume (operation type × time, <i>p</i> = 0.83). Volume reduction was equally effective in obese and superobese patients (weight category × time, <i>p</i> = 0.43). <b><i>Conclusion:</i></b> SG and RYGB lead to a significant thigh volume reduction in patients with lipoedema.

Background: Fluid in lymphedema tissue appears histologically as spaces around vessels and between dermal skin fibers. Lipedema is a painful disease of excess loose connective tissue (fat) in limbs, almost exclusively of women, that worsens by stage, increasing lymphedema risk. Many women with lipedema have hypermobile joints suggesting a connective tissue disorder that may affect vessel structure and compliance of tissue resulting in excess fluid entering the interstitial space. It is unclear if excess fluid is present in lipedema tissue. The purpose of this study is to determine if fluid accumulates around vessels and between skin fibers in the thigh tissue of women with lipedema. Methods: Skin biopsies from the thigh and abdomen from 30 controls and 80 women with lipedema were evaluated for dermal spaces and abnormal vessel phenotype (AVP): (1) rounded endothelial cells; (2) perivascular spaces; and (3) perivascular immune cell infiltrate. Women matched for body mass index (BMI) and age were considered controls if they did not have lipedema on clinical examination. Data were analyzed by analysis of variance (ANOVA) or unpaired t-tests using GraphPad Prism Software 7. p < 0.05 was considered significant. Results: Lipedema tissue mass increases beginning with Stage 1 up to Stage 3, with lipedema fat accumulating more on the limbs than the abdomen. AVP was higher in lipedema thigh (p = 0.003) but not abdomen skin compared with controls. AVP was higher in thigh skin of women with Stage 1 (p = 0.001) and Stage 2 (p = 0.03) but not Stage 3 lipedema versus controls. AVP also was greater in the thigh skin of women with lipedema without obesity versus lipedema with obesity (p < 0.0001). Dermal space was increased in lipedema thigh (p = 0.0003) but not abdomen versus controls. Dermal spaces were also increased in women with lipedema Stage 3 (p < 0.0001) and Stage 2 (p = 0.0007) compared with controls. Conclusion: Excess interstitial fluid in lipedema tissue may originate from dysfunctional blood vessels (microangiopathy). Increased compliance of connective tissue in higher stages of lipedema may allow fluid to disperse into the interstitial space, including between skin dermal fibers. Lipedema may be an early form of lymphedema. ClinicalTrials.gov: NCT02838277.

The article discusses the launching by MA Healthcare of the European Lipoedema Association as of October 2020 to improve the diagnosis and management of lipoedema by creating standard and evidence-based guidelines. The association was launched with the help of leading lymphology specialist Tobias Bertsch. Also cited is how lipoedema affects the adipose tissue.

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Compression hosiery is commonly used for the management of lymphoedema as well as lipoedema, but it is more commonly indicated for the lower limbs than for the upper limbs. The effects of compression hosiery on upper-limb lipoedema are poorly understood and researched. It is known that compression hosiery works in conjunction with activity or movement when standing or walking, which produces anti-inflammatory and oxygenating effects in the tissues. This effect is naturally difficult to realise in the upper limbs. Lymphoedema practitioners who treat those with lipoedema should bear in mind that compression treatment might not produce the same effects in upper-limb lipoedema as it does in lower-limb lipoedema. In these times of an overstretched health service, pragmatic resource use is essential.

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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.