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OBJECTIVES: Patient-relevant treatment benefit is traditionally measured with health-related quality of life (HRQoL) instruments. The Patient Benefit Index (PBI) methodology allows for a more direct measurement, with the patients rating both importance and achievement of treatment goals. Here, we developed and validated a PBI version specific for the assessment of benefit in lymphedema and lipedema treatment (PBI-L). METHODS: The development included five steps: (1) open item collection; (2) consensus of items in a multidisciplinary expert panel; (3) application of the German PBI-L in a cross-sectional study (n = 301); (4) translation into English; (5) application of the English PBI-L in a randomized clinical trial (n = 82). Subscales were developed using factor analysis. Construct validity was analyzed by correlating PBI-L and convergent criteria such as HRQoL and quality of care. To test for responsiveness, the association to change in HRQoL measures was computed. RESULTS: Floor and ceiling effects were low. There were few missing values. Two well-interpretable subscales were found with Cronbach's alpha >0.8 each. Global and subscale scores correlated with convergent criteria and with change in disease-specific HRQoL, but not with change in generic HRQoL. CONCLUSIONS: The PBI-L is an internally consistent, valid, and responsive instrument for the assessment of patient-relevant benefit of edema treatment.

Cankles refer to the area where the calf and ankle meet. Unaesthetic fat cankles, where definition between the calf and ankle is impossible, are a frustrating aesthetic deformity, which are exacerbated by their genetic conditioning and special resistance to diet. This article reports our experience with laser-assisted lipolysis (LAL) in cankle remodelling. A total of 30 patients were treated for unaesthetic fat cankles with LAL. The 924/975-nm diode laser used in this study consisted of two lasers, one emitting at 924 nm, and the other at 975 nm. According to our mathematical models, we assumed that to destroy 1 ml of fat, 0.1 kJ was required in dual emission mode at 924/975 nm. Patients were asked to file a satisfaction questionnaire. Ultrasound was used to measure the fat thickness pre- and postoperatively. Oedema in both lateral sulcus of the Achilles tendon was seen in all patients. It subsided after 4 weeks in nine cases and 6 weeks in 21 cases. Only two patients developed mild hyperpigmentation that disappeared, respectively, after 4 and 10 weeks. Pain during the anaesthesia and discomfort after the procedure were low with this technique. Mean down time was 1.0 day. Of the 30 patients, 29 would recommend this treatment. Overall, high patient and investigator satisfaction was confirmed by the sonography used to measure decrease in fat thickness. LAL in cankle remodelling is a safe and reproducible technique that is particularly appreciated by the patient. The procedure allows homogenous reduction of fatty tissue together with skin tightening.

In 2014 250 women with Lipoedema took part in our survey about their experiences with Lipoedema. The results give a clear picture of what living with Lipoedema is like and reveal that along with the pain and discomfort caused by Lipoedema, many simple tasks that other people regard as straightforward become extremely challenging for people with Lipoedema. Lipoedema UK’s Big Survey 2014 led to the development of the Royal College of GPs’ e-learning course, a half hour course, which enables GPs to recognise Lipoedema and diagnose patients Our survey was created by Lipoedema UK, the Lymphoedema Department at St George’s Hospital, London, and the support of the Lymphoedema Support Network.

BACKGROUND: Lipedema is a rare female disorder with a characteristic distribution of adipose tissue hypertrophy on the extremities, with pain and bruising. In advanced stages, reduction of adipose tissue is the only available effective treatment. In elderly patients with advanced lipedema, correction of increased skin laxity has to be considered for an optimal outcome. METHODS: We report on a tailored combined approach to improve advanced lipedema in elderly females with multiple comorbidities. Microcannular laser-assisted liposuction of the upper legs and knees is performed under tumescent anesthesia. Medial thigh lift and partial lower abdominoplasty with minimal undermining are used to correct skin laxity and prevent intertrigo. Postsurgical care with nonelastic flat knitted compression garments and manual lymph drainage are used. RESULTS: We report on three women aged 55-77 years with advanced lipedema of the legs and multiple comorbidities. Using this step-by-step approach, a short operation time and early mobilization were possible. Minor adverse effects were temporary methemoglobinemia after tumescent anesthesia and postsurgical pain. No severe adverse effects were seen. Patient satisfaction was high. CONCLUSION: A tailored approach may be useful in advanced lipedema and is applicable even in elderly patients with multiple comorbidities.

BACKGROUND: Functional pelvic disorders in patients undergoing conservative surgical approach for rectal cancer are considered a major public health issue and represent one third of cost of colorectal cancer. We investigated the hypothesis that lymphadenectomy, involves the pelvic floor results in a localized hides or silent pelvic lymphedema characterized by symptoms without signs. PATIENTS AND METHODS: We examined 13 colo-rectal cancer patients: five intra-peritoneal adenocarcinoma: 1 sigmoid and 4 upper third rectal cancer (1 male and 3 female) and 9 extra-peritoneal adenocarcinoma: 3 middle and 5 lower third rectal cancer (4 male and 5 female) using 1.5-T magnetic resonance, one week before and twelve months after discharged from hospital. RESULTS: Lymphedema was discovered on post-operative magnetic resonance imaging of all 9 patients with extra-pertitoneal cancer, whereas preoperative magnetic resonance imaging as well as a post-operative examination of 4 intra-peritoneal adenocarcinoma, revealed no evidence of lymphedema. Unlike the common clinical skin signs that typify all other sites of lymphedema, pelvic lymphedema is hides or silent, with no skin changes or any single symptom manifested. Magnetic resonance imaging showed that pelvic illness alone is accompanied by lymphedema related exclusively to venous congestion, and accumulation of liquid in adipose tissue or lipedema. CONCLUSIONS: Alteration of the pelvic lymphatic network during pelvic surgery can lead to lymphedema and, pelvic floor disease. Patients should be routinely examined for the possibility of developing this post-surgical syndrome and further studies are needed to establish diagnosis and to evaluate treatment preferences.

BACKGROUND: The management of lymphoedema is complex and should be based on guidelines. To date, no data assessing quality of care in lymphoedema in Germany are available. OBJECTIVE: We aimed at evaluating the quality of care of lymphoedema in the metropolitan area of Hamburg using guideline-based indicators. METHODS: Cross-sectional, community-based study including patients with lymphoedema. Assessment included a structured interview, clinical examination and patient-reported outcomes. Quality indicators derived from guidelines by a Delphi consensus were applied. RESULTS: 348 patients (median age 60.5 years) with lymphoedema (66.4%), lipoedema (9.5%) or combined oedema (24.1%) were included. 86.4% performed compression therapy, 85.6% received lymphatic drainage. On average 55.0% of the quality of care criteria were met; 64.8% were satisfied with care. The distribution curve of the health care index was almost normal. Treatment by specialists led to a higher quality of care index. CONCLUSION: Although overall quality of care in lymphoedema is fair, many patients are not treated properly according to guidelines.

Background: Butcher’s broom plant extract has been reported to improve lymphatic flow and the trace mineral, selenium, has been shown to improve lymphedema. This retrospective case study examines the effectiveness of Butcher’s broom in conjunction with selenium to decrease limb volume of a patient with lipedema, a common fat distribution disorder with excess adipose tissue fluid. Methods: Selenium (400 mcg) was initiated 6 days prior to limb volume evaluation utilizing perometry. The patient underwent physical therapy that consisted of manual lymph drainage (MLD) with Histological Variable Manual Technique (HIVAMAT), and compression bandaging. Butcher’s broom (one gram daily) was added on day 95 of treatment in addition to selenium and both were continued through day 293 (end of study). Results: Total volume reduction over the study period for the left and right upper extremities and left and right lower extremities was 525 ml and 225 ml (p<0.05), and 1769 ml and 1614 ml (p<0.0001), respectively. The total percent volume reduction during the time period when MLD with HIVAMAT and compression bandaging were performed for the left and right legs was 70.6 and 79.0%, respectively. In the absence of compression bandaging, the left and right arms lost 21.2 and 10% of initial volumes, respectively at the 6 month follow-up visit. During the latter part of the study when the patient was performing a home maintenance program, at which time selenium and Butcher’s broom were continued, the left and right lower extremities decreased an additional 29.4 and 20.9% of initial volumes, respectively, despite a lack of exercise due to a foot injury during the last 46 days. Conclusion: Butcher’s broom and selenium may offer new tools in conjunction with physical therapy to improve swelling and pain associated with lipedema.

Background: Butcher’s broom plant extract has been reported to improve lymphatic flow and the trace mineral, selenium, has been shown to improve lymphedema. This retrospective case study examines the effectiveness of Butcher’s broom in conjunction with selenium to decrease limb volume of a patient with lipedema, a common fat distribution disorder with excess adipose tissue fluid. Methods: Selenium (400 mcg) was initiated 6 days prior to limb volume evaluation utilizing perometry. The patient underwent physical therapy that consisted of manual lymph drainage (MLD) with Histological Variable Manual Technique (HIVAMAT), and compression bandaging. Butcher’s broom (one gram daily) was added on day 95 of treatment in addition to selenium and both were continued through day 293 (end of study). Results: Total volume reduction over the study period for the left and right upper extremities and left and right lower extremities was 525 ml and 225 ml (p<0.05), and 1769 ml and 1614 ml (p<0.0001), respectively. The total percent volume reduction during the time period when MLD with HIVAMAT and compression bandaging were performed for the left and right legs was 70.6 and 79.0%, respectively. In the absence of compression bandaging, the left and right arms lost 21.2 and 10% of initial volumes, respectively at the 6 month follow-up visit. During the latter part of the study when the patient was performing a home maintenance program, at which time selenium and Butcher’s broom were continued, the left and right lower extremities decreased an additional 29.4 and 20.9% of initial volumes, respectively, despite a lack of exercise due to a foot injury during the last 46 days. Conclusion: Butcher’s broom and selenium may offer new tools in conjunction with physical therapy to improve swelling and pain associated with lipedema.

The pathophysiology of skin diseases associated with monoclonal gammopathies is generally unknown. Our aim was to investigate whether a monoclonal gammopathy could be a causal factor in progressive lymphedema. We describe a 75 year old patient with a rapidly progressive lipo-lymphedema and a monoclonal gammopathy of unknown significance (MGUS) suspected as a key etiological factor. Dermal fibroblasts were cultured from lesional lower leg skin and non-lesional abdominal skin and compared to healthy control fibroblasts. We found 10-fold elevated basic fibroblast growth factor 2 (FGF-2) in the patient's serum and significantly increased basal FGF-2 production of lesional and non-lesional fibroblasts compared to healthy controls. Upon restimulation with patient or healthy control serum, lesional fibroblasts showed significantly increased proliferation rates and FGF-2 production in vitro. Non-lesional abdominal fibroblasts showed an intermediate phenotype between lesional and control fibroblasts. Our findings provide the first evidence that lesional dermal fibroblasts from lipo-lymphedema with plasma cell infiltration show increased proliferation and FGF-2 production and that both local tissue factors and altered FGF-2 serum levels associated with monoclonal gammopathies might contribute to this phenotype. Thus we propose a possible pathophysiologic link between the gammopathy-associated factors and the generation of lymphedema with initial fibrogenesis aggravating pre-existing lipedema.

Lipedema is a disproportional obesity due to unknown pathomechanism. Its major hallmark is frequent hematoma formation related to increased capillary fragility and reduced venoarterial reflex. Beyond microangiopathy, both venous and lymphatic dysfunction have also been documented. However, arterial circulation in lipedema has not been examined, and therefore we explored aortic elastic properties by echocardiography. Fourteen women with and 14 without lipedema were included in the study. Each subject consented to blood pressure measurement, physical examination, and transthoracic echocardiography. Aortic stiffness index (beta), distensibility, and strain were evaluated from aortic diameter and blood pressure data. Mean systolic (30.0 +/- 3.2 vs. 25.5 +/- 3.6, P < 0.05) and diastolic (27.8 +/- 3.3 vs. 22.3 +/- 3.1) aortic diameters (in mm) and aortic stiffness index (9.05 +/- 7.45 vs. 3.76 +/- 1.22, P < 0.05) were significantly higher, while aortic strain (0.082 +/- 0.04 vs. 0.143 +/- 0.038, P < 0.05) and distensibility (2.24 +/- 1.07 vs. 4.38 +/- 1.61, P < 0.05) were significantly lower in lipedematous patients compared to controls. Thus, lipedema is characterized with increased aortic stiffness.

BACKGROUND: Lipoedema is a painful disease in women with circumscribed increased subcutaneous fatty tissue, oedema, pain and bruising. Whereas conservative methods with combined decongestive therapy (manual lymphatic drainage, compression garments) have been well established over the past 50years, surgical therapy with tumescent liposuction has only been used for about 10years and long-term results are unknown. OBJECTIVES: To determine the efficacy of liposuction concerning appearance (body shape) and associated complaints after a long-term period. METHODS: A total of 164 patients who had undergone conservative therapy over a period of years, were treated by liposuction under tumescent local anaesthesia with vibrating microcannulas. In a monocentric study, 112 could be re-evaluated with a standardized questionnaire after a mean of 3years and 8months (range 1year and 1month to 7years and 4months) following the initial surgery and a mean of 2years and 11months (8months to 6years and 10months) following the last surgery. RESULTS: All patients showed a distinct reduction of subcutaneous fatty tissue (average 9846mL per person) with improvement of shape and normalization of body proportions. Additionally, they reported either a marked improvement or a complete disappearance of spontaneous pain, sensitivity to pressure, oedema, bruising, restriction of movement and cosmetic impairment, resulting in a tremendous increase in quality of life; all these complaints were reduced significantly (P<0·001). Patients with lipoedema stage II and III showed better improvement compared with patients with stage I. Physical decongestive therapy could be either omitted (22·4% of cases) or continued to a much lower degree. No serious complications (wound infection rate 1·4%, bleeding rate 0·3%) were observed following surgery. CONCLUSIONS: Tumescent liposuction is a highly effective treatment for lipoedema with good morphological and functional long-term results.

Background: Elevated stearoyl-CoA desaturase activity has been described in obese states, with an increased desaturation index (DI) suggesting enhanced lipogenesis. Differences in the DI among various phenotypes of abnormal adiposity have not been studied. Abnormal accumulation of subcutaneous adipose tissue occurs in rare adipose disorders (RADs) including Dercum’s disease (DD), multiple symmetric lipomatosis (MSL), and familial multiple lipomatosis (FML). Examining the DI in subcutaneous fat of people with DD, MSL and FML may provide information on adipose tissue fatty acid metabolism in these disorders. The aims of this pilot study were: 1) to determine if differences in adipose tissue DIs are present among RADs, and 2) to determine if the DIs correlate to clinical or biochemical parameters. Methods: Subcutaneous adipose tissue was obtained from human participants with DD (n = 6), MSL (n = 5), FML (n = 8) and obese Controls (n = 6). Fatty acid composition was determined by gas chromatography/mass spectrometry. The DIs (palmitoleic/palmitic, oleic/stearic, vaccenic/stearic ratios) were calculated from the gas chromatogram peak intensities. SCD1 gene expression was determined. Spearman’s correlations between the DIs and available clinical or biochemical data were performed. Results: In DD subjects, the vaccenic/stearic index was lower (p < 0.05) in comparison to Controls. Percent of total of the saturated fatty acid myristic acid was higher in DD compared with Controls and FML. Percent of monounsaturated vaccenic acid in DD trended lower when compared with Controls, and was decreased in comparison to FML. In MSL, total percent of the polyunsaturated fatty acids was significantly lower than in the Control group (p < 0.05). In the total cohort of subjects, the palmitoleic/palmitic and oleic/stearic DIs positively correlated with age, BMI, and percent body fat. Conclusions: The positive associations between the DIs and measures of adiposity (BMI and percent body fat) support increased desaturase activity in obesity. The lower vaccenic/stearic DI in DD SAT compared with Controls suggests presence of other factors involved in fat accumulation in addition to lifestyle. Other mechanisms driving fat accumulation in DD such as inflammation or lymphatic dysfunction should be investigated.

Lipedema is a disproportional obesity featuring light pressure-induced or spontaneous pain. On the basis of our clinical observations, lymphedema therapy, as practiced in our clinic, reduces the perception of pain beyond leg volume reduction. We therefore aimed to measure pain intensity prior and subsequent to treatment. 38 women with lipedema were enrolled in the study with 19 patients undergoing treatment and 19 serving as the control group using exclusively moisturizers. Treatment consisted of once daily manual lymph drainage (MLD), intermittent pneumatic compression (IPC), and multilayered short-stretch bandaging performed throughout a 5-day-course. Pain was evaluated with a 10-item questionnaire, a pain rating scale (PRS), and the Wong-Baker Faces scale. Treatment resulted in a significant reduction of pain with a decrease in mean scores of all three measures. In the control group, only PRS showed significant decrease. Our study results indicate that this treatment regimen not only reduces leg volume and capillary fragility, but also improves pain intensity in patients with lipedema.

BACKGROUND: There are many causes for a large lower limb in the pediatric age group. These children are often mislabeled as having lymphedema, and incorrect diagnosis can lead to improper treatment. The purpose of this study was to determine the differential diagnosis in pediatric patients referred for lower extremity "lymphedema" and to clarify management. METHODS: The authors' Vascular Anomalies Center database was reviewed between 1999 and 2010 for patients referred with a diagnosis of lymphedema of the lower extremity. Records were studied to determine the correct cause for the enlarged extremity. Alternative diagnoses, sex, age of onset, and imaging studies were also analyzed. RESULTS: A referral diagnosis of lower extremity lymphedema was given to 170 children; however, the condition was confirmed in only 72.9 percent of patients. Forty-six children (27.1 percent) had another disorder: microcystic/macrocystic lymphatic malformation (19.6 percent), noneponymous combined vascular malformation (13.0 percent), capillary malformation (10.9 percent), Klippel-Trenaunay syndrome (10.9 percent), hemihypertrophy (8.7 percent), posttraumatic swelling (8.7 percent), Parkes Weber syndrome (6.5 percent), lipedema (6.5 percent), venous malformation (4.3 percent), rheumatologic disorder (4.3 percent), infantile hemangioma (2.2 percent), kaposiform hemangioendothelioma (2.2 percent), or lipofibromatosis (2.2 percent). Age of onset in children with lymphedema was older than in patients with another diagnosis (p = 0.027). CONCLUSIONS: "Lymphedema" is not a generic term. Approximately one-fourth of pediatric patients with a large lower extremity are misdiagnosed as having lymphedema; the most commonly confused causes are other types of vascular anomalies. History, physical examination, and often radiographic studies are required to differentiate lymphedema from other conditions to ensure the child is managed appropriately.

BACKGROUND: Lipedema is a painful, genetically induced abnormal deposition of subcutaneous fat in the extremities of almost exclusively women. The pathogenesis is unknown and no curative treatment is available. Conservative therapy consisting of lymphatic drainage and compression stockings is often recommended, but is only effective against the edema. Some patients show a short-term improvement when treated in this way. The removal of the increased fat tissue of lipedema has become possible by employing advanced liposuction techniques which utilize vibrating microcannulas under tumescent local anesthesia. The effectiveness of this approach to lipedema is the subject of this study. PATIENTS AND METHODS: 25 patients were examined before liposuction and six months thereafter. The survey included the measurement of the volume of the legs and several parameters of typical pain and discomfort. The parameters were measured using visual analogue scales (VAS, scale 0-10). RESULTS: The volume of the leg was reduced by 6.99 %. Pain, as the predominant symptom in lipedema, was significantly reduced from 7.2 ± 2.2 to 2.1 ± 2.1 (p < 0.001). Quality of life as a measure of the psychological strain caused by lipedema improved from 8.7 ± 1.7 to 3.6 ± 2.5 (p < 0.001). Other parameters also showed a significant improvement and the over-all severity score improved in all patients. CONCLUSION: Liposuction reduces the symptoms of lipedema significantly.

OBJECTIVE. The objective of our study was to examine the frequency and significance of visualization of popliteal nodes during lymphoscintigraphy for the investigation of lower extremity swelling. MATERIALS AND METHODS. Technetium-99m–labeled nanocolloid was injected subcutaneously in the first web spaces of both feet of 204 consecutive patients (69 males, 135 females; age range, 11–79 years) undergoing routine, clinically indicated lymphoscintigraphy; imaging was performed 5, 45, and 150 minutes after injection. The patients were asked not to undertake any vigorous exercise between the injection and completion of imaging. RESULTS. No popliteal nodes were visualized in 29 patients in whom there was no evidence of lymphedema on clinical or lymphoscintigraphic examination (group 1). Unilateral or bilateral popliteal nodes were visualized in 10 of 39 patients (25.6%) with clinical evidence of lymphedema but normal lymphoscintigraphy findings (group 2) (p < 0.005 vs group 1). In 136 patients with clinical evidence of lymphedema and abnormal lymphoscintigraphy findings (group 3), unilateral or bilateral popliteal nodes were visualized in 59 (43.4%) (p < 0.0001 vs group 1). Popliteal nodes were visualized in 40 of 73 limbs with “dermal backflow” (54.8%) and 42 of 335 limbs without dermal backflow (12.5%) (p < 0.0001). CONCLUSION. Popliteal node visualization after subcutaneous foot web space injection is an important sign of abnormal lymphatic function in patients with clinical lymphedema of the lower extremities.

The aim of this study was to evaluate the prevalence of concomitant idiopathic cyclic edema with Grade II and III cellulite. All patients treated for Grade II and III cellulite were evaluated for idiopathic cyclic edema in a retrospective, quantitative and cross-sectional study. The study was carried out at the Godoy Clinic in the period from 2006 to 2010. All patients with body mass indexes > 25, Grade I cellulite and other causes of edema were excluded. The diagnosis of idiopathic cyclic edema was based on a clinical history and fluid retention throughout the day, in particular difficulty in removing rings on waking in the morning which improves later in the day. All patients with cyclic edema were treated with 75 mg aminaphtone three times daily. Statistical analysis considered the frequency of edema. Of the 82 women evaluated with ages between 18 and 58 years old (mean of 34.9 years) 41 (50.0%) were diagnosed with idiopathic cyclic edema. Idiopathic cyclic edema is an aggravating factor for cellulite and is frequently associated with the more advanced stages of the disease. Its control is essential in the treatment of cellulite.