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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.

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.

Background: As we did not find any reliable datas concerning the prevalance of lipoedema (in Middle Europe), we started an anamnestic, clinical and (duplex-)sonographic investigation in 62 professional women. We were endavoured to avoid a bias of assertion and to get a socially correct composition of the collective. Results: Including all stages of lipoedema, also the mild ones (lipohyperplasia), 39 % of women were concerned. Regarding only the moderate to distict findings of markedly filled in ankles, 9.7 % were concerned. Only the breeches-type was found in 4.8 %. There were no relevant differences in age, length and age of menarche in women without and with lipoedema. Women with lipoedema were heavier than those without and they had accordingly a higher body-mass index. The thickness of subcutis + cutis 8 cm above the medial malleolus was 11 ± 2.8 mm in women without lipoedema and about 16 mm in those with lipoedema (beyond the double standard deviation). The thickness of the cutis lied on average distictly under 2 mm in both groups. Sonography seems to be a safe tool in the judgment of the subcutis – also in epidemiological studies.

BACKGROUND AND PURPOSE: Unclear extremity complaints are common symptoms of inpatients. In a subset of these patients, a clinical suspicion of deep vein thrombosis (DVT) results; this needs to be quickly and definitively clarified by a vascular physician. The question arose of how often a clinical suspicion of DVT was confirmed in an inpatient population and which alternative diagnoses were able to be made by angiologists. PATIENTS AND METHODS: In a retrospective analysis, all inpatients in the Angiologic Vascular Diagnostics Center of the University Hospital Halle, Germany, examined in 2007 for a suspicion of DVT were evaluated with respect to the definitively made diagnosis. RESULTS: In 213 (28.6%) of 745 suspected cases of DVT, a DVT was confirmed. In 532 patients (71.4%), DVT was excluded. In 314 of these patients, 436 alternative diagnoses were recorded in the diagnostic reports of angiologic examinations. In 38.6% (n = 168), other venous causes could be confirmed as the most common alternative diagnosis. There were chronic venous diseases in 28% (n = 122), superficial thrombophlebitis (n = 27), and tumor-related pelvic vein compression (n = 19). 17.4% (n = 76) exhibited lymphedema. In 13.3% (n = 58), a generalized edema was diagnosed. Arthrogenic causes followed with 12.8% (n = 56). Lipedema (5.3%) and hematoma (5%) could be verified as other important differential diagnoses. Rare causes were symptomatic or ruptured Baker's cysts (2.5%), erysipelas (2.5%), abscess, aneurysm, muscle tears, and tumors. CONCLUSION: The variety of alternative diagnoses in patients with clinical suspicion of DVT is high. The knowledge and systematic examination of potential, even rare differential diagnoses after exclusion of DVT are part of the repertoire of the vascular physician. Unnecessary and expensive, as well as onerous, diagnostic procedures on the patient can be avoided. Anticoagulation that was begun as a result of the suspicion of DVT can quickly be stopped.

Adiposis dolorosa (AD) is a rare disorder of painful nodular subcutaneous fat accompanied by fatigue, difficulty with weight loss, inflammation, increased fluid in adipose tissue (lipedema and lymphedema), and hyperalgesia. Sequential compression relieves lymphedema pain; we therefore hypothesized that whole body cyclic pneumatic hypobaric compression may relieve pain in AD. To avoid exacerbating hyperalgesia, we utilized a touch-free method, which is delivered via a high-performance altitude simulator, the Cyclic Variations in Altitude Conditioning™ (CVAC™) process. As a pilot study, 10 participants with AD completed pain and quality of life questionnaires before and after 20-40 minutes of CVAC process daily for 5 days. Participants lost weight (195.5 ± 17.6-193.8 ± 17.3 lb; P = 0.03), and bioimpedance significantly decreased (510 ± 36-490 ± 38 ohm; P = 0.01). There was a significant decrease in scores on the Pain Catastrophizing Scale (P = 0.039), in average (P = 0.002), highest (P = 0.029), lowest (P = 0.04), and current pain severity (P = 0.02) on the Visual Analogue Scale, but there was no change in pain quality by the McGill Pain Questionnaire. There were no significant changes in total and physical SF-36 scores, but the mental score improved significantly (P = 0.049). There were no changes in the Pain Disability Index or Pittsburgh Sleep Quality Index. These data present a potential, new, noninvasive means of treating pain in AD by whole body pneumatic compression as part of the CVAC process. Although randomized, controlled trials are needed to confirm these data, the CVAC process could potentially help in treating AD pain and other chronic pain disorders.

BACKGROUND: Lipoedema is an accumulation of fat abnormally distributed in the lower limbs, and lymphoedema is oedema caused by a deficiency of the lymphatic system. High-resolution ultrasound operating at 20 MHz makes it possible to characterize dermal oedema. OBJECTIVES: The purpose of our study was to demonstrate that high-resolution ultrasound imaging of the skin can differentiate lipoedema from lymphoedema. METHODS: Sixteen patients with lymphoedema (22 legs), eight patients with lipoedema (16 legs) and eight controls (16 legs) were included. Patients with lipolymphoedema were excluded. Ultrasound examinations were carried out with a real-time high-resolution ultrasound device on three different sites for each lower limb. The images were then anonymized and examined by an independent dermatologist who was blind to the clinical diagnosis. A new series of images was examined by three dermatologists to check interobserver agreement. RESULTS: A significant difference in dermal thickness was observed between patients with lymphoedema and those with lipoedema and between patients with lymphoedema and controls. No significant difference in dermal thickness was shown between patients with lipoedema and controls at the thigh or ankle. Dermal hypo-echogenicity was found in at least one of the three sites in 100% of patients with lymphoedema, 12.5% of cases with lipoedema and 6.25% of the controls. Hypoechogenicity affected the entire dermis in all cases of lymphoedema except one. In cases of lipoedema and controls, hypoechogenicity was localized at the ankle and prevailed in the upper dermis. The expert correctly diagnosed all lower limbs with lymphoedema. No cases of lipoedema were diagnosed as lymphoedema. Exact interobserver agreement was excellent (0.98). CONCLUSIONS: High-resolution cutaneous ultrasonography makes it possible to differentiate lymphoedema from lipoedema. Obtaining a reliable diagnosis through high-resolution cutaneous ultrasonography might be valuable for improving the treatment of lipoedema and lymphoedema.

Lipedema is a condition characterized by swelling and enlargement of the lower limbs due to abnormal deposition of subcutaneous fat. Lipedema is an under-recognized condition, often misdiagnosed as lymphedema or dismissed as simple obesity. We present a series of pedigrees and propose that lipedema is a genetic condition with either X-linked dominant inheritance or more likely, autosomal dominant inheritance with sex limitation. Lipedema appears to be a condition almost exclusively affecting females, presumably estrogen-requiring as it usually manifests at puberty. Lipedema is an entity distinct from obesity, but may be wrongly diagnosed as primary obesity, due to clinical overlap. The phenotype suggests a condition distinct from obesity and associated with pain, tenderness, and easy bruising in affected areas.

AIM: We evaluated the outcome and risks of tumescence liposuction in patients with advanced lipedema or Dercum's disease. METHODS: Six patients treated at a single center during the years 2004 to 2008 have been included. All patients were female and obese (body mass index 34 to 41.9; mean 38.2+/-3.8) with an age range from 29 to 78 years (mean 55.7+/-20.5 years), five of them had co-morbidities. RESULTS: The total amount of lipoaspirates varied between 1500 mL and 4800 mL. Pain could be reduced in all four patients with Dercum's disease. Large adipose tissue removing implies a better the outcome for pain. Patient's satisfaction was "high" or "very high" in 5 and "medium" in one. The most common adverse effect was met-hemoglobulinemia (N.=4). CONCLUSION: Tumescence liposuction is a treatment option for lipedema and Dercum's disease. With careful monitoring the procedure is safe even for patients in advanced stages, higher age and with co-morbidities.

A prospective single-center study with mail questionnaire – in most cases combined with clinical controls – was carried out in 112 patients with lipedema after a period from 8 months to nearly 7 years following liposuction in tumescent local anesthesia.

Lipedema is a disease with unknown etiology presenting as bilateral and symmetric enlargement of the lower extremities due to subcutaneous deposition of the adipose tissue. Here we describe the histopathological features of the lipedema tissue and nonaffected adipose tissue obtained from a typical patient with severe lipedema. Immunohistochemical analyses indicated degenerative and regenerative changes of the lipedema tissue, characterized by crown-like structures (necrotizing adipocytes surrounded by infiltrating CD68+ macrophages; a feature commonly seen in obese adipose tissue) and proliferation of adipose-derived stem/progenitor/stromal cells (Ki67+CD34+ cells), respectively. These findings suggested increased adipogenesis in the lipedema tissue, which may further lead to hypoxia similar to that seen in obesity, resulting in adipocyte necrosis and macrophage recruitment. The confinement to the lower extremities and the difference from systemic obesity warrants further elucidation in future studies.

We report a familial Sotos syndrome in two children, boy and girl, aged 17 and 8 years, and in their 44 year old mother, who displayed normal intelligence at adult age, but suffered from insulin dependent diabetes mellitus, bronchial asthma, and severe lipedema. The underlying missense mutation, C2175S, occurred in a conserved segment of the NSD1 gene. Our findings confirm that familial cases of SS are more likely to carry missense mutations. This case report may prove useful to avoid underestimation of the recurrence rate of SS, and to demonstrate that the developmental delay may normalize, enabling an independent life and having an own family.

Einleitung: Bei einem Lipödem liegt eine anlagebedingte Fettgewebsvermehrung vor, bei der es zu einer symmetrischen Verdickung der Extremitäten kommt. Klinisch ist für das Lipödem eine Druckschmerzhaftigkeit charakteristisch. Bei länger bestehendem Lipödem kann man beobachten, dass bei einem Teil der Patientinnen zusätzliche Beschwerden auftreten. Die betroffenen Frauen erleben auf Grund der massiven Schwellungen dann, neben einem ausgeprägten Spannungs- und Schweregefühl der Beine, physische und psychische Belastungen, wodurch Sportangebote häufig nicht wahrgenommen werden und die soziale Interaktionen eingeschränkt ist. Studiendesign: Die vorliegende prospektive randomisierte Studie wurde zur Evaluation der Wirksamkeit eines vibrationsgestützten Krafttrainings mit dem Galileo-System im Vergleich zu einem herkömmlichen Training zur Aktivierung der Beinmuskelpumpe bei Patientinnen mit Lipödem durchgeführt. Die Probanden beider Gruppen erhielten 12 Anwendungen in einem Zeitraum von sechs Wochen. Zur Beurteilung des Therapieverlaufs wurde die Druckschmerzschwelle im Beinbereich mithilfe der visuellen Analogskala (VAS) und der Dolorimetrie an definierten Messpunkten erfasst. Nebenzielkriterien waren die Volumenreduktion und die Verbesserung funktioneller Parameter sowie der Lebensqualität. Die Erhebung der Outcomeparameter erfolgte vor der Intervention (U1), vor der letzen Trainingseinheit (U2) und 6 Wochen nach Therapieende (U3). Ergebnisse: Das Vibrationstraining (n=27) und die Venengymnastik (n=17) zeigten bezüglich der Schmerzlinderung signifikante Verbesserungen im Therapieverlauf. Die Druckschmerzschwelle konnte erhöht werden. Die Auswertung der Fragebögen zu Hämatomneigung und Schmerzempfinden ergab in der Interventionsgruppe eine signifikante Beschwerdelinderung im Therapieverlauf. In beiden Gruppen konnte eine subjektive Verbesserung der Mobilität und ein positiver Einfluss auf die Lebensstiländerung verzeichnet werden. Die im Gehtest beurteilte Ausdauerleistung verbesserte sich ebenfalls. Diskussion: Es konnte gezeigt werden, dass sowohl mit herkömmlicher Krankengymnastik als auch durch ein gerätegestütztes Vibrationstraining zwei wesentliche Symptome des Lipödems, nämlich Druckschmerzhaftigkeit und Hämatomneigung, positiv beeinflusst werden können. Somit lässt sich die Notwendigkeit unterstreichen, dass eine Therapie des Lipödems durch eine Bewegungstherapie ergänzt werden sollte.

OBJECTIVE: To assess for the first time the morphology of the lymphatic system in patients with lipedema and lipo-lymphedema of the lower extremities by MR lymphangiography. MATERIALS AND METHODS: 26 lower extremities in 13 consecutive patients (5 lipedema, 8 lipo-lymphedema) were examined by MR lymphangiography. 18 mL of gadoteridol and 1 mL of mepivacainhydrochloride 1% were subdivided into 10 portions and injected intracutaneously in the forefoot. MR imaging was performed with a 1.5-T system equipped with high-performance gradients. For MR lymphangiography, a 3D-spoiled gradient-echo sequence was used. For evaluation of the lymphedema a heavily T2-weighted 3D-TSE sequence was performed. RESULTS: In all 16 lower extremities (100%) with lipo-lymphedema, high signal intensity areas in the epifascial region could be detected on the 3D-TSE sequence. In the 16 examined lower extremities with lipo-lymphedema, 8 lower legs and 3 upper legs demonstrated enlarged lymphatic vessels up to a diameter of 3 mm. In two lower legs with lipo-lymphedema, an area of dermal back-flow was seen, indicating lymphatic outflow obstruction. In the 10 examined lower extremities with clinically pure lipedema, 4 lower legs and 2 upper legs demonstrated enlarged lymphatic vessels up to a diameter of 2 mm, indicating a subclinical status of lymphedema. In all examined extremities, the inguinal lymph nodes demonstrated a contrast material enhancement in the first image acquisition 15 min after injection. CONCLUSION: MR lymphangiography is a safe and accurate minimal-invasive imaging modality for the evaluation of the lymphatic circulation in patients with lipedema and lipo-lymphedema of the lower extremities. If the extent of lymphatic involvement is unclear at the initial clinical examination or requires a better definition for optimal therapeutic planning, MR lymphangiography is able to identify the anatomic and physiological derangements and to establish an objective baseline.

Lipoedema is a fat distribution disorder causing massive, bilaterally symmetrical enlargement of the lower and in some cases the upper extremities in women. The atraumatic, anatomically appropriate procedure of water jet-assisted liposuction available today represents a promising treatment for these patients who generally suffer from severe subjective and objective impairment. Liposuction treatment can bring long-term improvement if the operative technique focuses on lymph vessel preservation. Immunohistologic analyses show minimal evidence of lymph vessel structures in lipoaspirates. The histologic analysis of the aspirates documents a relatively specific removal ("apheresis") of primarily intact lipocytes with low vascular amount.