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Lymphedema and lipedema are chronic progressive disorders for which no causal therapy exists so far. Many general practitioners will rarely see these disorders with the consequence that diagnosis is often delayed. The pathophysiological basis is edematization of the tissues. Lymphedema involves an impairment of lymph drainage with resultant fluid build-up. Lipedema arises from an orthostatic predisposition to edema in pathologically increased subcutaneous tissue. Treatment includes complex physical decongestion by manual lymph drainage and absolutely uncompromising compression therapy whether it is by bandage in the intensive phase to reduce edema or with a flat knit compression stocking to maintain volume.

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.

At the 3rd International Lymphoedema Framework (ILF) conference in Toronto, an attempt was made to begin to try to address this issue. Over thirty people attended a lipoedema workshop run by the author. In this workshop, I had the opportunity to talk to this international group (Canada, USA, Netherlands, Australia, UK, Germany) who shared their experiences from across the world. One of the goals of the workshop was to create an initial broad consensus about the worldwide diagnosis and management of lipoedema.The outcomes of that workshop are presented in the following report.

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.

It is estimated that 3.5 million patients, both male and female, suffer from a congenital lipid distribution disorder of the arms and legs. Because these lipid distribution disorders are painful, owing to a lymphological high volume insufficiency and a structural drainage incompetence, the term lipohyperplasia dolorosa has become established as the name for the clinical picture of lipoedema. This lymphological clinical picture has also undergone a paradigm change with regard to the therapeutic options over the last 15 years: lipohyperplasia dolorosa is curable. This four part series reports on the principles and successes in operative lymphology. Zusammenfassung Geschätzt werden 3,5 Millionen PatientInnen, die an einer angeborenen Fettverteilungsstö-rung der Beine und Arme leiden. Da diese Fettverteilungsstörungen aufgrund einer lym -phologischen Hochvolumeninsuffizienz und einer strukturellen Drainageschwäche schmerzhaft ist, hat sich der Begriff Lipo-hyperplasia dolorosa zur Bezeichnung des Krankheitsbildes Lipödem durchgesetzt. Auch in Bezug auf die therapeutischen Optionen erlebte dieses lymphologische Krankheitsbild in den letzten 15 Jahren einen Paradigmenwechsel: Lipohyperplasia dolorosa ist heilbar. In einer vierteiligen Serie wird über Grundlagen und Erfolge der operativen Lymphologie berichtet werden.

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.

The article offers the insight of doctor Louis Habbema concerning the treatment of lipedema. The author says that liposuction with the use of super tumescent local anesthesia is being considered as an important advancement in lipedema treatment. Furthermore, he states that the liposuction treatment conducted in approximately 800 women has improved patients' symptoms and effectively and safely removes their fats.

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.

Lipedema is a symmetric fat disorder in women which affects legs and arms. Due to the fat bulges at the proximal inner thigh there is an abduction of the leg axis that leads to a change in the walking appearance and to an unnatural physiological strain on the leg joints (knock knee).

Lipedema is an infrequently recognized and often neglected clinical entity that nearly always affects women. It poses a diagnostic challenge as one of the common disorders that is easily confused with lymphedema.

Lipedema is an underdiagnosed syndrome of unclear etiology, characterized by symmetric painful enlargement of the buttocks and lower extremities, which spares the feet. This enlargement is caused by the deposition of adipose tissue. It was first described by Allen and Hines in 1940, who observed that it had a female predilection; patients commonly had an associated family history. We describe a patient with classic lipedema and multiple lipomas of her arms and trunk.

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.

The aim of the current study was to alert about dermatological alterations in the clinical evolution of lipo-lymphedema. The case of a 48-year-old patient is reported with clinical diagnosis of stage II lipo-lymphedema treated in the Angiology and Vascular Surgery Service of FAMERP. On physical examination the edema was hard, not reversible during rest, with nodules in the lower one-third of the limbs and a positive Stemmer's sign. The sizes of the nodules were different between legs. This report stresses the clinical findings and the differences in each disease. Additionally a review of publications is presented. The dermatological changes occurring in the evolution of lipedema may be associated with the evolution of lipo-lymphedema.

BACKGROUND: A 23-year-old male was referred to our clinic with diagnosis of idiopathic isolated growth hormone deficiency. A detailed family history revealed short stature and swelling of legs which only affected females in four generations of his family. METHODS: Combined pituitary function tests revealed growth hormone deficiency, secondary hypothyroidism and hypoprolactinemia in the proband. His mother had hypoprolactinemia and growth hormone deficiency. A diagnosis of inherited combined pituitary deficiency due to a PIT-1 mutation was suspected in view of the short stature with associated multiple pituitary hormone deficiencies. RESULTS: A mutation was identified in PIT-1 (POU1F1), 196C>T, which produces the amino acid change P24L in exon 1. The mutation was also found in the mother of the proband but not in his phenotypically normal half-sister. CONCLUSION: The case shows a novel association of two rare conditions Pit-1 mutation and lipoedema in a family that has not been described before. It also allows formulation of hypothesis on the interaction of growth hormone and sex steroids resulting in abnormal fat distribution in predisposed subjects at the time of puberty.