Proposed Framework for Research Case Definitions of Lipedema.

Background: Our aim is to propose a framework for the development of a research case definition of lipedema, based on current available literature and those observations that can be applied to future lipedema research with the intent to standardize and strengthen the scientific evidence base. Methods and Results: We conducted a narrative review of the literature, and identified consensus characteristics and disputed characteristics that could be included in a research case definition of lipedema. After considering the strength of the evidence and how each characteristic might be measured in a research study, we recommended an approach for the development of a research case definition of lipedema that would be based on consideration of five agreed-upon characteristics, and five disputed, or less substantiated, characteristics as additional evidence to enhance specificity. Conclusions: We present a case definition framework for lipedema drawn from the scientific literature that can be applied to future studies on lipedema. Utilizing this framework should help to increase the sensitivity and specificity of case definition and provide an opportunity for meta-analysis of clinical studies and facilitate future research intercomparisons.

Body mass index vs. waist-to-height-ratio in patients with lipohyperplasia dolorosa (vulgo lipedema).

BACKGROUND: Lipedema, also known as lipohyperplasia dolorosa (LiDo), is a painful condition affecting women, causing a disproportionate accumulation of subcutaneous adipose tissue in the extremities. It carries a lower risk of diabetes and cardio-metabolic dysfunctions compared to obesity, but coincident obesity can complicate diagnosis and treatment. PATIENTS AND METHODS: This retrospective study included 607 female LiDo patients, ≥ 18 years, stage 1-3, from Germany, the UK, and Spain. Data were collected as part of the standard initial assessment for LiDo patients. RESULTS: Based on waist-to-height-ratio (WHtR), 15.2% of patients were underweight, 45.5% normal weight, 22.1% overweight and 17.3% obese. There was a significant association between WHtR category and age group. Body mass index (BMI) is often overestimated, leading to misdiagnosis of obesity. CONCLUSIONS: The use of BMI also affects the recent decision of the German Federal Joint Committee on the reimbursement of liposuction costs by health insurance funds. Patients with BMI of more than 40 kg/m2 are excluded from cost coverage, and those with BMI between 35 kg/m2 and 40 kg/m2 must first receive conservative obesity therapy. In conclusion, the sole use of BMI in lipedema is unreliable and, in contrast to WHtR, leads to inaccurate diagnoses overestimating overweight and obesity.

Physical therapies in the decongestive treatment of lymphedema: A randomized, non-inferiority controlled study.

OBJECTIVE: To assess whether the treatment with intermittent pneumatic compression plus multilayer bandages is not inferior to classical trimodal therapy with manual lymphatic drainage in the decongestive lymphedema treatment. STUDY DESIGN: Randomized, non-inferiority, controlled study to compare the efficacy of three physical therapies' regimens in the Decongestive Lymphatic Therapy. PARTICIPANTS: 194 lymphedema patients, stage II-III with excess volume > 10% were stratified within upper and lower limb and then randomized to one of the three treatment groups. Baseline characteristics were comparable between the groups. INTERVENTION: all patients were prescribed 20 sessions of the following regimens: Group A (control group): manual lymphatic drainage + Intermittent Pneumatic Compression + Bandages; Group B: pneumatic lymphatic drainage + Intermittent Pneumatic Compression + Bandages; and Group C: only Intermittent Pneumatic Compression + Bandages. END-POINT: Percentage reduction in excess volume (PREV). RESULTS: All patients improved after treatment. Global mean of PREV was 63.9%, without significant differences between the groups. The lower confidence interval of the mean difference in PREV between group B and group A, and between group C and group A were below 15%, thus meeting the non-inferiority criterion. Most frequent adverse events were discomfort and lymphangitis, without differences between groups. A greater baseline edema, an upper-limb lymphedema and a history of dermatolymphangitis were independent predictive factors of worse response in the multivariate analysis. CONCLUSIONS: Decongestive lymphatic therapy performed only with intermittent pneumatic compression plus bandages is not inferior to the traditional trimodal therapy with manual lymphatic drainage. This approach did not increase adverse events.

Update in the management of lipedema.

INTRODUCTION: Lipedema is a chronic feminine disease that causes abnormal fat deposition in lower limbs and occasionally upper limbs. Easy bruising and pain are common. Lipedema patients suffer from both physical and psychological disability. Despite the relatively high prevalence and the impact on the quality of life, little is known about the disease. Most patients are misdiagnosed as lymphedema or obesity. The aim was to perform a non-systematic review on lipedema literature, related to diagnosis and therapy. EVIDENCE ACQUISITION: A literature search was performed by three researchers, to retrieve pertinent articles in PubMed Web of Science and ResearchGate from the last 20 years. The documents were selected from systematic reviews, reviews, meta-analyses, consensus documents and guidelines, randomized controlled trials and retrospective, cohort and clinical series. EVIDENCE SYNTHESIS: Regarding pathophysiology, alterations are not exclusively confined to adipose tissue. Lymphatic dysfunction, cardiovascular variations, blood capillary fragility and increased permeability are common. Patients often present with neurological alterations and hormonal malfunction. Elevated cutaneous sodium has been documented. Due to the absence of a defined etiology, evidence-based research is difficult in the management of lipedema. CONCLUSIONS: The setting of realistic expectations is important for both patient and medical care provider. The major aims of the management are multimodal for improvements in the quality of life; reduction in heaviness and pain, reshaping the affected limbs, weight control, improvements in mobility. Compression garments, physical therapy, exercise regimens, diet and psychological counseling are necessities. For selected patients, surgical treatment is an added option.

Mecánica rotacional ventricular izquierda invertida en un paciente con lipedema (del estudio de MAGYAR-Path) / Left ventricular twist rotation mechanics in a patient with lipoedema (MAGYAR-Path Study)

Resumen El lipedema es un trastorno crónico que se caracteriza por una hiperplasia del tejido adiposo subcutáneo simétrico, deformante, asociado a hematomas y dolor, que afecta fundamentalmente a mujeres tras la pubertad, que suele ser familiar y tiene influencia hormonal. Sin tratamiento, el lipedema puede progresar a linfedema, y se ha asociado al aumento en la rigidez de la aorta. La mecánica rotacional del ventrículo izquierdo (VI) tiene un papel significativo en la circulación normal. En circunstancias normales, la base del VI rota en el sentido de las agujas del reloj, mientras que el ápex del VI rota en sentido contrario, provocando un movimiento como de retorcer una toalla llamado torsión del VI. En el presente caso, mediante ecocardiografía speckle-tracking tridimensional se reveló una torsión del VI demostrando un ápex VI en el sentido de las agujas del reloj y la rotación antihoraria de la base del VI en un paciente con características clínicas de lipedema.

Abstract Lipoedema is a chronic disorder that is characterised by a symmetric, deforming, hyperplasia of the subcutaneous adipose tissue, and is associated with haematomas and pain. It mainly affects women after puberty, is usually familial and is influence by hormones. Untreated, the lipoedema can progress to lymphoedema, and has been associated with the increase in aortic stiffness. The mechanical rotation of the left ventricle (LV) has an important role in normal circulation. In normal circumstances, the base of the LV rotates clockwise, while the LV apex rotates in the opposite direction, leading to a movement like wringing a towel, called LV twist. In the present case, using three-dimension speckle-tracking echocardiography, an LV twist is observed, showing an LV apex in the clockwise direction and the anti-clockwise rotation of the LV base in a patient with clinical characteristics of lipoedema.

Ground reaction force patterns during gait in patients with lower limb lymphedema / Forças de reação ao solo durante a marcha de pacientes com linfedema de membro inferior

Although gait problems have been reported in patients with lower limb lymphedema (LLL), the gait pattern (GP) changes have not been documented yet. However, it is possible that patients with LLL show abnormal GP that can be related to biomechanical complications related to osteoarthritis or falls affecting the quality of life. Ground reaction force analysis during gait allows objective assessment of the patients and it can be used to plan a rehabilitation approach. Objective: To analyze the GRF during gait in patients LLL. Methods: An experimental descriptive study was realized with twenty-three LLL patients, both unilateral and bilateral and classified as moderate and severe, participated in the experiments. The patients walked on a force plate while the three ground reaction force (GRF) components, vertical, mediolateral (M-L) and anteroposterior (A-P), under their feet were recorded and analyzed. Results: In the patients with unilateral lymphedema, either moderate or severe, the vertical GRF components of the affected limb were similar to the sound one and also resembling those found in healthy adults. The M-L GRF was smaller in the non-affected side. In patients with bilateral lymphedema gait speed was significantly slower. More interestingly, the vertical GRF pattern was flat, not showing the typical 2-peak shape. Finally, the large M-L forces found suggest gait stability problems. Conclusions: The patients showed abnormal GRF patterns, including compensation with the non-affected leg. The GRF variability was higher in the patients with severe unilateral lymphedema. Bilateral lymphedema results in lower A-P forces. Stance phase duration was longer in patients with bilateral and severe lymphedema

[Postmastectomy pain syndrome in our region: characteristics, treatment, and experience with gabapentin]. / Sindrome de dolor posmastectomia en nuestro medio: caracteristicas, tratamiento y experiencia con gabapentina.

INTRODUCTION: Postmastectomy pain syndrome affects up to a half of the patients treated for breast cancer. AIMS: Characterization of this syndrome and its evolution. Measure pharmaceutical treatment (specifically with gabapentin). PATIENTS AND METHODS: A retrospective study of 89 patients with more than five years monitoring was carried out. RESULTS: Of the patients included in the study 88% had not been previously diagnosed. The mean age of the patients was 56.49 years old. The pain lasted on average 29.15 months. In 71% the neurological symptoms corresponded to the second intercostobrachial nerve. The average pain value for patients before treatment on the Visual Analog Scale was 66.5 and 13.14 on the Lattinen Index. The treatment with gabapentin at an average dose of 1,135 mg for 14 weeks reduced pain in 80% of the patients (p < 0.0001). In the majority of the patients the reduction was maintained in the long term. Ten percent continued the treatment whereas another 10% suspended treatment due to side effects. The average necessary number of patients to be treated was 2.13. Of those treated with gabapentin 35% relapsed while 15.68% changed medication compared to 50% of those treated with other indicated neuropathic drugs (p = 0.046). No difference in the effectiveness between both groups was found. CONCLUSIONS: Postmastectomy pain is an under-diagnosed pathology. Treatment (in particular with gabapentin) can be significantly effective and well tolerated in up to 90% of postmastectomy pain syndrome patients.

TITLE: Sindrome de dolor posmastectomia en nuestro medio: caracteristicas, tratamiento y experiencia con gabapentina.Introduccion. El sindrome de dolor posmastectomia puede afectar a mas de la mitad de pacientes intervenidas por cancer de mama. Objetivos. Revisar las caracteristicas clinicas de este sindrome y su evolucion, y evaluar la respuesta al tratamiento farmacologico. Pacientes y metodos. Estudio retrospectivo de 65 pacientes con un seguimiento superior a cinco años. Resultados. El 88% de las pacientes estaba sin diagnosticar. Su edad media era de 56,49 años y permanecieron con dolor un promedio de 29,15 meses. En el 71%, las alteraciones neurologicas correspondieron al segundo nervio intercostobraquial. La media en la puntuacion de dolor antes del tratamiento fue de 66,5 puntos en la escala analogica visual y de 13,14 en el indice de Lattinen. El tratamiento con gabapentina (dosis media: 1.135 mg/dia; duracion media: 14 semanas) disminuyo el dolor en el 80% de las pacientes (p < 0,0001). La mejoria se mantuvo a largo plazo. Un 10% continuo en tratamiento y el 10% debio suspenderlo por efectos secundarios. El numero necesario de pacientes que hubo que tratar fue de 2,13. Recidivo el 35% y preciso cambiar de farmaco el 15,68% de las tratadas con gabapentina, frente al 50% de las tratadas con otros farmacos (p = 0,046). No se encontraron diferencias en la eficacia entre ambos grupos. Conclusiones. El dolor posmastectomia es una patologia infradiagnosticada. El tratamiento (en especial con gabapentina) puede ser eficaz y bien tolerado hasta en el 90% de las pacientes de forma significativa.

Síndrome de dolor posmastectomía en nuestro medio: características, tratamiento y experiencia con gabapentina / Postmastectomy pain syndrome in our region: characteristics, treatment, and experience with gabapentin

Introducción. El síndrome de dolor posmastectomía puede afectar a más de la mitad de pacientes intervenidas por cáncer de mama. Objetivos. Revisar las características clínicas de este síndrome y su evolución, y evaluar la respuesta al tratamiento farmacológico. Pacientes y métodos. Estudio retrospectivo de 65 pacientes con un seguimiento superior a cinco años. Resultados. El 88% de las pacientes estaba sin diagnosticar. Su edad media era de 56,49 años y permanecieron con dolor un promedio de 29,15 meses. En el 71%, las alteraciones neurológicas correspondieron al segundo nervio intercostobraquial. La media en la puntuación de dolor antes del tratamiento fue de 66,5 puntos en la escala analógica visual y de 13,14 en el índice de Lattinen. El tratamiento con gabapentina (dosis media: 1.135 mg/día; duración media: 14 semanas) disminuyó el dolor en el 80% de las pacientes (p < 0,0001). La mejoría se mantuvo a largo plazo. Un 10% continuó en tratamiento y el 10% debió suspenderlo por efectos secundarios. El número necesario de pacientes que hubo que tratar fue de 2,13. Recidivó el 35% y precisó cambiar de fármaco el 15,68% de las tratadas con gabapentina, frente al 50% de las tratadas con otros fármacos (p = 0,046). No se encontraron diferencias en la eficacia entre ambos grupos. Conclusiones. El dolor posmastectomía es una patología infradiagnosticada. El tratamiento (en especial con gabapentina) puede ser eficaz y bien tolerado hasta en el 90% de las pacientes de forma significativa (AU)

Introduction. Postmastectomy pain syndrome affects up to a half of the patients treated for breast cancer. Aims. Characterization of this syndrome and its evolution. Measure pharmaceutical treatment (specifically with gabapentin). Patients and methods. A retrospective study of 89 patients with more than five years monitoring was carried out. Results. Of the patients included in the study 88% had not been previously diagnosed. The mean age of the patients was 56.49 years old. The pain lasted on average 29.15 months. In 71% the neurological symptoms corresponded to the second intercostobrachial nerve. The average pain value for patients before treatment on the Visual Analog Scale was 66.5 and 13.14 on the Lattinen Index. The treatment with gabapentin at an average dose of 1,135 mg for 14 weeks reduced pain in 80% of the patients (p < 0.0001). In the majority of the patients the reduction was maintained in the long term. Ten percent continued the treatment whereas another 10% suspended treatment due to side effects. The average necessary number of patients to be treated was 2.13. Of those treated with gabapentin 35% relapsed while 15.68% changed medication compared to 50% of those treated with other indicated neuropathic drugs (p = 0.046). No difference in the effectiveness between both groups was found. Conclusions. Postmastectomy pain is an under-diagnosed pathology. Treatment (in particular with gabapentin) can be significantly effective and well tolerated in up to 90% of postmastectomy pain syndrome patients (AU)

Predictive factors of response to decongestive therapy in patients with breast-cancer-related lymphedema.

BACKGROUND: Many studies have reported the benefits of Decongestive treatment in patients with breast-cancer-related lymphedema (BCRL) but few have study what are the predictive factors of response. METHODS: We performed a prospective, multicenter controlled cohort study of 171 patients with BCRL to identify independent predictive factors of response to decongestive treatment (CDT). Demographic data and clinical and lymphedema characteristics were collected prospectively. The end point was the "percentage reduction in excess volume (PREV)." Volumes were measured prior and at the end of CDT. Factors associated with response (PREV) were tested in univariate and multivariate analyses using linear regression techniques. RESULTS: Median age was 60.4 years (range 32-84); mean lymphedema chronicity 4 years [95% confidence interval (95% CI): 3.1-5.0]; mean baseline excess volume (EV) was 936 mL (95% CI: 846-1026), and mean percentage EV was 35.3% (95% CI: 32.0-38.7); compliance to bandages was good in 81.3% of patients. PREV was 71.7% (95% CI: 65.2-78.2). After univariate screening, 11 variables were found to be associated with PREV but only 4 variables were independent predictive factors of response to CDT in the multivariate analysis: Venous insufficiency, percentage of EV (the higher the EV, the lower the reduction with CDT); compliance to bandages (a good compliance improved PREV in 25%), and treatment in autumn (better results than during the rest of the year). CONCLUSIONS: This study shows that compliance to bandages during CDT is one of the most important predictors of response. Moreover, data support the idea that more severe lymphedemas have a worse response to treatment, and it should be recommended in early stages. The association between the season of treatment and response was also very strong, so weather conditions are an additional factor that must be taken into account in further studies.