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We report on a 29-year-old woman who presented with abdominal right upper quadrant pain after an outpatient liposuction procedure. A contrast-enhanced computed tomography scan revealed 4 hepatic perforation tracts with subcapsular liver hematoma and hematoperitoneum. The patient was treated by intravenous tranexamic acid and isotonic fluids and monitored on an intensive care unit. No intervention or surgery was necessary during her hospital stay. Follow-up imaging after 3 days using contrast-enhanced ultrasound still showed the perforation tracts in the liver but no expansion of subcapsular hematoma. After 7 days, the patient was discharged home with stable hemoglobin and reduced pain. Liver perforation is a rare complication of liposuction procedures. In patients with abdominal pain after liposuction, contrast-enhanced imaging studies should be performed to identify and characterize solid organ injury. Teams with expertise in angiography and visceral surgery need to be on standby.

BACKGROUND: When performing large volume liposuction, perioperative management of lipedema patients with coagulation disorders remains challenging due to a lack of clinical experience. With a prevalence of 1% of von Willebrand disease (VWD) in the general population, basic knowledge on diagnostic and adapted surgical strategies are essential for patients' safety. OBJECTIVE: Based on a selective literature review, the purpose of this article is to present a standardized algorithm for diagnosis and perioperative treatment of VWD patients undergoing large volume liposuction. METHODS: The databases MEDLINE (via PubMed) and Web of Science were selectively searched with the term "(((liposuction) OR (surgery)) OR (lipectomy)) AND (((VWD) OR (hemostaseology)) OR (von Willebrand disease))". Included were articles published in English or German until November 2020. RESULTS: The evidence for large volume liposuctions in patients with VWD is limited. Experience is largely based on operations with similar bleeding risks. A safe performance requires an adjustment of the surgical technique and a customized perioperative drug substitution plan. According to the current literature, perioperative thromboembolic events appear to be rare with adequate drug treatment. CONCLUSION: The implementation of the developed diagnostic and treatment algorithm may help further reducing bleeding complications and improve the safety for treated patients.

Background:  Lipedema is a loose connective tissue disease affecting the limbs of women, that is difficult to lose by diet, exercise, or bariatric surgery. Publications from Europe demonstrate that lipedema reduction surgery improves quality of life for women with lipedema. There are no comparable studies in the United States (USA). The aim of this study was to collect data from women with lipedema in the USA who have undergone lipedema reduction surgery in the USA to determine if quality of life, pain, and other measures improved after lipedema reduction surgery. Methods:  Subjects were recruited and consented online for a 166-item questionnaire in REDCap. In total, 148 women answered the questionnaire after undergoing lipedema reduction surgery in the USA. Significance set at P < 0.05 was determined by ANOVA, Tukey’s multiple comparison test, or paired t-test. Results:  Quality of life improved in 84% and pain improved in 86% of patients. Ambulation improved most in lipedema Stage 3 (96%). Weight loss occurred in all stages by 3 months after surgery. Complications included growth of loose connective tissue within and outside treated areas, tissue fibrosis, anemia, blood clots, and lymphedema. Conclusions:  Women with lipedema noticed significant benefits after lipedema reduction surgery in the USA. Prospective studies are needed to assess benefits and complications after lipedema reduction surgery in the USA.

Lipedema is an infrequently recognized disorder. It is characterized by bilateral painful enlargement of the legs due to abnormal depositions of subcutaneous fat, that can result in considerable disability. The diagnosis is established on the basis of medical history and clinical findings. Early diagnosis of this disorder is necessary to start adapted management and prevent progression and complications of the disease. Currently there is no curative treatment. The conservative treatment requires a multidimensional approach including manual lymph drainage, compression garments and management or prevention of obesity as well as other comorbidities. In some cases, surgery may be considered.

The tragedy of a Khoisan woman who may have had lipedema, a symbol of prejudice and stigma

Background: In the USA, the Orphan Drug Act of 1983 defines a rare disease as affecting under 200,000 individuals. Dercum’s disease (DD) is a loose connective (adipose) tissue disease characterized by painful lipomas. While considered a rare disease, the prevalence of DD has not been systematically assessed previously. The objective of this paper is to estimate the prevalence of DD to determine if it is rare or not. Results: Estimates of prevalence of DD using PubMed, the UK Biobank, the US Agency for Health Research and Quality Healthcare Cost and Utilization, physician practices, social media forums and internet searches found the prevalence of DD to be less than 200,000 individuals in the US. These prevalence likely overestimate the disease; however, underestimation may also occur because DD is not well known and may be misdiagnosed. Conclusion: DD meets requirements of the Orphan Drug Act to be classified as a rare disease. Further research should focus on representative population samples in the USA to better estimate the prevalence of DD. Estimating the prevalence is an important first step to increase recognition, research efforts and patient care for people living with DD.

Lipedema is a widespread in concern of etiology partially unknown disease especially in women. In many cases it is accompanied by bleeding complications. Our current work focuses on possible coagulation disorders as potential sources of such bleeding complications. Since only a minority of our patients showed a coagulation defect it is suggestive that the main underlying reason for bleeding in lipedema is of cutaneous origin what may only be forwarded by simultaneously existing coagulation disorders.

Lipedema is an underdiagnosed painful adipose tissue disorder that occurs almost exclusively in women, with onset manifesting at puberty or at times of hormonal change. Unlike many fat disorders, diet and exercise have little to no impact on the prevention or progression of this disease. Estrogens control the distribution of body fat and food intake, regulate leptin expression, increase insulin sensitivity, and reduce inflammation through signaling pathways mediated by its receptors, estrogen receptor alpha (ERα) and ERβ. This review will focus on understanding the role of estrogen in the pathogenesis of the disease and envisage potential hormonal therapy for lipedema patients.

Lipoedema is a chronic disease frequent in women, that causes an abnormal fat deposition in their lower limbs, with a remarkable disproportion between the upper and lower part of the body, easy bruising and pain. Despite the lack of pathognomonic tests for lipoedema, more and more patients in social networks are claiming they suffer from it, giving the disease more visibility but allowing a lot of fake information about lipoedema. Information for self-care and realistic expectations are important. Depending on the severity, the treatment includes: physical therapies, compression garments, exercise, diet, psychological support and surgical treatment.

PURPOSE OF REVIEW: Williams syndrome is a multisystem disorder caused by a microdeletion on chromosome 7q. Throughout infancy, childhood, and adulthood, abnormalities in body composition and in multiple endocrine axes may arise for individuals with Williams syndrome. This review describes the current literature regarding growth, body composition, and endocrine issues in Williams syndrome with recommendations for surveillance and management by the endocrinologist, geneticist, or primary care physician. RECENT FINDINGS: In addition to known abnormalities in stature, calcium metabolism, and thyroid function, individuals with Williams syndrome are increasingly recognized to have low bone mineral density, increased body fat, and decreased muscle mass. Furthermore, recent literature identifies a high prevalence of diabetes and obesity starting in adolescence, and, less commonly, a lipedema phenotype in both male and female individuals. Understanding of the mechanisms by which haploinsufficiency of genes in the Williams syndrome-deleted region contributes to the multisystem phenotype of Williams syndrome continues to evolve. SUMMARY: Multiple abnormalities in growth, body composition, and endocrine axes may manifest in individuals with Williams syndrome. Individuals with Williams syndrome should have routine surveillance for these issues in either the primary care setting or by an endocrinologist or geneticist.

Background: Lack of diagnostic awareness of lipedema and frequent confusion with obesity or lymphedema may be an obstacle for treatment. The clinical effects of conservative treatment methods are not clearly known. This study investigated the effects of exercise-based rehabilitation combined with complete decongestive therapy (CDT) or intermittent pneumatic compression therapy (IPCT) or alone in patients with severe lipedema. Methods: Thirty-three women with severe (type 3, stage III or IV) lipedema diagnosed according to the revised-Wold criteria were randomized into three groups: Group 1 (CDT plus exercises), Group 2 (IPCT plus exercises), and Group 3 (control-exercises alone). All groups received 30 sessions of combined (aerobic, strengthening, and stretching) exercise program. In addition, there were CDT in Group 1 and IPCT in Group 2 five times a week for 6 weeks. The primary outcome measure was the limb volume measurements. The secondary outcome measures were anthropometric measurements (body weight, body mass index, waist-to-height ratio, waist-to-hip ratio), 6-minute walk test, visual analog scale for pain, fatigue severity scale, Beck Depression Inventory, and Short Form Health Survey-36 (SF-36). Results: Thirty-one participants completed the interventions. Limb volumes (p = 0.017, ηp2 = 0.562 for right; p < 0.001, ηp2 = 0.775 for left), pain (p = 0.045, ηp2 = 0.199), and physical functioning subscore of SF-36 (p = 0.040, ηp2 = 0.465) differed significantly between treatments originating from Group 1. Conclusions: All programs improved outcome measurements after the intervention. However, when the difference between treatments was investigated, CDT administered in addition to the exercises has been shown to provide significant improvements in reducing limb volumes, pain, and physical function. Clinical trial registration number: The study was registered at the US National Institutes of Health (ClinicalTrials.gov) (NCT03924999) and available at https://clinicaltrials.gov/ct2/show/NCT03924999?term=lipedema&draw=2&rank=6.

Background: The aim of this study is to investigate the effect of complex decongestive physiotherapy (CDP) plus intermittent pneumatic compression (IPC) applications on lower extremity limb circumference and volume in patients with lipedema. Methods and Results: In measurement of limb volume and circumference measurement, the Perometer 400 NT was used before and after treatment. The perometer measurements in this study were performed in the certain five reference points (cB, cC, cD, cE, and CF). All participants included in the study were included in a treatment protocol consisting of CDP and IPC. It was seen that statistically significant reduction was found in the circumference of 3 of the 5 points of measurements performed in the left limb, whereas statistically significant reduction was found in the circumference of 4 of the 5 points of measurements performed in the right limb. When the assessments of limb volume performed with the perometer were compared before and after CDP, it was seen that statistically significant reduction was found in the volume of both limbs. Conclusion: This reduction indicates that CDP is effective in the treatment of lower extremity lipedema. Clinicaltrials.gov with an ID of NCT04492046.