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Background/objectives Patients with obesity and lipedema commonly are misdiagnosed as having lymphedema. The conditions share phenotypic overlap and can influence each other. The purpose of this study was to delineate obesity-induced lymphedema, obesity without lymphedema, and lipedema in order to improve their diagnosis and treatment. Subjects/methods Our Lymphedema Center database of 700 patients was searched for patients with obesity-induced lymphedema (OIL), obesity without lymphedema (OWL), and lipedema. Patient age, sex, diagnosis, cellulitis history, body mass index (BMI), and treatment were recorded. Only subjects with lymphoscintigraphic documentation of their lymphatic function were included. Results Ninety-eight patients met inclusion criteria. Subjects with abnormal lymphatic function (n = 46) had a greater BMI (65 ± 12) and cellulitis history (n = 30, 65%) compared to individuals with normal lymphatic function [(BMI 42 ± 10); (cellulitis n = 8, 15%)] (p < 0.001). Seventeen patients had a history of lipedema and two exhibited abnormal lymphatic function (BMI 45, 54). The risk of having lower extremity lymphedema was predicted by BMI: BMI < 40 (0%), 40–49 (17%), 50–59 (63%), 60–69 (86%), 70–79 (91%), ≥80 (100%). Five patients with OIL (11%) underwent resection of massive localized lymphedema (MLL) or suction-assisted lipectomy. Three individuals (18%) with lipedema were treated with suction-assisted lipectomy. Conclusions The risk of lymphedema in patients with obesity and lipedema can be predicted by BMI; confirmation requires lymphoscintigraphy. Individuals with OIL are at risk for cellulitis and MLL. Patients with a BMI > 40 are first managed with weight loss. Excisional procedures can further reduce extremity size once BMI has been lowered.

Significance: Primary lymphedema is a chronic condition without a cure. The lower extremities are more commonly affected than the arms or genitalia. The disease can be syndromic. Morbidity includes decreased self-esteem, infections, and reduced function of the area. Recent Advances: Several mutations can cause lymphedema, and new variants continue to be elucidated. A critical determinant that predicts the natural history and morbidity of lymphedema is the patient's body mass index (BMI). Individuals who maintain an active lifestyle with a normal BMI generally have less severe disease compared to subjects who are obese. Because other causes of lower extremity enlargement can be confused with lymphedema, definitive diagnosis requires lymphoscintigraphy. Critical Issues: Most patients with primary lymphedema are satisfactorily managed with compression regimens, exercise, and maintenance of a normal body weight. Suction-assisted lipectomy is our preferred operative intervention for symptomatic patients who have failed conservative therapy. Suction-assisted lipectomy effectively removes excess subcutaneous fibro-adipose tissue and can improve underlying lymphatic function. Future Directions: Many patients with primary lymphedema do not have an identifiable mutation and thus novel variants will be identified. The mechanisms by which mutations cause lymphedema continue to be studied. In the future, drug therapy for the disease may be developed.

Background: The Stemmer sign is a physical examination finding used to diagnose lymphedema. If the examiner cannot pinch the skin of the dorsum of the foot or hand then this positive finding is associated with lymphedema. The purpose of the study was to determine the accuracy of the Stemmer sign to predict lymphedema. Methods: All patients referred to our Lymphedema Program between 2016 and 2018 were tested for the Stemmer sign and underwent lymphoscintigraphy to define the patient’s lymphatic function. Patient age, lymphedema type (primary and secondary), disease location (arm and leg), lymphoscintigraphy findings, stage, severity, and body mass index were recorded. Comparison of predictive variables and Stemmer sign result was performed using Fisher’s exact test and Student’s t test. Results: One hundred ten patients were studied: patients with a positive Stemmer sign (n = 87) exhibited abnormal (n = 80) or normal (n = 7) lymphatic function by lymphoscintigraphy (sensitivity = 92%). False-positive Stemmer signs included individuals with obesity (n = 6) or spinal muscle atrophy (n = 1). Subjects with a negative Stemmer sign (n = 23) had normal (n = 13) or abnormal (n = 10) lymphatic function by imaging (specificity = 57%). Patients with a false-negative Stemmer sign were more likely to have a normal body mass index (P = 0.02) and Stage 1 disease (P = 0.01). Conclusions: A positive Stemmer sign is a sensitive predictor for primary and secondary lymphedema of the arms or legs and, thus, is a useful part of the physical examination. Because the test exhibits moderate specificity, lymphoscintigraphy should be considered for patients with a high suspicion of lymphedema that have a negative Stemmer sign.

Lymphedema often is confused with other causes of extremity edema and enlargement. Understanding the risk factors and physical examination signs of lymphedema can enable the health care practitioner to accurately diagnose patients ∼90% of the time. Confirmatory diagnosis of the disease is made using lymphoscintigraphy. It is important to correctly diagnose patients with lymphedema so that they can be managed appropriately.

Lymphedema is the chronic, progressive swelling of tissue due to inadequate lymphatic function. Over time, protein-rich fluid accumulates in the tissue causing it to enlarge. Lymphedema is a specific disease and should not be used as a generic term for an enlarged extremity. The diagnosis is made by history and physical examination, and confirmed with lymphoscintigraphy. Intervention includes patient education, compression, and rarely, surgery. Patients are advised to exercise, maintain a normal body mass index, and moisturize / protect the diseased limb from incidental trauma. Conservative management consists of compression regimens. Operative interventions either attempt to address the underlying lymphatic anomaly or the excess tissue. Lymphatic-venous anastomosis and lymph node transfer attempt to create new lymphatic connections to improve lymph flow. Suction-assisted lipectomy and cutaneous excision reduce the size of the area by removing fibroadipose hypertrophy.

Background: Lymphedema results from inadequate lymphatic function due to failure of lymphatic development or injury to a functioning lymphatic system. Patients suffer enlargement of the affected area, psychosocial morbidity, infection, and functional disability. The purpose of this study was to characterize the disease in a cohort of patients referred to a specialized center. Methods and Results: Our Lymphedema Program database was reviewed for all referrals between 2009 and 2019. Diagnosis was determined based on history, physical examination, and lymphoscintigraphy. Lymphedema type (primary, secondary, and obesity-induced), location of swelling, morbidity, previous management, accuracy of referral diagnosis, the geographic origin of the patients, and treatment in our center were analyzed. Seven hundred patients were referred with a diagnosis of "lymphedema"; 71% were female and 38% were children. Lymphedema was confirmed in 71% of the cohort: primary (62%), secondary (22%), and obesity-induced (16%). Twenty-nine percent of individuals labeled with "lymphedema" had another condition. One-half of patients had not received treatment, and 36% resided outside of our local referral area. One-third of subjects with lymphedema had an infection and 30% had >1 visit to the center. Patients with confirmed lymphedema were managed with compression stockings (100%), pneumatic compression (69%), and/or an excisional procedure (6%). Conclusions: Patients with lymphedema typically are adequately managed with conservative compression therapies and rarely require excisional operations. Diagnostic confusion is common and individuals with possible lymphedema are best managed by physicians focused on the disease.

Vascular anomalies and related conditions cause overgrowth of tissues. The purpose of this study was to determine the efficacy and safety of liposuction techniques for pediatric overgrowth diseases. Patients treated between 2007 and 2015 who had follow-up were reviewed. Seventeen patients were included; the median age was 12.7 years. The causes of overgrowth included infiltrating lipomatosis (n = 7), capillary malformation (n = 6), hemihypertrophy (n = 1), infantile hemangioma (n = 1), lipedema (n = 1), and macrocephaly-capillary malformation (n = 1). Forty-seven percent had enlargement of an extremity, 41 percent had facial hypertrophy, and 12 percent had expansion of the trunk. All subjects had a reduction in the size of the overgrown area and improved quality of life. Suction-assisted tissue removal is an effective technique for reducing the volume of the subcutaneous compartment for patients with pediatric overgrowth diseases. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Lipofibromatosis is a slow-growing, childhood soft-tissue neoplasm that is often confused with other conditions. We report a patient with lipofibromatosis causing extremity enlargement at birth. The lesion initially was thought to be a vascular anomaly or lipedema on clinical and MRI examination. When involving the lower extremity, diffuse lipofibromatosis must be differentiated from more common causes of lower limb enlargement in children: lymphatic malformation, lymphedema, or lipedema. Compared with these more frequent conditions, lipofibromatosis usually causes less morbidity. Management of the tumor includes observation or excision. Because complete extirpation of the lesion is difficult, the recurrence rate is high.

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.