Management of Primary and Secondary Lymphedema: Analysis of 200 Referrals to a Center

Background: Lymphedema is the chronic, progressive swelling of tissue due to inadequate lymphatic function. Although lymphedema is a specific condition, patients with a large extremity are often labeled as having “lymphedema”, regardless of the underlying cause. The purpose of this study was to characterize referrals to a center to determine if lymphedema should be managed by specialists.

Methods: Patients treated in our Lymphedema Program between 2009 and 2012 were reviewed. Diagnosis was determined based on history, physical examination, photographs, and imaging studies. Lymphedema type (primary, secondary), location of swelling, patient age, gender, and previous management were documented. The accuracy of referral diagnosis and the geographic origin of the patients also were analyzed.

Results: Two hundred patients were referred with a diagnosis of “lymphedema”; 72% were female and 29% were children. Lymphedema was confirmed in 71% of patients: primary (49%) and secondary (51%). Lymphedema was more common in females (67%). Primary lymphedema was more likely to affect the lower extremity (75%) than either the upper extremity (10%) or multiple sites (15%), while secondary lymphedema was more evenly distributed between the upper (41%) and lower (49%) extremities. Twenty-nine percent of patients labeled with “lymphedema” had another condition: lipedema (6%), obesity (8%), venous stasis (7%), musculoskeletal trauma (3%), rheumatologic (2%), or other (3%). Prior to referral only 5% of the cohort underwent a lymphoscintigram (the gold standard diagnostic test for lymphedema), while 35% of the lymphedema patients received unnecessary tests before being referred. More than 30% of patients resided outside of our local referral area. The average time between onset of lymphedema and referral to our Lymphedema Program was 8.3 years (range, <1 – 59 years).

Conclusions: Patients referred to a center with “lymphedema” often have another condition. Individuals with lymphedema often live with this disease for years before receiving interdisciplinary care, many have previously received incorrect treatment. Patients with suspected lymphedema should be referred to specialists focused on this disease.