title: Management of Primary and Secondary Lymphedema: Analysis of 225 Referrals to a Center.
abstract: BACKGROUND
  Lymphedema is the chronic, progressive enlargement 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 2013 were reviewed. Diagnosis was determined based on history, physical examination, photographs, and imaging studies. Lymphedema type (primary or secondary), location of swelling, patient age, sex, and previous management were documented. The accuracy of referral diagnosis and the geographic origin of the patients also were analyzed.
  
  
  RESULTS
  Two hundred twenty-five patients were referred with a diagnosis of "lymphedema"; 71% were women and 29% were children. Lymphedema was confirmed in 75% of the cohort: primary (49%) and secondary (51%). Twenty-five percent of patients labeled with "lymphedema" had another condition. Before referral 34% of patients with lymphedema received tests that are nondiagnostic for the disease, and 8% were given a diuretic which does not improve the condition. One third of patients resided outside our local referral area. The average time between onset of lymphedema and referral to our Lymphedema Program was 7.7 years (range, 1-59 years).
  
  
  CONCLUSIONS
  Patients presenting to a center with "lymphedema" often have another condition, and may be suboptimally managed before their referral. Patients with suspected lymphedema should be referred to specialists focused on this disease.
authors.affiliation: From the *Department of Plastic and Oral Surgery, and †Division of Nuclear Medicine and Molecular Imaging, Department of Radiology, Lymphedema Program, Boston Children's Hospital, Harvard Medical School, Boston, MA.
authors.forename: Reid A
authors.initials: RA
authors.lastname: Maclellan
authors.forename: Rafael A
authors.initials: RA
authors.lastname: Couto
authors.forename: Jorie E
authors.initials: JE
authors.lastname: Sullivan
authors.forename: Frederick D
authors.initials: FD
authors.lastname: Grant
authors.forename: Sumner A
authors.initials: SA
authors.lastname: Slavin
authors.forename: Arin K
authors.initials: AK
authors.lastname: Greene
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D003937:Diagnosis, Differential; D003951:Diagnostic Errors / Q000706:statistics & numerical data; D005260:Female; D006801:Humans; D008209:Lymphedema / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D012017:Referral and Consultation; D013038:Specialization; D014481:United States; D055815:Young Adult