A. A. Shah1,3, J. Roberson2, M. Petrosyan2,3, P. Guzzetta2,3 1Howard University College Of Medicine,Surgery,Washington, DC, USA 2George Washington University School Of Medicine And Health Sciences,Surgery,Washington, DC, USA 3Children’s National Medical Center,Surgery,Washington, DC, USA
Introduction: Primary lymphedema presenting in adolescence is known as lymphedema praecox. It is a rare disorder leading to failure of the lymphatic system to drain properly, almost exclusively in the lower extremities. The ensuing limb swelling can be debilitating and associated with adverse physical and psychosocial consequences. Patients are often subjected to a myriad of diagnostic tests which often have little influence on management. The purpose of this study is to review a large cohort of patients with a rare disorder presenting to a high-volume tertiary care children’s hospital, as well as to determine the fiscal impact of testing on these patients.
Methods: A 13-year retrospective institutional review was performed of patients between the ages of 7 and 21 with a diagnosis of lymphedema praecox. Information was obtained on demographic parameters, diagnostic studies performed, pre-morbid conditions, and clinical outcomes. Descriptive analyses were performed. This study was approved by the hospital’s IRB.
Results: Data were extracted from 49 patient records. The median age was 14 (range: 7-21) years. Participants were predominantly female (n=40, 81.6%). Thirty-one patients (64%) were African-Americans, although more than 50% of our outpatients are African-American. 19 patients had bilateral disease. In patients with unilateral disease, 16 were on the right and 14 were on the left. The diagnosis was made on clinical exam only in 14 patients. 19 patients had Doppler ultrasound (US) exams of which in 12 it was the only exam, 3 had US and MRI, 3 had US, MRI and lymphoscintigraphy, and 1 had US and lymphoscintigraphy. 11 patients had only MRI and physical exam for diagnosis. The charges for lower extremity Doppler ultrasound, lymphoscintigraphy, and MRI with contrast were $1,715, $1,269, and $6006 respectively.
Conclusion: We believe that in the adolescent female with physical findings consistent with lymphedema praecox, diagnostic imaging should be limited to a Doppler ultrasound to rule out deep venous thrombus or a mass as the cause of the swelling. Additional imaging does not aide in the diagnosis, but does add to the expense to and inconvenience of the patient and should be avoided.