Primary Lymphedema of the Upper Extremities: Clinical and Lymphoscintigraphic Features in 23 Patients

Abstract

Background: Primary idiopathic lymphedema is an uncommon condition that typically affects the lower extremities. Patients have a malformed lymphatic system that causes subcutaneous fluid and adipose deposition. Rarely, the disease also has been described in the upper extremities. The purpose of this study was to investigate a cohort of patients with primary arm lymphedema to better understand the disease.

Methods: Patients evaluated in our Lymphedema Program between 2008 and 2018 were reviewed for individuals with upper extremity primary lymphedema. Gender, age of onset, morbidity, associated features, and management were identified. Transit of radiolabeled tracer and dermal backflow on lymphoscintigraphy were recorded.

Results: Twenty-three patients of 234 individuals with primary lymphedema had upper extremity disease (9.8%). Eleven subjects were male. Age of onset was infancy (n = 15), adolescence (n = 5), or adulthood (n = 3). The disease affected the left arm (n = 11), right arm (n = 9), or both upper extremities (n = 3). Lymphoscintigraphy in 15 patients exhibited delayed transit of tracer and 2 illustrated dermal backflow. One-half of individuals also had primary lower extremity lymphedema (six unilateral and six bilateral). None of the patients in the cohort exhibited a family history of lymphedema. Two individuals had Turner syndrome. Morbidity included infection (n = 5), other lymphatic anomalies (n = 6), and lymphangiosarcoma (n = 1).

Conclusions: The upper extremities are a rare location for primary lymphedema and patients often also have lymphedema of the legs. Compared with that of the lower extremities, primary disease of the arm is more likely to be associated with systemic lymphatic dysfunction and has a lower risk of familial transmission.

Keywords: arm; extremity; lymphedema; lymphoscintigraphy; primary.