Surgical Management of Congenital Lymphedema in Infants and Children

• Of 67 children and infants with lymphedema, 28 had the congenital type. Congenital lymphedema appears during the first few weeks of life, frequently involves more than one extremity, and enlarges at a slower rate than general body growth. The swelling usually becomes less pronounced with age, and no specific therapy is required in two thirds of the patients. Seven of the 28 children had swelling of the upper extremities and a generalized lymphangiopathy syndrome. Subcutaneous lymphangiectomy was performed on ten of 28 patients who had moderate to severe swelling. Those with hand and arm involvement were particularly benefited; however, operations on the dorsum of the foot produced hypertrophic scars in one third of the cases. The operation is deferred until after age 2 years to permit optimal technical repair and to identify those patients whose conditions will improve spontaneously.

(Arch Surg 114:1133-1136, 1979)