Current Concepts in the Management of Primary Lymphedema

Abstract

Primary lymphedema is a heterogeneous group of conditions encompassing all lymphatic anomalies that result in lymphatic swelling. Primary lymphedema can be difficult to diagnose, and diagnosis is often delayed. As opposed to secondary lymphedema, primary lymphedema has an unpredictable disease course, often progressing more slowly. Primary lymphedema can be associated with various genetic syndromes or can be idiopathic. Diagnosis is often clinical, although imaging can be a helpful adjunct. The literature on treating primary lymphedema is limited, and treatment algorithms are largely based on practice patterns for secondary lymphedema. The mainstay of treatment focuses on complete decongestive therapy, including manual lymphatic drainage and compression therapy. For those who fail conservative treatment, surgical treatment can be an option. Microsurgical techniques have shown promise in primary lymphedema, with both lymphovenous bypass and vascularized lymph node transfers demonstrating improved clinical outcomes in a few studies.

Keywords: congenital lymphedema; lymphovenous anastomosis; lymphovenous bypass; primary lymphedema; vascularized lymph node transfer.

Figure 1

A patient with primary left lower extremity lymphedema that developed during adolescence.

Figure 2

Typical dorsal foot lymphedema. Accumulation of lymph with tissue overgrowth of the dorsal foot eliminates natural creases and precludes the pinching of the dorsal foot skin between the examiner’s fingertips (“stemmer sign”).

Figure 3

Management algorithm for primary lymphedema.

Figure 4

Lymphaticovenous bypass. End-to-side anastomosis between the lymphatic channel and a local vein used as a physiologic treatment for obstructive primary lymphedema.

Figure 5

Vascularized lymph node transfer. VLNT harvested from the jejunal mesentery based on branches from the superior mesenteric artery used as a physiologic treatment for aplastic primary lymphedema.