Diagnosis and treatment of venous lymphedema

Chronic venous disease (CVD) is a common cause of secondary lymphedema. Venous lymphedema is sometimes misdiagnosed as primary lymphedema and does not receive optimal treatment. We have routinely used intravascular ultrasound (IVUS) imaging in all cases of limb swelling. The aim of this study is to show that (1) routine use of IVUS can detect venous obstruction missed by traditional venous testing, and (2) iliac-caval venous stenting can yield satisfactory clinical relief and can sometimes reverse abnormal lymphangiographic findings.

The study comprised CVD patients who underwent iliac vein stenting. Lymphangiography was abnormal in 72 of 443 CEAP C(3) limbs, with leg swelling as the primary complaint (abnormal lymphangiography group). Clinical features and stent outcome were compared with a control group of 205 of 443 with normal lymphangiography (normal lymphangiographic group).

Clinical features were a poor guide to the diagnosis of lymphedema. Isotope lymphangiography was not helpful in differentiating primary from secondary lymphedema. Venography had 61% sensitivity to the diagnosis of venous obstruction. IVUS had a sensitivity of 88% for significant (≥50% area stenosis) venous obstruction. At 40 months, cumulative secondary stent patency was similar for the abnormal (100%) and normal lymphangiographic (95%) groups. Swelling improved significantly after stent placement in the abnormal lymphangiographic group (mean [standard deviation] swelling grade improvement 0.8 ± 1.1) but was less (P < .004) than in the control group (1.4 ± 1.3). Complete swelling relief was 16% and 44% (P < .001) and partial improvement (≥1 grade of swelling) was 45% and 66% (P < .01) in the abnormal and normal lymphangiographic groups, respectively. Associated pain was present in 50% and 36% of the swollen limbs in the abnormal and normal lymphangiographic groups. Pain relief (≥3 visual analog scale) at 40 months was 87% and 83%, respectively (P = .3), with 65% and 71%, experiencing complete pain relief. Quality of life criteria improved after stent placement in both groups but to a better extent in the normal lymphangiographic group. Abnormal lymphangiography improved or normalized in 9 of 36 (25%) of those tested after stent correction.

Prevailing practice patterns and diagnostic deficiencies probably result in the misdiagnosis of many cases of venous lymphedema as "primary" lymphedema. IVUS is recommended to rule out venous obstruction as the associated or initiating cause of lymphedema. Iliac venous stenting to correct the obstruction has excellent long-term patency and good clinical outcome, although results are not as good as in those with normal lymphatic function.