Life-threatening Pulmonary Lymphedema Secondary to Thoracic Duct Ligation

Abstract

We herein report an extremely rare adult case presenting with life-threatening pulmonary lymphedema secondary to generalized lymphedema. A 47-year-old woman with generalized lymphedema from her feet to below her chest, had undergone surgical ligation of the thoracic duct and bilateral pleurodesis for the treatment of intractable idiopathic chylothorax three years earlier. Chest computed tomography demonstrated bilateral ground-glass opacities, air-space consolidation and interlobular septal wall thickening, presenting as a crazypaving appearance predominantly on the gravity side. Bronchoalveolar lavage revealed marked lymphocytosis. She was treated with long-term oxygen therapy with noninvasive positive-pressure ventilation, followed by lymphovenous anastomoses of the lower extremities.

Correction of complete thoracic duct obstruction with lymphovenous bypass: A case report

“…Thoracic duct injury leading to lymphedema is considerably rare compared to chylothorax, but the clinical outcomes can be devastating. The patient may develop severe swelling of the limbs that is refractory to compression therapy and diuretics, and in extreme cases the patient may develop life threatening volume overload and pulmonary lymphedema (Ohnishi et al, ). In our patient, this manifested as dramatic swelling of the extremities and weight gain over a one year period that impaired the patient's immune function, respiratory status, mobility, and quality of life.…”

“…Typically, ligation of the thoracic duct does not lead to secondary lymphedema and collateralization of the lymphatic flow may be sufficient within 2 weeks (as evidenced by Ehrenhaft's studies of normalization of blood lipoprotein levels) (Ehrenhaft & Meyers, ). However, ligation of the thoracic duct has been reported to cause generalized secondary lymphedema (particularly to the lower extremities) (Ohnishi, Yamane, Shiota, & Yokoyama, ), and compared to chylothorax, treatment options are not well defined. We here present a case of thoracic duct bypass for treatment of secondary lymphedema after iatrogenic injury of the thoracic duct.…”