Practice Essentials
Lymphatic leakage often occurs after disruption of the lymphatic system and is classified as congenital, traumatic, or neoplastic. It may manifest in the following ways:
Lymphatic fistula
Lymphatic fistulas are epithelialized tracts that develop between the lymphatic system and the epidermis as a result of lymphatic injury after retroperitoneal, femoral, or other lymphadenectomy procedures; after infrainguinal reconstruction
; and after aortic aneurysm repairs. They can occur with the lymphatic channels and surrounding structures, such as the bladder, gastrointestinal (GI) tract, uterine cavity, or skin.
Chylous ascites is a collection in the abdomen or retroperitoneum resulting from injury to the thoracic duct or to the para-aortic or mesenteric lymphatics (eg, the cisterna chyli). Several case reports and much smaller clinical series (beginning in 1970) describe the development of chylous ascites after abdominal aortic reconstruction.
Chylothorax is a collection of lymph in the chest, usually resulting from disruption of the thoracic duct. A few case reports describing chylothorax following thoracoabdominal aortic reconstruction were submitted from 1979 onward. A more extensive review began in 1996 with the advent of congenital aortic coarctation repair.
Surgical procedures most commonly resulting in chylous leakage include lymphadenectomy, radical neck dissection, ligation of patent ductus arteriosus, surgery for coarctation of the aorta, aortic aneurysm repair, esophagectomy,
excision of mediastinal tumors, pneumonectomy, and sympathectomy. Chylothorax has been reported after heart-lung transplantation.
Low-output lymphatic fistulas respond to medical therapy, drainage, and parenteral nutrition. Failure of such treatment is an indication for diagnostic and therapeutic lymphangiography. Persistent leakage is an indication for operative repair. High-volume leakage (>1000 mL/day) should be aggressively treated with therapeutic lymphangiography, thoracic duct embolization (TDE), pleurodesis, surgery, or some combination thereof.
High-output chyle leakage is associated with a 50% mortality when surgical intervention is postponed; this is associated with nutritional, immunologic, or metabolic deterioration from large-volume lymph loss.
Optimal therapy for postoperative chylothorax remains controversial. In general, a low-fat diet or bowel rest, medical therapy, total parenteral nutrition (TPN), and drainage are warranted. High-output drainage is addressed early with interventional radiology techniques. Surgery is reserved for persistent drainage that does not respond to lymphangiography or TDE.