Lymphatic Leakage

Updated: May 30, 2023
  • Author: Michael Omidi, MD, FACS; Chief Editor: John Geibel, MD, MSc, DSc, AGAF  more...
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Overview

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 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 [1] ; 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. [2, 3]

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. [4]

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, [5]  excision of mediastinal tumors, pneumonectomy, and sympathectomy. Chylothorax has been reported after heart-lung transplantation. [6]

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. 

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Anatomy

Relevant anatomic considerations include the following:

  • Lymphatic fistula - Demonstration of isosulfan blue leakage within the reexplored infrainguinal incision aids in the repair of a lymphatic fistula; blue droplets appear from the site of lymphatic injury, which may then be suture-ligated, cauterized, or fibrin-glued
  • Chylous ascites - Several large mesenteric lymphatics located on the anteroinferior aspect of the left renal vein, along with the right and left lumbar lymphatics, form the cisterna chyli, which lies between the inferior vena cava and the abdominal aorta at the level of the second lumbar vertebra
  • Chylothorax - The thoracic duct lies to the right of the aorta and to the left of the azygos vein, beginning at the cisterna chyli and entering the posterior mediastinum through the aortic hiatus; in the superior mediastinum, the thoracic duct lies behind the aortic arch and subclavian artery, to the left of the esophagus, and enters the left brachiocephalic vein
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Pathophysiology

With a lymphatic fistula, transection of the infrainguinal lymphatic vessels results in lymphatic leakage. Collection of lymphatic fluid in the groin may result in wound infection, prosthetic graft infection, and lymphocele.

In chylous ascites, lymphatic leakage occurs from the para-aortic or mesenteric lymphatic vessels into a closed compartment (the intra-abdominal cavity); this has much greater implications for postoperative morbidity. When lymphatic leakage outstrips reabsorption, progressive abdominal distention occurs and can subsequently lead to pulmonary compromise. Malnourishment and infectious complications may result from the loss of proteins, fats, and vital immunologic complexes.

With a chylothorax, lymphatic leakage from the thoracic duct also occurs into a closed compartment (the thoracic cavity), inevitably resulting in respiratory difficulty. Malnourishment and infectious complications may also result from such a substantial lymphatic leak.

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Etiology

Etiologic factors contributing to lymphatic fistulas after infrainguinal reconstruction include failure to ligate injured lymphatic vessels and failure to approximate tissue layers meticulously at closure. Other risk factors for the development of lymphatic fistulas include diabetes mellitus, wound infections, reoperation, use of prosthetic grafts for vascular conduits, and excessive postoperative ambulation.

Failure to dissect meticulously around the abdominal aorta and failure to ligate the larger lumbar, para-aortic, and mesenteric lymphatic vessels may result in postoperative chylous ascites. Other causes of chylous ascites include neoplasms (eg, lymphoma), cirrhosis, trauma, congenital lymphatic abnormalities, infections (eg, peritoneal tuberculosis), inflammatory disorders, and surgical procedures (eg, pancreaticoduodenectomy, [7, 8] abdominothoracic esophagectomy, inguinal lymph node resection, and renal transplant). [9]

Inadvertent transection of the thoracic duct results in chylothorax. Like chylous ascites, chylothorax has many potential nontraumatic causes, including malignancy, congenital disorders, and various infectious and inflammatory processes. Congenital causes of chylothorax include thoracic duct atresia, birth trauma, and pleural thoracic duct fistula. Traumatic causes include surgery, blunt trauma, penetrating trauma, and diagnostic procedures (eg, lumbar arteriography and subclavian vein catheterization).

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Epidemiology

In a comprehensive review of lymphatic fistulas after infrainguinal reconstruction, Kalman et al reported an incidence of 1.1%. [1] Smaller series have reported an incidence of 0.8-6.4% after reconstructive procedures.

Chylous ascites can result from abdominal aortic aneurysm (AAA) repair, open cholecystectomy, gynecologic cancer surgery, liver transplant, and lymphatic abnormalities. The majority have been described after open AAA repair, accounting for 81% of all cases of postoperative chylous ascites reported and 38 cases from 1970. [10] The incidence of chylous ascites after AAA repair is 0.03-0.1%. [11]

Chylothorax after surgery involving the mediastinum and neck has a reported incidence of 0.42% for all general thoracic surgery procedures [12, 13] and an incidence of 3.9% for esophagectomies. [12, 14]

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Prognosis

In general, chylous ascites and chylothorax occurring secondary to traumatic injury during vascular reconstruction carry a better prognosis than the same conditions occurring secondary to underlying neoplasia. A mortality of approximately 18% has been reported for chylous ascites developing after aortic surgery. Mortality figures in the range of 44-83% have been reported for chylous ascites developing secondary to an underlying neoplasm.

High-output chylous leakage is associated with a 50% mortality when surgical intervention is not promptly initiated. This high mortality is associated with nutritional, immunologic or metabolic deterioration from large-volume lymph loss; it is reduced to 10% when surgical therapy is initiated in these patients. [15, 12, 16, 17, 18, 10]

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