Chyle fistula is defined as a leakage of lymphatic fluid from the lymphatic vessels, typically accumulating in the thoracic or abdominal cavities but occasionally manifesting as an external fistula. It is a rare but potentially devastating and morbid condition.
First described in the 17th century as complications of trauma, chyle fistulas most commonly occur secondary to lymphatic disease or malignancy or following abdominal, neck, or thoracic operations.[1, 2] Chyle fistulas also can form as a result of venous hypertension, and they have been described in patients with superior vena cava syndrome or thrombosis of the vena cava.
Patients with chyle fistulas usually give histories of some comorbid conditions such as malignancy or prior operations of the chest, neck, or abdomen. In postoperative patients, symptoms become evident after the start of oral feeding and depend on the site of obstruction.[3]
Chyle fistulas can cause extreme morbidity because of loss of fluids, electrolytes, and other nutrients. In addition, chyle fistulas can result in loss of lymphocytes and immune dysfunction. Finally, chyle fistulas are space-filling and exert pressure on surrounding tissues, creating symptoms that can range from minimal discomfort to life-threatening situations.
Treatment of chyle fistulas is determined by the following (see Treatment):
The cisterna chyli and thoracic duct drain lymph from the entire body except the head, neck, arms, and right thorax (which instead use the right bronchomediastinal, jugular, and subclavian lymph trunks to form the right lymph duct). The anatomy is highly variable; 50% of people do not have an identifiable cisterna chyli. In addition, half of the 4 L of lymph draining through the cisterna chyli and thoracic duct originates from the intestinal and hepatic lymphatics.
The cisterna chyli is found on the posterolateral edge to the right of the aorta at the level of vertebral bodies T12, L1, L2, and L3 as lymphatics from the mesentery, intercostal, and lumbar regions coalesce in the retroperitoneal space.
At approximately L1, the cisterna chyli ascends, becoming the thoracic duct (see the image below). The duct then enters the posterior mediastinum, crosses at T4 into the left retropleural space, and continues in a cephalad direction. The thoracic duct then enters the venous system at the junction of the left subclavian and internal jugular veins).
Leakage of lymph from damaged lymph vessels is common after surgery or trauma.[1] However, damaged lymphatics most often heal spontaneously or direct lymph centrally via rich interconnected lymphatic collaterals, without any significant morbidity. For chyle fistulas to form, either a scarcity of lymphatic collaterals must be present or the injury to the lymphatic channels must overwhelm the remaining lymphatic vessels. In addition, abnormal lymphatic vessels may be incapable of adequate lymph flow, leading to the accumulation of chyle.
Chyle flow varies dramatically depending on the quantity and quality of oral intake. During times of starvation, chyle flow is minimal. After meals, especially those with high contents of long-chain fatty acids, chyle flow increases dramatically. This basic knowledge provides the rationale for controlling dietary intake as part of the treatment of this disease.
Chyle fistulas are rare events. Typical causes include the following[1, 3] :
Because of the relatively low incidence of chyle fistulas, a high index of suspicion is required to make a timely diagnosis. Diagnosis usually is made after the patient has recovered from the injury and has started eating.[9]
Symptoms of chyloperitoneum include the following:
Symptoms of chylothorax include the following:
The characteristics of thoracocentesis or paracentesis aspirate are as follows:
Treatment of chyle fistulas is determined by the following:
Approaches to the management of chyloperitoneum and chylothorax are outlined in the images below.
Nutritional intervention remains the mainstay of nonoperative treatment.[9, 12, 13, 14, 15] It includes the following:
Several case reports and case series have reported the successful use of octreotide to treat chylous leaks in infants.[17, 18, 19, 20] One series reported 100% closure of chylous leaks from malignancy when 50 μg of subcutaneous octreotide was given three times per day. A retrospective study[21] and a prospective study[22] found octreotide to be effective in managing chylous leaks after neck dissection.
A retrospective case series by Chan et al suggested that chylous fistulae may be managed conservatively with octreotide and TPN but noted that long-term data would be needed to determine if and when surgical intervention is required for control.[23]
In a systematic review and meta-analysis comparing octreotide with oral dietary modification (ODM) for the treatment of chyle fistula after neck dissection, Molena et al found that whereas octreotide was associated with a high rate of spontaneous resolution, the available evidence was insufficient to allow universal recommendation of this option.[24] Additional data from randomized controlled trials would be required to determine whether octreotide has an independent treatment effect.
Although development of a chylous fistula is unusual after axillary dissection, one study of radical breast cancer procedures shows that they are manageable through conservative methods of treatment.[25]
Transjugular intrahepatic portosystemic shunts (TIPS) have been reported to successfully treat chylous ascites due to cirrhosis.[26]
A period of observation from one to several weeks should be allowed before determining whether these therapies are successful.
Repeated paracentesis/thoracentesis should be avoided, for the following reasons:
When accumulation of chyle causes a patient significant problems, such as shortness of breath and lung collapse, drainage through a thoracostomy tube or paracentesis may be necessary despite the obvious adverse effects that this form of therapy may produce.[27]
There is some evidence to suggest that negative-pressure wound therapy (NPWT) may be a potential treatment option for both high- and low-volume chylous fistulas after neck dissection.[28]
What the duration of medical therapy should be and when the decision should be made to proceed operatively are dependent on the individual physician. Because of the relative rarity of chyle fistulas, no definitive data are available to unequivocally direct the duration of therapy.
Surgery is undertaken when conservative therapies fail (~40% of cases).[29, 30] Surgical approaches vary significantly, depending on the site and etiology of the leak. A throacoscopic surgical approach to the thorax may be tried, provided that adequate expertise is available.[27]
If at all possible, ensure nutritional assessment and repletion before a surgical intervention; malnutrition increases morbidity significantly.[9] Administer gram-positive antibiotic coverage with a beta-lactamase inhibitor in the 24 hours prior to insertion of the shunt.
Because of the possible complication of disseminated intravascular coagulopathy (DIC), which is especially prevalent in patients with malignancy, include coagulation parameters in the preoperative evaluation.
Contraindications to surgical correction of chyle fistulas are based on the patient's comorbidities and his or her ability to tolerate surgery.
Surgical ligation generally can be successfully accomplished, provided that the site of the leak is identified and the primary pathology causing the leak has not caused disruption or blockage of other lymphatic vessels. Finding the leak may be aided by the use of scintigraphy. In some cases, the exact location of the disrupted lymphatic vessel is not identified, and surgeons may use flaps (eg, muscle flaps) to cover the area where the leak is occurring. Laparoscopic ligation of chyle leaks has been described.
Other forms of therapy to plug the leak have included fibrin glue and the use of chemical irritants such as tetracycline.
Interventions that may be considered in patients with lymphatic fistulas include the following[31] :
The use of peritoneovenous shunts (Denver or LeVeen) is a modality of treatment available when other treatments fail. Peritoneovenous shunts are especially useful for the management of chyle fistulas resulting from causes other than prior operations. The main objective of peritoneovenous shunts is to drain the chyle accumulated in the abdomen back to the venous circulation.
Denver valves use a slit system that reduces flow by impedance. Insertion of these shunts can be achieved as a minimally invasive procedure, and they can be inserted under local anesthetic, sparing the severely ill patient the trauma of a major operation.
The LeVeen valve is a closed-pressure system that opens at pressures of 3 cm H2O or less. This prevents leakage of blood into the tubing, thereby decreasing the chances of the system clotting (with subsequent failure).
Antibiotic coverage is continued empirically for 4 days after surgery. Any coagulopathy should be treated aggressively without delay.[3, 32] Patients should be monitored routinely until the resolution of the fistula. Postoperative visits should commence approximately 1 week after the patient is discharged from the hospital.[3]
Treatment of chyle fistula is very successful. However, unless the underlying etiology is reversed, the problem can be chronic and unrelenting.
If left untreated, chyle fistulas can be fatal, with patients dying from severe fluid and electrolyte abnormalities, malnutrition, and overwhelming infections, including peritonitis and empyema. Iatrogenic complications also can occur,[33] as well as complications from shunt placement, including DIC, shunt failure, and fluid overload.
Occasionally, when chyle leak is not apparent during surgery, ligation of the bed of the thoracic duct does not stop the leak. In three patients with chylothorax, pleurodesis was achieved using continuous pleural irrigation with minocycline versus intermittent pleurodesis.[34]