European Journal of Cardio-thoracic Surgery 32 (2007) 362369 www.elsevier.

com/locate/ejcts

Review

Aetiology and management of chylothorax in adults

Sukumaran K. Nair *, Matus Petko, Martin P. Hayward
Department of Cardiothoracic Surgery, The Heart Hospital, University College London NHS Trust, 16-18 Westmoreland Street, London W1G 8PH, United Kingdom Received 12 February 2007; received in revised form 15 April 2007; accepted 18 April 2007; Available online 18 June 2007

Summary Though rare in incidence, chylothorax can lead to signicant morbidity and mortality. Its occurrence corresponds to increased mortality following esophagectomy. Leakage of chyle and lymph leads to signicant loss of essential proteins, immunoglobulins, fat, vitamins, electrolytes and water. The presence of chylomicrons and a triglyceride level >110 mg/dl in the aspirated pleural uid conrms the diagnosis of chylothorax. Identifying the aetiology using different diagnostic tests is important in planning treatment. While therapeutic thoracentesis provides relief from respiratory symptoms, the nutritional deciency will continue to persist or deteriorate unless denitive therapeutic measures are instituted to stop leakage of chyle into the pleural space. Denitive therapy consists of obliteration and prevention of recurrence of chylothorax. Aggressive surgical therapy is recommended for post-traumatic or post-surgical chylothorax. # 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.
Keywords: Lymph; Thoracic duct; Chylothorax

1. Introduction Chylothorax represents chyle in the pleural cavity. Chyle represents lymphatic uid enriched with fat and its digestive products absorbed by the intestinal epithelium. The abundant presence of chylomicron in the pleural uid aspirate is diagnostic of chylothorax. Chyle is collected and transported by the thoracic duct into the circulation. Damage to the duct leads to leakage of chyle into the pleural space. Obstruction to the centripetal ow of chyle can lead to rupture of the lymphatics and leakage of chyle into the pleural spaces. We discuss the aetiology, clinical features, investigations and management of this rare condition.

2. Anatomy of the thoracic lymphatic system The lymphatic system includes cisterna chyli, thoracic duct, lymph glands, and lymphatic vessels. The thoracic duct most commonly originates from the cisterna chyli. Cisterna chyli is a lymphatic sac located anterior to the second lumbar vertebra and posterolateral to the abdominal aorta. Arising at the upper end of cisterna chyli, the thoracic duct passes posterior to the median arcuate ligament of the diaphragm between the azygos vein and the aorta in the right
* Corresponding author. Address: Hareeld Hospital, Hill End Road, Hareeld UB9 6JH, United Kingdom. Tel.: +44 1895 823737; fax: +44 1895 828666. E-mail address: suku50@hotmail.com (S.K. Nair).

hemithorax, at the level of the diaphragm. The thoracic duct then ascends in the posterior mediastinum immediately anterior to the vertebral column on the right side of the descending thoracic aorta, in the aorto-oesophageal recess. In the lower thorax, it is on the right side of the distal oesophagus, but crosses to the left at the level of the fth or sixth thoracic vertebra. It then ascends posterior to the aortic arch and anterior to the subclavian artery. After exiting the thorax via the superior thoracic aperture in the neck, it forms an arch anterior to the scalenus anterior muscle at the level of the seventh cervical vertebra by turning laterally and downwards to terminate at the junction of the left subclavian and internal jugular veins. The thoracic duct has an approximate length of 3645 cm and a diameter of 23 mm. It drains intestinal chyle to the bloodstream and the lymphatics of the body, except for the right side of the head and neck, right upper limb, right lung, right side of the heart and the convex surface of liver which in turn drain by the right lymphatic duct. Signicant variations to the above-described pattern can occur. Embryologically, the thoracic duct is a bilateral structure and hence many anatomical variations are possible. The pattern described above is true only in about 65% of the population [1]. The thoracic duct duplicates or triplicates itself in more than 40% of the population [14]. These branches may coalesce to form a plexus in the mid portion of the duct and end independently or as one duct [5]. Infrequently, the upper portion of the thoracic duct divides into two branches that drain separately, one in the usual

1010-7940/$ see front matter # 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved. doi:10.1016/j.ejcts.2007.04.024

S.K. Nair et al. / European Journal of Cardio-thoracic Surgery 32 (2007) 362369

363

manner and the other reaching the right subclavian vein [5]. This variation from the normal anatomical pattern explains the incidence of chyle leak despite care and attention the surgeon might have practised in identifying and protecting the main thoracic duct during an operation such as oesophagectomy.

3. Biochemistry of chyle Thoracic duct transports chyle and lymph from the intestines, liver, abdominal wall and lower extremities into the systemic venous system. The primary function of the thoracic duct is the transport of digestive fat into the venous system. Normally, in an adult the volume varies between 10 and >100 ml/kg body weight, depending on diet, intestinal absorption, and degree of physical activity [6]. Almost all the chyle is derived from the intestinal lacteal system, which gives it a characteristic milky appearance [6]. Up to 70% of absorbed dietary fat passes through the intestinal lacteal system. Chyle contains signicant quantities of chylomicron, triglyceride, cholesterol and fat-soluble vitamins. The other constituent, namely lymph, is derived mostly from the gastro-intestinal tract and liver. It contains a combination of lymphatic and gut-derived substances, including lymphocytes, immunoglobulins, enzymes, and products of digestion [7]. The electrolyte content of chyle is similar to that of serum. The thoracic duct transports 6070% of ingested fat to the bloodstream usually at a concentration of about 0.4 6 g/dl. The concentration of protein in chyle is 2.26 g/dl [7]. Chyle also contains from 400 to 6800 white blood cells/ ml. The majority of these are lymphocytes. Table 1 shows the general composition of chyle. Those fatty acids with fewer than 10 carbon atoms in the chain are absorbed directly by the portal venous system. This particular fact forms the basis for the use of medium-chain triglycerides as an oral diet in the conservative management of chylothorax.

implies disruption of the thoracic duct in the majority of instances. Trauma to the thoracic duct is the commonest mechanism of chylothorax. The commonest cause is thoracic surgery, particularly involving dissection of the mediastinum. Central venous catheterisation and similar procedures can lead to extensive venous thrombosis in the neck. This impedes drainage of lymph and chyle into the subclavian veins, followed by thoracic duct drainage, with resultant chylothorax. Often, a latency period of 27 days exists between the time of injury and clinical evidence of chylothorax if the injury is not a major one. This is because lymph accumulates in the posterior mediastinum until the mediastinal pleura ruptures, usually on the right side at the base of the inferior pulmonary ligament. Mediastinal lymphadenopathy can in turn lead to chylothorax. The enlarged lymph nodes compress the lymphatic channels and thoracic duct and impede centripetal drainage of lymphatic ow from the periphery of lung parenchyma and pleural surfaces. This leads to diffuse extravasation or oozing of chyle and lymph into the pleural space. 4.1. Rare mechanisms leading to chylothorax In hepatic chylothorax, chylous ascites crosses the diaphragm and accumulates in the pleural space. The abdominal source of chylothorax can be demonstrated by intraperitoneal injection of a radioisotope (99mTc-sulphur colloid) [8]. Chyloperitoneum results from leakage of chyle into the peritoneal cavity. Chylothorax as a complication of chyloperitoneum has been observed in various clinical conditions. As in hepatic chylothorax, chylous uid from chyloperitoneum can cross over to the pleural space to cause chylothorax. The mechanisms of chylothorax in idiopathic causes of chylothorax, such as Downs syndrome and Noonans syndrome, are unclear. The mechanisms involved behind chylothorax in Noonan syndrome may be multifactorial such as congenital heart disease, coagulation-factor deciency, pterygium colli, and lymphangiomatosis of the pleura, lungs, and chest wall. Yellow-nail syndrome, dened as a triad of slow-growing yellow nails, lymphoedema, and pleural effusion, is due to hypoplastic or dilated lymphatics [9,10]. Bilateral chylothoraces are more common in yellow-nail syndrome.

4. Mechanism of chylothorax The fundamental mechanism behind chylothorax is leakage of chyle into the pleural space. This generally
Table 1 Biochemistry of thoracic duct chyle Component Total fat Total cholesterol Total protein Albumin Globulin Fibrinogen Sugar Electrolytes Cellular elements Lymphocytes Erythrocytes Antithrombin globulin Prothrombin Fibrinogen Concentration (per 100 ml) 0.46 g 65220 mg 2.216 g 1.24.1 g 1.13.6 g 1624 g 48200 g Similar to plasma 4006800 per ml 50600 per ml 25% of plasma level 25% of plasma level 25% of plasma level

5. Effect of chylothorax Loss of chyle and lymph into pleural space can lead to drastic consequences because of the loss of essential proteins, immunoglobulins, fat, vitamins, electrolytes, and water. Large chylothoraces commonly lead to hypovolaemia due to the large volume loss. The rapidity with which decompensation occurs depends on the amount, rate, and duration of chyle loss. In the early stages, the patient may not demonstrate clinical symptoms or signs of loss of chyle. Advanced cases exhibit clinical features of severe malnutrition. Hyponatraemia, acidosis, and hypocalcaemia are the most commonly recognised phenomena [11]. Mortality is high in patients with recalcitrant or untreated chylothorax. While repeated therapeutic thoracentesis provides relief from

364

S.K. Nair et al. / European Journal of Cardio-thoracic Surgery 32 (2007) 362369

respiratory symptoms, the nutritional deciency will continue to persist or deteriorate unless denitive therapeutic measures are instituted to stop leakage of chyle into the pleural space. It is equally important to provide adequate nutritional support replenishing uid loss, vitamin deciencies and electrolyte loss while specic therapeutic measures are planned. Continued loss of proteins, immunoglobulins, and Tlymphocytes into the pleural space leads to immunosuppression [12,13]. Furthermore, B-lymphocyte-mediated immune functions are impaired by prolonged chyle drainage [12,13]. These factors predispose the patient to opportunistic infections [14]. However, infection of a chylothorax itself is very uncommon because chyle is inherently bacteriostatic. The bioavailability of certain drugs could be severely impaired in the presence of signicant chyle leak. Sequestration of drugs in chyle should be recognised early, to prevent subtherapeutic plasma levels in patients undergoing drainage of chylothorax. There are reports of this phenomenon causing subtherapeutic digoxin [15], amiodarone [16] and cyclosporine [17] levels in the serum of patients.

6. Aetiological classication of chylothorax There are a number of classications for chylothorax in the literature [18,19]. Table 2 shows a modied DeMeester classication of chylothorax into congenital, traumatic, neoplastic and miscellaneous [18]. Among traumatic chylothoraces, iatrogenic causes constitute the majority [20]. Thoracic surgery constitutes the majority of iatrogenic causes. Oesophageal resection is perhaps the most common iatrogenic cause of chylothorax, and an incidence of 4% has been noted in the literature [20]. The proximity of the thoracic duct to the oesophagus,
Table 2 Aetiological classication of chylothorax Congenital Atresia of thoracic duct Birth trauma Pleural thoracic duct stula Traumatic Blunt Penetrating Surgical Cervical Excision of lymph nodes Radical neck dissection Thoracic Ligation of patent ductus arteriosus Surgery for coarctation of aorta/aortic aneurysm Post-esophagectomy Surgery for mediastinal tumours Post-pneumonectomy Abdominal Post-sympathectomy Radical lymph node dissection Diagnostic procedures Lumbar arteriography Subclavian vein catheterisation Neoplasms Miscellaneous

presence of collateral channels, and highly variable course leads to its injury during oesophageal and pulmonary resection. Chylothorax is more likely in patients who undergo oesophageal resection by the transhiatal approach than the thoracic approach [21]. Other surgical procedures in the vicinity of the thoracic duct can inadvertently damage the thoracic duct. Laceration of the thoracic duct during catheterisation of the subclavian vein can lead to a chyle leak. Extensive venous thrombosis complicating central venous catheterisation has resulted in bilateral chylothorax and chylopericardium [22]. Thoracic duct injury can occur following hyperextension of the cervical vertebral column and fracture-dislocation of the spine [23]. Isolated thoracic duct injuries as a result of penetrating chest trauma have resulted in chylothorax [24]. Non-iatrogenic causes are responsible for approximately 20% of cases of traumatic chylothorax. Malignant obstruction of the thoracic duct is the commonest cause of nontraumatic chylothorax. Among the neoplastic aetiologies for chylothorax, lymphoma accounts for 70% of cases [20]. One of the less-recognised aetiologies of the chylothorax is hepatic cirrhosis [20]. As mentioned before, chylous pleural effusion results from the transdiaphragmatic passage of chylous ascitic uid. Majority of hepatic chylothoraces are rightsided in occurrence. Primary lymphatic disease is an uncommon cause of chylothorax. Mediastinal megalymphatics, congenital atresia of the thoracic duct [25] and localised chylous leak from the hilum of the lung can all lead to a chylothorax. Chylothorax can also occur as both an early or late complication of mediastinal radiotherapy when administered for a multitude of indications [26]. Lymphangioleiomyomatosis is an uncommon progressive thoracic pathology in females of childbearing age. It results in nodular and diffuse interstitial proliferation of the smooth muscle in the lungs, lymph nodes, and thoracic duct. Twothirds of the patients have chylous pleural effusion [2729]. Gorhams disease, also known as massive osteolysis, is a rare disorder in which vascular channels dilate within medullary bone and destroy the bone [30]. Chylothorax, as a result of leakage of chyle from lymphatic networks, is a lifethreatening complication accompanying Gorhams disease of the thoracic skeleton [31]. Incidence of chylothorax complicating Gorhams disease is about 17% [31].

7. Clinical features of chylothorax In the traumatic variety, the cause is usually obvious. Clinical features are typical of any pleural effusion. Dyspnoea, cough, and chest discomfort are the main symptoms. Pleuritic chest pain and fever are uncommon because chyle is not irritating to the pleural surface. The severity of symptoms depends on the size of the chylothorax. Rapid accumulation of large volume of pleural uid can lead to adverse haemodynamic consequences. In chronic cases, muscle wasting, weight loss, and signs of malnourishment and electrolyte imbalance can occur. The course of the thoracic duct explains why injury to the duct above the level of the fth thoracic vertebra usually produces left-sided chylothorax and injury below that level, a right-sided

S.K. Nair et al. / European Journal of Cardio-thoracic Surgery 32 (2007) 362369

365

chylothorax [19]. Half of all chylothoraces are right-sided, one-third is left-sided, and the remainder are bilateral [32]. Chylothorax may be the initial manifestation in many patients. This mode of presentation is more likely in patients with occult lymphoma or malignancy involving thoracic lymph nodes. Insidious onset of chylous pleural effusion is a common feature in patients with lymphangioleiomyomatosis. Such patients usually present with chest discomfort and dyspnoea, or pleural effusion as an incidental nding on chest radiograph.

8. Investigations In patients with postoperative and post-traumatic pleural effusions, attention should be directed to the anatomical location of surgery or trauma. Persistent drainage of pleural uid via the thoracostomy tube should raise suspicion. The pleural uid may not appear chylous if the pleural uid is mixed with blood or if the patient is fasting. Chest roentgenography with lateral views as well as decubitus views may be helpful in determining the size and location of the chylothorax. In complicated cases, other imaging techniques such as computed tomography (CT) of thorax may be required. CT is useful when chylothorax is associated with trauma, or where an underlying tumour is suspected. Patients with chylothorax and no obvious trauma should undergo CTof the chest to assess the mediastinum and hila for enlarged lymph nodes. Bipedal lymphangiography has been recommended to identify the cause and detect the site and size of the leak [33]. Lymphangiography can help detect anastomotic leaks in the postoperative patients and complete transection or partial laceration of the duct [34,35]. Oral or nasogastric tube feeding of food with high fat content in patients with suspected chylothorax results in a dramatic change in the colour and biochemical constituents of pleural uid [36]. Though in human subjects there is no compelling evidence to favour, a fat meal mixed with methylene blue leads to bluish-green discolouration of the pleural uid in chylothorax, thereby helping at times to localise the leak. Some have recommended this technique as a diagnostic test for chylothorax. However, methylene blue staining of the fat meal may not be helpful in post-traumatic chylothorax since discolouration of pleural uid can also occur in patients with oesophageal perforation. The most important diagnostic step is thoracentesis to obtain a sample of pleural uid for biochemical and other analyses. The appearance of the pleural uid can be misleading because not all chylous pleural effusions appear milky white or whitish. Approximately 50% of patients may demonstrate bloody, yellow or green turbid, serous, or serosanguineous effusions [37]. The usual milky appearance of the effusion may also be seen in pseudochylothorax or in empyema where the purulent uid contributes to the whitish colour. Even though typical chylous uid appears creamy or milky white, the denitive diagnosis of a chylous pleural effusion is based on presence of chylomicrons in the uid. Chylomicrons stain with Sudan III stain and cytological preparations of pleural uid thus stained may help identify chylomicrons. However, quantitative criteria have not been established for the diagnosis of chylous pleural effusion based

on Sudan III stain of cytological preparations. Normally, the diagnosis of chylothorax is based on the biochemical analysis of pleural uid. Lipoprotein analysis of pleural uid will conrm the presence of chylomicrons. Even though the presence of chylomicrons in the pleural uid is synonymous with chylothorax, lipoprotein analysis is not available in all medical centres. In lieu of lipoprotein analysis, quantitation of triglyceride in pleural uid can be used to diagnose chylothorax. Pleural uid triglyceride levels have been used in diagnosing chylothoraxW. Pleural uid triglyceride levels >110 mg/dl, presence of chylomicrons, low cholesterol level, and elevated lymphocyte count are diagnostic of a chylothorax. When the pleural uid triglyceride level is >110 mg/dl, there is <1% chance of it not being chylous, and pleural uid with a triglyceride value of <50 mg/dl has no more than a 5% chance of being chylous. When the triglyceride level is between 55 and 110 mg/dl, a lipoprotein analysis is indicated to detect chylomicrons. Other criteria for chylothorax include a pleural uid to serum triglyceride ratio >1, and a pleural uid to serum cholesterol ratio <1. In hepatic chylothorax, pleural effusion is usually a transudate, and has lower cholesterol (2264 mg/dl) levels than chylous effusions resulting from other causes [8]. In a given patient, the biochemical characteristics of hepatic chylothorax are identical to those of ascitic uid. In hepatic chylothorax, the abdominal source of chylothorax can be demonstrated by scintigraphy after the intraperitoneal injection of a radioisotope (99mTc-sulphur colloid), with the appearance of radioisotope in the pleural cavity within 90 min [8].

9. Pseudochylothorax versus chylothorax Pseudochylothorax, also known as chyliform pleural effusion or cholesterol pleural effusion, is the term applied to pleural uid that mimics true chylous pleural effusion in appearance but lacks the biochemical criteria for chylothorax. In contrast to true chylothorax, it is chronic and may be present for months to years. Pseudochylothorax is more likely to result from long-standing pleural effusion. The uid is yellow or opalescent green and may initially be mistaken for a chylous effusion, an empyema, or both [38]. High cholesterol levels are typical of a pseudochylous pleural effusion. Cholesterol levels are generally >200 mg/dl and may even exceed 1000 mg/dl [39]. The uid may demonstrate rhomboid-shaped cholesterol crystals on microscopy, which do not stain with Sudan III stain [39]. The precise origin of cholesterol in pseudochylothorax is unknown, but it is attributed to the continued breakdown of chronic inammatory cells in a long-standing effusion. Tuberculosis pleural effusions account for approximately 54% of all cases [40]. Rheumatoid arthritis and trapped lung syndrome are rare causes of pseudochylothorax [41]. Pseudochylous pleural effusions are usually sterile and require no treatment, unless they are large and cause progressive respiratory symptoms. In symptomatic patients, management with pleural drainage, alone or in combination with specic therapy is indicated. Complicated cases or

366

S.K. Nair et al. / European Journal of Cardio-thoracic Surgery 32 (2007) 362369

tuberculous pseudochylothorax that leads to progressive respiratory distress may require pleural decortication.

10. Management of chylothorax 10.1. Medical management There is universal agreement to the fact that the nutritional state of the patient worsens early after development of a thoracic duct leak and that this complication should be aggressively attended to. Patients almost always require aggressive nutritional support to reverse hypovolaemia, immunosuppression, and protein and electrolyte deciencies. Once the haemodynamic status and nutritional status are stabilised, attention should be diverted to specic therapy. The general approach to the problem varies in that some clinicians adopt early surgical intervention while others adopt a conservative approach to the problem. Table 3 summarises various treatment modalities practised in chylothoraces. Though conservative approach may have a role to play in small chylothoraces, therapeutic thoracentesis is the initial step in large chylothoraces that cause respiratory distress. Intercostal tube drainage is the preferred method of thoracentesis in most centres. During the period of excessive chyle leak, patients are generally advised nil by mouth or a diet rich in low-fat, medium-chain triglycerides. Mediumchain triglycerides are absorbed directly into the portal circulation. The latter approach resolves approximately 50% of congenital and traumatic chylothoraces [42]. In chronic chylothorax and in patients with rapid loss of nutrients into the pleural space, total parenteral nutrition is indicated [42]. Malignant chylothorax has been treated with radiotherapy and/or chemotherapy, with results that have not been consistently rewarding [43,44]. Generally, radiation therapy to a total dose of 2000 rads controls most cases of malignant chylothorax [44,45]. When surgical closure of the site of chyle leak is not practical, talc pleurodesis is an option in malignant chylothoraces [46]. One report of 19 patients with 24 chylothoraces secondary to lymphoma and refractory to chemotherapy or radiation therapy documented that talc pleurodesis via thoracoscopy under topical anaesthesia and conscious sedation resulted in a 100% success rate in the
Table 3 Summary of treatment modalities in chylothorax Conservative strategy Nil by mouth Medium-chain triglycerides by mouth Total parenteral nutrition Drainage of chylothorax Thoracentesis Intercostal tube drainage Ensuring complete lung expansion Operative strategy Direct ligation of thoracic duct Mass ligation of supradiaphragmatic thoracic duct Pleuroperitoneal shunting Pleurectomy Pleurodesis glue or talc Radiotherapy

prevention of recurrence of chylothorax at 30, 60 and 90 days following the procedure [47]. Other methods to control chylothorax include phrenic nerve crush and reimplantation of the thoracic duct into a vein or re-anastomosis of a torn thoracic duct [48]. Somatostatin or octreotide infusions have been used successfully to reduce intestinal chyle production and secondarily reduce chyle leak [4951]. Somatostatin treatment is an important adjuvant in the conservative management of chyle leak [52,53]. 10.2. Surgical management Aggressive surgical therapy is recommended for posttraumatic or post-surgical chylothorax. Mortality with conservative management of chylothoraces after oesophagectomy approaches 50% whereas with active surgical intervention, it drops to about 10% [21,54]. Many advocate conservative management for a maximum of 2 weeks in the absence of a strong indication for surgery [55,56]. Children who develop chylothorax after oesophageal surgery are more likely to develop overwhelming bacterial and fungal sepsis if a conservative approach alone is instituted [57]. The general consensus is that surgical management should be adopted if medical management fails. The clinical parameters that prompt surgical intervention and the type of intervention vary between centers. In general terms, surgical intervention offers better results than conservative management when the daily chyle leak exceeds 1.5 l in an adult or >100 ml/kg body weight per day in a child [55]. Surgical intervention is usually indicated when the chyle drainage rate is more than 1 l/day for a period more than 5 days [58]. Lymphangiography will help to delineate the anatomy of the lymphatic channels and thoracic duct as well as the site of leak, though this is laborious. Other methods which are helpful to locate the leak includes preoperative subcutaneous injection of 1% Evans blue dye in the thigh or enteral administration of a fat source like olive oil or cream. Methylene blue may be added to the fat source to highlight the site of leak. 10.3. Techniques of ligation of the thoracic duct Basically, if the chyle leak can be identied, direct ligation with nonabsorbable suture should be performed on either side of the leak. If the site of leak is not identiable easily, extensive dissection to nd this is strongly discouraged to minimise trauma and further leaks. Mass ligation of all tissue between the aorta, spine, oesophagus and pericardium is performed above the diaphragmatic hiatus via the right pleural space. This is traditionally performed through a sixth or seventh space thoracotomy. Parietal pleurectomy helps in these cases by promoting pleural symphysis. If the drainage volume is high or chylothorax occurs after an oesophageal operation, early re-operation should be considered. The surgical intervention of choice in thoracic duct injury is thoracic, abdominal or cervical ligation of the thoracic duct [59]. Many surgeons prefer to ligate the thoracic duct at the diaphragmatic level because this procedure has the advantage of stopping ow from any accessory ducts that may not be recognised [60,61].

S.K. Nair et al. / European Journal of Cardio-thoracic Surgery 32 (2007) 362369

367

Thoracoscopic ligation of thoracic duct has been well described [6264]. After enteral feeding of about 50 ml of cream, thoracoscopy is performed under single lung ventilation. The rst port is inserted in the right sixth or seventh intercostal space in the midaxillary line. A 308 scope is inserted through this port and the pleura inspected. A second port in the right eighth posterior intercostal space is used for dissection and division of the inferior pulmonary ligament. A third port in the anterior axillary line superiorly helps instrumentation to retract the lung. The pleural reection is incised above the diaphragm. If the thoracic duct is easily identiable at this stage it should be dissected carefully. A short segment of the duct is excised and the remaining ends clipped. If the duct is not identiable, mass ligation of all tissue as described above is done using clips. The operation is nished with the placement of an intercostals drain. The drain can be removed when the drainage ceases with the patient on a normal diet. Chyle leak following oesophagectomy occurs in about 10% of cases. If detected during the procedure, they should be closed immediately. Most postoperative chylothoraces in this setting do not heal with conservative management. Early surgical closure of the duct is the preferred option. Chylothorax noticed after pulmonary lobar resection is unusual. An initial course of conservative management is

logical if the lung is fully expanded closing the thoracic cavity. If a large residual space remains early surgical closure of the duct is recommended. In patients with unavoidable pleural spaces with a chyle leak, pleuroperitoneal shunts may be appropriate. When the thoracic duct is unidentiable, talc pleurodesis could be tried. This traditional technique has a success rate of 95% and negligible morbidity [62,65]. When the chylothorax is complicated, loculated, or the site of chyle leak cannot be established, pleural decortication and surgical pleurodesis may be indicated. Fibrin glue has also been used to seal chyle leak by inducing pleurodesis [6668]. Prophylactic ligation of the thoracic duct during oesophageal surgery has been recommended by some surgeons to prevent chylous stula [21,69]. Some advocate routine ligation of the thoracic duct, especially with tumours of the midoesophagus [69]. The incidence of post-oesophagectomy chylothorax reduced from 9 to 2.1% when elective ligation of the major thoracic duct was performed in a series of 255 patients [69]. The preferred site for elective ligation is in the upper abdomen or lower thorax, where there is more constant anatomy [59]. In the event that the duct cannot be identied, mass ligation of the tissue with teon-pledgetted non-absorbable sutures between the aorta and azygous vein may be performed [60]. Though this technique was originally

Fig. 1. Working algorithm in the management of chylothorax.

368

S.K. Nair et al. / European Journal of Cardio-thoracic Surgery 32 (2007) 362369 [16] Strange C, Nicolau D, Dryzer S. Chylous transport of amiodarone. Chest 1992;101:5734. [17] Repp R, Scheld H, Bauer J, Becker H, Kreuder J, Netz H. Cyclosporine losses by a chylothorax. J Heart Lung Transplant 1992;11:3978. [18] DeMeester T. The pleura. In: Spencer E, editor. Surgery of the chest. 4th ed., Philadelphia: WB Saunders; 1983. [19] Bessone L, Ferguson T, Burford T. Chylothorax: a collective review. Ann Thorac Surg 1971;12:52750. [20] McWilliams A, Gabbay E. Chylothorax occurring 23 years post-irradiation: literature review and management strategies. Respirology 2000;5:3013. [21] Bolger C, Walsh T, Tanner W, Hennessy T. Chylothorax after oesophagectomy. Br J Surg 1991;78:5878. [22] Kurekci E, Kaye R, Koehler M. Chylothorax and chylopericardium: a complication of a central venous catheter. J Pediatr 1998;132:10646. [23] Silen M, Weber T. Management of thoracic duct injury associated with fracture-dislocation of the spine following blunt trauma. J Trauma Injury Infect Crit Care 1995;39:11857. [24] Worthington M, Groot Md, Gunning A, Oppell UV. Isolated thoracic duct injury after penetrating chest trauma. Ann Thorac Surg 1995;60:2724. [25] Antonetti M, Manuck T, Schramm C, Hight D. Congenital pulmonary lymphangiectasia: A case report of thoracic duct agenesis. Pediatr Pulmonol 2001;32:1846. [26] Renterghem DV, Pauwels R. Chylothorax and pleural effusion as late complications of thoracic irradiation. Chest 1995;108:8867. [27] Carrington C, Cugell D, Gaensler E. Lymphagioleiomyomatosis: Physiologic-pathologic radiologic correlation. Am Rev Respir Dis 1977;116: 97795. [28] Corrin B, Liebow A, Friedman P. Pulmonary lymphangiomyomatosis. A review. Am J Pathol 1975;79:34882. [29] Urban T, Lazor R, Lacronique J, Murris M, Labrune S, Valeyre D, Cordier J. Pulmonary lymphangioleiomyomatosis. A study of 69 patients. Medicine 1999;78:32137. [30] Gorham L, Stout A. Massive osteolysis (acute spontaneous absorption of bone, phantom bone, disappearing bone). J Bone Joint Surg 1955;37A: 9851004. [31] Tie M, Poland G, Rosenow EI. Chylothorax in Gorhams syndrome. A common complication of a rare disease. Chest 1994;105:20813. [32] Goorwitch J. Traumatic chylothorax and thoracic duct ligature. J Thorac Surg 1955;29:46772. [33] Sachs P, Zelch M, Rice T, Geisinger M, Risius B, Lammert G. Diagnosis and localisation of laceration of the thoracic duct: usefulness of lymphangiography and CT. Am J Roentgenol 1991;157:7035. [34] Chavez C, Conn J. Thoracic duct laceration. Closure under conservative management based on lymphangiography evaluation. J Thorac Cardiovasc Surg 1966;51:7248. [35] Ngan H, Fok M, Wong J. The role of lymphography in chylothorax following thoracic surgery. Br J Radiol 1988;61:10326. [36] Robinson C. The management of chylothorax. Ann Thorac Surg 1985;39: 905. [37] Rahman N, Chapman S, Davies R. Pleural effusion: a structured approach to care. Br Med Bull 2005;72:3147. [38] Hillerdal G. Chylothorax and pseudochylothorax. Eur Respir J 1997;10: 115762. [39] Hamm H, Pfalzer B, Fabel H. Lipoprotein analysis in a chyliform pleural effusion: implications for pathogenesis and diagnosis. Respiration 1991;58:294300. [40] Garcia-Zamalloa A, Ruiz-Irastorza G, Aguayo F, Gurrutxaga N. Pseudochylothorax. Report of 2 cases and review of the literature. Medicine (Baltimore) 1999;78:2007. [41] Ferguson G. Cholesterol pleural effusion in rheumatoid lung disease. Thorax 1966;21:57782. [42] Fernandez Alvarez JR, Kalache KD, Grauel EL. Management of spontaneous congenital chylothorax: Oral medium-chain triglycerides versus total parenteral nutrition. Am J Perinatol 1999;16:41520. [43] Dajee H, Woodhouse R. Lymphangiomatosis of the mediastinum with chylothorax and chylopericardium: role of radiation treatment. J Thorac Cardiovasc Surg 1994;108:5945. [44] Johnson D, Klazynski P, Gordon W, Russell D. Mediastinal lymphangioma and chylothorax: the role of radiotherapy. Ann Thorac Surg 1986;41: 3258. [45] Heaton R, Arnold I, Howard N, Guz A. Successful treatment of chylothorax and superior vena cava obstruction by radiotherapy. Thorax 1987;42: 1534.

described by Murphy and Piper [70], it was later popularised by Patterson and colleagues [60]. If the leak is in the upper thorax or neck, ligation of the thoracic duct is performed in the Poiriers triangle, located between the internal carotid artery, arch of aorta, and vertebral column [59]. Pleuroperitoneal shunts have been used but these are of limited help in idiopathic cases [69,71]. Brofman et al. found that pleuroperitoneal shunts were effective in palliation of pleural effusions in yellow nail syndrome [72]. Therapy using externalised pleuroperitoneal shunting in chylothorax after surgical correction of congenital heart disease is considered safe, effective, and minimally invasive [73]. Though attempts have been successfully made by interventional radiologists to cannulate and embolise the leaking thoracic duct, success has been limited [74] and the procedure not reproducible at many centres. 10.4. Prognosis of chylothorax In the past, the mortality due to chylothorax was in excess of 50% [75]. Currently, the morbidity and mortality have improved due to the more aggressive management strategies adopted. Introduction of aggressive therapeutic measures to reverse the adverse effects of chyle loss has led to the lowering of mortality rates for post-traumatic chylothorax [76]. Malignant chylothorax, chronic debilitating chylothorax and bilateral chylothoraces have worse prognosis [77]. The success of managing large chyle leaks involves aggressive nutritional support and early surgical intervention when indicated. A step-wise algorithm shown in Fig. 1 summarises the management of chylothorax in adults.

Reference
[1] Davis M. A statistical study of the thoracic duct in man. Am J Anat 1915;171:212. [2] Pernis V. Variations of the thoracic duct. Surgery 1949;26:8069. [3] Cha E, Sirijintakam P. Anatomic variation of the thoracic duct and visualisation of mediastinal lymph nodes. Radiology 1976;119:458. [4] Anson B. An atlas of human anatomy. Philadelphia: Saunders; 1963. [5] Kinnaert P. Anatomical variations of the cervical portion of the thoracic duct in man. J Anat 1973;115:4552. [6] Paes M, Powell H. Chylothorax: and update. Br J Hosp Med 1994;51: 48290. [7] Zilversmit D. The composition and structure of lymph chylomicrons in dog, rat, and man. J Clin Invest 1965;44:161022. [8] Romero S, Martin C, Hernandez L, Verdu J, Trigo C, Perez-Mateo M, Alemany L. Chylothorax in cirrhosis of the liver: analysis of its frequency and clinical characteristics. Chest 1998;114:1549. [9] Emmerson P. Yellow nails, lymphoedema and pleural effusions. Thorax 1966;21:24753. [10] Samman P, White W. The yellow nail syndrome. Br J Dermatol 1964;76:1537. [11] Servelle M, Nogues C, Soulie J, Andrieux J, Terhedebrugge R. Spontaneous, postoperative and traumatic chylothorax. J Cardiovasc Surg 1980;21:47586. [12] Wasmuth-Pietzuch A, Hansmann M, Bartmann P, Heep A. Congenital chylothorax: lymphopenia and high risk of neonatal infections. Acta Paediatr 2004;93:2204. [13] Orange J, Geha R, Bonilla F. Acute chylothorax in children: selective retention of memory T cells and natural killer cells. J Pediatr 2003;143:2439. [14] Dumont A, Mayer D, Mulholland J. The suppression of immunologic activity by diversion of thoracic duct lymph. Ann Surg 1964;160:37383. [15] Taylor M, Kim S, Vaias L. Therapeutic digoxin level in chylour drainage with no detectable plasma digoxin level. Chest 1998;114:14824.

S.K. Nair et al. / European Journal of Cardio-thoracic Surgery 32 (2007) 362369 [46] Weissberg D, Ben-Zeev I. Talc pleurodesis. Experience with 360 patients. J Thorac Cardiovasc Surg 1993;106:68995. [47] Mares D, Mathur P. Medical thoracoscopic talc pleurodesis for chylothorax due to lymphoma: a case series. Chest 1998;114:7315. [48] Bresadola F, Mannella P, Sortini A, Buccoliero F, Pollinzi V. Internal drainage of the thoracic duct in the surgical treatment of cirrhotic ascites. 3-year follow-up. Minerva Chir 1976;31:10514. [49] Markham K, Glover J, Welsh R, Lucas R, Bendick P. Octreotide in the treatment of thoracic duct injuries. Am Surg 2000;66:11657. [50] Ulibari J, Sanz Y, Fuentes C, Mancha A, Aramendia M, Sanchez S. Reduction of lymphorrhagia from ruptured thoracic duct by somatostatin. Lancet 1990;336:258. [51] Rimensberger P, Muller-Schenker B, Kalangos A, Beghetti M. Treatment of a persistent postoperative chylothorax with somatostatin. Ann Thorac Surg 1998;66:2534. [52] Al-Zubairy S, Al-Jazairi A. Octreotide as a therapeutic option for management of chylothorax. Ann Pharmacother 2003;37:67982. [53] Rosti L, Bini R, Chessa M, Butera G, Drago M, Carminati M. The effectiveness of octreotide in the treatment of postoperative chylothorax. Eur J Pediatr 2002;161:14950. [54] Orringer M, Bluett M, Deeb G. Aggressive treatment of chylothorax complicating transhiatal oesophagectomy without thoracotomy. Surgery 1988;104:72650. [55] Marts B, Naunheim K, Fiore A, Pennington D. Conservative versus surgical management of chylothorax. Am J Surg 1992;164:5324. [56] Selle J, Snyder W, Schreiber J. Chylothorax: indications for surgery. Ann Surg 1973;177:2459. [57] Buttiker V, Fanconi S, Burger R. Chylothorax in children: guidelines for diagnosis and management. Chest 1999;116:6827. [58] Dugue L, Sauvanet A, Farges O, Goharin A, Mee JL, Belghiti J. Output of chyle as an indicator of treatment for chylothorax complicating oesophagectomy. Br J Surg 1998;85:11479. [59] Merrigan B, Winter D, OSullivan G. Chylothorax. Br J Surg 1997;84: 1520. [60] Patterson GA, Todd TR, Delarue NC, Ilves R, Pearson FG, Cooper JD. Supradiaphragmatic ligation of the thoracic duct in intractable chylous stula. Ann Thorac Surg 1981;32:449. [61] Miyamura H, Watanabe H, Eguchi S, Suzuki T. Ligation of the thoracic duct through transabdominal phrenotomy for chylothorax after heart operations. J Thorac Cardiovasc Surg 1994;107:316.

369

[62] Graham D, McGahren E, Tribble C, Daniel T, Rodgers B. Use of videoassisted thoracic surgery in the treatment of chylothorax. Ann Thorac Surg 1994;57:150711. [63] Kent R, Pinson R. Thoracoscopic ligation of the thoracic duct. Surg Endosc 1993;7:523. [64] Janssen J, Joosten J, Postmus P. Thoracoscopic treatment of postoperative chylothorax after coronary artery bypass surgery. Thorax 1994;49:1273. [65] Gingell J. Treatment of chylothorax by producing pleurodesis using iodised talc. Thorax 1965;20:2619. [66] Akaogi E, Mitsui K, Sohara Y, Endo S, Ishikawa S. Treatment of postoperative chylothorax with intrapleural brin glue. Ann Thorac Surg 1989;48:1168. [67] Stenzl W, Rigler B, Tscheleissnigg K, Beitzke A, Metzler H. Treatment of postsurgical chylothorax with brin glue. Thorac Cardiovasc Surg 1983;31:356. [68] Inderbitzi R, Krebs T, Stirneman T, Althaus Y. Treatment of postoperative chylothorax by brin glue application under thoracoscopic view with the use of local anaesthetic. J Thorac Cardiovasc Surg 1994;104:20910. [69] Dougenis D, Walker W, Cameron E, Walbaum E. Management of chylothorax complicating extensive oesophageal surgery. Surg Gynaecol Obstetr 1992;174:5016. [70] Murphy T, Piper C. Surgical management of chylothorax. Am Surg 1977;43:719. [71] Little A, Kadowaki M, Ferguson M, Staszek V, Skinner D. Pleuroperitoneal shunting. Alternative therapy for pleural effusions. Ann Surg 1988;208: 44350. [72] Brofman J, Hall J, Scottea W. Yellow nails, lymphedema and pleural effusion. Treatment of chronic pleural effusion with pleuroperitoneal shunting. Chest 1990;97:7435. [73] Wolff A, Silen M, Kokoska E, Rodgers B. Treatment of refractory chylothorax with externalised pleuroperitoneal shunts in children. Ann Thorac Surg 1999;68:10537. [74] Cope C, Salem R, Kaiser L. Management of chylothorax by percutaneous catheterisation and embolisation of the thoracic duct: prospective trial. J Vasc Interv Radiol 1999;10:124854. [75] Lampson R. Traumatic chylothorax. J Thorac Surg 1948;17:77891. [76] Cerfolio R, Allen M, Deschamps C, Trastek V, Pairolero P. Postoperative chylothorax. J Thorac Cardiovasc Surg 1996;112:13615. [77] Milsom J, Kron I, Rheuban K, Rodgers B. Chylothorax: an assessment of current surgical management. J Thorac Cardiovasc Surg 1985;89:2217.