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(Chest. 1999;116:682-687.)
© 1999 American College of Chest Physicians

Chylothorax in Children*

Guidelines for Diagnosis and Management

Vera Büttiker, MD; Sergio Fanconi, MD and Réne Burger, MD

* From the ICU, University Children's Hospital, Zürich, Switzerland.

Correspondence to: Vera Büttiker, MD, Steinwiesstr. 75, CH- 8032 Zürich, Switzerland; e-mail: BuettikerV{at}compuserve.com


    Abstract
 TOP
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
 References
 
Study objective: To establish guidelines for the diagnosis and management of chylothorax in children.

Design: Retrospective study.

Patients: Fifty-one patients with a diagnosis of chylothorax. Twelve patients were excluded because of incomplete data or incorrect diagnosis. The following parameters were analyzed: triglyceride level, total cell number, and lymphocyte percentage; amount of pleural effusion on day of diagnosis, day 5, and day 14; and total time of pleural effusion. Prospectively, the same parameters were analyzed in a control group of 10 patients with pleural drainage.

Intervention: Patients with chylothorax were treated primarily with fat-free oral nutrition; if chyle did not stop, total parenteral nutrition with total enteric rest was started. If conservative therapy was not successful, pleurodesis was performed.

Results: In children with chylothorax triglyceride, triglyceride content ranged from 0.56 to 26.6 mmol/L; all values except one were > 1.1 mmol/L. In 36 of 39 patients (92%), the cell count was > 1,000 cells/µL. In 33 of 39 patients (85%), lymphocytes were > 90%. In patients without chylothorax triglyceride, triglyceride levels ranged from 0.1 to 0.71 mmol/L (median, 0.38 mmol/L) and cell count was from 20 to 1400 cells/µL (median, 322 cells/µL), with a maximum of 60% lymphocytes. With fat-free nutrition, chyle disappeared in 29 of 39 patients. Five patients died, and five required pleurodesis.

Conclusions: Pleural effusion in children is chyle when it contains > 1.1 mmol/L triglycerides (with oral fat intake) and has a total cell count >= 1,000 cells/µL, with a lymphocyte fraction > 80%. Chylous effusions usually last long; however, after 6 weeks, the majority of the effusions (29 of 39 patients) had ceased. Late surgical interventions reduce the number of thoracotomies substantially, but can lead to very long hospitalization times. Early surgical interventions (after < 3 weeks) lead to a high number of thoracotomies, but certainly reduce hospitalization time.

Key Words: chyle • chylothorax • infants • newborn • pleural effusion • pleurodesis


    Introduction
 TOP
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
 References
 
In childhood, chylothorax is usually a postoperative complication, mainly occurring after cardiothoracic interventions or caused by thrombosis of the left or right subclavian vein. It is rarely due to the malformation of the pulmonary or thoracic lymphatic system that is associated with dysmorphic syndromes.1 2 3 In adults, common causes of chylothorax are thoracic or neck trauma or a malignancy at the upper thoracic aperture.4 5 6

The definition of chyle in adults is well established in the literature.5 For children, no clear definition exists and very often adult values are applied.4 5 7 One reason for this is the small number of chylothoraces in this age group. Values derived from adult patients are not necessarily applicable to children. The aim of this study was to define the content and distribution of cells and values of triglyceride concentration for chyle in pediatric patients. This should help distinguish between chyle and pleural fluid of a different etiology. Because therapy for chylothorax is often long and difficult, a precise diagnosis is mandatory.


    Materials and Methods
 TOP
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
 References
 
We retrospectively analyzed all patients with the diagnosis of chylothorax in our hospital database from the years 1985 to 1996. There were a total of 51 patients having this diagnosis, but we had to exclude 10 patients because of insufficient documentation. An additional two patients were excluded because an analysis of the available data dismissed the diagnosis of chylothorax.

The diagnosis of chylothorax was made according to the proposal of Staats et al4 and Straaten et al7 : triglyceride levels in pleural fluid had to be > 1.2 mmol/L, with a total cell number > 1,000 cells/µL and a predominance of mononuclear cells. The main diagnoses of the remaining 39 patients are presented in Table 1 .


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Table 1. Primary Diagnosis of Patients With Chylothorax

 
The following parameters were recorded: triglyceride level, total cell number, and content of lymphocytes in pleural effusion; amount of fluid on the day of diagnosis, and at day 5 and 14; and duration of pleural effusion after the initiation of therapy. Additionally, after feeding five patients a formula containing fat, we analyzed the change of triglyceride content. Therapy consisted of a diet with a fat-free formula. Total parenteral nutrition (TPN) with total enteric rest was begun when the diet was unsuccessful (after 2 to 3 weeks); surgery was performed when TPN was not effective. The length of each therapeutic modality was recorded.


    Results
 TOP
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
 References
 
There were 29 boys and 10 girls with the diagnosis of chylothorax. Their ages ranged from 5 days to 10.5 years old (median, 2.1 years old). The 10 patients in the control group were from 6 days to 7 years old (median, 1.3 years old). All received regular formula.

Thirty-three patients (85%) developed chylothorax after cardiac surgery. From the remaining six patients, two developed chylothorax after pneumonia. One of these patients had Down's syndrome; the second patient was a newborn baby with bilateral pneumonia and a consecutive bilateral pneumothorax. Two girls had idiopathic chylothorax. In one, the manifestation was at the age of 10 weeks. The other girl presented at the age of 3 months with chylothorax and then developed chyloascites and chylopericard; she died at 6 months of age. From the remaining two patients, one had chylothorax following an operation for a diaphragmatic hernia, and the other had esophageal atresia. Five patients died because of underlying disease and not because of complications resulting from chylothorax (Table 2 ): three had a severe congenital cardiac malformation with no satisfactory surgical correction; one developed a Budd-Chiari syndrome and finally died; and one died of acute respiratory distress after the aspiration of stomach contents.


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Table 2. Cause of Death in Five Patients

 
In 19 patients, chylothorax was on the right side; in 12 patients, it was left sided; and in 8 patients, it was bilateral.

Triglyceride content ranged from 0.56 to 26.6 mmol/L (Fig 1 ). All values except one were > 1.1 mmol/L: the one patient with a triglyceride level of 0.56 mmol/L was never fed a formula containing fat. The maximum value (26.6 mmol/L) was found in one patient with idiopathic chylothorax. After the ingestion of only a small amount of milk (six 10- to 15-mL formulas per day), the triglyceride concentration in five patients with chylothorax increased to > 1.1 mmol/L (Fig 2 ).



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Figure 1. Triglyceride concentration in pleural fluid. Each point represents one patient. There is a clear difference between patients with chylous effusion and those with nonchylous effusion.

 


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Figure 2. Triglyceride concentration in pleural fluid in five infants without enteral feeding and suspected chylothorax. Feeding small amounts of a formula increased triglyceride content and confirmed the diagnosis.

 
Triglyceride levels in 10 patients without chylothorax were measured for comparison; triglyceride content ranged from 0.18 to 0.71 mmol/L (median, 0.38 mmol/L; Fig 1 ).

In 36 of 39 patients (92%) with chylothorax, total cell count was > 1,000 cells/µL (Fig 3 ). In only 3 of 39 patients (7%), total cell count was < 1,000 cells/µL. In 33 of 39 patients (85%), lymphocytes were > 90%; in the remaining 6 patients, lymphocytes ranged from 57 to 89%. Total cell count of the control group ranged from 20 to 1400 cells/µL (median, 322 cells/µL), and lymphocytes ranged from 10 to 60% (Fig 3) .



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Figure 3. Total number of cells and lymphocytes. In chylous effusion, the fraction of lymphocytes is > 80%. Note the logarithmic scale.

 
The median amount of chyle at time of diagnosis was 214 mL (range, 55 to 730 mL).

After thoracotomy, chylothorax developed between postoperative days 1 to 18. With a fat-free formula, chyle disappeared within 14 days in 15 of 39 patients (38%). After 45 days of conservative therapy, effusion had stopped in 30 of 39 patients (77%). In two patients, chylous effusion ceased only after 50 and 51 days, respectively; two patients died after this time. In five patients (13%), pleural effusion lasted > 10 weeks and a pleurodesis was performed (Fig 4 ); this was successful in all patients. Effusion ceased 3 to 5 days after surgery.



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Figure 4. Duration of pleural drainage and time of pleurodesis. Squares indicate the number of days until the pleural effusions stopped. Plus signs indicate the number of days until the patient died. Circles indicate the number of days until pleurodesis was performed.

 

    Discussion
 TOP
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
 References
 
Chyle is lymphatic fluid enriched with fat secreted by intestinal cells. It is collected and then transported via the thoracic duct into the circulation. Measuring fat content and demonstrating the predominance of lymphocytes in pleural effusion serves to prove chyle. We used the definition of Staats et al4 to define chyle in our patients.

In our 39 patients, the diagnosis of chylothorax was confirmed by pleural effusion with a triglyceride level > 1.1 mmol/L and a cell count >1,000 cells/µL with a predominance of lymphocytes. Because oral fat intake, especially in infants, consists mainly of triglycerides and almost no cholesterol, it is preferable to measure triglyceride content in pleural effusion when chyle is suspected.

In the literature, it is reported1 6 8 9 10 11 that chylothorax usually occurs in newborn and pediatric patients as a complication of thoracic and cardiac surgery. This was also the case in 33 of our 39 patients. One patient had congenital chylothorax, probably as a result of birth trauma or caused by lymphatic malformation. This form of chylothorax is rarely described in the literature.7 12 13 Chylothorax is also described in children with trisomy 21 and Noonan's syndrome; in both instances, vascular and lymphatic malformations are found.3 Only one patient in our series had trisomy 21 and chylothorax develop after pneumonia. In older children and adults, nontraumatic chylothoraces that are caused by an obstruction of the thoracic duct secondary to fibrosis or tumors are described.6 In these cases, the spontaneous resolution of chylous leaks rarely occurs. Postoperative chylothoraces obviously have a traumatic origin; in our patients, they developed between day 1 and day 18 after thoracotomy. This has been also widely reported in the literature.1 6 9 10 14 There is a temporal relation between the time of diagnosis and the initiation of oral fat intake.

With one exception, all of our patients had a triglyceride content > 1.1 mmol/L. This is similar to the findings of Staats et al,4 who reported triglyceride levels >= 1.2 mmol/L in adult patients with chylous effusion. Our patient, who had a triglyceride level of 0.56 mmol/L and a diagnosis of chylothorax, had no oral fat intake at time of diagnosis; this child suffered massive effusion after repair of a congenital diaphragmatic hernia with intraoperative injury of the thoracic duct. In this situation, feeding the patient a formula containing fat was regarded as unnecessary. After cardiac surgery, especially in newborn infants, oral feeding is often delayed, but small amounts of formula were usually sufficient to allow the diagnosis of chylothorax. Without oral fat intake, the distinction between chylous and nonchylous effusion is difficult or even impossible to determine. On the other hand, in 13 patients, triglyceride content was > 1.1 mmol/L without oral fat intake; these children all had surgery at >= 3 months of age and had normal oral fat intake preoperatively. Obviously, their bowel still contained enough fat to allow the diagnosis. It seems, especially in newborn infants, that a minimal oral fat intake is mandatory to allow the diagnosis of chylothorax.

Staats et al4 and others5 also noted that a milky aspect, first described by Wallis and Schölberg15 in 1911, can be helpful in diagnosis. In fact, chylous effusion often has a turbid aspect; however, in our opinion, this can also be misleading: 2 of our 10 control patients without chylothorax had turbid effusions, but triglyceride content was only 0.12 mmol/L and 0.50 mmol/L, respectively. The milky aspect can, therefore, only serve as a hint for further investigations; it is not proof that the effusion is chylous.

The aspect of effusion also depends on total cell content. In almost all of our patients with chyle (92%), total cell count was > 1,000 cells/µL. In two thirds of our patients with chyle (69%), the lymphocyte fraction was > 90%; in the remaining patients, it was > 80%. Three patients had a total cell count < 1,000 cells/µL, but a high triglyceride level despite low oral fat intake. This low fat intake was the only common feature we could find as a possible explanation for the low cell number. Patients with lymphocytes between 80% and 90% of the total count had an increased number of polymorphonuclear granulocytes because of a possible irritation of the pleura by the operation or the drainage. In patients without chylothorax, lymphocytes were always < 60% of the total cell count.

Usually, initial therapy for postoperative chylothorax has been pleural space drainage, use of medium chain triglyceride (MCT) oil, fat-free oral alimentation, or enteric rest with TPN.2 9 14 16 17 In our patients, a fat-free formula consisting of proteins and starch was given; if effusion did not abate after 2 to 3 weeks, TPN was started together with total enteric rest. MCT oil was not used in our institution, as Pieterson and Jakobson18 demonstrated substantial increases of triglyceride content in pleural effusion when MCT oil was given.

The time of surgery usually is not defined as uniformly as is the initial therapy. Some authors1 8 recommend surgery if effusion persists for > 2 weeks; others1 8 19 regard an amount > 100 mL per year of age in children as an indication for surgery. Most authors, however, recommend an extended period of conservative management and do not proceed to surgical treatment until > 4 weeks of pleural effusion.10 11 14 Operative procedures include pleurodesis: the ligation of the main duct with adjacent leaking lymphatics.2 10 14 19 The right chest approach for duct ligation is not always successful because variations in ductal anatomy allow the lymph to bypass the area of ligation. Identification of leaking sites on the side of effusion and ligation of all tributaries together with the main duct is successful in as many as 90% of patients.20 However, this requires an extensive thoracotomy. Usually the decision to perform surgery is delayed, mainly with the intention of preventing an additional thoracotomy. A possible solution to this problem was presented by Murphy et al19 : as early as 8 days after diagnosis, they inserted a pleuroperitoneal shunt with good results in 75% of the patients. Especially in patients with chylothorax after thoracic procedures, pleuroperitoneal shunts were successful, less so in patients with caval thrombosis or high right atrial pressures. An advantage of this procedure is that it only requires the insertion of a pleural catheter. If successful, this technique avoids long parenteral nutrition and hospitalization time.

It is not clear from the small series of patients with chylothorax reported in the literature how often nonoperative management is successful. In our series, surgical interventions were performed very late in comparison. So, our patients practically represent the spontaneous course of this disease with a minimal number of surgical procedures (in 5 of 34 patients) that were performed for recalcitrant chylothoraces that were not abating spontaneously. This is in contrast to the report of Milsom et al,21 who performed a surgical procedure in 19 of 20 patients with chylothorax. Nevertheless, the rate of complications in our patients is not higher than those usually reported in the literature. Consequences of late surgical interventions are very long hospitalization times and imminent nutritional and infectious complications.22 Only one of our patients had septicemia, the preceding Ig level, however, was normal. If a surgical intervention in our series had been done after 3 weeks, then 20 of 39 patients would have undergone surgery. This explains why some authors report a high rate of surgical intervention in their patients. Opinions and recommendations about the length of conservative management vary considerably and depend also on the experience of the surgical team in performing a certain procedure. Advocates of operative interventions before 3 weeks have reported disappointing results with conservative management. We think that it can be worthwhile to wait > 3 weeks, provided that the nonoperative management is aggressive, ie, TPN with total enteric rest. To improve overall management, those patients with pertinacious chylothoraces should be identified early. In patients with high central venous pressures, thrombosis of the superior vena cava, or after caval-pulmonary anastomosis, conservative management usually fails.

From our patients, a considerable number (11 of 39 patients) had effusion lasting > 4 weeks; seven effusions were > 6 weeks. In five of the seven patients mentioned, a pleurodesis was performed successfully. In the remaining two patients, the cardiovascular situation was too unstable for a thoracotomy and they died. Retrospectively, we think that waiting 6 weeks or even longer as was done in two patients (105 days and 150 days, respectively) brings no advantage and is too long.

In conclusion, the diagnosis of chylothorax in newborn and pediatric patients can be made when analysis of the pleural fluid shows a triglyceride level of > 1.1 mmol/L and an absolute cell count > 1,000 cells/µL, with a lymphocyte fraction > 80%. This definition is simple and it allows, in most cases, a definite diagnosis, provided there is minimal oral fat intake. Surgery performed as early as 7 to 10 days after diagnosis of chylothorax certainly shortens hospitalization time. Waiting 2 to 4 weeks, however, reduces the need for surgical intervention substantially. Delaying surgery in a patient with pertinacious chylothorax > 4 weeks is not recommendable.


    Footnotes
 
Abbreviations: MCT = medium chain triglyceride; TPN = total parenteral nutrition

Received for publication April 14, 1999. Accepted for publication May 7, 1999.


    References
 TOP
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
 References
 

  1. Stringel, G, Stanley, M, Bass, J (1984) Surgical management of persistent postoperative chylothorax in children. Can J Surg 27,543-546[ISI][Medline]
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  3. Chernick, V, Reed, MH (1970) Pneumothorax and chylothorax in the neonatal period. J Pediatr 76,625-632
  4. Staats, BA, Ellefson, RD, Budahn, LL, et al (1980) The lipoprotein profile of chylous and nonchylous pleural effusions. Mayo Clin Proc 55,700-704[ISI][Medline]
  5. Ingram RH. Diseases of the pleura, mediastinum and diaphragm. In: Wilson JD, et al, eds. Harrison's principles of internal medicine. 12th ed. 1991; 1112
  6. Fairfax, AJ, McNabb, WR, Spiro, SG (1986) Chylothorax: a review of 18 cases. Thorax 41,880-885[Abstract]
  7. Straaten van, HL, Gerards, LJ, Krediet, TG (1993) Chylothorax in the neonatal period. Eur J Pediatr 152,2-5[ISI][Medline]
  8. Hargus, EP, Stanley, D, Carson, SD, et al (1978) Chylothorax and chylopericardial tamponade following Blalock Taussig anastomosis. J Thorac Cardiovasc Surg 75,642-645[Abstract]
  9. Feteih, W, Syamasundar, R, Whisennand, HH, et al (1983) Chylopericardium: new complication of Blalock-Taussig anastomosis. J Thorac Cardiovasc Surg 85,791-798[ISI][Medline]
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  11. Allen, EM, van Heeckeren, DW, Spector, ML, et al (1991) Management of nutritional and infectious complications of postoperative chylothorax in children. J Pediatr Surg 26,1169-1174[ISI][Medline]
  12. Koffler, H, Papile, LA, Burstein, RL (1978) Congenital chylothorax: two cases associated with maternal polyhydramnios. Am J Dis Child 132,638
  13. Brodman, RF, Zavelson, TM, Schiebler, GL (1975) Treatment of congenital chylothorax. NY State J Med 75,553-557
  14. Rheuban, KS, Kron, IL, Carpenter, MA, et al (1992) Pleuroperitoneal shunts for refractory chylothorax after operation for congenital heart disease. Ann Thorac Surg 53,85-87[Abstract]
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