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* 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 |
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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 |
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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 |
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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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| Results |
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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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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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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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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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| Discussion |
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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 |
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Received for publication April 14, 1999. Accepted for publication May 7, 1999.
| References |
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