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Experiences of postoperative chylothorax in children.

Eight cases of postoperative chylothorax in children are discussed. The age of the patients varied between 5 hours and 18 months. Chylothorax was diagnosed between 3 and 14 days after operation. In 6 cases chylothorax was certainly due to damage to the thoracic duct, or other big lymph vessels in the lungs or mediastinum. These patients had no typical pulmonary changes before chylothorax developed. In 2 cases there was probably no damage to the lymph vessels, but the venous pressure in the upper part of the body was elevated by constriction of the superior caval vein. The anatomical prerequisites for chylothorax in these cases is discussed. In these patients, before chylothorax, pulmonary changes appeared in the radiograph interpreted as widened lymph vessels and caused by altered lymph flow at the periphery. If pulmonary changes of this kind appear in patients with elevation of pressure in the superior caval vein, this may be a sign that chylothorax may develop. The importance of early diagnosis is stressed. Drainage of the pleura with continuous suction has proved to be the therapy of choice. Direct operation has not been necessary in any of our cases.

Chylothorax

Spontaneous chylothorax in Noonan syndrome. Treatment with prednisone.

OBJECTIVE: To describe a case of spontaneous chylothorax in a child with Noonan syndrome successfully treated with prednisone. DESIGN: Case report. SETTING: A pediatric cardiology referral center for the Rocky Mountain region. PATIENT: An 18-month-old girl with Noonan's syndrome, pulmonary stenosis, and hypertrophic cardiomyopathy who presented with spontaneous chylothorax. INTERVENTIONS: The child's chylothorax did not respond to thoracic duct ligation, tetracycline pleurodesis, and pleurectomy during a 2-month period. A low-fat diet was helpful but did not eliminate the problem. Prednisone was started orally at 1 mg/kg per dose twice daily and slowly tapered during 3 months. The chylothorax did not recur during 8 months of follow-up. CONCLUSIONS: Prednisone may be useful in the treatment of chylothorax in Noonan syndrome. A controlled clinical trial would be helpful but would be difficult in such a rare complication of an uncommon syndrome.

Chylothorax

Recurrent congenital chylothorax.

Congenital chylothorax is an uncommon but well-described condition. Recurrent congenital chylothorax is extremely rare. Yancy and Spock (1967) reviewed 31 reported cases of spontaneous chylothorax which occurred in the first 2 months of life and noted that male infants were affected twice as commonly as females. Two other cases of recurrent congenital chylothorax in male offspring (Defoort and Thiery, 1978; Reece et al., 1987) led to the suggestion of possible X-linked recessive inheritance. We describe a case of recurrent congenital chylothorax in which two consecutive female infants in a sibship were affected. The underlying cause of this disorder remains unknown. Inheritance as an X-linked recessive is not possible and this case is suggestive of autosomal recessive inheritance. The case also serves to emphasize the need for caution in counselling for recurrence risks when the underlying aetiology of the disorder is unknown.

Adult

Postoperative chylothorax in children: differences between vascular and traumatic origin.

Twenty-four children with postoperative chylothorax were encountered among 1,264 consecutive thoracic operations over a 7-year period and form the basis of this study. Chylothorax was caused by direct lesion to the thoracic duct or lymphatic vessels in 17 patients and was associated with superior vena cava (SVC) obstruction in seven. Of the latter, five had bilateral chylothorax. Chylothoraces secondary to venous hypertension and thrombosis have a longer interval between operation and diagnosis compared with direct trauma as well as a longer duration and larger volume of chylous drainage. Treatment was entirely nonoperative in 16 patients and operative in 8. Nonoperative treatment consisted of pleural needle aspiration or suction drainage in association with a medium chain triglyceride (MCT) diet (n = 11) or total parenteral nutrition (TPN) after failure of MCT (n = 5). Direct operation on the thoracic duct was performed in 5 patients, four had pleurodesis, and 2 had pleuroperitoneal shunts inserted. All patients were cured of their chylothorax and there were no deaths. Patients with major vein thrombosis were the most difficult to treat. On the basis of this experience, we suggest a step-by-step approach: (1) insertion of chest tube after 3 to 4 pleural punctures; (2) 1-week trial of MCT diet, with intravenous support to correct protein losses; (3) TPN if chylothorax increases or persists with large volumes; (4) Doppler echocardiography or phlebography to rule out obstruction of major thoracic veins; and (5) insertion of TPN line in inferior vena cava in case of such obstruction; and (6) direct surgical approach to the thoracic duct after 4 weeks of unsuccessful nonoperative treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Child

Conservative versus surgical management of chylothorax.

Chylothorax is a potentially life-threatening disorder that has profound respiratory, nutritional, and immunologic consequences. Treatment for this problem is controversial, and the results have been variable. From 1985 to 1990, 29 patients (16 males, 13 females; mean age: 20.1 years; range: 5 days to 76.8 years) were diagnosed as having chylothorax (18 right, 6 left, 5 bilateral). Etiologies included surgical trauma (26) and nonsurgical trauma (3). Initial conservative treatment consisted of tube thoracostomy drainage (mean duration: 13.3 days; range: 1 to 62 days; mean total volume: 4,030 mL) and dietary modification (low-fat diet, total parenteral nutrition). This resulted in resolution of the chylothorax in 23 patients (79% success), although 2 patients died of unrelated causes while hospitalized (myocardial infarction and cardiopulmonary arrest). Five adult patients and one infant (21%) required ligation of the thoracic duct, with resolution of the chylothorax in all six (100% success). Despite successful duct closure, one infant died of respiratory failure unrelated to the operation, and one adult died as the result of a cerebrovascular accident 6 weeks postoperatively, yielding an operative mortality of 33% and an overall mortality of 14% (4 of 29). Our experience demonstrates that initial treatment of chylothorax with thoracostomy drainage and dietary modification is successful in the majority of patients and is not associated with high morbidity or mortality rates. Surgical intervention for chylothoraces that fail to respond to initial conservative measures will be required in a minority of patients but appears to be associated with a higher risk of complications.

Adolescent

[Chylothorax in the neonatal period].

The data of 11 infants with chylothorax in the neonatal period, seven with congenital chylothorax and 4 infants with iatrogenic chylothorax are reported. Chylothorax in the neonatal period may cause birth asphyxia and serious respiratory problems. Among the infants with congenital chylothorax diagnosis can be established before birth by ultrasound technique, followed by optimal resuscitation to prevent asphyxia. In most of the cases, conservative treatment with continuous drainage and total parenteral nutrition is sufficient.

Cardiac Surgical Procedures

[Chylothorax developed after cardiac surgery in children: a report of three cases].

Two children developed chylothorax after median sternotomy and a third child developed chylothorax after a Blalock-Taussig operation under the left thoracotomy. The first patient had a closure of ASD and a pulmonary valvotomy and then later developed chylothorax. The thoracic duct was ligated 10 cm just above the diaphragm at 28 days after the first operation because of unsuccessful conservative treatment. The second patient had a Blalock-Taussig operation and the third patient had radical operation for ECD and both developed chylothorax. The latter two patients were cured with conservative treatments such as the administration of Medium Chain Triglyceride or total parenteral nutrition with thoracic drainage. Children who developed chylothorax after cardiac surgery should be treated conservatively for at least the early postoperative period when circulatory and/or respiratory conditions are still unstable.

Child, Preschool

Persistent chylothorax. Treatment by talc pleurodesis.

Two patients with persistent chylothorax resistant to therapy by special diet, thoracenteses, and tube thoracostomy drainage were successfully treated by talc pleurodesis. In one patient with advanced lymphosarcoma involving pleura and mediastinal nodes, the chylothorax was managed by open thoracotomy and talc powder. The other patient developed chylothorax following resection of a thoracic aortic aneurysm. She was successfully treated by talc suspension introduced through a thoracostomy tube. Pleurodesis with talc should be considered only when the chylothorax persists after an adequate period of medical treatment and pleural decompression. Intrapleural instillation of talc through a thoracostomy tube should be successful if the chylothorax can be evacuated and the underlying lung fully expanded.

Chylothorax

Combined occurrence of chyloperitoneum and chylothorax after surgery and chemotherapy for Wilms' tumor.

Chyloperitoneum is an extremely rare complication of abdominal surgery in children and a combined occurrence of chylothorax and chyloperitoneum after abdominal surgery has never been reported in children. Chylous ascites usually occurs as a result of operative trauma to the thoracic duct, cisterna chyli, or its tributaries. About one third of all patients with chylous ascites after retroperitoneal lymph node dissection also develop secondary chylothorax. Diaphragmatic defects have been shown to be responsible for the occurrence of chylothorax secondary to chyloperitoneum. Congenital diaphragmatic weakness may result in evagination of the peritoneum causing diaphragmatic blebs, the rupture of which results in the movement of the peritoneal fluid into the pleural cavity. In the authors' patient, the rent in the diaphragm that occurred during surgery was probably responsible for the chylothorax. The role of chemotherapy, if any, in the pathophysiology of this complication is unknown. Total parenteral nutrition (TPN) is a simple and effective treatment for postoperative chylous effusions. Surgical treatments such as abdominal exploration for the repair of leaking lymphatics and peritoneovenous shunt should be reserved for patients who fail TPN.

Chylothorax

Chylothorax after oesophagectomy.

Chylothorax is an uncommon complication of oesophagectomy. In a review of 537 oesophageal resections there were 11 cases of chylothorax, an incidence of 2.0 per cent. There was no correlation with site, size, penetration, lymph node status, length or type of tumour but there was a significant correlation between chylothorax and the type of operative procedure carried out. The incidence in 95 transhiatal resections was 10.5 per cent. The incidence following 442 transthoracic procedures was 0.2 per cent (P less than 0.001) with one chylous fistula occurring after a three-stage oesophagectomy. Initial management was conservative with chest drainage and total parenteral nutrition. Thoracotomy and duct ligation was subsequently carried out in three patients and was successful in two. The third patient died. Conservative management alone was successful in four out of eight patients, with closure of the fistula at a median of 35 days (range 14-42 days). Four patients treated conservatively died. Transhiatal oesophagectomy greatly increases the risk of chylothorax, a condition that carries a high mortality rate (46 per cent in this series) whether managed conservatively or by surgical intervention.

Chylothorax

[Pathogenesis, clinical application and therapy of chylothorax (author's transl)].

With the growth of traffic density and the spread of thoracic surgery, the clinical picture of the chylothorax can be observed with increasing frequency. Above all, the spontaneous or symptomatic chylothorax presents diagnostic problems, since, in about 70% of the cases, it only represents the concomitant symptom of a malignant tumor. Only after exact diagnostics and the exclusion of a malignant primary disease can one be content with the cursory diagnosis of an idiopathic chylothorax. However, this requires strict postoperative control and observation of the patients. Two patients are presented in whom the genesis of the chylothorax still remained unclear 3 1/2 years after the successful ligature of the thoracic duct.

Accidents, Traffic

[Congenital and post-operative chylothorax in the neonatal period].

In a retrospective survey of the patients admitted to the Neonatology Department during a 15 year period (october 1975-september 1990), we found 7 cases of chylothorax: 4 subsequent to cardiac surgery and 3 congenital. The incidence of chylothorax relative to the total number of admissions to NICU was 1.06% and 1.2% in respect to total admissions after cardiac surgery. The clinical outcome was good for all patients except one who died during resuscitation maneuvers following delivery. Only one patient required surgery to control chylothorax and the rest were successfully managed with medical treatment. We found that patients with postsurgical chylothorax drained a larger volume of pleural fluid, needed larger volumes of replacement fluid and required parental nutrition and mechanical ventilation for a longer period of time.

Cardiac Surgical Procedures

Management of chylothorax complicating extensive esophageal resection.

Between January 1983 and May 1987, 255 esophagectomies were performed for carcinoma of the middle (40 patients) or lower (215 patients) esophagus. All patients were operated upon through a left thoracolaparotomy and underwent a radical en bloc resection of the tumor along with all palpable mediastinal nodes. Ten patients had chylothorax develop postoperatively. There were seven men and three women with a mean age of 65.7 years (range of 37.0 to 81.0 years). Parameters that were statistically evaluated for possible correlation to increased incidence of chylous fistula were age, sex, site and size of tumor, histologic type, mediastinal lymphatic involvement and elective prophylactic ligation of the major thoracic duct (MTD) at the conclusion of the procedure. It was found that elective ligation of the MTD was associated with a lower occurrence of chylothorax (2.1 per cent) as compared with those with no routine ligation (9 per cent), p less than 0.05. The leak was successfully treated by repeated thoracotomy and mass ligation of the MTD in eight patients, while one patient underwent closed tube thoracostomy. In one instance, only a pleuroperitoneal shunt was performed. The over-all hospital mortality rate from chylothorax was 10 per cent and there was a late death because of pneumonia. We recommend prophylactic ligation of the MTD in all instances of extensive esophageal resection for the prevention of chylothorax, as well as early thoracotomy for the management of established leaks.

Aged

Hyponatremia and hyperkalemia associated with idiopathic or experimentally induced chylothorax in four dogs.

Two dogs with idiopathic chylothorax and 2 dogs with experimentally induced (ie, ligation of the cranial vena cava) chylothorax were treated by intermittent thoracic drainage. Of these 4 dogs, 3 that did not have evidence of renal failure had normal or near-normal serum sodium and potassium concentrations before thoracic drainage began, and all 3 developed repeatedly marked hyponatremia and hyperkalemia during thoracic drainage. Another dog became weak and depressed, ostensibly because of hyperkalemia. Serum sodium and potassium concentrations in 1 dog with spontaneous chylothorax returned to normal after chylothorax resolved and thoracic drainage was stopped. The other 3 dogs died or were euthanatized, and the effect of stopping thoracic drainage could not be evaluated. In 3 dogs in which it was measured, normal-to-high plasma cortisol concentration was observed before and after adrenocorticotropin administration, and 2 dogs also had hyperaldosteronemia. Hyponatremia was hypothesized to be caused by sodium loss via thoracic drainage whereas hyperkalemia may have been multifactorial in origin, but probably was attributable, at least, in part to decreased renal potassium clearance.

Animals

Congenital chylothorax in a trisomy 21 newborn.

We here report a case of trisomy 21 with congenital chylothorax. The patient was a male newborn who had been diagnosed as having trisomy 21 with congenital chylothorax. This is the fifth case of the both conditions in English literature. Congenital chylothorax is very rare in a trisomy 21 patient. There, however, may be causal relationship between the two conditions.

Chylothorax

Pleuroperitoneal shunts for refractory chylothorax after operation for congenital heart disease.

Between 1980 and 1990, 10 of 12 children with a symptomatic chylothorax after operation for congenital heart disease failed to respond to traditional medical therapy (thoracentesis, tube thoracostomy, low-fat diet). All 10 patients underwent placement of a pleuroperitoneal shunt, with complete resolution of the chylothorax in 9 patients (90%). Cardiac catheterization, performed before placement of the pleuroperitoneal shunt in 5 patients, demonstrated elevated right atrial pressure in all patients (range, 10 to 25 mm Hg). The pleuroperitoneal shunt functioned effectively in 4 patients with moderately elevated right atrial pressures (range, 10 to 16 mm Hg; median, 13.5 mm Hg) but not in 1 patient with a right atrial pressure of 25 mm Hg. Pleuroperitoneal shunting as treatment for chylothorax after operation for congenital heart disease is safe and effective, even in the face of moderate elevations in right atrial pressure.

Chylothorax

Dietary modification of chyle composition in chylothorax.

Nutrition support has played a major role in the treatment of chylothorax, both to prevent malnutrition and to minimize chyle production and flow. This report evaluates chyle composition in a patient with chylothorax who was placed on a low-fat diet, medium-chain triglyceride diet, and total parenteral nutrition in sequence. Both triglyceride content and volume of chyle declined, but drainage persisted, ultimately requiring thoracic duct ligation. The chyle triglyceride while on total parenteral nutrition, which presumably originates from both the intestine and plasma, contained more long-chain unsaturated fatty acids than the circulating serum triglyceride. Of particular interest was the detection of an appreciable amount of medium-chain fatty acids in the chyle triglyceride, constituting 20% of the triglyceride fatty acids when an enteral formulation with medium-chain triglyceride as a sole fat source was administered. The finding of almost threefold more decanoic acid (C10:0) than octanoic acid (C8:0), despite the presence of considerably more octanoic acid in the original diet, suggests that trioctanoin may be a preferable medium-chain triglyceride substrate for the nonsurgical treatment of chylothorax.

Adult

Congenital chylothorax and mediastinal neuroblastoma.

This is the first reported association of congenital chylothorax and mediastinal neuroblastoma in a newborn infant. The infant exhibited features of both congenital and acquired chylothorax. Resolution of the chylothorax occurred following ligation of thoracic duct after failure of conservative management with multiple thoracentesis and chest tube drainage.

Chylothorax