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PubMed1994

Massive chylothorax associated with lymphangiomatosis of the bone.

Chylothorax in the absence of tumor or trauma is uncommon. Lymphangiomatosis of the bone, although extremely rare, has been associated with chylothorax. The authors describe the case of a 12-year-old boy who presented with a symptomatic left chylothorax associated with lymphangiomatosis of the ribs, scapula, and clavicle. Despite tube thoracostomies and the initiation of total parenteral nutrition, massive losses of chyle persisted, resulting in hypoproteinemia and severe lymphopenia. Control of the chylothorax was achieved by a parietal pleurectomy and application of fibrin glue (Tisseel). In the literature there are 16 cases of chylothorax associated with lymphangiomatosis of the bone. Their presentation, treatment, and outcome are reviewed. Conservative treatments such as dietary manipulations or thoracenteses were rarely successful. Thoracotomy with parietal pleurectomy on the side of the effusion is usually effective in controlling the chylothorax. Lymphangiomatosis should be considered a diagnostic possibility for any child who presents with a chylothorax.

Biopsy↗

PubMed2004

Use of Monogen for pediatric postoperative chylothorax.

BACKGROUND: Postoperative chylothorax is a potentially serious complication of pediatric cardiac surgery. The purpose of this study was to report the use of Monogen enteral formula for the management of pediatric postoperative chylothorax. METHODS: A retrospective, single-institution 2-year study of all patients with a diagnosis of postoperative chylothorax was conducted. Chylothorax was diagnosed in 25 patients after a total of 535 cardiac operations in children younger than 10 years, for an incidence of 4.7%. Eighteen patients had been given Monogen, an enteral low long-chain triglyceride formula, as initial treatment. Six had been given total parenteral nutrition. The following variables were related to outcome and response to Monogen: age, sex, weight, underlying condition, type of surgery, interval between surgery and chylothorax diagnosis, duration and daily volume of chyle leak, central venous pressure, residual lesions, and weight loss. RESULTS: Enteral feeding with Monogen was successful for 14 of 18 patients with a response to treatment evident by the end of the third day. No variables predicted which patients would respond to Monogen. Body weight was maintained or increased in 14 of the 17 surviving patients taking Monogen. A return to normal diet at 4 +/- 1 weeks from the day of pleural drain removal did not result in recurrent chylothorax. CONCLUSIONS: A trial of Monogen is recommended as initial treatment for postoperative chylothorax unless enteral feeding is contraindicated.

Algorithms↗

PubMed2000

Chylothorax complicating esophagectomy for cancer: a plea for early thoracic duct ligation.

OBJECTIVE: Postoperative chylothorax remains an uncommon but potentially life-threatening complication of esophagectomy for cancer, and the ideal management is still controversial. The aim of the study was to compare the outcomes of patients treated nonoperatively with those of patients promptly undergoing reoperation. METHODS: From 1980 to 1998, 1787 esophagectomies for esophageal or cardia cancer were performed, and 19 (1.1%) patients had postoperative chylothorax. We analyzed type of operation, surgical approach, delay of diagnosis of chylothorax, daily chest tube output, type of management, major complications, death, hospital stay, and final outcome. RESULTS: Of the 19 patients with chylothorax, 11 were initially managed nonoperatively (group A): 4 (36%) patients had spontaneous resolution of chylothorax, and the other 7 required reoperation for the persistence of a high-volume output. There were three infectious complications and one postoperative death in this group. No reliable predictive criteria of successful versus unsuccessful nonoperative management could be found. The 8 most recent patients underwent early reoperation (group B). All patients recovered, and no major complications possibly related to chylothorax or hospital deaths were observed. They were discharged after a median of 22 days (range, 12-85 days) compared with a median of 36 days (range, 21-64 days) for patients of group A. CONCLUSIONS: Early thoracic duct ligation is the treatment of choice for chylothorax occurring after esophagectomy. Reoperation should be performed immediately after the diagnosis is made to avoid the complications related to nutritional and immunologic depletion caused by prolonged nonoperative treatment.

Adult↗

PubMedDate not supplied

Postoperative chylothorax: differences between patients who received median sternotomy or lateral thoracotomy for congenital heart disease.

BACKGROUND: Chylothorax after surgery for congenital heart disease is rare. We wanted to compare the different presentations of chylothorax in patients who received median sternotomy or lateral thoracotomy. PATIENTS AND METHODS: We retrospectively studied pediatric patients with congenital heart disease who received palliative or corrective surgeries and developed postoperative chylothorax between January 1992 and July 2003. Patients were divided into two groups by the type of surgery: median sternotomy and lateral thoracotomy. The average daily fluid amount (mL/kg/24 hours), latency period, duration of chylothorax, and requirement for surgery were compared. RESULTS: Seventeen patients (11 boys, 6 girls; mean age, 14.0 +/- 12.8-month-old) were enrolled. Diagnoses were tetralogy of Fallot (n = 8), right isomerism with complex heart defects (n = 3), patent ductus arteriosus (n = 2), transposition of the great artery (n = 1), ventricular septal defect (n = 1), and endocardial cushion defect (n = 2). There were 9 in the lateral thoracotomy group and 8 in the median sternotomy group. Only one patient required surgery because of the failure of conservative treatment. The lateral thoracotomy group had a significantly lower average body weight (6.9 +/- 2.9 kg vs. 11.0 +/- 3.8 kg) and longer average latency period before postoperative chylothorax (15.1 +/- 9.2 days vs. 7.2 +/- 4.7 days). CONCLUSION: The majority of pediatric patients who develop chylothorax after cardiac surgery can be successfully managed by medical treatment only. To avoid complications in pediatric patients after cardiac surgery, chylothorax should be suspected for patients with unexplainable, prolonged, and abundant pleural effusion.

Cardiac Surgical Procedures↗

PubMed2006

Postoperative chylothorax in patients with a thoracic aortic aneurysm.

OBJECTIVES: Postoperative chylothorax in patients with a thoracic aneurysm is generally infrequent. We report a mode of surgery to reduce the incidence of paraplegia. We review our experience with chylothorax after resection of an aneurysm to find its cause and to evaluate the success of management. PATIENTS AND METHODS: For descending thoracic aneurysms, intercostal arteries in the aneurysm were exposed before incising the aneurysm and, they were only sacrificed no change in motor-evoked potentials (MEPs) occurred after temporary occlusion. Between January 2001 and December 2003, out of a total of 147 aneurysms including thoracic and thoracoabdominal, 4 consecutive patients (2.7%) with chylothorax were reviewed. RESULTS: The chylothorax was diagnosed 1.5 days after operation (range 1 to 2 days). All patients were initially treated by the cessation of oral intake. This treatment was successful for 2 patients and the remaining 2 required surgical intervention to control the fistula. Chylothorax was cured in all patients. CONCLUSION: We postulate that chylothorax is caused by injury of the thoracic duct or its branch during the exposing the intercostal arteries. In the management of chylothorax, early intervention is recommended when the volume of chylous fluid is not decreased by conservative treatment.

Aged↗

PubMed1989

Pleuroperitoneal shunts in the management of persistent chylothorax.

Between June 1981 and June 1988, we placed pleuroperitoneal shunts in 16 patients for the management of refractory chylothorax on the Pediatric Surgical Service, University of Virginia. The cause of the chylothorax was caval thrombosis from central venous catheters in 5 patients, idiopathic in 3, and mediastinal lymphangioma in 2, and in 6, it developed after a cardiac procedure. Chylothorax in each patient was unresponsive to thoracentesis, tube thoracostomy, and dietary manipulations. A Denver double-valved shunt system is currently employed and is implanted using general anesthesia. Manual pumping is required postoperatively for several months. Twelve (75%) of the 16 patients had excellent results with complete elimination of the chylothorax and resolution of symptoms. In 10 of these 12, the shunt has been removed. Four had an unsatisfactory result: 3 had inferior vena cava hypertension, and 3 were low-birth-weight premature infants. Four patients seen early in this series required revision of the position of the pleural catheter, with successful drainage in each instance. Pleuroperitoneal shunting is a safe, simple, and effective treatment of chylothorax in infants and children. In view of our success in treating chylothorax with these shunts, we recommend early shunting before the development of nutritional or immunological depletion.

Adolescent↗

PubMed2006

Chylothorax in children after congenital heart surgery.

BACKGROUND: A definitive management strategy for postoperative chylothorax remains elusive. We reviewed our experience in the management of chylothorax in children after congenital heart surgery. METHODS: The case records of 51 patients, with a median age of 11 months (range, 4 days to 19.6 years), diagnosed to have postoperative chylothorax between 1981 and 2004 were reviewed. The responses of patients to nutritional modifications, octreotide therapy, and surgical interventions were noted. RESULTS: The prevalence of postoperative chylothorax, which developed at a median of 9 days after operation (range, 0 to 24 days), was 0.85% (51 of 5,995). Four patients died, and among the 47 survivors the median duration and total volume of chylous drainage was 15 days (range, 1 to 89 days) and 156 mL/kg (range, 3 to 6,476), respectively. The duration of chyle output was significantly longer after the Fontan-type procedures (p = 0.0006). Twenty-one patients were diagnosed between 1981 and 1999 and managed by nutritional modifications, 2 of whom required further surgical interventions. Of the 30 patients diagnosed between 2000 and 2004, 12 responded to nutritional modifications alone while 18 were started on octreotide therapy at a median of 19.5 days (range, 7 to 35 days) after the onset of chylothorax. Fifteen of the 18 (83%) patients responded to octreotide therapy at 15.3 +/- 5.5 days after starting octreotide, while 3 required further surgical interventions. None developed side effects from octreotide therapy. CONCLUSIONS: Octreotide has been incorporated into the management algorithm of postoperative chylothorax and appears to be a useful adjunctive therapy.

Adolescent↗

PubMed2006

Video-assisted right supradiaphragmatic thoracic duct ligation for non-traumatic recurrent chylothorax.

BACKGROUND: Chylothorax is an uncommon disorder with respiratory, nutritional and immunological manifestations. Surgical management is indicated in case of recurrence or failure after conservative treatment. We report our experience with video-assisted right-sided supradiaphragmatic thoracic duct ligation for non-traumatic, non-postoperative persistent or recurrent chylothorax. PATIENTS AND METHODS: The medical records of six patients operated at our institution between 1999 and 2004 were retrospectively reviewed. A right-sided chylothorax was found in four patients, a left-sided in one, and a bilateral in one. Three patients developed chylothorax after chemotherapy and chest irradiation for malignant diseases (lymphoma in two patients and breast cancer in one), one in the context of lymphangioleiomyomatosis, one due to a non-diagnosed lymphoma, and one after heart transplantation. RESULTS: The mean operative time was 102 min, with an average length of hospital stay of 14 days. Persistent cessation of chylous effusion within 7 days after surgery was observed in 5/6 patients without recurrence during a mean follow-up time of 41 months. One patient with undiagnosed mediastinal lymphoma required re-operation and thoracic duct ligation on day 8 by right-sided thoracotomy due to persistent chylothorax. No 30-day mortality was recorded. Two patients presented postoperative complications including respiratory insufficiency requiring mechanical ventilation in one, and chylous ascites development requiring peritoneo-venous LeVeen shunting in one patient. CONCLUSIONS: Recurrent or persistent non-traumatic chylothorax may be successfully treated by video-assisted right supradiaphragmatic thoracic duct ligation.

Adult↗

PubMed2006

The role of somatostatin in the treatment of persistent chylothorax in children.

OBJECTIVE: To analyze the success rate of somatostatin in children with persistent chylothorax who failed dietary treatment options (fat-free nutrition, total parenteral nutrition) and to work out predictive factors for a successful therapy with somatostatin. METHODS: Retrospective cohort study over a 5-year period (2000-2004) in a neonatal and pediatric intensive care unit of a tertiary university hospital. We analyzed the data of 85 neonatal and pediatric patients. Treatment of chylothorax occurred according to a multistage protocol with progressing invasiveness: (1) fat-free enteral nutrition, (2) total parenteral nutrition, (3) somatostatin infusion, (4) surgery. The percentages of patients successfully treated at the progressing steps were recorded. The somatostatin group was analyzed regarding to physiologic, diagnostic, treatment and outcome parameters. Somatostatin-responders were compared with non-responders. RESULTS: Seventy-six of the 85 patients had chylothorax after cardiac surgery. Sixty-six percent could be treated with fat-free nutrition alone, 19% needed treatment with total parenteral nutrition and in 15% somatostatin was added. Of the whole sample, 4.7% required a surgical intervention. Of the 13 patients treated with somatostatin, all had bilateral chylothorax. Six patients (46%) responded to somatostatin. Responders and non-responders did not differ significantly regarding age, day of postoperative diagnosis of chylothorax, amount of chylous effusion before somatostatin infusion, triglyceride concentration and lymphocyte percentage in chylous, and central venous pressure (p=0.066). CONCLUSIONS: Somatostatin, integrated in a treatment algorithm, was successful in resolving persistent chylothorax in around 50% of patients. With this strategy, some children may be prevented from undergoing an operation. However, factors predicting successful therapy with somatostatin could not be elicited.

Algorithms↗

PubMed1981

Management of nontraumatic chylothorax.

Twenty-two patients with chylothorax have been treated at the National Institutes of Health since 1955. In 9 of these patients, the condition resulted from an antecedent operation and in 13, it occurred without a history of prior operation (nontraumatic). All 6 of the patients with tumors in whom nontraumatic chylothorax developed had a lymphoma. Four of these 6 also had a chylous ascites, while 6 of the 7 patients without tumors had an associated chylous ascites. Only 3 of the 13 patients with nontraumatic chylothorax responded to nonoperative therapy alone with stabilization of the pleural effusions. A single patient with systemic lupus erythematosus responded to steroid therapy. In contrast, 3 of 4 patients who underwent thoracotomy for nontraumatic chylothorax had permanent relief of their chylous pleural effusions. In the absence of medically treatable disease, thoracotomy with ligation of the thoracic duct and/or pleurectomy or pleurodesis can provide substantial palliation for patients with nontraumatic chylothorax, even when a discrete source of lymph leakage cannot be localized or ascites is present. Early surgical therapy of nontraumatic chylothorax is advocated in such circumstances.

Adolescent↗

PubMed1986

Chylothorax: a review of 18 cases.

Eighteen patients who had developed a chylothorax during a 25 year period, 1955-80, were reviewed. The chylothoraces occurred as a complication of cardiothoracic surgery in 11 patients, of whom eight were children in the first decade of life. Five cases followed operations for coarctation of the aorta and two occurred as a complication of a Blalock shunt for Fallot's tetralogy. The chylothorax was evident within 48 hours of the operation in all but two patients. In seven cases a second operation was performed to prevent further chylous leakage and in two infants the thoracic duct was ligated. The remainder of the postsurgical chylothoraces responded to either continuous drainage or repeated aspiration and a low fat diet. There were no late sequelae of chylothorax following surgery. Spontaneous chylothorax was identified in seven patients and in five of these it was bilateral. Patients with spontaneous chylothorax were all adults and, despite treatment, had a poor prognosis. Three with malignant disease and two with pulmonary lymphangioleiomyomatosis had died within two years of the appearance of the chylothorax. Two patients with chronic idiopathic chylothoraces survived for more than two years and one of these developed a secondary fibrothorax.

Adult↗

PubMed2006

Pleural fluid/serum immunoglobulin ratio is a diagnostic marker for congenital chylothorax in utero.

OBJECTIVE: To distinguish congenital chylothorax from other causes of hydrothorax in utero. METHODS: Serum and pleural fluid samples from 8 fetuses with congenital chylothorax and 5 control fetuses with other causes of hydrothorax were tested for total protein, albumin, IgG, IgA, and IgM. RESULTS: Fetuses with congenital chylothorax had higher levels of IgG in pleural fluid, but not the other four proteins, than control fetuses (P<0.05). There were no significant differences in serum proteins among fetuses. When we examined pleural fluid to serum ratios, the IgG ratio in fetuses with congenital chylothorax was significantly higher than that of control fetuses (P<0.05). The IgG ratio in chylothorax was greater than 0.6 regardless of lymphocyte count. CONCLUSION: Pleural fluid/serum IgG ratio may be a diagnostic marker for congenital chylothorax in utero.

Albumins↗

PubMed2000

The treatment of chylothorax.

OBJECTIVES: To understand and grasp the diagnosis and treatment of chylothorax caused by variou reasons. METHOD: The treatment results of 31 cases of chylothorax in PUMC hospital from 1963 approximately 1997 were retrospectively analyzed. RESULTS: Among 31 cases, 18 underwent surgery, 14 of 18 were cured, 2 died. In the 13 treated conservatively, 2 were cured, 3 died. Eleven cases were congenital, iatrogenic and traumatic chylothorax, 8 of them received surgical treatment and 6 of 8 were cured. The spontaneous chylothorax of unknown cause were 10 cases, 7 were treated by surgery and 6 were cured. CONCLUSION: Surgical intervention should be aggressively recommended for the traumatic, congenital, and iatrogenic chylothorx. The definite reason must be found out for the spontaneous chylothorax, corresponding management will be given according to the reason. Surgical ligation of the thoracic duct will contribute good result for the chylothorax of unknown cause, but combination of multiple treatment measures will be necessary for a successful management.

Adolescent↗

PubMed2006

Use of octreotide, a long-acting somatostatin analogue, in the treatment of chylothorax of idiopathic etiology.

We describe a case of chylothorax of idiopathic etiology, treated with octreotide, a long-acting somatostatin analogue. A 30-year-old man with a left supraclavicular mass, and chylothorax, initially diagnosed by outpatient thoracentesis, underwent diagnostic surgery to ascertain the etiology of the pleural effusion. Biopsies of the left supraclavicular mass, lymphatic tissue and lymph nodes were benign. Triglyceride level in the pleural fluid was 396 mg/dL, diagnostic of chylothorax. Treatment included intravenous total parenteral nutrition (TPN) and a nil per os (NPO) diet. Subsequent surgical interventions included left lung decortication and glue-mediated control of chylothorax, combined with TPN and a strict low-fat diet. Given the persistency of chylothorax, thoracic duct ligation was also performed, and octreotide subcutaneous injections were started, with dramatic resolution of pleural effusion, after 1 week of treatment, in absence of any side effects. The patient fully recovered, and no relapse has been observed during a follow-up period of over 1 year. In conclusion, octreotide showed to be a valid and safe noninvasive approach for the treatment of chylothorax, whose early clinical use may also reduce the need for surgical intervention.

Adult↗

PubMed1995

Management of chylothorax after pulmonary resection.

BACKGROUND: Conservative management with intrapleural drainage and total parenteral nutrition (TPN) has been the first choice of treatment for postoperative chylothorax. With this approach, however, it usually takes several weeks for the chylothorax to resolve and it is sometimes unsuccessful. In this study, we reviewed seven patients who had chylothorax develop after pulmonary resection for primary carcinoma of the lung. STUDY DESIGN: The patients were treated according to a "one-week trial" that consisted of one week of observation with intrapleural drainage and maximum parenteral nutritional support followed by operative intervention if the effect of the conservative therapy was not adequate. When the chylous leak was decreased to less than 100 mL/day or less than 15 percent of the maximum daily drainage volume after the "one-week trial," the conservative management was continued for two more weeks. After observation for three weeks, oral intake was begun and a final evaluation of the treatment was made. RESULTS: One patient did not consent to the "one-week trial" and underwent operative treatment on the third postoperative day. Two patients had chylous leaks less than 100 mL/day or less than 15 percent of the maximum daily chylous leak after one week observation. Conservative management with TPN was continued in these patients for two more weeks and operation was performed in one on the 20th day and in the other on the 22nd postoperative day. The remaining four patients underwent operative treatment on the seventh or eighth postoperative day. All of the operations for chylothorax were successful, and chest tubes were removed promptly. These results show that operative management of chylothorax was reliable and safe. The "one-week trial," however, offered few advantages in determining the therapeutic strategy for postoperative chylothorax.

Adult↗

PubMed1992

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↗

PubMed1991

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↗

PubMed2005

Chylothorax in Henoch-Schonlein purpura: a case report and review of the literature.

Henoch-Schonlein purpura (HSP) is the most common acute vasculitis in the pediatric population, with an incidence of 10-14 per 100,000. The classic presentation of this disorder includes erythematous papules followed by palpable purpura in the lower extremities, trunk, and face, arthralgia or arthritis, abdominal pain, gastrointestinal bleeding, and nephritis. While renal abnormalities in HSP are common, the classic pulmonary manifestations, such as hemorrhage and pneumonitis, are thought to be infrequent. Subclinical pulmonary manifestations, including diffusion defects and radiographic anomalies, seem to be quite frequent in patients with HSP but are not commonly reported. Other respiratory manifestations include pleural effusion and chylothorax, but these are rarely mentioned in the literature. Chylothorax was only reported once in an adult patient with HSP in whom the mechanism of formation was demonstrated to be secondary to transdiaphragmatic passage of chylous fluid from the peritoneal cavity. Here we describe an 8-year-old girl with HSP, nephrotic syndrome, and chylothorax, and we report the results of a review of the literature regarding respiratory complications in HSP. The present case is the first pediatric patient reported with HSP and chylothorax. The therapeutic measures utilized were effective in resolving her edema, ascites, and chylothorax, and we advocate the use of these measures as first-line therapy in future patients with similar complications from HSP.

Child↗