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[Relapse of idiopathic chylothorax 17 years after remission of congenital chylothorax].

We report a case of chylothorax in a 17-year-old male. As a neonate, had had congenital chylothorax, and was successfully treated with medium-chain triglycerides; but recently presented with dyspnea after an episode of severe coughing. Radiographic examination disclosed abundant effusion in the right chest. The effusion was milky brown, had a creamy supernatant and a high triglyceride level (3085 mg/dl). This condition was diagnosed as idiopathic chylothorax. Lymphangiography showed a rupture of the right thoracic duct. Since the effusion was resistant to conservative therapy, we performed thoracoscopic clipping of the thoracic duct, which reduced the amount of pleural effusion. We speculated that coughing may have caused the rupture of a congenitally weakened thoracic duct.

Adolescent↗

[A case of chylothorax resulting from malignant lymphoma--pathogenesis of chylothorax: a new concept].

We report a case of chylothorax in a 51-year-old male with non-Hodgkin's lymphoma. Combination chemotherapy reduced the size of mediastinal lymph nodes dramatically, but retroperitoneal lymph nodes remained almost the same, and chylothorax subsequently developed. Lymphangiogram showed reticular spread of contrast material from the thoracic duct downwards but not above the diaphragm. These findings suggest that an obstruction of the infra-diaphragmatic but not the supra-diaphragmatic thoracic duct caused development of collateral lymphatic channels penetrating the diaphragm into the thoracic cavity, with subsequent formation of chylothorax.

Adolescent↗

Chylothorax in lymphangioleiomyomatosis.

STUDY OBJECTIVE: Pulmonary lymphangioleiomyomatosis (LAM) is a rare, serious disorder characterized by proliferation of abnormal smooth-muscle cells and affects almost exclusively women of childbearing age. Optimal management of chylothorax, a well-recognized complication of LAM, in these patients has not been defined. This study was performed to characterize the clinical course and identify appropriate management options for chylothorax occurring in patients with LAM. DESIGN: Identification and retrospective review of available medical records on patients with LAM and chylothorax. SETTING: Tertiary-referral medical center. PATIENTS: All patients with LAM seen at Mayo Clinic, Rochester, MN, from January 1, 1976, to December 31, 2000. INTERVENTION: None. MEASUREMENT AND RESULTS: Eight of 79 patients (10.1%) with LAM had chylothorax. All were women aged 33 to 51 years, and four patients had underlying tuberous sclerosis complex. These eight women represented 3.5% of the 229 patients with chylothorax seen over this 25-year period at Mayo Clinic Rochester. Six patients had unilateral pleural effusion and two patients had bilateral effusions at initial presentation. The size of the chylothorax varied and was not necessarily progressive. Management of chylothorax ranged from thoracentesis only to thoracotomy with thoracic duct ligation and parietal pleurectomy. When needed, pleurodesis by instillation of sclerosing agents or parietal pleurectomy appeared to be effective in controlling chylothorax. CONCLUSIONS: Chylothorax occurring in patients with LAM has a variable clinical course. Although pleurodesis with or without thoracic duct ligation appears to be effective in controlling intractable chylothorax, less invasive treatments such as thoracentesis or observation may suffice in some cases. Management of chylothorax in patients with LAM should be individualized depending on the size and clinical effects of the chylous pleural effusion, as well as comorbid factors and local expertise.

Adult↗

[Congenital and acquired chylothorax].

BACKGROUND/PURPOSE: Chylothorax in paediatric age is a life-threatening clinical entity that cause serious respiratory, nutritional and immunologic complications. Chylothorax in the absence of trauma or tumour is uncommon and lymphangiomatosis of the bone, although extremely rare, has been associated with these condition. The authors describe the case of a two-year-old girl who presented with a massive chylothorax associated with hip and paravertebral lymphangioma and spread lymphangiomatosis of the spine. The authors also review the literature and their experience of congenital and postoperative chylothorax in order to establish guidelines for the diagnosis and management of both primary and postoperative chylothorax in paediatric age. METHODS: From 1990 and 1999, 14 children had chylothorax. 9 patients had pleural effusion after surgical procedure, 5 patients had congenital chylothorax (both in prenatal and neonatal time), one of whom with bone lymphangiomatosis associated. RESULTS: Postoperative chylothorax has been successfully treated by conservative approach (starvation, total parenteral nutrition and chest tube) in 6 out of 7 cases (two patients died because of complex cardiac malformation). Conservative approach is useful in case of congenital chylothorax, but not with bone lymphangiomatosis associated. CONCLUSIONS: Postoperative and congenital chylothorax is well managed with conservative treatment. Chylothorax with bone lymphangiomatosis associated needs early and aggressive surgical approach.

Bone Neoplasms↗

Lymphangiographic evaluation of experimentally induced chylothorax after ligation of the cranial vena cava in dogs.

Ligation of the cranial vena cava (CrVC) distal to the entrance of the azygous vein resulted in chylothorax in 7 of 10 dogs. Of the remaining 3 dogs, 1 developed a serosanguineous effusion that did not become chylous, and 2 dogs did not develop pleural effusion. In 2 of the 7 dogs developing chylothorax, the pleural effusion became serosanguineous within 2.5 weeks after CrVC ligation. Mesenteric lymphangiography was performed 2 to 6 weeks after ligation of the CrVC. Lymphangiectasia was seen in 4 dogs with chylothorax, but was not seen in the 3 dogs with serosanguineous effusions or the 2 dogs that did not develop effusions. One dog with chylothorax died prior to repeat lymphangiography. Less dye entered the thoracic duct, and alternate lymphaticovenous communications to the caudal vena cava were evident in the dogs without chylothorax. Ligation of the thoracic duct at the lymphaticovenous junction was performed in 3 dogs. These dogs did not develop pleural effusion. Lymphangiography was performed immediately after ligation and indicated filling of abdominal lymphatics but not of the thoracic duct. Lymphangiographic findings 6 weeks after ligation also indicated filling of intestinal lymphatics. Results of the present study indicated that ligation of the CrVC causes chylothorax, and that thoracic lymphangiectasia is a consistent finding in animals with experimental chylothorax. Obstruction of the thoracic duct did not induce lymphangiectasia or chylothorax. Impedence of thoracic duct flow into the CrVC may be a cause of clinical chylothorax in the dog.

Animals↗

Etiology and management of pediatric chylothorax.

OBJECTIVES: To determine the incidence and etiology of chylothorax and to assess our therapeutic management approach. STUDY DESIGN: We reviewed 51 patients diagnosed with chylothorax over a 12-year period. Cause, interval between operation and diagnosis, duration of chylothorax, and total volume loss per weight were recorded. RESULTS: Chylothorax was diagnosed in 46 children after cardiothoracic surgery, giving an incidence of 2.5% (46/1842); in 1 child chylothorax occurred after chest trauma, and in 4 the chylothorax was congenital or a manifestation of lymph angiomatosis. Three etiologic groups were identified: group 1, direct injury to the thoracic duct (33/51 = 65%); group 2, thrombosis and/or high venous pressure in the superior vena cava (14/51 = 27%); and group 3, congenital (4/51 = 8%). Conservative treatment was the only treatment in 80% of the patients. Surgical procedures consisted of 4 ligations of the thoracic duct, placement of 7 pleurodesis shunts, and placement of 2 pleuroperitoneal shunts. Patients in groups 2 and 3 were at higher risk for failure of conservative treatment (P <. 005). Longer duration of chylothorax and higher volume of drainage were present in group 2 compared with group 1 (P <.01). CONCLUSION: Conservative treatment was successful in 80% of the patients with our management approach. Prevention, early recognition, and treatment of potential complications, such as superior vena cava thrombosis or obstruction, may further improve success of conservative treatment. Congenital chylothorax seems different and may require a specific approach.

Angiomatosis↗

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↗

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↗

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↗

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↗

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↗