Refractory Chylothorax Research Articles

Therapeutic Effectiveness of Diagnostic Lymphangiography for Refractory Postoperative Chylothorax and Chylous Ascites: Correlation With Radiologic Findings and Preceding Medical Treatment

The purpose of this study was to assess the therapeutic effect of lymphangiography for refractory postoperative chylothorax and chylous ascites and analyze the relation between the clinical outcomes and radiologic findings or response to the preceding medical treatment. Between January 2004 and June 2012, 14 patients underwent lymphangiography. All patients had been unresponsive to at least two medical treatments for 3-62 days (median, 13.5 days) before lymphangiography. Leaks were classified as major, minor, and undetectable on the basis of radiologic findings including CT and conventional radiography after lymphangiography. The clinical outcomes were correlated with the radiologic findings or the changes of the daily chylous output after the preceding medical treatments. The leaks were healed in nine of 14 patients (64.3%) by 3-29 days (median, 8 days) after lymphangiography. Healing was achieved for two of seven major leaks, and all of the minor leaks (n=4) and undetectable leaks (n=3) after lymphangiography. The remaining five major leaks were not healed after a median follow-up of 15 days. The daily output decreased more than half after medical treatment in seven of the 14 patients, and the leak was healed in six of these patients (85.7%) by 5-18 days (median, 10 days). Lymphangiography might be useful for the treatment of refractory postoperative chylothorax and chylous ascites.

Successful Treatment of Refractory Idiopathic Chylothorax with Thoracoscopic Ligation of the Thoracic Ducts and Pleurodesis

Successful Treatment of Refractory Idiopathic Chylothorax with Thoracoscopic Ligation of the Thoracic Ducts and Pleurodesis

Management of Refractory Chylothorax After Pediatric Cardiovascular Surgery

We investigated the optimal treatment for refractory chylothorax after pediatric cardiovascular surgery. We retrospectively reviewed the cases of 15 consecutive patients who developed chylothorax after congenital heart surgery performed between December 2004 and November 2010. Among the 15 patients (12 male and 3 female; median age 13.9 months) who developed postoperative chylothorax, 10 recovered with conservative therapy, such as a low-fat diet, medium chain triglyceride-enriched diet, or total parenteral nutrition. Of the remaining 5 patients who underwent surgical treatment followed by conventional therapy, 4 showed improvement, and 1 died from cardiac failure. Surgical treatment was performed at a median of 19 days after diagnosis of chylothorax. Average drainage output of thoracocentesis for the first 5 days before thoracic duct ligation was 33.1 ml/kg/day. Duration of chylous fluid drainage was significantly longer in surgical patients than in patients who recovered with conservative therapy (p < 0.01). Surgical patients tended to be younger with lower body weight. Significant risk factors for surgical intervention were age <4 months, body weight <4 kg, and duration of drainage >10 days. In cases of refractory postoperative chylothorax, surgical therapy such as thoracic duct ligation should be considered when discharge from the drainage tube is >30 ml/kg/day or chylothorax is not improved within 10 days.

Successful pleurodesis for refractory chylothorax due to chronic lymphocytic leukemia

IntroductionChylothorax is a very rare complication of chronic lymphocytic leukemia. Presentation of caseWe describe the case of an 83-year old woman with chronic lymphocytic leukemia, complicated by recurrent chylothorax and ultimately treated by pleurodesis with bleomycin. DiscussionThere are several options for management of patients presenting with chylothorax due to chronic lymphocytic leukemia. ConclusionPleurodesis is a reasonable and effective treatment modality for patients with refractory chylothorax.

Octreotide as therapeutic option for congenital idiopathic chylothorax: a case series

Octreotide, a somatostatin analogue, is used for the management of patients with refractory chylothorax although its safety and efficacy in neonates have not been evaluated in controlled clinical trials. We present one of the largest case series about the use of octreotide in congenital idiopathic chylothorax. Six cases of congenital chylothorax (CC) were prospectively collected, who were managed with same unit protocol for octreotide. Mean (SD) gestation was 34.5 (±2.2) weeks, and birthweight was 3410 (±840.4) g. All infants required chest drains from day 1 of life, and the mean (SD) duration of insertion was 36.1 (±8.5) days. Octreotide was commenced at a median age of 13.5 days (range 8-22), given for a median duration of 20 days (range 12-27). The starting dose was 0.5-1 μg/kg/h with an increment of 1-2 μg/kg/day to a maximum of 10 μg/kg/day. Resolution of chylothorax was achieved in five patients, being resistant to treatment in the sixth patient. None had adverse effects from octreotide. Full enteral feeds were reached at a mean age of 44 days. Early commencement of octreotide is recommended although further reports to evaluate the safety and efficacy would add to the profile of this medication in the treatment of CC.

Tc-99m Human Serum Albumin Lymphoscintigraphy With SPECT/CT in Chylothorax

A 45-year-old man who had refractory right chylothorax after esophagectomy for esophageal cancer, underwent lymphoscintigraphy with Tc-99m human serum albumin. Focal abnormal uptake was seen in the mid-abdomen on planar image 30 minutes after the tracer injection. SPECT/CT delineated the extent of the accumulation between the anastomotic site and the right pleural effusion area. SPECT/CT effectively detected the site of lymphatic leakage.

A New Technique of Continuous Pleural Irrigation with Minocycline Administration for Refractory Chylothorax

Chylothorax is a rare and serious complication of thoracic surgery and can lead to respiratory insufficiency, protein loss, fluid imbalance, and immunodeficiency. Chylothorax is often difficult and complex to manage. Although multiple approaches for the treatment of chylothorax have been reported, there is still no clear consensus on its optimal management. A surgical approach involving careful dissection and clipping of lymphatic vessels can help effectively manage chylous fistula in a majority of cases. Occasionally, chyle leak may not be apparent during surgery, and thus ligation of the bed of the thoracic duct may not be sufficient to stop the chyle leak. We report here on 3 patients with chylothorax in whom pleurodesis was successfully achieved via continuous intrapleural irrigation with minocycline (800 mg minocycline and 800 mg of 2 % lidocaine hydrochloride in 1000 mL normal saline, set to 100 mL per hour) instead of conventional intermittent pleurodesis.

Intralipid application and massive ligation around the aorta for refractory chylothorax: a case report

A 58 years old male patient underwent esophagectomy for esophageal cancer. Refractory chylothorax happened after operation in spite of different treatment including conservation therapy and thoracic duct ligation. He was enterally administrated 250 mL 30% intralipid before operation. Exploration showed there was a milky leakage from the soft tissue near the anastomotic stoma behind aortic arch where is difficult to handle. We gently mobilized the aorta in the front of the ninth spine and bundled it with its adjacent tissue including azygos vein and thoracic duct with a dacron patch. The leakage ceased rightly and the following recovery went smoothly.

Octreotide for the treatment of chylothorax in neonates

Routine care for chylothorax in neonate includes either conservative or surgical approaches. Octreotide, a somatostatin analogue, has been used for the management of patients with refractory chylothorax not responding to conservative management. To assess the efficacy and safety of octreotide in the treatment of chylothorax in neonates. We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library), MEDLINE and EMBASE (to March 7, 2010). We assessed the reference lists of identified trials and abstracts from the annual meetings of the Pediatric Academic Societies published in Pediatric Research (2002 to 2009) without language restrictions. We planned to include randomised or quasi-randomised controlled trials of octreotide in the treatment of congenital or acquired chylothorax in term or preterm neonates, with any dose, duration or route of administration. Data on primary (amount of fluid drainage, respiratory support, mortality) and secondary outcomes (side effects) were planned to be collected and analysed using mean difference, relative risk and risk difference with 95% confidence intervals. No randomised controlled trials were identified. Nineteen case reports of 20 neonates with chylothorax in whom octreotide was used either subcutaneously or intravenously were identified. Fourteen case reports described successful use (resolution of chylothorax), four reported failure (no resolution) and one reported equivocal results following use of octreotide. The timing of initiation, dose, duration and frequency of doses varied markedly. Gastrointestinal intolerance and clinical presentations suggestive of necrotizing enterocolitis and transient hypothyroidism were reported as side effects. No practice recommendation can be made based on the evidence identified in this review. A prospective registry of chylothorax patients and a subsequent multicenter randomised controlled trial are needed to assess the safety and efficacy of octreotide in the treatment of chylothorax in neonates.

Refractory chylothorax following a transhepatic gunshot wound to the abdomen requiring unorthodox surgical treatment.

An 18 year-old-male sustained a gunshot wound to the abdomen which required an uneventful hepatorrhaphy. He later returned with a large right effusion and was diagnosed with a chylothorax. The output was persistent despite conservative measures. Thoracotomy with attempted thoracic duct ligation was unsuccessful at decreasing the output. Re-exploration and ligation of the thoracic duct was required thru an abdominal approach.

Clinical analysis of patients with pulmonary lymphangioleiomyomatosis (PLAM) in mainland China

There have been no clinical reports on pulmonary lymphangioleiomyomatosis (PLAM) based on large studies or epidemiological surveys in mainland China. The purpose of this study was to provide a retrospective analysis of PLAM patients in mainland China by reviewing the clinical data of PLAM cases reported. The China Academic Journals Full-text Database search engine was used to collect related cases in mainland China through the end of 2008. 120 cases met the study's inclusion criteria and were reviewed for this analysis. The average age of the 120 patients upon confirmed diagnosis was 37.3+/-6.4 years. The average duration from onset of symptoms to a confirmed diagnosis was 29.6+/-35.8 months, with 80 person-time patients having experienced misdiagnosis before the confirmed diagnosis. The major clinical manifestations of PLAM included progressive dyspnea, recurrent pneumatothorax, refractory chylothorax. Pulmonary function abnormalities included obstructive pulmonary ventilation disorders and degenerated diffusing capacity. Ten patients were found to be complicated with renal angiomyolipoma and 17 with abdominal or pelvic lymphangioleiomyoma. Half of the patients had undergone antiestrogen therapies such as progesterone, and four patients received pulmonary transplantation. The average duration from the confirmed diagnosis to death was 36.4+/-48.9 months among the 28 cases of death. Doctors in mainland China are becoming increasingly vigilant to PLAM, although misdiagnosis or missed diagnosis still exists. Provider attention to the correlation between PLAM and tuberous sclerosis complex, as well as to the possible involvement of multiple organs, is insufficient.

Thoracic Duct Ligation for Persistent Chylothorax After Pediatric Cardiothoracic Surgery

There is considerable literature on incidence and medical management of postsurgical chylothorax in children but little is known about outcomes of thoracic duct ligation (TDL) for patients refractory to medical therapy. A retrospective review of patients undergoing TDL after cardiothoracic surgery (1992 through 2007) was done. Data on demographics including cardiac morphology, characteristics of chylous drainage, medical management, and post-TDL course were collected. When available, imaging studies of the upper body venous drainage vessels were examined. Twenty patients (median age, 0.65 years; range, 0.03 to 11 years; weight, 7.0 kg; range, 2.6 to 30 kg) had a diagnosis of chylothorax made 8.5 days (range, 2 to 118 days) after initial operation. Median duration of pre-TDL medical management was 17.5 days (range, 7 to 69 days). Median drainage for 5 days preceding TDL was 34.5 mL x kg(-1) x d(-1) (range, 15 to 135 mL x kg(-1) x d(-1)) with maximal output of 65 mL x kg(-1) x d(-1) (range, 30 to 200 mL x kg(-1) x d(-1)). After TDL, there was a decrease in median drainage to 13 mL x kg(-1) x d(-1) (range, 4 to 160 mL x kg(-1) x d(-1); p = 0.003). Chest tubes were removed 8.5 days (range, 4 to 34 days) after TDL. There were 4 deaths (none attributed to TDL), 2 treatment failures (post-TDL chest tube drainage > 2 mL x kg(-1) x d(-1) > 14 days), and 2 recurrences (after initial chylothorax resolution and hospital discharge). Three patients had documented upper body venous thrombosis. Univariate analysis demonstrated thrombosis of upper body venous vessels (p = 0.02) and prolonged post-TDL chest tube drainage (p = 0.01) were risk factors for death, treatment failure, or chylothorax recurrence. Thoracic duct ligation leads to a major reduction in chest tube drainage and prompt tube removal in most pediatric patients and should be considered early in refractory postoperative chylothorax. Patients with upper body venous thrombosis associated with chylothorax are at a high risk for failure of TDL and mortality.

Complete Remission of a Lymphoma-Associated Chylothorax by Radiotherapy of the Celiac Trunk and Thoracic Duct

A chylothorax is a rare complication of mostly advanced malignant lymphomas. A case of a refractory chylothorax unresponsive to chemotherapy and successfully treated with radiotherapy is reported. A 45-year-old woman with recurrent stage IV low-grade follicular non-Hodgkin's lymphoma and a progressive chylothorax is described. The CT scans showed bulky lymphadenopathy at the thoracic trunk but no detectable enlargement of mediastinal lymph nodes. After ineffective pretreatment including chemotherapy and chest drainage, fractionated radiotherapy to the celiac trunk (20.4 Gy) and the thoracic duct (15 Gy) was performed. Already after 7.5 Gy a rapid decline of chylothorax was noted and the chest drain could be removed. A complete remission of the chylothorax could be achieved after 20.4 Gy. During a follow-up of 16 months no recurrence of chylothorax occurred. CT scans showed nearly complete remission of the lymphadenopathy of the celiac trunk 12 months after radiotherapy. Radiotherapy with limited total doses is an effective treatment option for lymphoma-associated chylothorax and should always be taken into consideration, especially in cases unresponsive to chemotherapy.

Persistent chylothorax in lymphangioleiomyomatosis treated by intrapleural instillation of povidone

Lymphangioleiomyomatosis (LAM) is a rare, idiopathic disease affecting young women often complicated by refractory and difficult to manage chylothorax. We report one case of a patient who received a bilateral lung transplantation for LAM presenting a refractory chylothorax. The instillation of povidone iodine in the pleural space was effective in inducing pleurodesis.

Successful treatment of amegakaryocytic thrombocytopenia with anti-CD20 antibody (rituximab) in a patient with systemic lupus erythematosus

Amegakaryocytic thrombocytopenia is an extremely rare disorder in systemic lupus erythematosus, and its mechanism and treatment are still largely unknown. We describe a 42-year-old woman with systemic lupus erythematosus who presented various clinical manifestations of life-threatening amegakaryocytic thrombocytopenia (10,000 platelets/mm3 with a marked decrease of megakaryocytes in the bone marrow), proteinuria, psychosis, refractory chylothorax, ascites, and type II diabetes caused by the anti-insulin receptor autoantibody. She was initially treated with prednisolone (25-50 mg/day) and cyclosporine A (200 mg/day) without any improvement in severe thrombocytopenia. However, her clinical symptoms, including platelet counts, dramatically improved, with a concurrent decrease in the anti-c-Mpl antibody, an autoantibody against the thrombopoietin receptor, after a subsequent treatment with rituximab (375 mg/m2 intravenously, weekly, for two consecutive weeks). Our case suggested that amegakaryocytic thrombocytopenia in patients with systemic lupus erythematosus might be mediated by the anti-c-Mpl antibody and could be treated with rituximab through elimination of pathogenic B cells producing autoimmune antibodies.

A clinical study of congenital chylothorax and octreotide therapy

Purpose : Congenital chylothorax is an accumulation of lymphatic fluid within the pleural space. It is a common cause of unidentified hydrops fetalis. We examined the perinatal history, clinical manifestation, diagnosis, treatment, and outcome in 6 newborns diagnosed to have congenital chylothorax with hydrops fetalis. We also studied the effect of octreotide therapy for congenital chylothorax in relation to conservative treatment. Methods : We retrospectively reviewed the medical records of 6 patients diagnosed to have congenital chylothorax with hydrops fetalis among 27,907 newborns who were born at the Cheil General Hospital and Womens Healthcare Center between January 2004 and July 2007. The diagnosis of chylothorax is based on the analysis of pleural fluid before and after milk feeding. Results : Incidence of congenital chylothorax in this study was 0.021%. All 6 cases were noted in over the 92% lymphocyte in pleural analysis. Transudate was changed into chyle with increasing triglyceride levels above 200 mg/dL after milk feeding. Three of 6 infants improved with conservative treatment, including thoracostomy and assisted ventilation. The others had persistent symptoms despite conservative treatment and responded to octreotide therapy. A complication, specifically vomiting was noted in 1 case during octreotide therapy. Conclusion : In this study, octreotide therapy resulted in a safe and excellent outcome. Therefore, octreotide therapy is considered in severe refractory congenital chylothorax in conservative treatment. Further studies are required to determine appropriate guidelines for octreotide therapy.

One-Stage Excision of Massive Cervicomediastinal Lymphangioma in the Newborn

We report the first neonate successfully treated with one-stage complete excision of massive cervicomediastinal lymphangioma producing alarming respiratory distress. Early postoperative course was complicated by refractory bilateral chylothorax, which effectively responded to insertion of external pleuroperitoneal shunts. At the 6-year follow-up, the child remains symptom free and recurrence free. One-stage resection for cervicomediastinal lymphangioma is curative and well tolerated even shortly after birth.

Thoracoscopic Thoracic Duct Sealing with LigaSure in Two Children with Refractory Postoperative Chylothorax

Chylothorax is an uncommon complication of cardiac surgery. We report two cases of the thoracoscopic management of persistent postoperative chylothorax by thoracic duct thermofusion and section with the LigaSure device. A 6-year-old boy and a 3-year-old girl developed persistent chylothorax following an aortic coarctation repair and a Fontan procedure, respectively. The former presented with a left chylothorax due to direct surgical injury, and the latter, a right chylothorax due to indirect lymphatic rupture secondary to increased venous pressure. In both patients, voluminous chylous drainage persisted for more than 3 weeks despite maximal medical treatment. Ultimately, a right thoracoscopic coagulation and section of the thoracic duct with the 5-mm LigaSure device was performed. The LigaSure is a computer-controlled bipolar diathermy system designed to seal blood vessels up to 7 mm in diameter, with no specific recommendations regarding the lymphatics. The lymph leakage ceased within 2 days in the first patient and immediately in the second one, with no recurrence after oral intake resumption on day 5. Both patients are free of recurrence at 1 year. Thoracoscopic coagulation and section of the thoracic duct above the diaphragm with the LigaSure device appears to be a simple, effective, and safe therapeutic option for treatment of refractory postoperative chylothorax in children.

Nonimmune Hydrops Fetalis Due to Generalized Lymphatic Dysplasia in an Infant with Robertsonian Trisomy 21

We report the first case of generalized lymphatic dysplasia and trisomy 21 presenting with nonimmune hydrops fetalis. This infant showed intractable chylothorax, chylous ascites, and periodic bouts of edema. A karyotype analysis revealed Robertsonian trisomy 21: 46,XY,t(14q21q)(q10;q10) +21. This patient died of multiple organ failure at 400 days of life, despite the management of chylous effusions. The lymphoscintigraphy and histopathological findings led to the final diagnosis of generalized lymphatic dysplasia, which might also contribute to the development of hydrops. Refractory chylothorax in trisomy 21 patients may emphasize the need for intensive scrutiny of lymphatic disorders.

Transjugular Intrahepatic Portosystemic Shunt Creation as Treatment for Refractory Chylous Ascites and Chylothorax in a Patient with Cirrhosis

The etiology of chylothorax is usually considered to consist of four major categories: tumors, trauma, idiopathic conditions, and miscellaneous conditions. It appears that chylothorax is a rare and underreported manifestation of cirrhosis resulting from transdiaphragmatic passage of chylous ascites. This condition can be debilitating as a result of respiratory compromise from a large volume of pleural fluid, as well as metabolic derangements, malnutrition, and immunologic impairment from loss of vital lymphatic constituents. Herein the authors present a case of a 46-year-old male patient with cirrhosis and complications of high-volume chylous ascites and chylothorax who was successfully treated with creation of a transjugular intrahepatic portosystemic shunt.