Chylous Effusion Research Articles

Cytologic, Immunocytochemical and Ultrastructural Characterization of Lymphangioleiomyomatosis Cell Clusters in Chylous Effusions of Patients with Lymphangioleiomyomatosis

To establish the cytologic and immunocytochemical features of lymphangioleiomyomatosis (LAM) cell clusters (LCCs) and to clarify its diagnostic significance for LAM.We evaluated 17 samples of LAM-associated chylous effisions from 13 patients with LAM. We performed Papanicolaou staining and immunocytochemistry for muscular antigens, melanoma-related antigens, female 'hormone receptors and markers for lymphatic endothelial cells (LECs).The cytologic features of LCCs were a well-organized, globular cluster consisting of LAM cells enveloped by LECs. The LAM cells were observed to form a tightly cohesive core and had a moderate nuclear/cytoplasmic ratio. These are distinct characteristics from cancer cell clusters. Immunocytochemical examinations revealed the LAM cells to be positive for muscular antigens, melanoma-related antigens and progesterone receptor, but only 2 of 7 specimens were positive for estrogen receptor. The surface monolayer cells were confirmed to be immunopositive for various LEC markers. Ultrastructural study confirmed that LCCs were covered by LECs.LCCs were detected in all LAM-associated chylous effusion samples. The cytologic and immunocytochemical examinations of chylous effusions are thus considered to have diagnostic significance for LAM that may therefore enable patients to avoid undergoing such invasive tests as lung biopsies.

Serum Vascular Endothelial Growth Factor-D Levels in Patients With Lymphangioleiomyomatosis Reflect Lymphatic Involvement

Lymphangioleiomyomatosis (LAM) is a rare multisystem disorder affecting primarily women of child-bearing age, and characterized by cystic lung destruction, tumors of the kidney (angiomyolipomas [AMLs]), and involvement of the axial lymphatics (lymphangioleiomyomas). Patients with LAM experience loss of pulmonary function attributed to the proliferation of abnormal-appearing smooth muscle-like cells (LAM cells). It is possible to group the LAM population by the presence or absence of extrapulmonary involvement (eg, AMLs, lymphangioleiomyomas, chylous effusions). Serum vascular endothelial growth factor (VEGF)-D, a lymphangiogenic factor, is higher in LAM patients than in healthy volunteers and has been proposed as a tool in the differential diagnosis of cystic lung disease. We assessed serum VEGF-D concentrations in relationship to clinical phenotype in LAM patients. Serum VEGF-D levels were quantified by enzyme immunosorbent assay for 111 patients with LAM and 40 healthy volunteers. VEGF-D levels in patients with pulmonary LAM, with or without extrapulmonary manifestations, were compared to those of healthy volunteers. Serum VEGF-D levels were greater in patients with LAM compared to those of healthy volunteers (p < 0.001). However, when patient samples were grouped based on the extent of lymphatic extrapulmonary involvement (eg, lymphangioleiomyomas and adenopathy), the statistical difference was maintained only for patients with LAM with lymphatic involvement (p < 0.001), not for those patients whose disease was restricted to the lung. Serum VEGF-D levels are a good biomarker for lymphatic involvement (area under the curve [AUC], 0.845; p < 0.0001), and a fair predictor for LAM disease (AUC, 0.751; p < 0.0001). Serum levels correlated to CT scan grade (p = 0.033). Serum VEGF-D concentration is a measure of lymphatic involvement in patients with LAM.

Ectopic Thyroid Carcinoma Causing Right Ventricular Outflow Tract Obstruction in a Dog

A 9-year-old Bouvier des Flandres was presented with coughing, lethargy, chylous pleural effusion, and a heart murmur. An echocardiogram revealed the presence of an intracardiac mass causing right ventricular outflow tract obstruction. The mass was successfully removed surgically, using total inflow occlusion. Histopathology and immunohistochemistry identified the tumor as an ectopic thyroid carcinoma. The dog was euthanized 11 months after diagnosis at the request of the owner because of nonresolving chylothorax.

Peritoneoatrial Shunting for Intractable Chylous Ascites Complicating Thoracic Duct Ligation

Thoracic duct ligation for chylothorax is considered a safe and efficient procedure. However, we observed two cases that were complicated by intractable chylous ascites. Refractory chylous ascites are usually cured by surgical peritoneovenous shunting, but in both patients successful treatment required peritoneoatrial shunting. Actually, a peritoneovenous shunt was impossible because of extensive venous thrombosis in jugular and superior vena cava in one patient and failed because of constrictive pericarditis requiring pericardectomy in the other, both underlying diseases also accounting for the thoracic duct ligation complications.

Octreotide for Management of Chylothorax following Bidirectional Glenn in a Three-Month-Old Infant

Chylothorax developing after open heart surgery for congenital heart defects can be a challenging problem. Established therapies are not always efficacious. This report summarizes our experience using octreotide as an adjunct in the management of an intractable chylous effusion. The patient described underwent a bilateral bidirectional cavopulmonary anastomosis at 3 months of age. Postoperatively, the patient demonstrated significant quantities of milky fluid drainage from the right pleural chest tube. Initial management included diuretics, afterload reduction, and a non-fat diet. These maneuvers appeared to be effective. However, the patient experienced a recurrence of the effusion requiring re-admission to the hospital and re-insertion of a right pleural chest tube. Given the recalcitrant nature of the effusion, an infusion of octreotide was begun. An octreotide infusion was begun at 1 mcg/kg/hr and gradually increased to 3 mcg/kg/hr. Chest tube drainage diminished allowing chest tube removal 13 days later. The octreotide infusion was weaned off over the subsequent 10 days without any further effusion problems. Octreotide may be a useful adjunct in the management of intractable chylous effusions following congenital heart surgery.

Chylothorax Associated With Tricuspid Dysplasia and Atrial Septal Defect in a Bullmastiff

Transudate pleural effusion associated with tricuspid dysplasia and ostium secundum-type atrial septal defect was diagnosed in a 14-month-old bullmastiff. Following administration of furosemide and an angiotensin-converting enzyme (ACE) inhibitor, the dog remained free of pleural effusion for 10 months, until he showed severe dyspnea due to chylothorax. Medical therapy was unsuccessful to avoid recurrence of life-threatening pleural chylous effusion. Ligation of the thoracic duct and apposition of an omental pedicle flap were effective in the resolution of pleural chylous leakage.

Octreotide for Management of Chylothorax following Bidirectional Glenn in a Three-Month-Old Infant. Comment

Background: Chylothorax developing after open heart surgery for congenital heart defects can be a challenging problem. Established therapies are not always efficacious. This report summarizes our experience using octreotide as an adjunct in the management of an intractable chylous effusion. Methods: The patient described underwent a bilateral bidirectional cavopulmonary anastomosis at 3 months of age. Postoperatively, the patient demonstrated significant quantities of milky fluid drainage from the right pleural chest tube. Initial management included diuretics, afterload reduction, and a non-fat diet. These maneuvers appeared to be effective. However, the patient experienced a recurrence of the effusion requiring re-admission to the hospital and re-insertion of a right pleural chest tube. Given the recalcitrant nature of the effusion, an infusion of octreotide was begun. Results: An octreotide infusion was begun at 1 mcg/kg/hr and gradually increased to 3 mcg/kg/hr. Chest tube drainage diminished allowing chest tube removal 13 days later. The octreotide infusion was weaned off over the subsequent 10 days without any further effusion problems. Conclusions: Octreotide may be a useful adjunct in the management of intractable chylous effusions following congenital heart surgery.

Pleural fluid characteristics of chylothorax.

To determine the biochemical parameters of chylous pleural fluids and better inform current clinical practice in the diagnosis of chylothorax. We retrospectively reviewed 74 patients with chylothorax (defined by the presence of chylomicrons) who underwent evaluation during a 10-year period from January 1, 1997, through December 31, 2006. The biochemical parameters and appearance of the fluid assessed during diagnostic evaluation were analyzed. The study consisted of 37 men (50%) and 37 women (50%), with a median age of 61.5 years (range, 20-93 years). Chylothorax was caused by surgical procedures in 51%. The chylous pleural fluid appeared milky in only 44%. Pleural effusion was exudative in 64 patients (86%) and transudative in 10 patients (14%). However, pleural fluid protein and lactate dehydrogenase levels varied widely. Transudative chylothorax was present in all 4 patients with cirrhosis but was also seen with other causes. The mean +/- SD triglyceride level was 728+/-797 mg/dL, and the mean +/- SD cholesterol value was 66+/-30 mg/dL. The pleural fluid triglyceride value was less than 110 mg/dL in 10 patients (14%) with chylothorax, 2 of whom had a triglyceride value lower than 50 mg/dL. Chylothoraces may present with variable pleural fluid appearance and biochemical characteristics. Nonmilky appearance is common. Chylous effusions can be transudative, most commonly in patients with cirrhosis. Traditional triglyceride cutoff values used in excluding the presence of chylothorax may miss the diagnosis in fasting patients, particularly in the postoperative state.

Bilateral idiopathic chylothorax associated with light physical exercise

Chylothoraces are associated with multiple etiologies including non-Hodgkin lymphoma and intrathoracic surgical trauma. In about 15% of cases no reason can be found for the chylous pleural effusion: most of these idiopathic chylothoraces are thought to be related to minor trauma. We present a case of bilateral idiopathic chylothorax temporally associated with light physical exercise. A 74-year-old woman was brought to the hospital for evaluation of dyspnea and right-sided pleuritic chest pain. A chest roentgenogram demonstrated bilateral pleural effusion. The patient reported that the pain at the right side of her chest started abruptly 48 h prior to admission, when she twisted right and hyperextended her spine to take up an object from the floor. Routine laboratory studies were within normal limits. Thoracentesis yielded a creamy–milky appearing fluid, with elevated tryglicerides levels (>575 mg/dL). A computed tomography scan of the chest and abdomen was normal, except for the bilateral pleural effusion. After 72 h of rest and low-fat diet the effusion completely resolved. Idiopathic chylothorax in adults is an uncommon type of pleural effusion. Its diagnosis requires to rule out other etiologies. The treatment of choice is conservative.

Reconstruction of occluded thoracic duct for treatment of chylopericardium: A novel surgical therapy

Chylopericardium is an uncommon disease predominantly caused by trauma. Prolonged chyle depletion may result in nutritional, metabolic, and immunologic deficiencies due to loss of essential proteins, immunoglobulins, fat, vitamins, electrolytes, and water. Medical treatment includes a low-fat diet with medium-chain triglyceride restriction, cardiac support, diuretic medications, and drainage of the pericardial effusion. Conventional surgical therapy consists of pericardial fenestration and thoracic duct ligation. We report a case of massive secondary chylous pericardial effusion successfully treated with microsurgical lymphovenous anastomosis, reconnecting the occluded thoracic duct to the internal jugular vein. This case highlights features and management strategies of this perplexing clinical condition.

Transudative chylous pleural effusion: Case report

Transudative chylous pleural effusion is a very rare entity. Hereby we present a 46 year old man a case of chronic renal failure and nephrotic syndrome with chylous ascites, lower extremity edema and chylous transudative pleural effusion with slight response to ultrafiltration.

Sirolimus Ameliorated Post Lung Transplant Chylothorax in Lymphangioleiomyomatosis

We report a successful case of sirolimus treatment for chylous pleural and peritoneal effusions of lymphangioleiomyomatosis after lung transplantation. A 32-year-old woman underwent living donor lung transplantation. Persistent chylous pleural and peritoneal effusions were seen postoperatively. Pleurodesis by intrathoracic injection of OK-432, minomycin, and somatostatine analog failed to control chylous effusions. However, sirolimus treatment reduced the amount of chylous drainage and improved both chylous pleural and peritoneal effusions.

Chylothorax and Thymic Lymphoma in a Pet Rabbit ( Oryctolagus cuniculus)

Abstract A 6-year-old dwarf rabbit ( Oryctolagus cuniculus) presented for chronic epiphora and tachypnea. Results of a complete blood count were unremarkable, and a plasma biochemical profile revealed elevated concentrations of blood glucose and alkaline phosphatase and decreased globulin and potassium levels. Thoracic radiography showed a mediastinal mass and significant pleural effusion. Thoracocentesis revealed a chylous effusion, which was confirmed with laboratory testing. Thoracic ultrasonography confirmed the presence of the mass, and fine-needle aspirates were obtained. The final diagnosis was thymic lymphoma and resulting chylothorax. Chylothorax should be considered in the differential diagnosis for rabbits with pleural effusion. Although it is a rare condition in rabbits, chylothorax likely occurs for the same reasons as in dogs and cats.

Manejo nutricional e digestibilidade no quilotórax canino

O quilotórax corresponde ao acúmulo de fluido linfático em um ou em ambos os espaços pleurais, resultante do extravasamento de linfa do ducto torácico ou de uma de suas divisões principais. Este pode estar associado à ascite quilosa em pacientes com distúrbios linfáticos sistêmicos, como na linfangiectasia, em decorrência de defeitos no transporte linfático-venoso do quilo da cavidade torácica para a circulação sistêmica. A linfangiectasia intestinal é um distúrbio obstrutivo que envolve o sistema linfático do órgão e revela-se como a causa mais comum de enteropatias associadas à má absorção e perda de proteínas em cães. São apresentados no presente relato o controle clínico com o uso de dieta caseira com baixa gordura e a quantificação da função digestiva, por meio da determinação da digestibilidade do alimento, em um cão acometido por quilotórax secundário à linfangiectasia intestinal. A dieta caseira incluiu arroz, peito de frango, cenoura, carbonato de cálcio, levedura de cerveja, suplemento vitamínico e mineral e sal. O ensaio de digestibilidade foi conduzido pelo método de coleta total de fezes. A dieta caseira foi eficaz em interromper o acúmulo de efusão quilosa torácica, restabelecer as concentrações de proteína total e albumina e promover ganho de peso no paciente. Os resultados da digestibilidade demonstraram menor aproveitamento de todos os nutrientes no cão com linfangiectasia em relação ao controle sadio.

Chylothorax: A Rare Complication of Tube Thoracostomy

Chylothorax resulting from chest tube injury to the thoracic duct is very rare and underreported. The purpose of this case report is to exemplify this rare but potentially significant complication of chest tube thoracostomy. An 86-year-old woman presented with sepsis and a massive right pleural effusion; she developed a chylous effusion with the pleural fluid triglyceride level of 158 mg/dL 2 days after a traumatic chest tube insertion. All investigations excluded common causes of non-traumatic chylothorax. The chylothorax improved after fasting and implementation of a medium-chain triglyceride diet. The optimal depth of insertion of the chest tube typically ranges from 5 to 15 cm, ensuring all sideports are within the chest and the proximal port is at least 2 cm beyond the rib margin. Traumatic chylothorax secondary to chest tube insertion should be included in the differential diagnosis of patients presenting with chylothorax after a thoracostomy tube.

Treatment of refractory lymphangioleiomyomatosis–associated chylous effusion with a pleuroperitoneal window and omental flap

Lymphangioleiomyomatosis (LAM) is a rare progressive cystic disease of the lung that predominantly affects women. Clinical features of the disease include spontaneous pneumothoracices, dyspnea, cough, as well as the formation of chylous effusions. These effusions are often refractory to treatment. We describe a case of a 74-year-old woman with LAM associated chylous effusions that was refractory to standard treatments including bowel rest with total parental nutrition (TPN), thoracic duct ligation, and pleurectomy but was successfully managed with a novel combination of oral progesterone, rapamycin, and doxycycline combined with the creation of a pleuroperitoneal shunt with omental flap placement in the pleural cavity.

Sirolimus Amelioration of Clinical Symptoms of Recurrent Lymphangioleiomyomatosis After Living-donor Lobar Lung Transplantation

In this study we report the case of a 28-year-old female patient with recurrent lymphangioleiomyomatosis (LAM) in the allografts after bilateral living-donor lobar lung transplantation. Although her post-operative course under immunosuppression with tacrolimus and prednisolone had been uneventful without rejection episodes, she had developed shortness of breath and a progressive chylous effusion with diffuse cystic changes in both lungs 5 years after transplantation. In spite of a diagnosis of having a recurrence of LAM based on radiologic findings and deteriorating pulmonary function, her clinical symptoms, which included dyspnea and chylothorax, were significantly improved after treatment with sirolimus. Although a beneficial effect of sirolimus in the treatment of LAM has not been definitively determined, this report may provide useful information for management of recurrent LAM after lung transplantation.

Chylopericardium in adults: A literature review over the past decade (1996–2006)

We present a systematic overview of the literature on reported cases of chylous pericardial effusion in adults over the past 10 years, assessing clinical presentation, etiology, diagnosis, and treatment. We conducted an evidence-based literature review searching for all reported adult cases of chylopericardium in the past 10 years (January 1996-December 2006). A total of 33 reported cases of chylopericardium were identified through a systematic literature search. There was no gender bias in the incidence of chylous pericardial effusion. Age at diagnosis varied from 18 to 68 years with a mean +/- standard deviation of 36.78 +/- 14.71 years. Time from symptom onset to diagnosis was variable (ranging from acute presentation to several years). The most common reported etiology was idiopathic. The diagnosis was made by pericardiocentesis in all patients. A search for secondary causes included computed tomography of the chest, lymphoscintigraphy, and lymphangiography along with radioactive oral (131)I-triolein. Conservative therapy was prescribed in 28 patients and failed in 16, in whom subsequent surgery was necessary. Overall, 21 patients underwent various types of surgery. Thoracic duct ligation with creation of a pericardial window was the most common surgical procedure. Chylopericardium is a rare entity. The fluid appearance, triglyceride content, cytologic characteristics, and negative cultures are crucial to make the correct diagnosis. Treatment is dependent on the etiology. Surgical management is the most successful. Conservative therapy is reserved for patients with idiopathic chylopericardium, those with an untreatable etiology, those considered at high risk for surgical treatment, or those with a predictably short lifespan.

Pleural Fluid Analysis in Chylous Pleural Effusion

Chyle is a noninflammatory, lymphocyte-predominant fluid that may cause a pleural effusion as a consequence of thoracic duct leakage into the pleural space. Although chyle is reported to have protein concentrations in the transudative range, chylous effusions are typically exudative, as defined by the standard criteria. We hypothesized that chylous effusions from a thoracic duct leak alone have low lactate dehydrogenase (LDH) concentrations due to the absence of inflammation and are lymphocyte-predominant, protein-discordant exudates. Consequently, pleural effusions that do not meet these criteria but with triglyceride concentrations of > 110 mg/dL or are positive for chylomicrons should be associated with other diagnoses contributing to pleural fluid formation. Retrospective. The pleural fluid analyses of 876 consecutive thoracenteses were reviewed. All cases with a triglyceride concentration of > 110 mg/dL or the presence of chylomicrons were retrieved. The effusions were then classified as transudates, concordant exudates, protein-discordant exudates, and LDH-discordant exudates, and according to lymphocyte predominance (> 50%). The causes of these pleural effusions were determined after the review of the medical record. Twenty-two pleural effusions had elevated triglyceride concentrations and/or were positive for chylomicrons. Eleven effusions were lymphocyte-predominant, protein-discordant exudates, and two of these were associated with chylous ascites. The remaining effusions were transudates (n = 7) or concordant exudates (n = 4); all were associated with conditions known to cause pleural effusion apart from chyle leakage. Chylous effusions caused solely by conditions known to cause chylothorax were lymphocyte-predominant, protein-discordant exudates. Protein concentrations in the transudative range or elevated LDH concentrations were associated with a coexisting condition that may impact the management of these chylous effusions.

Chylous vaginal discharge in a patient with lymphangioleiomyomatosis

We present the case of a patient having chylous vaginal discharge, which may be a symptom of the secondary to an established diagnosis of the systemic disorder, lymphangioleiomyomatosis, a disease predominantly affecting women during their reproductive years. Lymphangioleiomyomatosis causes dyspnea, pneumothorax, chylous pleural effusions, and eventually respiratory failure.