A transudative chylothorax associated with superior vena cava syndrome.
ABSTRACT: The chylothorax is a lymphocyte predominant protein-discordant exudative pleural effusions with low lactate dehydrogenase and elevated triglyceride levels. Transudative chylothoraces associated with Superior Cava syndrome (SVC) are an extremely rare clinical entity. In this manuscript, we describe a case of transudative chylothorax due to SVC obstruction secondary to thrombosis of a peripheral inserted central venous catheter, which ultimately resolved after endovascular intervention. In our review of the literature, only five cases of transudative chylothorax associated with SVC syndrome were identified with 60% of cases associated with thrombosis and complications due to catheters in the central venous circulation. Treatment of the underlying cause is key to resolution of the chylothorax. Thoracentesis is an initial intervention for diagnostic and therapeutic purposes. Endovascular intervention is the primary mode of treatment for SVC thrombosis and stenting is preferred for malignant causes, however anticoagulation alone has been reported in the resolution of chylothorax. In patients with recurrent chylothorax despite of relief of SVC obstruction, a medium-chain triglyceride diet and octreotide can be prescribed in order to decrease the chyle flow in the thoracic duct. Surgical ligation of the thoracic duct can be considered if medical management and endovascular treatment fails.
Project description:Chylous pleural effusion (chylothorax) frequently accompanies lymphatic vessel malformations and other conditions with lymphatic defects. Although retrograde flow of chyle from the thoracic duct is considered a potential mechanism underlying chylothorax in patients and mouse models, the path chyle takes to reach the thoracic cavity is unclear. Herein, we use a novel transgenic mouse model, where doxycycline-induced overexpression of vascular endothelial growth factor (VEGF)-C was driven by the adipocyte-specific promoter adiponectin (ADN), to determine how chylothorax forms. Surprisingly, 100% of adult ADN-VEGF-C mice developed chylothorax within 7 days. Rapid, consistent appearance of chylothorax enabled us to examine the step-by-step development in otherwise normal adult mice. Dynamic imaging with a fluorescent tracer revealed that lymph in the thoracic duct of these mice could enter the thoracic cavity by retrograde flow into enlarged paravertebral lymphatics and subpleural lymphatic plexuses that had incompetent lymphatic valves. Pleural mesothelium overlying the lymphatic plexuses underwent exfoliation that increased during doxycycline exposure. Together, the findings indicate that chylothorax in ADN-VEGF-C mice results from retrograde flow of chyle from the thoracic duct into lymphatic tributaries with defective valves. Chyle extravasates from these plexuses and enters the thoracic cavity through exfoliated regions of the pleural mesothelium.
Project description:Chylous leaks, such as chylothorax and chylopericardium, are uncommon effusions resulting from the leakage of intestinal lymphatic fluid from the thoracic duct (TD) and its tributaries, or intestinal lymphatic ducts. The cause can be either traumatic (thoracic surgery) or nontraumatic (idiopathic, malignancy). Treatment has traditionally consisted of dietary modification (nonfat diet) and/or surgery (TD ligation, pleurodesis). Thoracic duct embolization (TDE) has become a viable treatment alternative due to it high success rate and minimal complications. In this article, the authors describe the etiologies of chylothorax, patient population, outcomes, and long-term follow-up of TDE patients. Relevant lymphatic anatomy and physiology are reviewed, with special attention paid to the formation of the duct by tributaries at the cisterna chyli (CC). The technique of TDE is outlined, including bilateral pedal lymphangiography, TD cannulation, and embolic agents used for the procedure.
Project description:Accurate assessment of the lymphatic system has been limited due to the lack of optimal diagnostic methods. Recently, we adopted noncontrast magnetic resonance (MR) lymphangiography to evaluate the central lymphatic channel. We aimed to investigate the feasibility and the clinical usefulness of noninvasive MR lymphangiography for determining lymphatic disease.Ten patients (age range 42-72 years) with suspected chylothorax (n?=?7) or lymphangioma (n?=?3) who underwent MR lymphangiography were included in this prospective study. The thoracic duct was evaluated using coronal and axial images of heavily T2-weighted sequences, and reconstructed maximum intensity projection. Two radiologists documented visualization of the thoracic duct from the level of the diaphragm to the thoracic duct outlet, and also an area of dispersion around the chyloma or direct continuity between the thoracic duct and mediastinal cystic mass.The entire thoracic duct was successfully delineated in all patients. Lymphangiographic findings played a critical role in identifying leakage sites in cases of postoperative chylothorax, and contributed to differential diagnosis and confirmation of continuity with the thoracic duct in cases of lymphangioma, and also in diagnosing Gorham disease, which is a rare disorder. In patients who underwent surgery, intraoperative findings were matched with lymphangiographic imaging findings.Nonenhanced MR lymphangiography is a safe and effective method for imaging the central lymphatic system, and can contribute to differential diagnosis and appropriate preoperative evaluation of pathologic lymphatic problems.
Project description:Chylothorax is most common on the left side owing to the position of the thoracic duct. Malignancy-associated chylothorax is not uncommon. However, bilateral chylothorax is rare and malignancy should be a consideration in absence of trauma. We report a case of a patient with follicular lymphoma who presented with bilateral pleural effusion; pleural fluid analysis confirmed chylothorax. The patient did not have any significant peripheral or axial lymphadenopathy, which made the diagnosis difficult in absence of histopathology. Pleural fluid analysis was negative for malignant cells, however, the flow cytometry markers were suggestive of follicular lymphoma. Primary effusion lymphoma, which could have been another possibility, was ruled out by the flow cytometry markers. We conclude that pleural fluid flow cytometry markers play an important role where there is no significant lymphadenopathy and in absence of histopathological diagnosis. This demands further evaluation.
Project description:Background:Chylothorax is a rare clinical condition that results from thoracic duct damage with leakage of chyle from the lymphatic system to the pleural space. Rarely, constrictive pericarditis has been associated with chylothorax, but to our knowledge only in relation to secondary causes such as tuberculosis, HIV, or malignancy. Case summary:A previously healthy 63-year-old man presented with effusive-constrictive pericarditis, recurrent right-sided pleural effusion, and chylothorax. There was no history of co-morbidities, surgical illness, or cardiac procedures. No single aetiologic factor was identified despite comprehensive assessment. Substantial immunosuppressive therapy was given without a sufficient clinical response. Pericardiectomy resulted in resolution of the constrictive haemodynamics and terminated chylous effusion. Discussion:The hypothesized mechanisms for development of chylothorax in association with constrictive pericarditis are the increased effective capillary infiltration secondary to central venous hypertension and reduced lymphatic drainage due to high pressure in the left subclavian vein. Increased capillary filtration may result in excessive lymph formation. However, the mechanism is not completely understood.
Project description:Objectives:Postoperative chylothorax is a serious complication after transthoracic esophagectomy, and is associated with major morbidity due to dehydration and malnutrition. For patients with high-output fistula, re-thoracotomy with ligation of the thoracic duct is the treatment of choice. Radiologic interventional management is an innovative procedure that has the potential to replace surgery in the treatment algorithm. Methods:Four patients with high-output chylous leaks following esophagectomy are presented. Ultrasound-guided lymphangiography with embolization of the thoracic duct and/or disruption of the cisterna chyli was performed to occlude the leakage site. Radiologic interventions and procedure-related outcomes are described in detail. Results:In all four patients, ultrasound-guided lymphangiography of the groin with injection of Lipiodol was able to detect and visualize the leakage site in the lower mediastinum. In three patients, the leak could be successfully occluded by Lipiodol embolization. In one patient, embolization failed and the disruption technique was successfully performed. No procedure-related complications were observed. Conclusions:In case of a postoperative chylothorax, radiologic intervention is feasible and safe. The procedure is indicated for high-output chylous fistulas after esophagectomy, and should be applied early after the diagnosis of this postoperative complication.
Project description:Chylothorax originating in a patient with Schimmelpenning syndrome is rare and poses a problem in diagnosis and treatment. A 22-year-old male was admitted with dyspnea indicative of a large pleural chylous effusion. Besides conservative dietary treatment measures, the chylous effusion was drained (2,000 mL/day). Computed tomography-lymphography after ligation of the thoracic duct and pleurectomy revealed a small collateral flow of chylous fluid toward the chest wall and entering the thorax. Eventually, local radiation therapy with 36 Gy effectively treated the chylothorax. Five months later, an epitheloid angiosarcoma developing from a preexisting cutaneous lesion was detected and treated by surgical resection.
Project description:The number of cases of superior vena cava syndrome (SVCS) increased due to increased cardiac devices and central venous catheters. Management of benign SVCS is still controversial. A 51-year-old male known to have ischemic cardiomyopathy and chronic renal failure on regular hemodialysis. In the last 12 months, he had progressive shortness of breath and swelling of his upper part of the body. Examination revealed engorgement of the neck veins, facial puffiness, and pitting edema of both upper limbs. Venography showed occluded SVC. We applied a 50 Watt of energy via electrocautery pen to a Hi-Torque 0.014 Astato guidewire to cross the occluded segment retrogradely. We used 2 stents 39 mm, mounted on BIB 20/40 mm. Final angiography revealed full restoration of SVC flow. Diathermy use to cross a chronic total SVC obstruction is feasible and safe. Endovascular techniques are suitable as initial management of benign SVC syndrome.
Project description:We often observe patients with antiphospholipid syndrome (APS) presenting with both venous and arterial thrombi. Anticoagulant therapy is effective for venous and peripheral arterial embolisms in these patients; however, it has opposite effects when applied for thoracic aortic mural thrombosis because of the risk of new arterial embolisms. Recently, thoracic endovascular aortic repair (TEVAR) has been used to prevent arterial embolisms due to aortic thrombosis. However, we generally hesitate to implant artificial materials in patients in a hypercoagulable state because this can cause new thrombi to develop. Here, we present a case of successful treatment by anticoagulant therapy and TEVAR in an APS patient presenting with pulmonary embolisms (PEs) and multiple arterial embolisms due to thoracic aortic mural thrombosis. A 46-year-old man was referred to our hospital due to dyspnea and leg pain. Since contrast-enhanced computed tomography revealed PEs, thoracic aortic mural thrombosis, and lower limb arterial embolisms, we administered anticoagulation therapy. Three days later, contrast-enhanced computed tomography revealed new arterial embolisms in the right kidney. To prevent further arterial embolisms due to thoracic aortic mural thrombosis, we performed emergent TEVAR in addition to anticoagulant therapy. Thereafter, no venous or arterial embolisms recurred during the 13-month follow-up period. <Learning objective: An optimal therapy has not been established for patients in a hypercoagulable state who are threatened by venous thrombi and multiple arterial embolisms due to thoracic aortic mural thrombosis. In such patients, in addition to anticoagulant therapy, thoracic endovascular aortic repair for thoracic aortic mural thrombosis can be a promising option to prevent further arterial embolisms.>.
Project description:Chylothorax resulting from thoracic duct damage is often difficult to identify and repair. We hypothesized that near-infrared fluorescent light could provide sensitive, real-time, high-resolution intraoperative imaging of thoracic duct anatomy and function.In 16 rats, 4 potential near-infrared fluorescent lymphatic tracers were compared in terms of signal strength and imaging time: indocyanine green, the carboxylic acid of IRDye 800CW (LI-COR, Lincoln, Neb), indocyanine green adsorbed to human serum albumin, and IRDye 800CW conjugated covalently to human serum albumin. Optimal agent was validated in 8 pigs approaching human size (n = 6 by open surgery with FLARE imaging system [Beth Israel Deaconess Medical Center, Boston, Mass] and n = 2 by video-assisted thoracoscopic surgery minimally invasive [m-FLARE] imaging system [Beth Israel Deaconess Medical Center]). Lymphatic tracer injection site, dose, and timing were optimized.For signal strength, sustained imaging time, and clinical translatability, the best lymphatic tracer was indocyanine green, which is already Food and Drug Administration approved for other indications. In pigs, a simple subcutaneous injection of indocyanine green into lower leg (? 36 ?g/kg), provided thoracic duct imaging with onset of about 5 minutes after injection, sustained imaging for at least 60 minutes after injection, and signal-to-background ratio of at least 2. With this technology, normal thoracic duct flow, collateral flow, injury models, and repair models could all be observed under direct visualization.Near-infrared fluorescent light could provide sensitive, sustained, real-time imaging of thoracic duct anatomy and function during both open and video-assisted thoracoscopic surgery in animal models.