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.     

Cervical thoracic duct cyst

Thoracic duct cysts are rare lesions presenting as mediastinal or supraclavicular masses. We report a case of a 77-year-old female who presented with a left supraclavicular mass. A cervical thoracic duct cyst was suspected after contrast-enhanced computed tomography (CT) of the neck and chest. Diagnosis was confirmed by ultrasound-guided cyst aspiration and lymphangiography. Therapeutic embolization of the thoracic duct was unsuccessful. Definitive treatment was achieved with thoracic duct ligation using video-assisted thoracoscopic surgery (VATS) followed by surgical excision of the cyst. This is believed to be the first report of using minimally invasive surgery for thoracic duct ligation in the treatment of a thoracic duct cyst.     

Traumatic injury of the thoracic duct

Injuries to the thoracic duct are infrequent but may become life-threatening when chylous leakage persists. This report describes 6 patients with such injuries in whom the leakage resolved spontaneously in one, was corrected using microsurgical lymphatic repair or lymphatic-venous anastomosis in two, successfully treated either by ligation of the thoracic duct or insertion of a peritoneovenous shunt in two, and was eventually controlled after bilateral pleurodesis and thoracic duct ligation by insertion of a peritoneo-venous shunt in one. Conventional lymphography is superior to lymphoscintigraphy and is usually required to document disruption of the thoracic duct.     

Management of chylothorax via percutaneous embolization

PURPOSE OF REVIEW: The purpose of this review is to present a novel radiologic percutaneous transabdominal technique for treating high-output chylothorax by thoracic duct embolization, and to demonstrate that it can be potentially safer than the traditional treatment by surgical open-chest thoracic duct ligation. RECENT FINDINGS: Pedal lymphography is initially performed to opacify large retroperitoneal lymph channels; a suitable duct more than 2 mm in diameter is then punctured transabdominally to allow catheterization and embolization of the thoracic duct under fluoroscopic guidance. If feeding lymphatic channels are too small for catheterization, they can often be occluded by needle disruption. This percutaneous interventional technique, which has been used in 60 patients with mostly high-output chylothorax caused by thoracic surgery, resulted in a 65% cure rate with no morbidity. Back-up surgical thoracic duct ligation was performed promptly on suitable lower risk patients when the percutaneous procedure failed. SUMMARY: We have found that two thirds of patients presenting with life-threatening chylothorax can be safely treated by percutaneous transabdominal thoracic duct blockage. When successful, this novel interventional procedure can obviate repeat major thoracic surgery and shorten hospital stays.     

Primary idiopathic chylopericardium presenting with cardiac tamponade

Primary idiopathic chylopericardium is an extremely rare condition especially in children and young adults. Although the exact pathophysiology of primary chylopericardium has not been established, the reflux of chylous fluid into the pericardial space was suggested as the etiology. Damage to the thoracic duct valves and the communication of the thoracic duct to the pericardial lymphatics or abnormally elevated pressure in the thoracic duct could cause chylous fluid reflux. In this report, we described the case of a 4-year-old boy with primary idiopathic chylopericardium presenting as cardiac tamponade who was treated with video-assisted thoracoscopic window and then surgical duct ligation.     

胸外科术后乳糜胸诊治分析

目的总结胸外科术后乳糜胸的诊治经验。方法分析我院31例胸外科术后发生乳糜胸的临床资料。结果出现乳糜胸的时间大多数为术后2～10d，26例经保守治疗治愈，5例再次开胸，其中4例治愈，1例术后仍再发，继续保守治疗治愈。结论胸外科术后乳糜胸大多数可经保守治疗治愈，再次开胸作为补救，若高度怀疑损伤胸导管及其主要分支应预防性束状盲扎胸导管。     

Isolated primary chylopericardium

A 16-year-old man was found to have an enlarged cardiac silhouette. Primary chylopericardium was diagnosed when pericardiocentesis yielded the characteristic milky-white fluid. The thoracic duct was easily identified by giving milk and butter and an injection of ethylene blue immediately before the operation. Intraoperative thoracic ductography showed no abnormal findings. Mass ligation of the thoracic duct above the diaphragm and partial pericardiectomy were successfully performed through a right thoracotomy approach. In addition, many of the lymphatics were ligated above the diaphragm. The right thoracotomy approach was a useful method for resection and ligation of the thoracic duct just above the diaphragm. Follow-up showed no accumulation of pericardial fluid or pleural effusion.     

Mediastinal thoracic duct cyst

A healthy 34-year-old man had a mediastinal cyst on the imaging study. Surgical treatment was performed. The cyst was diagnosed as a thoracic duct cyst from its anatomic location and contents. Pathologic examination found it to be consistent with thoracic duct cyst. Endothelial cells on its luminal surface were identified by an immunohistologic stain with the factor VIII-related antigen. Twenty-six cases of thoracic duct cysts have been reported. We report an additional case and review the previously reported cases. We found that the ligation of the inferior pedicle of the cyst is essential to prevent postoperative chylothorax.     

Chyle leakage after blunt trauma.

Three patients developed chylous leakage after major blunt trauma. In one patient with non-remitting right-sided chylothorax, lymphangioscintigraphy as well as conventional oil contrast lymphography demonstrated disruption of the thoracic duct at the aortic hiatus which eventually required transpleural mediastinal ductal ligation proximal to the fistula. The other two patients had chylous retroperitoneum and/or chylous peritoneum which was self-limited although one patient (a three-year-old boy) died of multiorgan failure from associated pulmonary contusions and cervical spine injuries. Chylous leak after non-penetrating trauma is usually attributed to hyperflexion-extension of the vertebral column with shearing of tethered lymphatics. Alternatively, sudden compression of lipemic and engorged mesenteric lymphatics, adjacent nodes and the lower thoracic duct aggravated by deformations associated with stretching and tearing motions may also directly disrupt chyle-containing lymphatics.     

Transabdominal ligation of the thoracic duct as treatment of choice for postoperative chylothorax after esophagectomy

Postoperative chylothorax after injury of the thoracic duct during esophagectomy is a rare but severe complication which may lead to serious problems such as loss of fat and proteins, and immunodeficiency. Without treatment mortality can rise to over 50%. From 1988 to 2005, we treated 10 patients with postoperative chylothorax after 409 resections of the esophagus (2.4%). Of these 10 patients nine underwent transthoracic esophagectomy with gastric pull-up to enable an intrathoracic (n = 7) or cervical (n = 2) anastomosis and one patient received a transhiatal esophagectomy with gastric pull-up and cervical anastomosis. The average amount of postoperative chylus was 2205 mL (200-4500 mL) per day. After a median postoperative interval of 10 days, relaparotomy and transhiatal double ligation of the thoracic duct was performed in nine out of 10 patients. One patient could be managed conservatively. The average amount of chylus was reduced to 151 mL per day (90.5%). Seven patients had no complications, and three suffered from postoperative pneumonia. Two of the patients with pneumonia recovered, and one died. Discharge from hospital, after ligation of the thoracic duct, was possible after a median time of 18 days (11-52). Ligation of the thoracic duct via relaparotomy appeared to be a simple and safe method to treat postoperative chylothorax.     

Chylothorax following cardiac surgery caused by unusual lymphatic anatomy

Chylothorax due to injury to the thoracic duct and lymphatic channels during left internal thoracic artery harvest is well described. However, high volume leakage of chyle due to disruption of aberrant thymic collateral lymphatic channel in the anterior mediastinum has not been described previously. We describe such a case which was managed by early surgical exploration, ligation of the aberrant duct, and insertion of a pleuro-peritoneal shunt.     

Sealing the thoracic duct with ultrasonic coagulating shears

BACKGROUND/AIMS: Ultrasonic coagulating shears were developed as an endosurgical device that allows cutting of vessels without ligation. In this study, we obtained basic data on the feasibility of dividing and sealing the thoracic duct by using ultrasonic coagulating shears. METHODOLOGY: We obtained the thoracic duct and the left gastric artery from surgical specimens of 27 patients. After one end of each vessel was sealed using ultrasonic coagulating shears, we recorded the bursting pressure. The sealed ends of the vessels were also examined histopathologically. RESULTS: The mean bursting pressure of the thoracic duct was high enough to support the clinical use of this device, and was significantly higher than that of the left gastric artery (p<0.001). Microscopic examination of the sealed vessels showed that degenerated collagen fibers were more homogeneous and covered a significantly larger area in the thoracic duct than in the left gastric artery (p<0.001). CONCLUSIONS: The present study provides a basis for using ultrasonic coagulating shears to seal the thoracic duct and possibly lymph node dissection.     

Massive chylopericardium after coronary artery bypass surgery

Massive isolated chylopericardium is a rare postoperative complication of coronary artery bypass surgery. In the following case, massive chylopericardium developed after a coronary artery bypass procedure in which the left internal mammary artery was used for revascularization. The chylopericardium resulted from direct trauma to the thoracic duct during mobilization of the left internal mammary artery to its origin at the subclavian artery. With adequate drainage, the problem was resolved. In cases in which drainage persists, ligation of the thoracic duct may be necessary.     

The effect on survival of thoracic duct ligation in experimental peritonitis

BACKGROUND/AIMS: It has been shown that systemic bacteremia and endotoxemia in peritonitis is mainly related to lymphatic transport via the thoracic duct. This study was performed to investigate the effect on mortality of thoracic duct ligation in experimental peritonitis. METHODOLOGY: Thirty dogs were divided into three groups. Groups I, II, and III were control, unligated, and ligated thoracic duct peritonitis groups, respectively. Liver biopsy, blood and peritoneal fluid cultures were taken and survival time was established. RESULTS: Bacteria were determined in peritoneal fluid in all animals in groups II and III. Growing bacteria numbers in group III were two times higher than in group II. While bacterium was grown on blood cultures in all group II animals, growing was determined on blood cultures in only 2 animals in group III. Diffuse necrosis was determined in the liver of 2 animals who died within 72 hours in group II. Another 8 animals had minimal focal necrosis in their livers. Diffuse and progressive necrosis was determined in the liver of all animals in group III. The difference between liver necrosis in group II and group III was found to be statistically significant (p = 0.002). CONCLUSIONS: This experimental study demonstrates that thoracic duct ligation decreases bacteremia rates clearly but that mortality increases significantly.     

Massive primary chylopericardium in an infant

We report a case of massive spontaneous primary chylopericardium in a 2-month-old infant who was successfully treated with thoracic duct ligation and creation of a posterior pericardial window.     

Chylothorax following extended thymectomy for myasthenia gravis

The effectiveness of extended thymectomy for the treatment of myasthenia gravis is well documented. Most of the postoperative complications have been related to respiratory distress or wound complication, but chylothorax following thymectomy has been reported as a rare complication. From January 1995 to December 2004, 217 patients underwent extended thymectomy for myasthenia gravis at Taipei Veterans General Hospital. Three cases (1.38%) developed chylothorax after operation. Injury to the unseen division of the mediastinal lymphatics and branches from the thoracic duct during extensive dissection of perithymic fat tissue, which is seldom performed in classical thymothymectomy procedures, may have been the main cause of this complication. Two of the cases received conservative treatment and recovered uneventfully. The other patient (0.46%) underwent ligation of the thoracic duct 3 months later, which also resulted in the complication being cured. Conclusions: Post-thymectomy chylothorax is rare and seems to be related to extended thymectomy. Even a small invasive procedure such as VATS for extended thymectomy formyasthenia gravis could be complicated by chylothorax. We recommend that if chylothorax develops after thymectomy, conservative treatment is the treatment of choice; however, thoracic duct ligation is a useful method for treating long-term unhealed chylothorax.     

Chylothorax after childbirth

We report a case of chylothorax which appeared in a mother after childbirth. Disruption of the thoracic duct occurred with the high intrathoracic pressures generated by the Valsalva maneuver used by the patient during labor to "push." No evidence of other trauma or malignancy were found and the patient did well after use of total parenteral nutrition, thoracotomy with thoracic duct ligation, and pleurodesis.     

Surgery for chylothorax

Chyle is lymph fluid of intestinal origin containing fat digestion products. Chylothorax is produced by leaks from the thoracic duct or from one of its collaterals subsequent to valve incompetence. These leaks may be due to trauma (post-surgical chylothorax, the most frequent) or to spontaneous rupture of a lymph vessel distended by chyle reflux, the thoracic duct itself being pathological. When the thoracic duct is interrupted (obstruction, agenesis), chylothorax may occur from leakage due to reflux within substitution collateral pathways diverting the flow of chyle into the venoux confluents of the neck. Medical treatment is always attempted first: evacuation of chylothorax by drainage and fat-free diet or parenteral nutrition. Recently, treatment with octreotide has been found to be beneficial. Surgery consists in thoracic duct ligation or suture of leaking collaterals. In difficult cases, when the chyle leakage cannot be identified, pleurodesis is the only option.     

Chylothorax

Chylothorax or chylous pleural effusion occurs when chyle accumulates in the pleural space usually secondary to disruption of thoracic lymphatics. Chyle is a milky, white, opalescent fluid that is formed when long-chain triglycerides in the diet are transformed into chylomicrons and very-low-density lipoproteins, which are then secreted into intestinal lacteals. These lymphatic channels coalesce to form the thoracic duct, which transports chyle and ultimately drains it into the left subclavian vein. Any injury to the duct (or its major tributaries) as it courses through the thoracic cavity can lead to a chylous effusion. Diagnosis depends on direct analysis of the fluid by assaying the triglyceride content and, at times, lipid electrophoretic pattern (chylomicrons). Management depends on the underlying cause and the individual clinical circumstances. Nonoperative options include observation, treatment of the underlying disease, dietary modification employing strict medium-chain triglyceride diet or total parenteral nutrition, therapeutic thoracentesis, tube thoracostomy with chemical pleurodesis, and embolization of the thoracic duct. Surgical management may include pleurectomy, talc poudrage, pleuroperitoneal shunting, and repair or ligation of the thoracic duct via thoracoscopy or thoracotomy.     

Pressure within the thoracic duct modulates lymph composition

The amount of lymph received by the thoracic duct depends on each contributing organ's ability to produce interstitial fluid and generate a pressure differential moving lymph into the central lymphatic circulation. It has been reported that varying the pressure within the thoracic duct could alter each organ's contribution to thoracic duct flow. The thoracic duct above the diaphragm was cannulated to obtain lymph from the liver, gut, and lower body. Pressure within the thoracic duct was elevated serially by increasing the lymphatic cannula outflow height. This caused lymph protein concentration to increase while chyle concentration (measured by absorbance) decreased. The data demonstrate that as thoracic duct pressure increases, the percentage contribution of gut lymph flow (as represented by chyle concentration) decreases while the contribution of lymph originating within the liver (as indicated by higher protein concentration) increases. We conclude that pressure variation within the central lymphatic system affects the amount of lymph or edema fluid leaving any given organ.