chylothorax

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Chylothorax:

7/10/2012 amr badreldin hamdy MD FCCP 1 Chylothorax Amr Badreldin Hamdy MD, FCCP

Definition:

7/10/2012 amr badreldin hamdy MD FCCP 2 Definition A chylothorax is formed when the thoracic duct is disrupted and chyle enters the pleural space.

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7/10/2012 amr badreldin hamdy MD FCCP 3 Drainage from the thoracic duct is called chyle. Ductal lymph is clear during fasting and becomes milky after a fatty meal. Principal function of the thoracic duct is the transport of digestive fat to the venous system.

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7/10/2012 amr badreldin hamdy MD FCCP 4 Chyle appears grossly as milky, opal- escent fluid that usually separates into three layers upon standing: > a creamy uppermost layer containing chylomicrons, > a milky intermediate layer, >a dependent layer containing cellular elements.

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7/10/2012 amr badreldin hamdy MD FCCP 5 The flow rate through the duct is affected by the rate of lymph formation in the gastrointestinal tract.

Anatomy:

7/10/2012 amr badreldin hamdy MD FCCP 6 Anatomy

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7/10/2012 amr badreldin hamdy MD FCCP 7 The thoracic duct (TD) is constant only in its variability. It originates from the cisterna chyli which overlies the anterior surface of the second lumbar vertebra, posterior to and to the right of the aorta. It passes through the esophageal hiatus of the diaphragm into the thoracic cavity.

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7/10/2012 amr badreldin hamdy MD FCCP 8 The TD ascends extrapleurally in the posterior mediastinum along the right side of the anterior surface of the vertebral column and lies between the azygos vein and the descending aorta in close proximity to the esophagus and pericardium.

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7/10/2012 amr badreldin hamdy MD FCCP 9 At T5 - T7 it crosses to the left behind the aorta and ascends on the left side of the esophagus. Once the TD passes the thoracic inlet, it arches 3 to 5 cm above the clavicle and passes anterior to the sublcavian artery, vertebral artery and thyrocervical trunk to terminate in the region of the left jugular and subclavian veins.

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7/10/2012 amr badreldin hamdy MD FCCP 10 A bicuspid valve prevents entry of blood into the lymphatic system. The right duct is small ( 2cm in length) and drains lymph from right head and chest.

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7/10/2012 amr badreldin hamdy MD FCCP 11 Unidirectional flow is ensured by: - multiple valves throughout the duct, - intrinsic wall contraction, - thoracic pressure gradient.

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7/10/2012 amr badreldin hamdy MD FCCP 12 Daily between 1,500 and 2,500 mL of chyle normally empties into the venous system. Ingestion of fat can increase the flow of lymph in the TD by 2-10 times the resting level for several hours.

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7/10/2012 amr badreldin hamdy MD FCCP 13 Ingestion of liquid increases the chyle flow, whereas the ingestion of protein or carbohydrates has little effect on lymph flow. The protein content is usually above 3g/dL, the electrolyte composition of chyle is similar to that of serum.

Composition of Chyle:

7/10/2012 amr badreldin hamdy MD FCCP 14 Composition of Chyle

Lipids:

7/10/2012 amr badreldin hamdy MD FCCP 15 Lipids Fat is the main component of chyle. Sixty to seventy percent of ingested fat absorbed by intestinal lymphatics is conveyed to the blood by the thoracic duct.

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7/10/2012 amr badreldin hamdy MD FCCP 16 Lymphatic fat is transported as chylo-microns. Fatty acids with less than 10 carbon atoms are absorbed directly into the venous portal system.

Proteins:

7/10/2012 amr badreldin hamdy MD FCCP 17 Proteins Lymphatics are the main pathway for return of extra-cellular proteins to the vascular space. The protein content is half the concentration of plasma.

Electrolytes:

7/10/2012 amr badreldin hamdy MD FCCP 18 Electrolytes Electrolyte composition is similar to plasma.

Cells:

7/10/2012 amr badreldin hamdy MD FCCP 19 Cells Lymphocytes are the main cellular elements. Ninety percent are T-lymphocytes. The primary cell in chyle is the small lymphocyte (400 - 6,800/mm3). Prolonged drainage of a chylous PE can result in profound T-lymphocyte depletion.

Pathophysiology of Chyle:

7/10/2012 amr badreldin hamdy MD FCCP 20 Pathophysiology of Chyle

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7/10/2012 amr badreldin hamdy MD FCCP 21 @ it leads to cardiopulmonary abnormalities and metabolic immuno- logic deficiencies. @ it can compress the lung resulting in dyspnea and respiratory distress. @ empyema is a rare complication due to the bacteriostatic nature of lecithin and fatty acids.

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7/10/2012 amr badreldin hamdy MD FCCP 22 @ loss of proteins and vitamins, more than fat, leads to metabolic and nutritional defects, immunodeficiency, coagulopathy, malnutrition and death. @ because it is bacteriostatic and non- irritating, it does not cause fibrothorax.

Etiology:

7/10/2012 amr badreldin hamdy MD FCCP 23 Etiology 1. Trauma. 2. Tumor. 3. Miscellaneous. 4. Idiopathic.

Surgical Trauma:

7/10/2012 amr badreldin hamdy MD FCCP 24 Surgical Trauma 1. Cardiovascular surgery (coronary artery by-pass when the internal mammary artery is harvested; heart transplant, high lumbar aortography). 2. Esophageal surgery( sclerotherapy for esophageal varices). 3. Pulmonary surgery. 4. Costo-vertebral surgery.

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7/10/2012 amr badreldin hamdy MD FCCP 25 5. Neck surgery (thoraco-lumbar fusion for correction of kyphosis). 6. Diaphragm surgery.

Non-surgical Trauma:

7/10/2012 amr badreldin hamdy MD FCCP 26 Non-surgical Trauma 1. Blunt chest trauma. 2. Hyperextension of the spine or fracture of a vertebra. 3. External cardiac massage. 4. Aortic angiography. 5. Subclavian vein catheterization.

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7/10/2012 amr badreldin hamdy MD FCCP 27 6. The injury may be less impressive,and chylothoraces have been attributed to coughing, vomiting, and weight lifting, or vigorous stretching while yawning.

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7/10/2012 amr badreldin hamdy MD FCCP 28 Chylothorax secondary to closed trauma is usually on the right side, and the site of rupture is most commonly in the region of the ninth or tenth thoracic vertebra (e.g. fall from height, motor vehicle accidents, compression injuries to the trunk, heavy blows to the back or stomach, and childbirth).

Tumors:

7/10/2012 amr badreldin hamdy MD FCCP 29 Tumors

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7/10/2012 amr badreldin hamdy MD FCCP 30 Fifty percent of chylothoraces in adults are caused by tumors. Seventy five percent are lymphomas. Other forms include chronic lymphocytic leukemia, metastatic disease and lung cancer.

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7/10/2012 amr badreldin hamdy MD FCCP 31 Benign and malignant tumors may involve the TD through lymphatic permeation, direct invasion or tumor embolus.

Miscellaneous:

7/10/2012 amr badreldin hamdy MD FCCP 32 Miscellaneous 1. On rare occasions, a chylothorax is associated with heart failure or nephrotic syndrome and the effusion is a transudate. 2. Liver cirrhosis. 3. Thrombosis of the SVC or the sub- clavian vein.

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7/10/2012 amr badreldin hamdy MD FCCP 33 4. Primary lymphangioleiomatosis (LAM). 5. Tuberus sclerosis. 6. Lymphangiomatosis. 7. Sarcoidosis. 8. Yellow nail syndrome. 9. Tuberculosis.

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7/10/2012 amr badreldin hamdy MD FCCP 34 10. Amyloidosis. 11. Castleman’s disease (Giant Lymph Node hyperplasia). 12. Familial lymphedema. 13. Hypothyroidism. 14. Obstruction of the SVC secondary to Behcet’s syndrome.

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7/10/2012 amr badreldin hamdy MD FCCP 35 15. Filariasis. 16. Radiation-induced mediastinal fibrosis. 17. Kaposi sarcoma in AIDS patients. 18. Gorham’s syndrome.

Congenital Chylothorax:

7/10/2012 amr badreldin hamdy MD FCCP 36 Congenital Chylothorax It is the most common cause of pleural effusion in the newborn infant. Is twice as often in males. Prognosis is good and perinatal morbidity is between 15-30%. The infant develops respiratory distress in the first few days of life.

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7/10/2012 amr badreldin hamdy MD FCCP 37 In some cases, a congenital chylothorax is associated with Turner’s syndrome, Noonan’s syndrome, or Down’s syndrome.

Clinical :

7/10/2012 amr badreldin hamdy MD FCCP 38 Clinical

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7/10/2012 amr badreldin hamdy MD FCCP 39 The main danger to patients with chylothorax is that they become mal- nourished and immunocompromised because of the removal of large amounts of protein, fat, electrolytes and lymphocytes from the body with repeat- ed thoracentesis and chest tube drain- age.

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7/10/2012 amr badreldin hamdy MD FCCP 40 The loss of chyle might result in hypo- natremia, hypocalcemia, acidosis, hypovolemia, reduction of venous return to the heart, and lymphocytic depletion.

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7/10/2012 amr badreldin hamdy MD FCCP 41 With non-traumatic chylothorax, the onset of symptoms is usually gradual. The initial symptoms of chylothorax are usually related to the presence of space occupying fluid in the thoracic cavity e.g. dyspnea. Pleural chest pain and fever are rare.

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7/10/2012 amr badreldin hamdy MD FCCP 42 A latent period of 2-10 days usually occurs between the trauma and the onset of the pleural effusion. Lymph collects extrapleurally in the mediastinum after the initial duct disruption, forms a CHYLOMA, and produces a posterior mediastinal mass.

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7/10/2012 amr badreldin hamdy MD FCCP 43 The mediastinal pleura eventually ruptures, chyle gains access to the pleural space, and dyspnea is produced by the chyle compressing the lung. At times, hypotension, cyanosis, and extreme dyspnea occur when the chyloma ruptures into the pleural space.

Diagnosis:

7/10/2012 amr badreldin hamdy MD FCCP 44 Diagnosis

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7/10/2012 amr badreldin hamdy MD FCCP 45 Lipid measurements might be indicated in all patients with pleural effusion of unknown etiology in order to rule out the diagnosis of chylothorax.

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7/10/2012 amr badreldin hamdy MD FCCP 46 High levels of lipid accumulate in the pleural space in two situations: * When the thoracic duct is disrupted, chyle can enter the pleural space to produce a chylous effusion.

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7/10/2012 amr badreldin hamdy MD FCCP 47 * In long standing pleural effusions, large amounts of cholesterol or lecithino- globulin complexes can accumulate in the pleural fluid to produce a chyliform pleural effusion.

Chyle or Pus?:

7/10/2012 amr badreldin hamdy MD FCCP 48 Chyle or Pus? Chyle may be mistaken for pus but there is no odor and the cultures are negative. Gram stain reveals lymphocytes rather than PMLs with no bacteria.

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7/10/2012 amr badreldin hamdy MD FCCP 49 The milkiness with empyema is caused by the suspended white blood cells, and debris and if such fluid is centrifuged, the supernatant is clear.

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7/10/2012 amr badreldin hamdy MD FCCP 50 The best way to establish the diagnosis of chylothorax is by measuring the triglyceride and cholesterol levels in the pleural fluids.

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7/10/2012 amr badreldin hamdy MD FCCP 51 If the pleural fluid triglyceride level is above 110mg/dL and the ratio of the pleural fluid to serum cholesterol is less than 1.0, the diagnosis of chylothorax is established.

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7/10/2012 amr badreldin hamdy MD FCCP 52 The cholesterol ratio is used to exclude pseudochylothorax because some patients with chyliform pleural effusion also have triglyceride levels above 110 mg/dL, but their pleural fluid to serum cholesterol ratio will exceed 1.0 .

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7/10/2012 amr badreldin hamdy MD FCCP 53 NB/ the only other situation in which the pleural fluid triglyceride is above 110mg /dL is when IV fluid containing high levels of triglycerides leaks from a central vein into the pleural space.

Chylomicrons:

7/10/2012 amr badreldin hamdy MD FCCP 54 Chylomicrons The demonstration of chylomicrons in the pleural fluid by lipoprotein analysis establishes the diagnosis of chylothorax .

Lipophilic Dye Ingestion:

7/10/2012 amr badreldin hamdy MD FCCP 55 Lipophilic Dye Ingestion Ingestion of a fatty meal with a lipop- philic dye, followed by a thoracentesis 30 to 60 min later, to ascertain whether the pleural fluid has changed in color.

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7/10/2012 amr badreldin hamdy MD FCCP 56 With congenital chylothorax, the pleural fluid is initially serous and turns chylous only when milk feedings are started.

Lymphoscintigraphy:

7/10/2012 amr badreldin hamdy MD FCCP 57 Lymphoscintigraphy Technetium-99m human serum albumin is injected into the dorsum of the foot or hand and subsequently the thoracic duct is imaged in nuclear medicine.

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7/10/2012 amr badreldin hamdy MD FCCP 58 Oral ingestion of iodine labeled BMIPP, and after ingestion of this tracer by approximately 80 minutes the thoracic duct can be imaged.

Treatment:

7/10/2012 amr badreldin hamdy MD FCCP 59 Treatment

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7/10/2012 amr badreldin hamdy MD FCCP 60 The general aims are: Relief of dyspnea by removal of chyle. Preventing dehydration. Maintenance of nutrition. Reduction in the rate of chyle formation.

Conservative Treatment:

7/10/2012 amr badreldin hamdy MD FCCP 61 Conservative Treatment Tube drainage. Medium-chain fatty acid diet. NOP and total parenteral nutrition (TPN) is the most effective method of decreasing chyle production. Fluid and electrolyte support.

Decrease flow of chyle:

7/10/2012 amr badreldin hamdy MD FCCP 62 Decrease flow of chyle The patient’s nutritional status can be maintained with IV hyperalimination. This is preferred than medium-chain triglycerides which are unpalatable and are recommended when one wishes to reduce the flow of chyle.

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7/10/2012 amr badreldin hamdy MD FCCP 63 The flow of chyle is also decreased if the patient stays in bed because any lower extremity movement increases the flow of lymph.

Somatostatin:

7/10/2012 amr badreldin hamdy MD FCCP 64 Somatostatin It requires continuous IV infusion. Usual starting dose is 3.5mg/kg/hr which can be increased to 10mg/kg/hr.

Octreotide:

7/10/2012 amr badreldin hamdy MD FCCP 65 Octreotide It is given by SC route. Usual dose in the adult is 50mg/8 hrs, in children 0.3-1mg/kg/hr. Primary side effects include suppression of GIT motility and secretion (loose stools, malabsorption, nausea and flatulance).

Mechanism of action:

7/10/2012 amr badreldin hamdy MD FCCP 66 Mechanism of action They decrease triglyceride absorption and lymphatic flow.

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7/10/2012 amr badreldin hamdy MD FCCP 67 One must treat the chylothorax definitively, such as with thoracic duct ligation or pleuroperitoneal shunt implantation, before the patient becomes too cachectic to tolerate the operation.

Pleuroperitoneal Shunt:

7/10/2012 amr badreldin hamdy MD FCCP 68 Pleuroperitoneal Shunt The optimal method to remove chyle. Chyle is shunted to the peritoneal cavity where it is absorbed without creating significant ascitis. It can be inserted with local anaesthesia as opposed to general anaesthesia which is required for thoracic duct ligation.

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7/10/2012 amr badreldin hamdy MD FCCP 69 The shunt can be removed 30-90 days after its insertion. No dietary restriction is needed. Should not be inserted if chylous ascitis is present.

Percutaneous Transabdominal Thoracic Duct Ligation:

7/10/2012 amr badreldin hamdy MD FCCP 70 Percutaneous Transabdominal Thoracic Duct Ligation

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7/10/2012 amr badreldin hamdy MD FCCP 71 Minimally invasive. Pedal lymphography is initially performed to opacify large retroperitoneal lymph channels.

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7/10/2012 amr badreldin hamdy MD FCCP 72 A suitable duct (>2 mm diameter) is punctured transabdominally to allow catheterization and embolization of the thoracic duct under fluoroscopic guidance.

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7/10/2012 amr badreldin hamdy MD FCCP 73 The embolization is performed using platinum microcoils or micro particles. Glue may be used singly or in combination with coils.

Pleurodesis :

7/10/2012 amr badreldin hamdy MD FCCP 74 Pleurodesis Pleurodesis through a chest tube is not generally recommended for patients with chylothorax. Thoracoscopy with talc insufflation of 2gm talc or with pleural abrasion or partial parietal pleurectomy is done and is effective.

Ligation of Thoracic Duct:

7/10/2012 amr badreldin hamdy MD FCCP 75 Ligation of Thoracic Duct Until Lampson initially described successful ligation of the thoracic duct in 1948, the mortality rate from chylothorax was 50%. It is the definitive treatment.

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7/10/2012 amr badreldin hamdy MD FCCP 76 Causes no ill defects (because of the multiple anastamosis among various lymphatic channels and direct lymphatico-venous communications. If the chylothorax is bilateral, a right thoracotomy should be performed because the duct is more readily approached from that side.

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7/10/2012 amr badreldin hamdy MD FCCP 77 The actual point of leakage from the duct must be determined and ligation of the duct on both sides of the leak is done. Preoperative lymphangiogram should be done.

Chyliform Pleural Effusion:

7/10/2012 amr badreldin hamdy MD FCCP 78 Chyliform Pleural Effusion Is a pleural effusion that is turbid or milky from high lipid content not resulting from disruption of the thoracic duct.

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7/10/2012 amr badreldin hamdy MD FCCP 79 Pseudochylothoraces may be seperated into those with cholesterol crystals (pseudochylous effusions), and those without cholesterol crystals (chyli- form pleural effusions). But no practical reason exists for making this distinction.

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7/10/2012 amr badreldin hamdy MD FCCP 80 Most patients with chyliform pleural effusion have long standing pleural effusion (mean >5y), and have thickened and sometimes calcified pleura.

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7/10/2012 amr badreldin hamdy MD FCCP 81 Most of the cholesterol in chyliform PE is associated with high-density lipo- proteins in contrast to the cholesterol in acute exudates that is mostly bound to low-density lipoproteins.

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7/10/2012 amr badreldin hamdy MD FCCP 82 The origin of cholesterol and other lipids is not definitely known, but one possibility is from degenerating RBCs and WBCs in the pleural fluid.

Causes:

7/10/2012 amr badreldin hamdy MD FCCP 83 Causes The two most common causes of the effusion initially are rheumatoid pleuritis and tuberculosis. Many pleural effusions secondary to paragnomiasis contain cholesterol crystals.

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7/10/2012 amr badreldin hamdy MD FCCP 84 Chyliform effusions are usually unilateral. Differential diagnosis of chyliform pleural effusions are empyema and chylothorax. In an empyema, centrifugation results in a clear supernatant.

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7/10/2012 amr badreldin hamdy MD FCCP 85 Chyliform pleural effusions contain cholesterol crystals. Microscopically, the cholesterol crystals present a typical rhomboid configuration. Pleural fluid cholesterol levels > 200 mg/dL strongly suggest a chyliform effusion. Some have a high (>250mg/dL ) trigyceride level.

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7/10/2012 amr badreldin hamdy MD FCCP 86 When a patient is diagnosed as having a chyliform pleural effusion, the possibility of TB should always be entertained. Decortication should be considered if the patient is symptomatic and the under- lying lung is believed to be functional.

Pleurectomy:

7/10/2012 amr badreldin hamdy MD FCCP 87 Pleurectomy If the thoracic duct cannot be successfully ligated at thoracotomy, a parietal pleurectomy should be performed to obliterate the pleural space. One must not delay thoracotomy too long.

Role of Thoracoscopy:

7/10/2012 amr badreldin hamdy MD FCCP 88 Role of Thoracoscopy Ligation of the thoracic duct is done with the videothoracoscope. Thoracoscopy permits the entire pleural space to be visualized, as well as allowing direct suture of a lymphatic leak.

References:

7/10/2012 amr badreldin hamdy MD FCCP 89 References Light R.W.: Pleural Diseases. Fifth ed. Lippincott Williams & Williams ( 2007). Miller JI Jr: Diagnosis and management of chylothorax. Chest Surg Clin North Am, 6:139 (1996). Hillerdal G: Chylothorax and pseudo- chylothorax. Eur Respir J; 10:1157 (1997).

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7/10/2012 amr badreldin hamdy MD FCCP 90 Merrigan BA et.: Chylothorax. Br J Surg 84:15 (1997). Williams KR, Burford TH: The manage- ment of chylothorax. Ann Surg 160:131 (1964). Roy PH et al.: The problem of chylo- thorax. Mayo Clin Prod 42:457 (1967).

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7/10/2012 amr badreldin hamdy MD FCCP 91 Chernick V, Reed MH: Pneumothorax and chylothorax in the neonatal period. J Pediatr; 76:624 (1970). Hughes RL et al: The management of chylothorax. Chest; 76:212 (1979). Ross JK: A review of the surgery of the thoracic duct. Thorax; 16:12 (1961).

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7/10/2012 amr badreldin hamdy MD FCCP 92 Hamdan MA, Gaeta ML: Octreotide and low-fat breast milk in postoperative chylothorax. Ann Thorac Surg; 77:2215 (2004). Buettiker V et al.: Somatostatin: a new therapeutic option for the treatment of chylothorax. Intensive Care Med; 27: 1083 (2001).

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7/10/2012 amr badreldin hamdy MD FCCP 93 Demos NJ et al.: Somatostatin in the treatment of chylothorax. Chest; 119: 964 (2001). Kalomendis I: Octreotide and chylothorax. Cur Opin Pul Dis; 12:264 (2006) Little AG et al.: Pleuroperitoneal shunting: alternative therapy for persistent chylothorax. Ann Thorac Surg; 208:443 (1988).

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7/10/2012 amr badreldin hamdy MD FCCP 94 Adler RH, Levinsky L: Persistent chylo- thorax. J Thorac Cardiovasc Surg; 76: 859 (1978). Robinson CLN: The management of chylothorax. Ann Thorac Surg; 39: 90 (1985). Perry RE et al.: Pleural effusion in the neonatal period. J Pediatr 62:838 (1963

THANK YOU:

7/10/2012 amr badreldin hamdy MD FCCP 95 THANK YOU