|Year : 2015 | Volume
| Issue : 2 | Page : 73-76
Bilateral chylothorax in a patient with chronic central vein thrombosis and chronic thromboembolic pulmonary hypertension
Avdhesh Bansal, Trilok Chand, Kapil Sharma
Department of Respiratory, Critical Care and Sleep Medicine, Indraprastha Apollo Hospitals, New Delhi, India
|Date of Web Publication||16-Jun-2015|
B-241, Sarita Vihar, New Delhi - 110 076
Source of Support: None, Conflict of Interest: None
The chylothorax is not a common presentation, and bilateral chylothorax in patients with chronically high central venous pressure secondary to venous thrombosis is a rare in incidence. We reported a case of bilateral chylothorax in a patient of chronic deep vein thrombosis (DVT) in central veins with chronic thromboembolic pulmonary hypertension who presented with 2 weeks history of increased breathlessness, bilateral chest discomfort and weakness. Work-up with chest X-ray and ultrasonography-chest showed gross left sided and mild right sided pleural effusion, thoracocentesis was consistent with chylothorax. Contrast enhanced computed tomography-chest showed multiple collateral formation of left side subclavian vein, venous Doppler showed old DVT in right and left subclavian veins and two-dimensional echocardiogram showed finding of severe pulmonary hypertension. After 24 h of fasting and conservative management, pleural drain became clear and decreased in the amount. Patient's video assisted thoracoscopic surgery was done, and thoracic duct was ligated and cut down at diaphragmatic level and bilateral talc pleurodesis done. Patient improved clinically and radiologically.
Keywords: Chronic thromboembolic pulmonary hypertension, chylothorax, deep vein thrombosis
|How to cite this article:|
Bansal A, Chand T, Sharma K. Bilateral chylothorax in a patient with chronic central vein thrombosis and chronic thromboembolic pulmonary hypertension. J Assoc Chest Physicians 2015;3:73-6
|How to cite this URL:|
Bansal A, Chand T, Sharma K. Bilateral chylothorax in a patient with chronic central vein thrombosis and chronic thromboembolic pulmonary hypertension. J Assoc Chest Physicians [serial online] 2015 [cited 2018 Nov 16];3:73-6. Available from: http://www.jacpjournal.org/text.asp?2015/3/2/73/158884
| Introduction|| |
Central venous thrombosis is not uncommon but is only rarely associated with chylothorax. The formation of chylothorax in patients with central vein thrombosis is due to transmission of the lymphatic flow to the venous circulation through collateral channels if the thoracic duct outflow is obstructed due to central venous thrombosis. Bilateral chylothorax associated with chronic thromboembolic pulmonary hypertension (CTEPH) and chronic deep vein thrombosis (DVT) is very rarely reported.
| Case Report|| |
A 44-year-old Indian lady, chronic intravenous drug abuser presented with 2 weeks history of increased breathlessness, bilateral chest discomfort, and weakness. She is a diagnosed case of chronic DVT and CTEPH and she is on regular treatment for last 5 years. On examination, she found to be hypoxic, decreased breath sounds and dull note on the Left side of chest but she didn't show any sign of infection.
Patient's routine blood investigations were within normal limits except slightly high international normalized ratio (1.6) and low serum calcium (6.7 mg/dl). Her chest X-ray (CXR) revealed left side pleural effusion and cardiomegaly [Figure 1]. Patient's ultrasonography chest done, which revealed gross left sided and mild right sided pleural effusion, on thoracocentesis we noticed a pale yellow-turbid appearance [Figure 2] of fluid, which was sent for investigations.
In view of yellowish turbid appearance of fluid and suspected pus or chyle, we put the chest drain on the left side of the chest, which drained 1000 ml of milky colored fluid.
Patient's lab reports of fluids showed white blood cell 2400/Cumm (100% Lymphocytes), red blood cell 900/Cumm, glucose 97 mg/dl, protein 2.6 g/dl, lactate dehydrogenase 121 IU/L. The lipid profile of the fluid showed triglycerides 204 mg/dl, cholesterol 37 mg/dl and chylomicrons were positive. Pleural fluid's all stains and cultures found to be negative.
We reviewed the literatures to find out the etiology, and we get done the contrast enhanced computed tomography (CECT-chest), venous Doppler of lower limb and deep neck veins and echocardiography. Patient's CECT-chest showed multiple collateral formation of left side subclavian vein [Figure 3] but no lymphadenopathy, venous Doppler showed old DVT in right and left subclavian and internal jugular veins [Figure 4] and [Figure 5] and two-dimensional echocardiogram showed markedly dilated right atrium and right ventricle, paradoxical IVS and pushed to left, pulmonary artery systolic pressure - 90 mmHg, severe pulmonary arterial hypertension and stumped flow in main pulmonary artery, right and left pulmonary arteries [Figure 6].
|Figure 3: Computed tomography-chest showing left pleural effusion and multiple thoracic collateral formations|
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|Figure 5: Venous Doppler showing thrombus in right internal jugular vein|
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|Figure 6: Two-dimensional echocardiogram showing grossly dilated right heart chambers|
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Patient was started on conservative management, her hydration and electrolyte balance was maintained. Patient was kept fasting and only clear liquids were allowed and total parenteral nutrition (TPN) was started. After 24 h of fasting pleural drain became clear and decreased in the amount. Patient again started increased dyspnea and chest pain, CXR was done which showed increased pleural effusion on the right side also, which was therapeutically drained, and it was same consistency and appearance as the left side was. We put the patient on octreotide 50 mcg s/c TDS to decrease the drain amount, and thoracoscopic intervention was planned. After the evaluation of all pros and cons, video assisted thoracoscopic surgery (VATS) was done. In VATS thoracic duct was ligated and cut down at diaphragmatic level, and talc pleurodesis was done bilaterally. Patient improved clinically and radiologically. On follow-up visits, we found that chylothorax was did not reappear.
| Discussion|| |
The milky appearance of pleural fluid may be due to chylothorax or pseudochylothorax. Chylothorax represents chyle in pleural fluid and chyle is a lymph or lymphatic fluid enriched with fat, small lymphocytes, minerals, fat soluble vitamins and digestive products absorbed by the intestinal epithelium.
A chylothorax is formed when the thoracic duct is disrupted, and chyle enters the pleural space.  The etiology of chylothorax can be grouped into four different categories, namely, trauma, tumor, miscellaneous, and idiopathic. Trauma is the leading cause of chylothorax. This trauma is usually a cardiovascular, pulmonary, or esophageal surgical procedure. Chylothorax appears particularly frequently following operations in which the left subclavian artery is mobilized. Penetrating trauma to the chest or neck such as gunshot or knife wounds can also sever the thoracic duct and may lead to chylothorax. Trauma in which the spine is hyperextended or a vertebra is fractured is most likely to cause chylothorax, particularly if the injury occurs after the recent ingestion of a fatty meal.  A chylothorax secondary to closed trauma is usually on the right side, and the site of rupture is most commonly in the region of the 9 th or 10 th thoracic vertebra. Such trauma includes falls from a height, motor vehicle accidents, and compression injuries to the trunk, heavy blows to the back or stomach, and childbirth. The injury may be less impressive, and chylothoraces have been attributed to coughing, vomiting, and weight lifting.
Another leading cause of chylothorax is malignancy. Extrinsic compression of the thoracic duct or direct invasion of the thoracic duct may cause rupture and leakage of chyle. Lymphomas are the most common type of tumor associated with chylothorax (60-70% of cases) however; any mediastinal tumor (e.g., teratoma and neuroblastoma) has the potential to cause chylothorax. 
Thrombosis of the superior vena cava or the subclavian vein is becoming one of the more common causes of chylothorax.  Berman et al., reviewed the case histories of 37 infants and children with thrombosis of their superior vena cava in a newborn and pediatric intensive care unit and reported that 9 (24%) had a chylothorax.  Chylothorax can also complicate innominate vein or left subclavian vein thrombosis.  Extensive venous thrombosis complicating central venous catheterization has resulted in bilateral chylothorax and chylopericardium. 
The possible cause of bilateral chylothorax in central vein thrombosis is that when the pressure of the venous system exceeds the pressure in the thoracic duct, rupture of thoracic duct and/or its collaterals occur  and formation of chylothorax is expected because accessory conduits function of accessory lymphatic channels can be compromised by venous hypertension, which lead to elevated lymphatic pressures and pleural lymphatic engorgement, and eventually accessory lymphatic rupture or leakage. 
On rare occasions, a chylothorax is associated with heart failure or the nephrotic syndrome and the effusion is also a transudate in these instances. The fourth category of chylothorax is idiopathic, including most cases of congenital chylothorax. Most cases of idiopathic chylothorax in the adult are probably due to minor trauma, such as coughing or hiccupping, after the ingestion of fatty meals. Half of all chylothoraces are right-sided, one-third is left-sided, and the remainders are bilateral. 
Clinically these patients present with dyspnea, cough and chest discomfort due space-occupying fluid in the pleural cavity. Due to the nonirritating nature of chyle, pleuritic chest pain and fever are rarely present. The severity of symptoms depends on the amount of the chylothorax. Rarely, these patients can develop a tension chylothorax with compromise of the systemic circulation. Chronic cases have muscle wasting, weight loss, and features of malnutrition and electrolyte imbalance. Sometimes pleural effusion as an incidental finding on chest radiograph in dyspneic patient.
The diagnosis of chylothorax is based on the biochemical analysis or lipid profile of pleural fluid. Pleural fluid triglyceride levels >110 mg/dl, presence of chylomicrons, low cholesterol level, and elevated lymphocyte count are diagnostic of a chylothorax.
The approach to management of chylothorax is the same regardless of the cause of the chylothorax. It is divided into two categories; one is medical or nonsurgical management which includes drainage by thoracocentesis or intercostals tube drainage, nutritional support in form of fat-free diet, medium-chain triglyceride diet and TPN, somatostatin analogue, and pleurodesis. Second category is surgical treatment, which includes the ligation of the thoracic duct by thoracotomy or VATS, thoracoscopic pleurodesis, and pleuroperitoneal shunt.
| Conclusion|| |
Central vein thrombosis is not uncommon, but is only rarely associated with chylothorax, and bilateral chylothorax in a patient with CTEPH and central vein thrombosis is very rarely reported. Our patient was initially managed by medical means, but finally surgical option was opted, and patient improved. Hence, these kinds of patients managed only by surgery in the form of thoracic duct ligation or cut down.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]