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Year : 2019  |  Volume : 36  |  Issue : 1  |  Page : 87-89  

Chylothorax due to sarcoidosis: A unique occurrence

Department of Pulmonary Medicine, T. N. Medical College, BYL Nair Hospital, Mumbai, Maharashtra, India

Date of Web Publication31-Dec-2018

Correspondence Address:
Dr. Jyotsna M Joshi
Department of Pulmonary Medicine, T. N. Medical College, BYL Nair Hospital, Mumbai, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/lungindia.lungindia_408_18

Clinical trial registration CTRI/2015/07/005962( registered on 3/7/2015)

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How to cite this article:
Gupta A, Utpat K, Desai U, Joshi JM. Chylothorax due to sarcoidosis: A unique occurrence. Lung India 2019;36:87-9

How to cite this URL:
Gupta A, Utpat K, Desai U, Joshi JM. Chylothorax due to sarcoidosis: A unique occurrence. Lung India [serial online] 2019 [cited 2019 Sep 21];36:87-9. Available from: http://www.lungindia.com/text.asp?2019/36/1/87/249171


Chylothorax is a rare condition that results from the thoracic duct damage with chyle leakage from the lymphatic system into the pleural space. Sarcoidosis[1] is an unconventional cause for chylothorax, and high index of suspicion is required to diagnose it. We herein report a rare case of sarcoidosis presenting as chylothorax in a young female.

A 22-year-old female, student by occupation nonaddict, symptomatic for 15 days with complaints of cough and dyspnea with insignificant history had been reported. Chest radiograph showing right-sided effusion and intervened with implantable cardioverter-defibrillator (ICD) following which she was referred to us for further management. On examination, vitals were stable and respiratory system examination revealed movements decreased on the right side with volume loss signs with reduced breath sounds in the right lower lobe areas with ICD in situ draining milky white fluid. Pleural fluid triglycerides level was 234 milligrams per deciliter (mg/dl), pleural fluid cholesterol level was 5.6 mg/dl, chylomicrons were present, and no cholesterol crystals were seen. Contrast-enhanced computed tomography (CT) thorax showed right-sided hydropneumothorax with the right middle lobe consolidation with pericardial effusion with subtle lung nodules [Figure 1]. She underwent Cope's percutaneous closed needle pleural biopsy, and both fluid and biopsy were negative for Mycobacterium tuberculosis (MTB). CT-guided biopsy of the right middle lobe consolidation lesion was performed which showed noncaseating granuloma [Figure 2] on histopathology and microbiologically did not detect MTB. She also underwent bronchoscopy, where tracheobronchial tree was normal, and bronchial washing again turned out be negative for MTB. Her serum angiotensin-converting enzyme level was normal, and tuberculin skin test was negative. Her spirometry was suggestive of a restrictive abnormality with forced vital capacity (FVC) of 0.71 L and 31% predicted. Her two-dimensional echocardiography showed nontappable pericardial effusion along with bicuspid aortic valve labeled as congenital heart disease. In view of the multisystemic involvement, a congenital syndromic association like Turner's or Noonan syndrome were contemplated but were ruled out on karyotyping. Direct nodal CT lymphangiography showed obstruction of the thoracic duct due to left inguinal adenopathy. Hence, a final diagnosis of sarcoidosis leading to thoracic duct obstruction due to inguinal lymphadenopathy culminating in chylothorax was made. The patient was managed with oral corticosteroids prednisolone 1 mg/kg body, weight medium-chain triglyceride (MCT) diet, and octreotide 100 mcg subcutaneous injections thrice daily. She responded in the form of symptomatic improvement and decrease in the amount of drain and conversion of chylous fluid to serous. Her ICD was removed, and she was discharged in stable state with steroids in tapering doses. The patient followed up at 1 month with further improvement in symptoms and improvement in FVC by 420 ml. The steroid therapy was tapered with total duration to be of 6 months.
Figure 1: Contrast-enhanced computed tomography (lung window) showing moderate right-sided hydropneumothorax with internal septation with patchy consolidation with interstitial septal thickening with ill-defined ground glass centrilobular nodules in left lung with implantable cardioverter-defibrillator in situ

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Figure 2: Photomicrograph of the computed tomography-guided lung biopsy histopathology showing interstitium with nonnecrotizing naked epithelioid granulomas along with moderate, diffuse inflammation composed of lymphocytes, histiocytes, and plasma cells

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DeMeester classified chylothorax causes into congenital, traumatic, neoplastic, and miscellaneous.[2] The most common cause for chylothorax is thoracic surgery. The other customarily encountered causes of chylothorax include trauma to the thoracic duct, malignancies notoriously lymphomas, lymphangioleiomyomatosis, and infections such as tuberculosis and filariasis.

Imaging studies such as CT thorax and abdomen may also help in identifying the underlying causes of chylothorax such as lymphadenopathy or mass. CT or magnetic resonance imaging lymphoangiogramhas a diagnostic value in localizing the site of the thoracic duct leak or blockage and has shown therapeutic benefit in some cases; however, direct nodal lymphoscintigraphy[3] is usually a safer and less invasive option. Treatment of chylothorax is a comprehensive endeavor which comprises treatment of the underlying condition, conservative management like MCT diet, ICD placement, somatostatin analog like octreotide[4] can be given and surgical management of the thoracic duct trauma if needed. Surgical therapy[5] is recommended in cases where despite conservative management the patient drains >1.5 l/day in an adult or >100 ml/kg body weight per day in a child, leaks chyle >1 l/day × 5 days or has a persistent chyle flow for >2 weeks or severe electrolyte imbalance or severe malnutrition. Thus, in our case, it was the inflammation associated with sarcoidosis and the adenopathy led to insult to the thoracic duct and the small lymphatic channels in the lungs, which subsequently manifested as chylothorax.

A systematic search of the PubMed database (from inception till August 1, 2018) with the following search terms was undertaken: (“Chylothorax” or “Sarcoidosis” or “Sarcoidosis-related chylothorax”). The search yielded 31 citations, of chylothorax which were identified to be associated with sarcoidosis. Most of the search emphasized genetic mutation involved in the disease and recent advances in the treatment of the disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Soskel NT, Sharma OP. Pleural involvement in sarcoidosis. Curr Opin Pulm Med 2000;6:455-68.  Back to cited text no. 1
Nair SK, Petko M, Hayward MP. Aetiology and management of chylothorax in adults. Eur J Cardiothorac Surg 2007;32:362-9.  Back to cited text no. 2
Lv S, Wang Q, Zhao W, Han L, Wang Q, Batchu N, et al. Areview of the postoperative lymphatic leakage. Oncotarget 2017;8:69062-75.  Back to cited text no. 3
Markham KM, Glover JL, Welsh RJ, Lucas RJ, Bendick PJ. Octreotide in the treatment of thoracic duct injuries. Am Surg 2000;66:1165-7.  Back to cited text no. 4
Selle JG, Snyder WH 3rd, Schreiber JT. Chylothorax: Indications for surgery. Ann Surg 1973;177:245-9.  Back to cited text no. 5


  [Figure 1], [Figure 2]


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