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Home , Pulmonary embolism

Diagnostic Snapshot

What Caused ? This Fluid?

Courtney Robb, RN, MS, CRNFA, FNP-C From Department of Cardiothoracic Surgery, The University of Texas MD Anderson Center, Houston, Texas Figure 1 The author has no conflicts of interest to disclose. Correspondence to: Courtney Robb, RN, MS, CRNFA, FNP-C, 1327 Lake Pointe Pkwy, Sugar Land, TX 77478. E-mail: [email protected] https://doi.org/10.6004/jadpro.2018.9.5.10 HISTORY sweats, chills, fatigue, or dizziness. She reported Mrs. C is a 58-year-old Caucasian female with a histo- feeling slightly short of breath with exertion.

exercise. In 2014, a thoracentesis that ruled out can- PHYSICAL AND DIAGNOSTIC STUDIES cer with unknown cause was performed on the fluid. Mrs. C’s vital signs were stable, with a ox- Her pulmonologist referred her to an oncologist for imetry of 100%. Mrs. C was well-nourished and closer observation and 1 year with serial CT imaging demonstrated no fatigue and no distress. There was no palpable lymphadenopathy of the or PMH includes hypothyroidism, breast augmenta- supraclavicular region. Her was unla- tion, and tubal ligation. She takes levothyroxine and bored with no signs of dyspnea. Her breath sounds a multivitamin daily. She is very active and does not were clear bilaterally on , except they smoke. She has a family history of melanoma. were slightly diminished in the left lower lobe. There was dullness to in the left lower CHIEF COMPLAINT . There were no , rhonchi, or crack- In September 2016, Mrs. C reported to her local les. On auscultation of the heart, there were no PCP with chest tightness and a hard, dry that murmurs or gallops. Upon examination of her ab- developed after strenuous exercise and would not domen, there were no palpable masses, no guard- subside. She also stated she felt something “pop” ing or tenderness, and normal bowel sounds in all in her chest. Her local physician ordered a chest x- four quadrants. There was no or clubbing ray, and she was noted to have bilateral pleural ef- of the nail beds, and she had good refill. fusions. Due to this finding, a CT angiogram of the There was no edema in her extremities. A PET, This article is distributed under the terms of Creative Commons Attribution Non-Commercial License, which permits

unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. chest with contrast was ordered, revealing bilateral thoracentesis of the left lung, and lymphangiog- raphy were ordered for further evaluation. Labo- and a small amount on the right. The angiogram did not show pulmonary emboli. There was no paren- studies were performed and returned chymal consolidation or pulmonary mass. There normal. Thoracentesis showed triglyceride level was no evidence of intrathoracic lymphadenopa- of 220 mg/dL of milky fluid. The study was nega- thy (Figure 1). She denied , , night tive for malignant cells. © 2018 Harborside™ J Adv Pract Oncol 2018;9(5):549–551

WHAT IS THE MOST LIKELY DIAGNOSIS?

TIAL EN TIAL A Malignant B Idiopathic FF ER DIAGNOSIS C Pulmonary SEE BACK FOR ANSWER DI CORRECT ANSWER B less than 50 mg/dL triglyceride level, it is associ- Idiopathic Chylothorax. A chylothorax is the ated with a less than 5% change of being a chylous presence of the lymphatic fluid chyle in the pleural fluid, helping to rule out a chylothorax with a high space. Chylothorax makes up about 2% of all pleu- degree of sensitivity (Bender et al., 2016). ral effusions in adults, and in 15% of those cases, no known cause is identified, therefore classify- EXPLANATION OF ing them as spontaneous and idiopathic (Bender, INCORRECT ANSWERS Murthy, & Chamberlain, 2016). Chyle is secreted Malignant Pleural Disease. In 25% of cases, by intestinal cells that are transported through the pleural effusions are caused by malignant disease thoracic duct and can accumulate in the pleural (Sato, 2006). Pleural effusions are common in space due to leakage or obstruction of the thoracic patients with cancer; about half of patients with duct or one of its major branches (Nataprawira, cancer develop pleural effusions. Pleural effusions Rosmayudi, & Effendy, 2004). Chylothorax is a are especially common in Hodgkin lymphoma, rare occurrence and a serious and potential life- with an incidence of up to 60% (Hunter, Dhakal, threatening event if not treated accordingly. Voci, Goldstein, & Constine, 2014). There is up There are two types of chylothorax: spontane- to a 20% incidence of bilateral pleural effusions ous and traumatic. Traumatic chylothoraces are seen in patients with non-Hodgkin lymphoma, caused by trauma or surgery. Spontaneous chy- most often at presentation (Hunter et al., 2014). lothorax can develop due to infection, neoplasm, Patients with malignant can pres- superior vena cava , cirrhosis, or for ent with unilateral or bilateral pleural effusions. an unknown cause. In more rare cases, it has been The number 1 cause of pleural effusion in a study shown to occur after strenuous exercise or valsal- with 3,077 patients was cancer. Of those 3,077 pa- va maneuver. It can also present at any age, includ- tients, the most common primary tumors with ing in neonates (Bender et al., 2016). malignant pleural effusion were lung (37%) and Clinical manifestations of a chylothorax are breast (16%) tumors. However, the presentation related to the presence of fluid accumulation in was typically unilateral (Porcel, Esquerda, Vives, the thoracic cavity. The differentiation among & Bielsa, 2014). In another study of 264 patients, diagnoses of malignancy, parapneumonic effu- 17% of the pleural effusions were lung cancer, 10% sion, or chylothorax occurs with evaluation of the were metastatic tumors, and 9% were mesothe- pleural fluid with thoracentesis. Upon gross ex- lioma. Overall, malignant effusions account for amination of the fluid, chyle will present as a chy- approximately 20% to 25% of all effusions. There- lous milky fluid, as seen with Mrs. C. However, it fore, when a person presents with bilateral pleu- can also appear this way in a pseudochylothorax. ral effusion, malignant cause should be ruled out A pseudochylothorax is a pleural effusion that is (Tingquist & Steliga, 2018). frequently associated with long-standing inflam- Pulmonary Embolism. Pulmonary embolism matory disorders such as rheumatoid arthritis and (PE) is the fourth leading cause of pleural effu- tuberculosis. It is different from a chylothorax at sion, and the possibility of PE should be evaluated the microscopic level (Chong, Chauhan, Di Nino, for all patients with undiagnosed pleural effusion. Brien, & Casserly, 2012). The pleural fluid under- The most common cause of pleuritic goes lipid electrophoresis with microscopic and and pleural effusion in patients younger than 40 chemical evaluation. A pseudochylothorax will years old is PE. Pleural effusions can develop due present with greater than 250 mg/ to PE depending on the size, number, and location dL and triglycerides less than 110 mg/dL. With a of thrombosis. Pleural effusions that develop due chylothorax, triglyceride levels are greater than to PE typically occupy less than one-third of the 110 mg/dL and chylomicrons are present (Bender hemithorax (Light, 2001). et al., 2016). If the test is positive for triglycerides greater than 110 mg/dL, there is a less than 1% MANAGEMENT chance it is not a chylothorax, thus supporting the Treatment of a chylothorax may be conservative diagnosis with high specificity. If the fluid shows or surgical. The conservative measure aims at re- ducing chyle production through diet control and well and was discharged home the same day. reducing symptoms by draining the chylous fluid She returned in 3 months with a repeat chest CT from the thorax (Bender et al., 2016). Reduction in showing no signs of pleural effusions; they had intake with a nonfat diet may allow for spon- resolved. She also reported feeling well with res- taneous closure of the lymphatic vessels. The clo- olution of her symptoms. l sure is typically completed within a week’s time or less if the patient adheres well to the diet. In pa- References tients with potential malnutrition issues, consult- Bender, B., Murthy, V., & Chamberlain, R. S. (2016). The ing a nutritionist will be important for close moni- changing management of chylothorax in the modern era. European Journal of Cardio-Thoracic Surgery, 49(1), toring (Sriram, Meguid, & Meguid, 2016). More 18–24. https://doi.org/10.1093/ejcts/ezv041 invasive interventions target the cause and either Chong, S. G., Chauhan, Z., Di Nino, E., Brien, A. O., & Casserly, directly close the site of the leak through surgery B. P. (2012). Effusion under the microscope.Monaldi Ar- chives for Chest Disease, 77(1), 32–34. or embolize the lymphatic vessels percutaneously Hunter, B. D., Dhakal, S., Voci, S., Goldstein, N. P. N., & Con- (Itkin, 2016). stine, L. S. (2014). Pleural effusions in patients with Hodg- There is also new research evaluating pharma- kin Lymphoma: Clinical predictors and associations with cotherapy treatments for chylothorax. Octreotide, outcome. Leukemia and Lymphoma, 55(8), 1822–1826. https://doi.org/10.3109/10428194.2013.836599 a somatostatin analog, has two modes of action to Itkin, M. (2016). Interventional treatment of pulmonary help stop a chyle leak. It acts on the somatostatin lymphatic anomalies. Techniques in Vascular and In- receptors of the lymph ductal endothelial cells, terventional , 19(4), 299–304. http://dx.doi. org/10.1053/j.tvir.2016.10.005 causing smooth muscle contraction of the lymph Light, R. W. (2001). Pleural effusion due to pulmonary em- duct to decrease leakage. It also prevents intesti- boli. Current Opinion Pulmonary Medicine, 7(4), 198– nal fat absorption by hindering hormone secretion 201. Retrieved from https://www.ncbi.nlm.nih.gov/ from the pancreas and gastrointestinal tract, thus pubmed/11470974 Nataprawira, H. M., Rosmayudi, O., & Effendy, S. (2004). Recur- reducing the quantity of flowing lymph fluid. This rent idiopathic chylothorax. Paediatrica Indonesia, 44(6), can be administered intravenously and monitored 253–258. https://doi.org/10.14238/pi44.6.2004.253-8 for its response (Tabata et al., 2016). Porcel, J. M., Esquerda, A., Vives, M., & Bielsa, S. (2014). Etiology of pleural effusions: Analysis of more than 3,000 consecutive thoracenteses. Archivos de Bronco- OUTCOME neumologia, 50(5), 161–165. https://doi.org/10.1016/j.ar- Mrs. C underwent percutaneous image-guided bres.2013.11.007 Sato, T. (2006). Differential diagnosis of pleural effu- lymphangiogram with possible thoracic duct sions. Japan Medical Association Journal, 49(9), 315– by . The 319. Retrieved from http://www.med.or.jp/english/ lymphangiogram was successful, with lipiodol in- pdf/2006_09+/315_319.pdf jected via the bilateral inguinal and right pelvic Sriram, K., Meguid, R. A., & Meguid, M. M. (2016). Nutrition- al support in adults with chyle leaks. Nutrition, 32(2), lymphatic system. Slow-flow lymphatics without 281–286. http://dx.doi.org/10.1016/j.nut.2015.08.002 any large active thoracic duct leak were noted. Tabata, H., Ojima, T., Nakamori, M., Nakamura, M., Katsuda, On final CT, lipiodol was visualized at the M., Hayata, K.,…Yamaue, H. (2016). Successful treatment of chylothorax after esophagectomy using octreotide and thoracic duct confluence within the left subcla- etilefrine. Esophagus: Official Journal of the Japan Esoph- vian . It was determined at that time, the ageal Society, 13(3), 306–310. https://doi.org/10.1007/ lymphangiogram could be considered therapeu- s10388-016-0526-8 tic and was attributed to mild inflammatory ef- Tingquist, N. D., & Steliga, M. A. (2018). Diagnosis and man- agement of pleural metastasis and malignant effusions fects of lipiodol, and thoracic duct embolization in breast cancer. The Breast, 5, 934–941. http://doi. was not needed. Mrs. C tolerated the procedure org/10.1016/B978-0-323-35955-9.00072-6