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Chyloptysis with chylopericardium, a rare case and mini-review
- Xuming Luo†1,
- Zhuhua Zhang†1,
- ShiQiang Wang1,
- XianDong Gu1 and
- Xiongbiao Wang1Email authorView ORCID ID profile
© The Author(s). 2018
Received: 18 April 2017
Accepted: 11 January 2018
Published: 29 January 2018
Chyloptysis is reflux of chyle into the lungs and is a rare manifestation of primary chylous disorders.
Over 29 months, on the basis of x-rays, a 33-year-old female was repeatedly misdiagnosed with recurrent right-sided pneumonia; her symptoms included a recurrent cough, the appearance of cheese-like sputum, and diffuse pulmonary exudates. There was a history that abundant fluid in the pericardium was confirmed with echocardiography to have been present and asymptomatic for 4 years. Lymphangiography and surgery confirmed that the terminal portion of the thoracic duct was compressed by thick fibrous tissue and the vascular sheath of the internal jugular vein. Chyloptysis caused by high peribronchial lymphatic pressure was diagnosed and surgical intervention relieved the symptoms.
Chyloptysis is rare and easy to misdiagnose but is a typical symptom of chylous reflux syndrome.
Chylous disorders are uncommon; chylous ascites, chylothorax, and chylopericardium are relatively more frequent, and chyloptysis is extremely rare. Chyloptysis is defined as the expectoration of milky-white sputum rich in chyle. Initially 11 cases reported in the literature were reviewed in 2012 by Kato ; no more than 20 cases have been reported overall [2–8]. Patients and primary care physicians often do not notice this sputum type, which can lead to misdiagnosis . Here we described one such case that persisted for 29 months until we confirmed and corrected the disorder.
During surgery, the end of the thoracic duct, ampulla, and various lymphatic trunks were found to be parceled and compressed by thicker fibrous tissue and a vascular sheath of the internal jugular vein. Chylous reflux was observed in the lymphatic tubes of the neck, bronchomediastinal, and subclavian trunks. Lymph fluid was not flowing freely into the blood. Thicker fibrous tissue and the vascular sheath were separated, yet the end of the thoracic duct remained compressed by the neck trunk. The thoracic duct and ampulla were at an angle owing to displacement by the chest wall lymphatic tubes, so lymphatic drainage was limited. Therefore the involved chest wall lymphatics were ligated, compression of the thoracic duct was relieved, and lymph flow returned to normal. Chylous reflux in the neck and subclavian trunks was significantly alleviated. After surgery, the patient felt severe tightness in her chest, and the pericardial effusion increased significantly for a week. There was a moderate amount of pleural effusion on the left and a small amount on the right. Pleural fluid on the left was aspirated and triglycerides were 12.63 mmol/L. Symptoms improved after pericardial and hydrothorax aspiration, and both cough and expectoration were diminished. The dimensions of the chylopericardium were as follows: apex, 18 mm; left rear, 37 mm; left, 28 mm; right front, 12 mm. The patient is being followed even now.
Discussion and conclusions
Chyloptysis is the expectoration of chyle ; its rarity caused the patient depicted here to be misdiagnosed with recurrent pneumonia for 29 months, since the chyloptysis was not recognized and the pericardial effusion not aspirated. Another limitation was the difficulty in diagnosing the etiology of the reflux. Thus, to our knowledge, this is the first case in the literature regarding primary chyloptysis caused by mechanical compression and pulling of the thoracic duct with recurrent episodes of chyloptysis associated with chylopericardium. The etiology of the fibrous tissue around thoracic duct might be congenital, as there was no history of trauma or surgery. The obstruction was located near the end of thoracic duct, leading to the patient’s intrathoracic lymphatic reflux disorder. The obstruction induced chylopericardium and chyloptysis, but no symptoms of a systemic lymphatic reflux disorder were noted.
The diagnosis of chyloptysis is not difficult if the characteristic sputum is recognized. If chylous pleural or pericardial effusions accompany chyloptysis, diagnosis is easier. A cough producing white sputum occasionally slightly streaked with bloodcould indicate recurrent episodes but without bronchial infection, and an acellular sputum may indicate chyloptysis . The production of chylous sputum is not always postprandial and not always associated with the intake of fatty foods. In addition to the quantification of cholesterol and triglycerides, lipoprotein electrophoresis is useful for confirming the chylous nature of the sputum. Chylous mucus in the airway can solidify overnight resulting in formation of chylous bronchial casts . Plastic bronchitis could be formed by many compositions. Recognition of the characteristic appearance and differential diagnosis of mucus plugs will hopefully facilitate diagnosis and management [10, 11].
The main causes of chyloptysis
obstruction of the thoracic duct
surgery, radiation, trauma
an abnormality of the lymphatics
incompetence of lymphatic valves
(including yellow nail syndrome)
high venous pressure
coronary artery disease, untreated heart failure
Chyloptysis is primarily diagnosed by lymphangiography , contrast lymphangiography, or MRI. Lymphangiography is recommended to identify the anatomy and site of the lymphatic leak. Lymphoscintigraphy, which is noninvasive, has historically been the imaging modality of choice . Contrast lymphangiography can be used to confirm anatomy, lymphangiectasia, and the site of the lymphatic leak; it is also helpful in planning surgical treatment. Three-dimensional MRI may offer extensive information about the anatomy of the lymphatic vasculature and the effects of lymphatic dysfunction on local structures and tissue composition . HRCT is the most sensitive modality to identify the characteristic pathognomonic features of LAM, which are pulmonary cysts, ranging between a few mm and 1 cm in diameter with a thin wall with clear borders from the underlying normal parenchymal image, scattered evenly over all normal lung fields . Fat content should be tested on the bronchial casts or bronchial alveolar lavage fluid. Additionally, a bronchoscopy should be performed to look for an irregular connection between the pulmonary and lymphatic systems.
Treatment for chyloptysis is focused on treating the primary cause and decreasing chyle formation. In most patients, symptoms can be relieved by eliminating the underlying cause or bypassing the thoracic duct . A low-fat diet may also decrease the production of chyle . Management of the bronchial casts includes bronchoscopic extraction, which may minimize the occurrence of inflammation [5, 20].
Chyloptysis is a rare but typical symptom of chylous disorders and is easy to misdiagnose. In this case mechanical compression and pulling of the thoracic duct might have been induced by a congenital disorder, causing recurrent chyloptysis accompanied by chylopericardium. Surgery was effective for the chyloptysis, although the chylopericardium could not be resolved, likely because this area does not absorb fluid well or another pathological process is ongoing.
We would like to thank Accdon for language editing.
This work was supported by the fund of the clinical key discipline program of Putuo district of Shanghai(2016PTZK03) and the leading talent fund of Putuo district of Shanghai (2014-A-23).
Availability of data and materials
All data are contained within the manuscript.
Acquisition of Data: LX, ZZ. Drafting the manuscript: WS, GX. Revisions for intellectual content: WX. All authors read and approved the final manuscript.
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