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Cardiothoracic Surgery |

Fibrosing Mediastinitis With Lymphatic Obstruction and Chyloptysis

Allen Burks, MD; Derek Byers, MD; Chad Witt, MD; Constantine Raptis, MD
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Washington University in St. Louis, St Louis, MO


Chest. 2015;148(4_MeetingAbstracts):44A. doi:10.1378/chest.2230121
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Abstract

SESSION TITLE: Thoracic Surgery Cases

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Tuesday, October 27, 2015 at 07:30 AM - 08:30 AM

INTRODUCTION: Chyloptysis is the clinical entity of sputum laced with chyle. We present a case of chyloptysis due to fibrosing mediastinitis that presented with recurrent airway obstruction and respiratory failure.

CASE PRESENTATION: A 57 year-old man was referred with a 9-year history of frequent episodes of dyspnea and a cough productive of copious white mucus that worsened after eating fatty meals. Five years prior to referral, he was treated for cryptococcal pneumonia and had recurrent episodes of respiratory failure related to tracheobronchial stenosis that improved with bronchoscopic dilations and aspiration of airway secretions. Physical examination was remarkable for bilateral rhonchi and fullness of the right axilla and chest wall. Bronchoscopy revealed thick, white, mucoid secretions, and stenosis of the trachea and bilateral main bronchi with white plaques on the mucosa (Image 1). Triglycerides, cholesterol, and chylomicrons were detected in the bronchial washings and endobronchial biopsies demonstrated abundant lipid-filled macrophages with dilated CD31+ and D2-40+ lymphatic channels. An MRI lymphangiogram showed mediastinal lymphangiectasis communicating with a malformation involving the right chest and axilla. The patient underwent ultrasound-guided doxycycline sclerosis of the lymphatic malformation.

DISCUSSION: A history of cough productive of copious white sputum following fatty meals should raise the specter of chyloptysis. It can develop from dilated bronchopulmonary lymphatics or a bronchopleural fistula communicating with a chylous effusion. This case is an unusual presentation of chyloptysis that resulted from lympangiectasis and obstruction associated with fibrosing mediastinitis and airway narrowing following cryptococcal bronchopneumonia. Upon sclerosis of the lymphatic malformation and adoption of a strict low fat diet, the patient has remained symptom-free at 18 months and has had dramatic improvements pulmonary function (Table 1).

CONCLUSIONS: Chyloptysis is confirmed by the presence of triglycerides, cholesterol, and chylomicrons in sputum or bronchial washings. Multiple diagnostic modalities may be required to identify the etiology. Here, we used bronchial washings with endobronchial biopsies and MRI lymphangiography to confirm the diagnosis. This case highlights the diagnostic utility of MRI as a noninvasive alternative to traditional lymphangiography and the therapeutic efficacy of dietary fat restriction for chyloptysis.

Reference #1: Lim KG, et al. Chyloptysis in adults. Chest. 2004;125:336-40

DISCLOSURE: The following authors have nothing to disclose: Allen Burks, Derek Byers, Chad Witt, Constantine Raptis

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