SESSION TITLE: Lung Pathology Cases
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Tuesday, October 27, 2015 at 04:30 PM - 05:30 PM
INTRODUCTION: Plastic bronchitis (PB) is a rare occurrence of unknown etiology primarily described in children with congenital heart disease (CHD). To our knowledge, this represents the first reported case of PB in a patient with Kaposi sarcoma (KS).
CASE PRESENTATION: A 25-year-old man with HIV/AIDS and pulmonary KS presented with dyspnea and productive cough of “white-red” tissue. Physical exam revealed hypoxia, bibasilar rhonchi, and multiple violaceous plaques. Chest CT showed diffuse peribronchovascular consolidative opacities and interlobular septal thickening (Fig 1). Infectious workup was negative. Bronchoscopy revealed tissue floating throughout the tracheobronchial tree, forming bronchial casts. Pathology showed non-inflammatory fibrinous fragments, consistent with PB. The patient continued to expectorate large casts despite inhaled mucolytics, bronchodilators, steroids, and chest percussion therapy. He ultimately required mechanical ventilation and recurrent bronchoscopy to clear the casts (Fig 2). Despite therapies aimed at reducing lymphatic flow (including midodrine, octreotide, sirolimus, and total parenteral nutrition), the patient ultimately expired. Autopsy revealed extensive pulmonary KS with profound compression of the lymphatics.
DISCUSSION: PB is a rare phenomenon in which patients expectorate thick branching bronchial casts in the mold of the tracheobronchial tree. It has been described in patients of all ages and in association with numerous coexisting illnesses including any cause of bronchial inflammation (asthma, ABPA, pneumonia), cardiac pathology (especially CHD), or disorders of lymphatic drainage (lymphangiectasia, lymphangiomatosis). To our knowledge, no cases of PB in the setting of KS, an angioproliferative disorder of the vascular and lymphatic endothelium, have been described. KS is well known to cause lymphatic obstruction and severe lymphedema of the extremities but similar lymphatic stasis in the lungs has not been described. We postulated that the development of PB in this patient was secondary to lymphatic stasis in the lungs thus therapies aimed at reducing lymphatic flow were implemented though to no avail.
CONCLUSIONS: PB is the rare occurrence of large branching bronchial casts associated with many coexisting illnesses. This case demonstrates a previously unreported cause of PB and highlights the importance of investigation into its pathogenesis and therapeutic alternatives.
Reference #1: Eberlein MH et al. Plastic bronchitis: a management challenge.Am J Med Sci 2008;335(2):163-169.
DISCLOSURE: The following authors have nothing to disclose: Sheila Habib, Phoebe King, Krayton Blower, Diego Maselli, Gregory Anstead
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