Abstract

SESSION TITLE: Pulmonary Pathology SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/20/2019 1:00 PM - 2:00 PM INTRODUCTION: Plastic bronchitis is the expectoration of branching bronchial casts, often secondary to a defined pathologic entity like asthma, cystic fibrosis, or bronchiectasis.[1] Overall, it is a rare syndrome primarily affecting children after congenital heart surgery, and much rarer still in adults. Increasingly, lymphatic anomalies have been identified as the underlying etiology in cases previously considered idiopathic.[2] It is classically managed with mucolytics, aggressive pulmonary hygiene, and treatment of the underlying disease.[3] Mucus plugs form casts in the shape of the bronchi, and larger casts can obstruct the airway. When conservative measures fail, patients require bronchoscopic evacuation. If large enough, airway casts can lead to rapid clinical decline and even cause death by asphyxiation. Venovenous extracorporeal membrane oxygenation (VV ECMO) can secure ventilation in patients with central airway obstruction, but this has not been done previously for cases of plastic bronchitis in adults. CASE PRESENTATION: We report a case of a 34 year old female presenting with worsening cough and dyspnea in a setting of pulmonary lymphangiectasia. She described coughing up tree-like casts and noted numerous prior bronchoscopies. Early in her course, her work of breathing increased requiring intubation and mechanical ventilation. Despite aggressive pulmonary hygiene, she continued to decline. Flexible bronchoscopy revealed a large, rubbery cast descending from the tip of endotracheal tube into both main stem bronchi, occluding the airway. The obstruction could not be completely removed. With rising hypercapnia, she became more somnolent and hypoxic. An attempt at rigid bronchoscopy was deferred in favor of starting VV ECMO for stabilization. Her acidosis and hypoxemia were then rapidly corrected. With ventilation no longer compromised, her larynx was suspended and the endotracheal tube was removed, allowing unencumbered access to the airway. Alternating between rigid and flexible bronchoscopy, larger graspers were used to extract multiple portions of the cast. The airway was then widely patent. She was subsequently treated with inhaled alteplase and dornase alfa to thin secretions. She was weaned off ECMO and extubated within a week of original presentation. DISCUSSION: Among adults with plastic bronchitis, this is the first reported use of VV ECMO paired with rigid bronchoscopy. This is also the first case at our institution where ECMO was used for support in the face of central airway obstruction. The extracorporeal life support provided important stabilization without need for mechanical ventilation. Without the threat of immediate respiratory arrest, rigid bronchoscopy was safely performed to clear the occluded airway. CONCLUSIONS: Plastic bronchitis remains a rare disorder, but combined treatment with ECMO and rigid bronchoscopy may be a successful treatment option in select cases. Reference #1: Eberlein MH, Drummond MB, Haponik EF. Plastic bronchitis: a management challenge. Am J Med Sci 2008;335:163-169. Reference #2: Itkin MG, McCormack FX, Dori Y. Diagnosis and treatment of lymphatic plastic bronchitis in adults using advanced lymphatic imaging and percutaneous embolization. Ann Am Thorac Soc 2016;13:1689-1696. Reference #3: Brooks K, Caruthers RL, Schumacher KR, Stringer, KA. Pharmacotherapy challenges of Fontan-associated plastic bronchitis: a rare pediatric disease. Pharmacotherapy 2013;33:922-934. DISCLOSURES: no disclosure on file for Hatim Al-Jaroushi; No relevant relationships by Kyle Chapman, source=Web Response No relevant relationships by Jeremiah Hayanga, source=Web Response

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