SESSION TITLE: Bronchology/Interventional Procedures Student/Resident Cases
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 28, 2014 at 07:30 AM - 08:30 AM
INTRODUCTION: Double aortic arch is typically diagnosed during infancy and surgically repaired. We describe an elderly patient with progressive dyspnea and wheezing, in part due to tracheal compression from a congenital double aortic arch.
CASE PRESENTATION: A 79 year-old female presented with progressive dyspnea, wheezing and dysphagia to the ICU. She reported a lifelong history of exertional dyspnea, and had been on supplemental oxygen for 3 years. Her exertional symptoms escalated over the preceding months, and she received 3 courses of antibiotics for pneumonia. CT images revealed a double aortic arch with a 4 vessel branching pattern, and significant mass effect on the trachea and esophagus (Figure 1). The aorta had significant atherosclerotic calcification.The narrowest point on the trachea measured 8mm, and an esophagram demonstrated smooth narrowing at the level of the aortic arch with proximal dilation and accumulation of contrast. In an effort to control her dyspnea, bronchoscopy was performed with anticipation to place a tracheal stent at the area of tracheal compromise due to the double aortic arch. Rather than focal stenosis, diffuse, profound tracheobronchomalacia (TBM) was identified and a Y stent was placed (Figure 2). While some symptomatic improvement was noted post-procedure, she developed significant mucus plugging requiring bronchoscopic suctioning on two occasions.
DISCUSSION: Double aortic arch is a congenital abnormality typically diagnosed as a child. While rare, elderly patients can present with late onset of symptoms related to tracheal and/or esophageal obstruction.1,2 Age-related elongation of the aorta combined with atherosclerotic burden is felt to contribute to increasing compressive effects upon adjacent structures. In considering a differential for atypical wheezing and dysphagia, aortic arch abnormalities should be considered. Congenital vascular abnormalities are usually surgically corrected. In advanced age, these abnormalities may lead to focal narrowing at the site of compression, or malacic airways due to chronic pressure. We found diffuse TBM and attempted symptom alleviation via Y-stent placement. This has not been previously reported in the literature.
CONCLUSIONS: Congenital double aortic arch rarely presents in adulthood. Concurrent development of dysphagia and dyspnea should raise suspicion about a vascular variant causing compressive effects on adjacent soft tissue structures. Further study is needed to explore non-surgical approaches such as airway stenting to palliate symptoms, especially when dealing with a geriatric population.
Reference #1: Keskin O, Erden A, Soykan I. An unusual cause of dysphagia. Double aortic arch. Gastroenterology 2013; 144(4): 694
Reference #2: Lone GN, Rathore SS, Malik JA et al. Double aortic arch masquerading as bronchial asthma for five decades. Asian Cardiovasc Thorac Ann 2012; 20(3): 338-340
DISCLOSURE: The following authors have nothing to disclose: Michael Perkins, Margaret Pisani, Jonathan Puchalski, Shyoko Honiden
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