EVALUATION AND MANAGEMENT OF UNILATERAL DIAPHRAGMATIC PARALYSIS IN SYMPTOMATIC CYSTIC FIBROSIS PATIENT

Abstract

SESSION TITLE: Medical Student/Resident Genetic and Developmental Disorders Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Diaphragmatic paralysis is a condition commonly associated with trauma, neurologic compromise, or systemic disease. Unilateral diaphragmatic paralysis (UDP) is more common than bilateral disease and uncommonly results in respiratory symptoms. However, this case details a presentation of UDP in a 23-year-old CF patient that resulted in progressively worsening lung function necessitating surgical correction. CASE PRESENTATION: TL is a 23 year old male with CF who presented with a 4-week history of worsening dyspnea, productive cough, chest tightness, and decreased exercise capacity. He was hospitalized and started on IV antibiotics. During hospitalization, patient underwent radiology which showed progression of disease via CT, PFT, and BD testing. Patient was discharged home after 6 weeks with a lack of improvement in lung function and given home IV antibiotics. A clinic follow-up appointment was scheduled to assess lung function following home IV. At 4-week follow up he reported worsening shortness of breath and his PFT did not show improvement. Physical exam showed significant increase in work of breathing lying down as compared to sitting upright. Chest X-ray was concerning for significant elevation of right hemi-diaphragm. A repeat chest CT confirmed elevation of right hemi diaphragm with progressive fibrosis. He underwent bronchoscopy and BAL which confirmed patent airways. He underwent sniff testing using flouroscopic guidance which confirmed paralyzed right hemi diaphragm with paradoxical motion. The combined results were felt to be consistent with idiopathic unilateral diaphragmatic paralysis leading to rapid decline in lung function. The patient underwent right thoracotomy and plication of right hemi diaphragm. Patient tolerated surgery well. At 6 weeks follow-up he had significant improvement in his shortness of breath, quality of life and exercise capacity. His PFT recovered to baseline and are now stable. DISCUSSION: UDP is often asymptomatic but underlying heart or lung disease, such as CF, significantly worsens dyspnea in patients with UDP(1). The most common causes of UDP include postsurgical complications, trauma, infection and neurological disease. CF drugs like aminoglycosides, colistin, linezolid and atypical infections can potentially lead to diaphragmatic weakness with significant loss of lung function (2). The work-up for diagnosing diaphragmatic weakness is extremely important, especially in CF, and this case demonstrates the breadth of etiologies that can be investigated. CONCLUSIONS: Diaphragmatic dysfunction is an important and underdiagnosed cause of shortness of breath. It can negatively affect quality of life, indicate disease severity, and be potentially prognostic. Although paralysis can often be due to phrenic nerve dysfunction, this presentation is an example of drug and/or infection-induced paralysis that can lead to significant loss of lung function. Reference #1: Armstrong, J. D. (2012). Dysfunction of the diaphragm [4]. New England Journal of Medicine, 366(21), 2036–2037 Reference #2: Wilcox, P.G., Pardy, R.L. (1989) Diaphragmatic weakness and paralysis. Lung 167, 323–341 DISCLOSURES: Advisory Committee Member relationship with PARI Please note: $1001 - $5000 Added 03/31/2020 by Nauman Chaudary, source=Web Response, value=Consulting fee Advisory Committee Member relationship with Novartis Please note: $1001 - $5000 Added 03/31/2020 by Nauman Chaudary, source=Web Response, value=Consulting fee No relevant relationships by Radu Mitran, source=Web Response