Diaphragm Dysfunction (Paralysis) in a Cystic Fibrosis Patient

Abstract

Diaphragmatic Paralysis (DP) is a rare disease and usually occurs secondary to systemic processes, although idiopathic forms have been described. Management of diaphragmatic paralysis depends on multiple factors, including the etiology, presence, or absence of symptoms, and more importantly, the presence of nocturnal hypoventilation. DP has been described in both unilateral and bilateral forms. In unilateral DP, most patients are asymptomatic and often do not require intervention. Symptomatic patients with unilateral DP, however, will require plication. In bilateral DP, phrenic nerve pacing or nasal Continuous Positive Airway Pressure, Bilateral Positive Airway Pressure (CPAP BiPAP), or intermittent positive pressure ventilation by the nasal or oral mask is recommended. We describe a case of unilateral DP in a cystic fibrosis patient treated by surgical plication.