Dramatic improvement after bilateral diaphragmatic plication in Charcot-Marie-Tooth disease

A 52-year-old woman with Charcot-Marie-Tooth disease presented with severe dyspnea due to bilateral diaphragmatic paralysis severely compromising respiratory function. There was little in the available literature to guide us regarding management of this unusual condition, and after deliberation, we decided to treat her with a staged plication of bilateral hemidiaphragms. Postoperatively, she demonstrated very good symptomatic relief supported by objective evidence, including improvement in lung function tests. We describe our management of this difficult condition, including the surgical and anesthetic considerations, and would recommend bilateral diaphragmatic plication as an effective option in patients with this unfortunate disease.