Goto T, Hurford W E
Department of Anesthesia, Massachusetts General Hospital, Boston.
J Clin Anesth. 1994 Sep-Oct;6(5):434-6. doi: 10.1016/s0952-8180(05)80019-4.
We present the case of a patient with Charcot-Marie-Tooth (CMT) disease who required prolonged ventilatory support following a thoracotomy due to respiratory muscle weakness. Although CMT was traditionally believed to affect only skeletal muscles, recent evidence suggests that respiratory involvement is relatively common, even in the absence of pulmonary symptoms. Assessment of respiratory muscle strength using measurements of vital capacity and negative inspiratory force is helpful in evaluating pulmonary reserve in patients with CMT.