Combined general and epidural anesthesia for major abdominal surgery in a patient with Pompe disease

We present a case of combined general anesthesia with muscle relaxant and epidural analgesia for hemicolectomy in a 56-year-old woman with Pompe disease. Progressive pulmonary function loss predisposes Pompe disease patients to an increased risk of aspiration pneumonia, atelectasis, and all pulmonary infections. Given the impaired cough resulting from abdominal muscle weakness, patients with Pompe disease who undergo abdominal major surgery are prone to great risks of postoperative pulmonary complications. In our case, to optimize the patient's pulmonary toilet during the postoperative period, epidural block was provided as well as general anesthesia. Although she had a severe scoliotic spine and a worst pulmonary function test, the attempt of epidural block provided excellent pain control and pulmonary toilet care.