Postoperative acute respiratory failure caused by adult-onset Pompe disease

Pompe disease, which leads to dysfunction of the enzyme acid a-glucosidase, is a genetic disorder seen in 1 in 40000 births. Adult-onset Pompe disease is known as a slowly progressive myasthenia with or without respiratory dysfunction. We herein report two cases of adult-onset Pompe disease, in which postoperative acute respiratory failure was the the initial manifestation. The two patients showed no symptoms of ambulatory and respiratory dysfunction before operation. The diagnosis of Pompe disease was determined by muscle biopsy and acid a-glucosidase assay in the blood. Rapid deterioration of already struggling diaphragmatic function induced by stress of surgery and anesthesia were thought to be the main reason of postoperative acute respiratory failure. Physicians should be aware of the existence of an adult form of Pompe disease which may present with postoperative acute respiratory failure.