Postoperative use of nasal intermittent positive pressure in a patient with spinal muscular atrophy type II.

We report the successful use of nasal intermittent positive pressure ventilation (NIPPV) in the perioperative period of a 51 yr-old woman with a type II spinal muscular atrophy (SMA II). The patient was treated chronically with nocturnal NIPPV at home and scheduled for endoscopic retrograde cholangiopancreatography (ERCP) under general anesthesia. Some criteria of difficult intubation were present (forced mouth opening of 1.5 cm, short neck and thyromental distance of 5 cm). Nasal endotracheal fiberoptic intubation during spontaneous breathing under sedation with propofol was performed. The ERCP procedure was conducted without complications. At the end of the procedure, IPPV was maintained until recovery of respiratory function. After extubation, NIPPV was continued in the recovery room. The patient was discharged from the post-anesthesia care unit 4 hours after the procedure. Management of patients with SMA remains a challenge and clinicians must be aware that the use of NIPPV may be a useful and life-saving tool in the perioperative period for these patients.