• Satoshi Toyama, Fumimaro Hatori, Ayako Shimizu, and Toshio Takagi.
• Department of Anesthesia and Intensive Care Unit, National Center for Child Health and Development, Tokyo, Japan.
• J Anesth. 2008 Jan 1;22(2):182-5.

AbstractWe performed anesthetic management for a patient with severe Williams-Campbell syndrome, which is a congenital deficiency of cartilage in the subsegmental bronchial tree. An 11-year-old girl with this syndrome had labored breathing because of abdominal distension caused by a giant ovarian tumor, and removal of the tumor was scheduled. Because she had been receiving home oxygen therapy for 10 years due to hypoxia, it was possible that positive-pressure ventilation may have increased the risk of perioperative pulmonary complications. So we selected combined spinal and epidural anesthesia with general anesthesia, using a ProSeal (Laryngeal Mask Company, Henley on Thames, UK) laryngeal mask airway. We placed an epidural catheter and induced spinal anesthesia blockade under general anesthesia as the main analgesia technique, in order to maintain spontaneous breathing. The surgery was completed uneventfully and the patient emerged from anesthesia without dyspnea. She had an uneventful recovery and was discharged home.

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