[Difficult intubation and anesthesia in Pfaundler-Hurler disease]

Because of increasing dyspnea, adenoidectomy and tonsillectomy were indicated in a 6-year-old girl with Pfaundler-Hurler disease. The main symptoms were cardiac insufficiency and chronic bronchitis. Because of the large skull with hypoplastic cervical spine, anomalies of facial bones, larynx and jawbone normal intubation was impossible. We therefore performed intubation during spontaneous breathing under inhalation anesthesia and topical anesthesia of the larynx. After exploratory laryngoscopy a preformed Woodbridge tube was inserted into the trachea blind and by ear during of inspiration. The patient's cardiac insufficiency and chronic bronchitis made a balanced anesthesia with reduced dosage of rapifen and halothane necessary. In spite of the poor general prognosis, after the operation a considerable relief of the patient's suffering was noted, because she was able to breathe freely.