title: [Anesthetic management of a child with Hunter syndrome associated with sleep apnea].
abstract: A 2-year-old boy with Hunter syndrome was scheduled for adenotomy under general anesthesia. Preoperative examinations of this patient revealed that he had stridor, sleep apnea, short neck, and a hypertrophic pharyngeal structure as revealed by fiberoptic pharyngoscopy, from which we expected to encounter difficulty in mask ventilation and tracheal intubation. On standby with settings for an emergency fiberoptic laryngoscopy and an emergency tracheotomy, we first attempted slow induction technique preserving spontaneous breathing. Thereafter mask ventilation was possible and a muscle relaxant was administered. On the second trial, almost blind orotratcheal intubation was successful. Postoperatively, mechanical ventilation was performed for 12 hours, in consideration of the airway obstruction due to pharyngeal bleeding and edema. The most severe anesthetic complication in the case of Hunter syndrome is the difficulty of intubation and respiratory disorder. Preoperative evaluation of the upper airway and careful perioperative monitoring and observation of respiration are of the greatest importance.
authors.affiliation: Department of Anesthesia, Chugoku Rosai General Hospital, Kure.
authors.forename: K
authors.initials: K
authors.lastname: Yamada
authors.forename: I
authors.initials: I
authors.lastname: Nakagawa
authors.forename: M
authors.initials: M
authors.lastname: Kubota
authors.forename: H
authors.initials: H
authors.lastname: Niinai
authors.forename: T
authors.initials: T
authors.lastname: Kamiya
mesh_terms: D000233:Adenoidectomy; D000402:Airway Obstruction / Q000209:etiology / Q000517:prevention & control / Q000601:surgery; D000768:Anesthesia, General; D002675:Child, Preschool; D006801:Humans; D007442:Intubation, Intratracheal; D008297:Male; D016532:Mucopolysaccharidosis II / Q000150:complications*; D011182:Postoperative Care; D011183:Postoperative Complications / Q000517:prevention & control; D012121:Respiration, Artificial; D012891:Sleep Apnea Syndromes / Q000209:etiology*