title: Anesthetic and airway management of general anesthesia in a patient with Meckel-Gruber syndrome.
abstract: Meckel-Gruber syndrome, characterized by occipital encephalocele, microcephaly, polydactyly, cleft lip or palate, mandibular micrognathism, and anatomical abnormality of the larynx and tongue, along with other associated malformations, is in the list of diseases associated with difficult airway. However, there has been no report on the management of general anesthesia and airway management for such patients. A 2-year-old girl with Meckel-Gruber syndrome was scheduled for cardioplasty and gastrostomy for gastroesophageal reflux under general anesthesia. Preoperative examination revealed obesity, microgenia, dysspondylism, proteinuria, hypoplastic kidneys, and stenosis of the anal canal. Although we anticipated some difficulty with the intubation and prepared several alternative methods for intubation, such as a bronchofiberscope and a laryngeal mask airway, tracheal intubation was completed without difficulty using conventional laryngoscopy after inhalational induction with sevoflurane. Because most patients with this syndrome die before and shortly after delivery, those who survive to some age might have less severe deformities.
authors.affiliation: Department of Anesthesiology and Medical Crisis Management, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467-8602, Japan.
authors.forename: Mitsunori
authors.initials: M
authors.lastname: Miyazu
authors.forename: Kazuya
authors.initials: K
authors.lastname: Sobue
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Ito
authors.forename: Takafumi
authors.initials: T
authors.lastname: Azami
authors.forename: Shoji
authors.initials: S
authors.lastname: Ito
authors.forename: Akinori
authors.initials: A
authors.lastname: Takeuchi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Sasano
authors.forename: Takako
authors.initials: T
authors.lastname: Tsuda
authors.forename: Hirotada
authors.initials: H
authors.lastname: Katsuya
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery*; D000768:Anesthesia, General / Q000379:methods*; D018685:Anesthetics, Inhalation; D002675:Child, Preschool; D005260:Female; D005764:Gastroesophageal Reflux / Q000601:surgery*; D006801:Humans; D007442:Intubation, Intratracheal; D007828:Laryngoscopy; D008738:Methyl Ethers; D000077149:Sevoflurane; D013577:Syndrome; D014132:Trachea
chemical_list: D018685:Anesthetics, Inhalation; D008738:Methyl Ethers; D000077149:Sevoflurane