title: General anesthesia for a patient with deletion 6q syndrome in addition to laryngomalacia undergoing dental treatment.
abstract: Chromosome 6 deletions are very rare (1,2), and deletion 6q syndrome is clinically characterized by mental and/or neuromotor retardation and microcephaly (3). Other alterations frequently observed are decreased biparietal diameter, hypertelorism, hypotelorism, absent eyebrows, prominent eyes with ptosis, receding chin, dysmorphic ears, large extremities, prominent nasal bridge, long philtrum, epicthus, strabismus, and micrognathia (3-5). Laryngomalacia is the most common congenital laryngeal anomaly and the most frequent cause of stridor in infants (6-11). We report the case of a 14-year-old male patient with both deletion 6q syndrome and laryngomalacia, who we treated for multiple dental caries. He had a medical history of tracheotomy at age 11 years for laryngomalacia, and has suffered from epileptic attacks and aspiration pneumonia over the last 2 and 6 years, respectively. Since he was mentally retarded and in a poor respiratory state, dental treatment under general anesthesia was scheduled in our hospital. General anesthesia was induced and maintained using 30% nitrous oxide and 1-3% sevoflurane in oxygen through the tracheotomy tube. Pre- and intraoperative endotracheal suction improved the condition of both lungs markedly and the procedures were uneventful and completed in 2 h and 58 min.
authors.affiliation: Department of Dental Anesthesiology, School of Dental Medicine, Tsurumi University, Yokohama, Japan. fujii-keiko@tsurumi-u.ac.jp
authors.forename: Keiko
authors.initials: K
authors.lastname: Fujii-Abe
authors.forename: Mami
authors.initials: M
authors.lastname: Sasao
authors.forename: Haruhisa
authors.initials: H
authors.lastname: Fukayama
mesh_terms: D000293:Adolescent; D000766:Anesthesia, Dental / Q000379:methods*; D000769:Anesthesia, Inhalation / Q000379:methods*; D018685:Anesthetics, Inhalation / Q000008:administration & dosage; D002872:Chromosome Deletion; D002896:Chromosomes, Human, Pair 6 / Q000235:genetics*; D017742:Dental Care for Chronically Ill; D003731:Dental Caries / Q000628:therapy*; D004827:Epilepsy / Q000473:pathology; D006801:Humans; D008607:Intellectual Disability; D007442:Intubation, Intratracheal; D055092:Laryngomalacia / Q000473:pathology*; D008297:Male; D008738:Methyl Ethers / Q000008:administration & dosage; D009609:Nitrous Oxide / Q000008:administration & dosage; D010100:Oxygen / Q000008:administration & dosage; D011015:Pneumonia, Aspiration / Q000473:pathology; D000077149:Sevoflurane; D013396:Suction; D013577:Syndrome; D014139:Tracheostomy
chemical_list: D018685:Anesthetics, Inhalation; D008738:Methyl Ethers; D000077149:Sevoflurane; D009609:Nitrous Oxide; D010100:Oxygen