title: Assessment of obstructive apnea by using polysomnography and surgical treatment in patients with Beckwith-Wiedemann syndrome.
abstract: OBJECTIVE
  Obstructive apnea is sometimes seen in patients with Beckwith-Wiedemann syndrome. The cause of apnea is not limited to macroglossia, and the surgical indication for obstructive apnea has not yet been established. The authors performed polysomnography for the assessment of apnea.
  
  
  METHODS
  Overnight polysomnograms were obtained in 2 patients who developed obstructive apnea after 1-stage repair for omphalocele. CASE 1: Apnea index (AI), defined as apneic events per hour, indicated 17.3, and SpO2 below 95% occupied 80% of the total sleep time. Computed tomography and magnetic resonance imaging indicated obstruction of the airway between macroglossia and the hypopharynx. Central tongue resection and the division of the frenulum linguae for associated ankyloglossia were performed 97 days after birth. One month after surgery, apneic events disappeared and SpO2 below 95% occupied only 1% of the total sleep time. CASE 2: Obstructive AI indicated 28.1. Division of the frenulum linguae and anterior glossopexy were performed 55 days after birth. Postoperative polysomnogram indicated a marked reduction of AI.
  
  
  CONCLUSIONS
  These results indicated that polysomnography was useful for evaluating obstructive apnea and that advancement of the tongue by division of the frenulum linguae may be recommended for the treatment of obstructive apnea in patients with Beckwith-Wiedemann syndrome.
authors.affiliation: Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan. kamata@pedsurg.med.osaka-u.ac.jp
authors.forename: Shinkichi
authors.initials: S
authors.lastname: Kamata
authors.forename: Masafumi
authors.initials: M
authors.lastname: Kamiyama
authors.forename: Toshio
authors.initials: T
authors.lastname: Sawai
authors.forename: Keisuke
authors.initials: K
authors.lastname: Nose
authors.forename: Noriaki
authors.initials: N
authors.lastname: Usui
authors.forename: Hisayoshi
authors.initials: H
authors.lastname: Kawahara
authors.forename: Masahiro
authors.initials: M
authors.lastname: Fukuzawa
mesh_terms: D001506:Beckwith-Wiedemann Syndrome / Q000150:complications*; D003964:Diaphragm / Q000002:abnormalities / Q000601:surgery; D005260:Female; D006554:Hernia, Umbilical / Q000601:surgery; D006801:Humans; D007013:Hypopharynx / Q000002:abnormalities; D007231:Infant, Newborn; D008035:Lingual Frenum / Q000601:surgery; D008260:Macroglossia / Q000150:complications / Q000601:surgery; D010100:Oxygen / Q000097:blood; D017286:Polysomnography; D020181:Sleep Apnea, Obstructive / Q000097:blood / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery
chemical_list: D010100:Oxygen