摘要
Purpose: Obstructive apnea is sometimes seen in patients with Beckwith- Wied emann syndrome. The cause of apnea is not limited to macroglossia, and the surgi cal indication for obstructive apnea has not yet been established. The authors p erformed polysomnography for the assessment of apnea. Method: Overnight polysomn ograms were obtained in 2 patients who developed obstructive apnea after 1- sta ge repair for omphalocele. Case 1: Apnea index (AI), de fined as apneic events per hour, indicated 17.3, and SpO2 below 95% occupie d 80% of the total sleep time. Computed tomography and magnetic resonance imag ing indicated obstruction of the airway between macroglossia and the hypopharynx . Central tongue resection and the division of the frenulum linguae for associat ed ankyloglossia were performed 97 days after birth. One month after surgery, ap neic events disappeared and SpO2 below 95% occupied only 1% of the total sle ep time. Case 2: Obstructive AI indicated 28.1. Division of the frenulum linguae and anterior glossopexy were performed 55 days after birth. Postoperative polys omnogram indicated a marked reduction of AI. Conclusions: These results indicate d that polysomnography was useful for evaluating obstructive apnea and that adva ncement of the tongue by division of the frenulum linguae may be recommended for the treatment of obstructive apnea in patients with Beckwith- Wiedemann syndro me.
Purpose: Obstructive apnea is sometimes seen in patients with Beckwith- Wied emann syndrome. The cause of apnea is not limited to macroglossia, and the surgi cal indication for obstructive apnea has not yet been established. The authors p erformed polysomnography for the assessment of apnea. Method: Overnight polysomn ograms were obtained in 2 patients who developed obstructive apnea after 1- sta ge repair for omphalocele. Case 1: Apnea index (AI), de fined as apneic events per hour, indicated 17.3, and SpO2 below 95% occupie d 80% of the total sleep time. Computed tomography and magnetic resonance imag ing indicated obstruction of the airway between macroglossia and the hypopharynx . Central tongue resection and the division of the frenulum linguae for associat ed ankyloglossia were performed 97 days after birth. One month after surgery, ap neic events disappeared and SpO2 below 95% occupied only 1% of the total sle ep time. Case 2: Obstructive AI indicated 28.1. Division of the frenulum linguae and anterior glossopexy were performed 55 days after birth. Postoperative polys omnogram indicated a marked reduction of AI. Conclusions: These results indicate d that polysomnography was useful for evaluating obstructive apnea and that adva ncement of the tongue by division of the frenulum linguae may be recommended for the treatment of obstructive apnea in patients with Beckwith- Wiedemann syndro me.
