title: Anesthetic considerations of two sisters with Beckwith-Wiedemann syndrome.
abstract: Anesthetic considerations of 21-mo-old and 4-yr-old sisters with Beckwith-Wiedemann syndrome during surgical repair of cleft palate and reduction of macroglossia are presented and discussed. This syndrome is characterized by exomphalos, macroglossia, gigantism, hypoglycemia in infancy, and many other clinical features. This syndrome is also known as exomphalos, macroglossia, and gigantism (EMG) syndrome. Principal problems associated with anesthetic management in this syndrome are hypoglycemia and macroglossia. Careful intraoperative plasma glucose monitoring is particularly important to prevent the neurologic sequelae of unrecognized hypoglycemia. It is expected that airway management would be complicated by the macroglossia, which might cause difficult bag/mask ventilation and endotracheal intubation following the induction of anesthesia and muscle paralysis, so preparations for airway difficulty (e.g., awake vocal cord inspection) should be considered before induction. A nasopharyngeal airway is useful in relieving postoperative airway obstruction.
mesh_terms: D000402:Airway Obstruction / Q000517:prevention & control; D000766:Anesthesia, Dental / Q000379:methods*; D000768:Anesthesia, General / Q000379:methods*; D001506:Beckwith-Wiedemann Syndrome / Q000150:complications; D002675:Child, Preschool; D002972:Cleft Palate / Q000601:surgery; D003730:Dental Care for Persons with Disabilities / Q000379:methods*; D005260:Female; D006801:Humans; D007003:Hypoglycemia / Q000209:etiology; D007223:Infant; D008260:Macroglossia / Q000150:complications / Q000209:etiology; D016343:Monitoring, Intraoperative