Laryngologic management of infants with the Chiari II syndrome

Abstract

As a part of a prospective multi-disciplinary study, all children born with a Chiari II malformation within the Uppsala region during a 3-year period were evaluated for any difficulties in breathing or swallowing. The evaluation was repeated at regular intervals during their first 18 months. Direct laryngoscopies were performed using flexible fiberscopes. Four out of 22 children were found to have disturbed breathing. Among those, two suffered from central apnoeic spells as well as bilateral vocal fold motion impairment, one from apnoeic spells only and one from bilateral vocal fold motion impairment only. All four also had dysphagia with aspiration. Three of the children developed respiratory symptoms within the first 3 months and the symptoms of the fourth begun within the first 6 months. One infant with severe symptoms expired at the age of 3 months. The vocal fold paralysis, apnoeic spells and swallowing difficulties of another infant resolved following active neurosurgical management. The conclusion that laryngologic issues are prominent in the severe Chiari II syndrome was further supported by a review of four more cases managed in recent years. Laryngologic assessment in the neonatal period can help to raise the issue of early neurosurgical intervention as well as identify some of the infants who will need extensive habilitation. Screening of laryngeal function by means of flexible fiberoptic laryngoscopy is recommended.