Incidence and Characterization of Oral Motor, Pharyngeal, and Cervical Esophageal Swallowing Dysfunction in Pediatric Patients with Chiari 1 Malformation

Abstract

Chiari I malformation is as an abnormality of the brain at the cervicomedullary junction. The cerebellar tonsils extend through the foramen magnum and into the upper cervical spinal canal. Compression of the brainstem and lower cranial nerves may occur. Disruption in cerebrospinal fluid flow dynamics and the formation of syrinxes in the spinal cord may also be present. Consequently, a variety of clinical manifestations may occur including problems with oropharyngeal and cervical esophageal swallowing function.

The present study was a retrospective review of 251 pediatric patients with a new diagnosis of Chiari I. Clinical signs and symptoms as reported during clinic visits to the Cincinnati Children’s Neurosurgery Department were documented and compared to age. Logistic regression was utilized to estimate clinical signs and symptoms likely to be present in conjunction with characteristics of the Chiari (degree of cerebellar tonsillar descent, tonsillar pointing, tonsillar pistoning, and disruption of cerebrospinal fluid flow). Signs and symptoms of oropharyngeal and cervical esophageal swallowing dysfunction were correlated with objective findings during instrumental assessments of swallowing to determine associations.

Results indicated that clinical manifestations of oropharyngeal and cervical esophageal swallowing dysfunction varied according to age category. Swallowing problems, oral motor dysfunction, gagging, and coughing/choking with feeding were shown to have a statistically significant linear relationship with age, and tended to be reported most often in the youngest age category of 10 months to 3 years: p < .0001. Oral dysfunction, swallowing problems, and gagging were predicted to occur with tonsillar descent greater than 9 millimeters, and in the presence of tonsillar pointing, tonsillar pistoning, and abnormal cerebrospinal fluid flow dynamics. Videofluoroscopic assessment of swallowing function (VSS) and Fiberoptic Endoscopic Evaluation of Swallowing (FEES) revealed associations between coughing/choking and globus sensation with objective findings of premature spillage prior to swallowing, residue after swallowing, and abnormal cricopharyngeal function. FEES with sensory testing provided information about sensory threshold responses.

This study provides evidence that signs and symptoms of oropharyngeal and cervical esophageal swallowing dysfunction are likely to accompany Chiari I malformation in pediatric patients and should be considered in the differential diagnosis of feeding and swallowing problems.