Vocal Cord Paralysis in Pediatric Chiari Malformation: A Systematic Review

Introduction: Arnold-Chiari Malformation (ACM) is one possible cause of congenital pediatric vocal cord paralysis (VCP). The prognostic factors affecting VCP in children with ACM are not well characterized. We hypothesized that younger age at onset of stridor or VCP would be associated with poorer prognosis and that early posterior fossa decompression (PFD) would be associated with better prognosis.

Methods: A systematic review was performed according to PRISMA guidelines. PubMed was searched for relevant studies yielding over 200 articles. Study abstracts were reviewed by three separate examiners. Sixty studies underwent full text review. The following data were extracted: age at onset of stridor or VCP, ACM type, laryngoscopy findings, type and timing of neurosurgery, and tracheostomy history. Analyses utilized Chi-square tests.

Results: Over 90% had bilateral vocal cord paralysis. For both ACM 1 and 2, there were no statistically significant associations between age at VCP onset and rates of complete resolution of VCP or tracheostomy. For both ACM 1 and 2, there were no statistically significant associations between time from VCP onset to PFD and rate of tracheostomy placement, rate of decannulation, or rate of complete resolution of VCP. For children with ACM type 2, younger age at symptom onset was correlated with decreased rate of tracheostomy decannulation (n=21, p=0.026).

Conclusions: A meta-analysis of the literature suggests an association between younger age at onset of VCP in ACM and lower rate of tracheostomy decannulation, reinforcing prior findings of earlier symptom onset’s conferring poorer prognosis. Additional prospective studies are needed to elucidate the natural history and utility of early intervention in children with vocal cord paralysis secondary to Chiari malformation.