Difference in clinical presentation and surgical outcomes in pediatric and adult patients with Chiari malformation type 1: a single center retrospective study

Abstract

Introduction

Chiari malformation type 1 (CM1) is a common congenital disorder affecting both children and adults. Although pediatric and adult CM1 patients share many characteristics, the differences between the groups are not fully described.

Method

A comparative analysis was made of two previously defined cohorts of adult and pediatric non-syndromic CM1, surgically treated at the study center. Clinical outcomes were assessed using the Chicago Chiari outcome scale (CCOS) and radiological outcomes were measured as change in cerebellar tonsil and syringomyelia status.

Results

A total of 209 patients (73 pediatric, 136 adults) were included, with median ages of 11 and 33 years, respectively. The proportion of female patients (62% vs 78%) was higher in the adult population (p = 0.012). Headache (p = 0.007), neck pain (p = 0.000), vertigo (p = 0.007), and sensory symptoms (p = 0.000) were more common in adults, while scoliosis (p = 0.000) and sleep apnea (p = 0.015) were more common in the pediatric population. Preoperative imaging findings did not differ significantly. After posterior fossa decompression, both groups scored a median CCOS of 15 at early follow-up (3 vs 4 months), though the pediatric population had a more favorable distribution of CCOS scores (p = 0.003). Postoperatively, syringomyelia status did not differ significantly between groups, but cerebellar tonsil status improved more frequently in adults (64% vs 88%, p = 0.000).

Conclusion

This study demonstrates that while headache is the most common presenting symptom in both pediatric and adult CM1 patients, pediatric patients are more likely to present with scoliosis and sleep apnea. In contrast adult patients more frequently experience headache, neck pain, vertigo, and sensory symptoms. There were no differences in other preoperative imaging variables and outcomes were favorable for most patients in both groups.