Clinical Characteristics, Imaging Findings and Surgical Outcomes of Chiari Malformation Type I in Pediatric and Adult Patients

Abstract

A growing number of children and adolescents are being diagnosed as Chiari malformation type I (CM-I) for behavioral disorders, developmental delay, seizures, or abnormal orpharyngeal function. The aim of this study was to compare the clinical characteristics, imaging findings and surgical outcomes of CM-I in pediatric and adult patients. Between January 2014 and June 2017, 84 patients with CM-I underwent surgical treatment in our department. We divided the patients into two groups: pediatric group (n=11, age <18 years) and adult group (n=73, age ≥18 years). Data on clinical characteristics, imaging findings, surgical outcomes, and prognosis were retrospectively reviewed and compared between these two groups. For clinical presentation, scoliosis (36.4%) and developmental delay (36.4%) were more common in pediatric patients, whereas, sensory disturbance (58.9%) and motor weakness (41.1%) were more common in adult patients. Imaging findings showed that the incidence of hydrocephalus and craniovertebral junctional abnormalities was significantly higher in pediatric group than in adult group (P<0.05). Compared to adult group, pediatric group showed a better improvement or resolution of syrinx and tonsillar herniation after surgical treatments (P<0.05). The total Chicago Chiari Outcome Scale (CCOS) score in pediatric patients at the last followup was significantly higher than that in adult patients (P=0.002). In conclusion, the clinical characteristics and imaging findings appeared to be different in pediatric and adult patients with CM-I. The surgical outcomes of pediatric patients were shown to be significantly better than those of adult patients.

Keywords

Chiari malformation type I / adult patients / pediatric patients / clinical manifestations / surgical outcome

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This project was supported by grants from the National Natural Science Foundation of China (No. 81702478 and No. 81270865) and China Postdoctoral Science Foundation (No. 2016M600596).