Multicenter comparison of Chiari malformation type I presentation in children versus adults

J Neurosurg Pediatr. 2024 Feb 23;34(1):49-56. doi: 10.3171/2023.12.PEDS22366. Print 2024 Jul 1.

Abstract

Objective: Treatment for Chiari malformation type I (CM-I) often includes surgical intervention in both pediatric and adult patients. The authors sought to investigate fundamental differences between these populations by analyzing data from pediatric and adult patients who required CM-I decompression.

Methods: To better understand the presentation and surgical outcomes of both groups of patients, retrospective data from 170 adults and 153 pediatric patients (2000-2019) at six institutions were analyzed.

Results: The adult CM-I patient population requiring surgical intervention had a greater proportion of female patients than the pediatric population (p < 0.0001). Radiographic findings at initial clinical presentation showed a significantly greater incidence of syringomyelia (p < 0.0001) and scoliosis (p < 0.0001) in pediatric patients compared with adult patients with CM-I. However, presenting signs and symptoms such as headaches (p < 0.0001), ocular findings (p = 0.0147), and bulbar symptoms (p = 0.0057) were more common in the adult group. After suboccipital decompression procedures, 94.4% of pediatric patients reported symptomatic relief compared with 75% of adults with CM-I (p < 0.0001).

Conclusions: Here, the authors present the first retrospective evaluation comparing adult and pediatric patients who underwent CM-I decompression. Their analysis reveals that pediatric and adult patients significantly differ in terms of demographics, radiographic findings, presentation of symptoms, surgical indications, and outcomes. These findings may indicate different clinical conditions or a distinct progression of the natural history of this complex disease process within each population, which will require prospective studies to better elucidate.

Keywords: CSF; Chiari malformation; spinal cord; syringomyelia; syrinx.

Publication types

  • Multicenter Study
  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Arnold-Chiari Malformation* / complications
  • Arnold-Chiari Malformation* / diagnostic imaging
  • Arnold-Chiari Malformation* / surgery
  • Child
  • Child, Preschool
  • Decompression, Surgical* / methods
  • Female
  • Humans
  • Male
  • Middle Aged
  • Retrospective Studies
  • Scoliosis / diagnostic imaging
  • Scoliosis / surgery
  • Syringomyelia / complications
  • Syringomyelia / diagnostic imaging
  • Syringomyelia / surgery
  • Treatment Outcome
  • Young Adult