Management of Sleep Apnea in Children with Chiari I Malformation: A Retrospective Study

Pediatr Neurosurg. 2022;57(3):175-183. doi: 10.1159/000523779. Epub 2022 Feb 24.


Introduction: The literature indicates that decompression of Chiari I malformations (CM-1) may resolve symptoms of sleep apnea. This study aims to identify the incidence of obstructive sleep apnea (OSA), central sleep apnea (CSA), and mixed sleep apnea in a cohort of pediatric CM-1 patients treated at our institution. We also assessed apnea-hypopnea index and symptomatology before and after surgery to investigate if Chiari decompression is a viable treatment for sleep apnea in CM-1 patients. Improvement relative to ENT surgical intervention was also considered.

Methods: We identified 75 patients who underwent polysomnography (PSG) from our database of 465 CM-1 patients. Sleep apnea diagnosis was based on the sleep physician's overall interpretation of the PSG. Symptomatology pre- and post-surgery was analyzed.

Results: Of the 75 CM-1 patients that underwent PSG, 23 were diagnosed with sleep apnea. Sixteen had OSA, 6 had CSA, and 1 had mixed apnea. Twelve OSA patients received ENT intervention. Eight improved and 2 further improved after Chiari decompression. Of the 4 patients that did not improve, one of those later improved following Chiari decompression. Of the 6 CSA patients, 2 underwent Chiari decompression, but only one improved. The mixed apnea patient underwent several ENT interventions that did not relieve symptoms but improved following Chiari decompression.

Discussion/conclusions: Based on our results, sleep apnea in CM-1 patients may be obstructive, central, or mixed and is likely multifactorial. A multidisciplinary approach to the management of these patients is important, including neurosurgery, otolaryngology, and sleep medicine. Future prospective studies will lend further insight into this condition and its management.

Keywords: Chiari I malformation; Decompression surgery; Sleep apnea.

MeSH terms

  • Arnold-Chiari Malformation* / complications
  • Arnold-Chiari Malformation* / epidemiology
  • Arnold-Chiari Malformation* / surgery
  • Child
  • Humans
  • Prospective Studies
  • Retrospective Studies
  • Sleep Apnea Syndromes* / diagnosis
  • Sleep Apnea Syndromes* / epidemiology
  • Sleep Apnea Syndromes* / surgery
  • Sleep Apnea, Central* / diagnosis
  • Sleep Apnea, Central* / epidemiology
  • Sleep Apnea, Central* / surgery
  • Sleep Apnea, Obstructive* / complications
  • Sleep Apnea, Obstructive* / epidemiology
  • Sleep Apnea, Obstructive* / surgery