Impact of sleep‐disordered breathing on the management of children with Chiari malformation type I. Issue 12 (6th September 2022)

• Record Type: Journal Article
• Authors: Vagianou, Foteini; Khirani, Sonia; De Saint Denis, Timothée; Beccaria, Kevin; Amaddeo, Alessandro; Breton, Sylvain; James, Syril; Paternoster, Giovanna; Arnaud, Eric; Zerah, Michel; Fauroux, Brigitte
• Is Part Of: Pediatric pulmonology. Volume 57:Issue 12(2022)
• Page Start: 2954
• Page End: 2962
• Publication Date: 2022-09-06
• Subjects: central sleep apnea -- Chiari malformation -- craniosynostosis -- poly(somno)graphy -- polymalformative syndromes; Pediatric respiratory diseases -- Periodicals; Pediatrics -- Periodicals; 618.922
• DOI: 10.1002/ppul.26113
• Languages: English
• ISSNs: 8755-6863

Abstract

Abstract: Objectives: Children with Chiari malformation type I (CM‐I) have an increased risk of sleep apnea. The aim of the study was to describe the management of CM‐I patients in relation to symptoms, magnetic resonance imaging (MRI) findings and sleep apnea syndrome (SAS). Methods: We performed a retrospective analysis of clinical charts of all 57 CM‐I patients seen between September 2013 and April 2017. Results: A total of 45 patients had isolated CM‐I or associated co‐morbidity (CM‐Iia), 5 had craniosynostosis (CM‐Ics), and 7 a polymalformative syndrome (CM‐Ipm). The prevalence of SAS, defined as an apnea‐hypopnea index >1 event/h, was high in CM‐I ranging from 50% to 80% according to the CM‐I group. The prevalence of central sleep apnea (CSA) was low, with 5 (9%) patients having CSA and only 3 patients with CM‐Iia having isolated CSA. A total of 17 patients (30%) had foramen magnum decompression (FMD). Neither positive symptoms of CM‐I nor MRI findings alone, nor both combined were good indicators for FMD. No correlation was observed between the cerebellar tonsil descent and SAS in CM‐I. But all 5 patients with CSA had a FMD. The combination of MRI findings and/or symptoms of CM‐I together with moderate‐to‐severe SAS best discriminated patients who needed a FMD. Conclusion: Our findings highlight the importance of a combined evaluation of symptoms, MRI and polygraphy results in the management of CM‐I patients.