Impact of sleep‐disordered breathing on the management of children with Chiari malformation type I

• Published in: Pediatric pulmonology Vol. 57; no. 12; pp. 2954 - 2962
• Main 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
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01.12.2022
• Subjects: Arnold-Chiari Malformation - complications; Arnold-Chiari Malformation - diagnostic imaging; Arnold-Chiari Malformation - epidemiology; central sleep apnea; Chiari malformation; Child; craniosynostosis; Humans; Magnetic resonance imaging; Magnetic Resonance Imaging - methods; poly(somno)graphy; polymalformative syndromes; Polysomnography - adverse effects; Retrospective Studies; Sleep apnea; Sleep Apnea Syndromes - complications; Sleep Apnea Syndromes - epidemiology; Sleep Apnea Syndromes - therapy; Sleep Apnea, Central - complications; Sleep Apnea, Central - epidemiology; Sleep Apnea, Central - therapy; craniosynostosis; polymalformative syndromes; poly(somno)graphy; central sleep apnea; Chiari malformation
• ISSN: 8755-6863; 1099-0496; 1099-0496
• DOI: 10.1002/ppul.26113

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.