{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T09:49:13Z","timestamp":1767260953292,"version":"3.37.3"},"reference-count":15,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2023,4,4]],"date-time":"2023-04-04T00:00:00Z","timestamp":1680566400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2023,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Rationale<\/jats:title><jats:p>Patients with congenital central hypoventilation syndrome (CCHS) require long\u2010term ventilation to ensure gas exchange and to prevent deleterious consequences for neurocognitive development. Two ventilation modes may be used for these patients depending on their tolerance, one invasive by tracheostomy and the other noninvasive (NIV). For patients who have undergone a tracheostomy, transition to NIV is possible when they meet predefined criteria. Identifying the conditions favorable for weaning from a tracheostomy is critical for the success of the process.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>The aim of the study was to share our experience of decannulation in a reference center; we hereby describe the modality of ventilation and its effect on nocturnal gas exchange before and after tracheostomy removal.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Retrospective observational study at Robert Debr\u00e9 Hospital over the past 10 years. The modalities of decannulation and transcutaneous carbon dioxide recordings or polysomnographies before and after decannulation were collected.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sixteen patients underwent decannulation following a specific procedure for transition from invasive to NIV. All decannulations were successful. The median age at decannulation was 12.6 [9.4; 14.1] years. Nocturnal gas exchange was not significantly different before and after decannulation, while expiratory positive airway pressure and inspiratory time increased significantly. An oronasal interface was chosen in two out of three patients. The median duration of hospital stay for decannulation was 4.0 [3.8; 6.0] days.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our study underlines that decannulation and transition to NIV are achievable in CCHS children using a well\u2010defined procedure. Patient preparation is crucial to the success of the process.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.26399","type":"journal-article","created":{"date-parts":[[2023,4,4]],"date-time":"2023-04-04T13:15:44Z","timestamp":1680614144000},"page":"1761-1767","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Decannulation in congenital central hypoventilation syndrome"],"prefix":"10.1002","volume":"58","author":[{"given":"Zina","family":"Ghelab","sequence":"first","affiliation":[{"name":"Division of Paediatric Otolaryngology, Robert Debr\u00e9 Hospital AP\u2010HP and University of Paris Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Plamen","family":"Bokov","sequence":"additional","affiliation":[{"name":"AP\u2010HP, H\u00f4pital Robert Debr\u00e9, Service de Physiologie P\u00e9diatrique\u2010Centre du Sommeil\u2014CRMR Maladies Respiratoires Rares\u2014Hypoventilations Alv\u00e9olaires Rares\u2014Syndrome d'Ondine, INSERM NeuroDiderot Universit\u00e9 de Paris\u2010Cit\u00e9 Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Natacha","family":"Teissier","sequence":"additional","affiliation":[{"name":"Division of Paediatric Otolaryngology, Robert Debr\u00e9 Hospital AP\u2010HP and University of Paris Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Delphine","family":"Micaelli","sequence":"additional","affiliation":[{"name":"Assistance Publique\u2010H\u00f4pitaux de Paris, Paediatric Intensive Care Unit, Robert Debr\u00e9 University Hospital Universit\u00e9 de Paris Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1366-8726","authenticated-orcid":false,"given":"Maxime","family":"Patout","sequence":"additional","affiliation":[{"name":"Service des Pathologies du Sommeil (D\u00e9partement R3S)\u2014CRMR Hypoventilations Centrales Cong\u00e9nitales, INSERM, UMRS1158 Neurophysiologie Respiratoire Exp\u00e9rimentale et Clinique, AP\u2010HP, H\u00f4pital de la Piti\u00e9\u2010Salp\u00eatri\u00e8re Sorbonne Universit\u00e9 Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aur\u00e9lie","family":"Hayotte","sequence":"additional","affiliation":[{"name":"Assistance Publique\u2010H\u00f4pitaux de Paris, Paediatric Intensive Care Unit, Robert Debr\u00e9 University Hospital Universit\u00e9 de Paris Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"St\u00e9phane","family":"Dauger","sequence":"additional","affiliation":[{"name":"Assistance Publique\u2010H\u00f4pitaux de Paris, Paediatric Intensive Care Unit, Robert Debr\u00e9 University Hospital Universit\u00e9 de Paris Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2786-0812","authenticated-orcid":false,"given":"Christophe","family":"Delclaux","sequence":"additional","affiliation":[{"name":"AP\u2010HP, H\u00f4pital Robert Debr\u00e9, Service de Physiologie P\u00e9diatrique\u2010Centre du Sommeil\u2014CRMR Maladies Respiratoires Rares\u2014Hypoventilations Alv\u00e9olaires Rares\u2014Syndrome d'Ondine, INSERM NeuroDiderot Universit\u00e9 de Paris\u2010Cit\u00e9 Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0827-616X","authenticated-orcid":false,"given":"Benjamin","family":"Dudoignon","sequence":"additional","affiliation":[{"name":"AP\u2010HP, H\u00f4pital Robert Debr\u00e9, Service de Physiologie P\u00e9diatrique\u2010Centre du Sommeil\u2014CRMR Maladies Respiratoires Rares\u2014Hypoventilations Alv\u00e9olaires Rares\u2014Syndrome d'Ondine, INSERM NeuroDiderot Universit\u00e9 de Paris\u2010Cit\u00e9 Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,4,4]]},"reference":[{"issue":"1","key":"e_1_2_9_2_1","first-page":"1","article-title":"Guidelines for diagnosis and management of congenital central hypoventilation syndrome","volume":"15","author":"Trang H","year":"2020","journal-title":"Orphanet J Rare Dis"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-020-01601-7"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.siny.2019.101037"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.25503"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOO.0000000000000204"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11325-021-02368-2"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24448"},{"issue":"10","key":"e_1_2_9_9_1","first-page":"729","article-title":"The technique of weaning from tracheostomy. 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