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Decannulation in congenital central hypoventilation syndrome
  • +6
  • Benjamin Dudoignon,
  • Zina Ghelab,
  • Plamen Bokov,
  • Natacha Teissier,
  • Delphine Micaelli,
  • Maxime Patout,
  • Aurelie Hayotte,
  • Stéphane Dauger,
  • Christophe Delclaux
Benjamin Dudoignon
Assistance Publique - Hopitaux de Paris

Corresponding Author:[email protected]

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Zina Ghelab
Assistance Publique - Hopitaux de Paris
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Plamen Bokov
Assistance Publique - Hopitaux de Paris
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Natacha Teissier
Assistance Publique - Hopitaux de Paris
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Delphine Micaelli
Assistance Publique - Hopitaux de Paris
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Maxime Patout
Hopital Universitaire Pitie Salpetriere Service des Pathologies du Sommeil
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Aurelie Hayotte
Assistance Publique - Hopitaux de Paris
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Stéphane Dauger
Assistance Publique - Hopitaux de Paris
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Christophe Delclaux
Assistance Publique - Hopitaux de Paris
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Abstract

Rationale: Patients with congenital central hypoventilation syndrome (CCHS) require long-term 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 it critical for the success of the process. Objective: 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. Methods: Retrospective observational study at Robert Debré Hospital over the past 10 years. The modalities of decannulation and transcutaneous carbon dioxide recordings or polysomnographies before and after decannulation were collected. Results: 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 [9.7; 15.0] 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 mean duration of hospital stay for decannulation was 4.0 [3.0; 6.0] days. Conclusion: Our study underlines that decannulation and transition to NIV are achievable in CCHS children using a well-defined procedure. Patient preparation is crucial to the success of the process.
01 Dec 2022Submitted to Pediatric Pulmonology
01 Dec 2022Submission Checks Completed
01 Dec 2022Review(s) Completed, Editorial Evaluation Pending
01 Dec 2022Assigned to Editor
06 Dec 2022Reviewer(s) Assigned
07 Jan 2023Editorial Decision: Revise Major
07 Feb 20231st Revision Received
07 Feb 2023Submission Checks Completed
07 Feb 2023Review(s) Completed, Editorial Evaluation Pending
07 Feb 2023Reviewer(s) Assigned
07 Feb 2023Assigned to Editor
08 Mar 2023Editorial Decision: Revise Minor
10 Mar 20232nd Revision Received
10 Mar 2023Assigned to Editor
10 Mar 2023Submission Checks Completed
10 Mar 2023Review(s) Completed, Editorial Evaluation Pending
10 Mar 2023Reviewer(s) Assigned
16 Mar 2023Editorial Decision: Accept