Decannulation in congenital central hypoventilation syndrome

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 is 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 [9.4; 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. 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.

Congenital central hypoventilation syndrome: Heterogeneous clinical presentation, ventilatory modalities and outcome

“…Despite being the first option in early-onset disease with unequivocal efficacy, IMV imposed serious concerns about speech and psychosocial development (21). Additional possible complications associated with tracheostomy tube (infections, bleeding, frequent admissions for tracheostomy tube replacement) coupled with a better understanding of the nature of the disease led to the necessity of introducing NIV and broadening indications for it (15,20).…”

“…The age of the child remains an important factor of ventilatory modality, particularly when it comes to decannulation. Namely, after the failure of an early transition from invasive ventilation to NIV had been reported, some authors suggested the period from six years to adolescence as the prime time for decannulation and introduction of NIV (15,20,25,26). Still, meticulous patient recruitment can render NIV possible after removing the tracheostomy tube, even at an early age (27).…”

“…While chronic invasive mechanical ventilation (IMV) represents a traditional treatment modality, the importance of noninvasive ventilation (NIV) has been popularized and highlighted in recent years in selected patients, e.g., after decannulation or in those with late-onset disease (14-16). With better overall medical care directed at each aspect of the disease, the field of indications for NIV has become wider in the past decade (14)(15)(16).…”