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Decannulation in children affected by congenital central hypoventilation syndrome: A proposal of an algorithm from
Maria Giovanna Paglietti1, Federica Porcaro1, Aleksandar Sovtic2
1Pediatric Pulmonology & Respiratory Intermediate Care Unit, Sleep and Long-Term Ventilation Unit, Academic Department of Pediatrics, Bambino Gesù Children's Hospital, Rome, Italy.
Insights
Decannulation from invasive mechanical ventilation (IMV) is achievable for children with congenital central hypoventilation syndrome (CCHS). Transitioning to noninvasive ventilation (NIV) and decannulation can be successfully managed with a proposed algorithm.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
- Critical Care
Background:
- Congenital central hypoventilation syndrome (CCHS) necessitates long-term ventilatory support for survival in pediatric patients.
- Invasive mechanical ventilation (IMV) via tracheostomy is standard but carries risks like impaired speech and airway infections.
- Limited data exists on transitioning CCHS patients from IMV to noninvasive ventilation (NIV) and decannulation.
Purpose of the Study:
- To report the experience of a decannulation program in CCHS-affected children.
- To propose an algorithm for transitioning CCHS patients from IMV to NIV.
Main Methods:
- Retrospective study of four CCHS patients from two European centers undergoing tracheostomy removal.
- Pre-decannulation training included tracheostomy capping and endoscopic airway assessment.
- Initiation of NIV training at a mean age of 106.25 months.
Main Results:
- Decannulation was successfully achieved in all four patients 12 months post-training.
- No patient required reintroduction of the tracheal cannula during short or long-term follow-up.
- Endoscopic assessment confirmed upper and lower airway suitability for decannulation.
Conclusions:
- Effective liberation from IMV, transition to NIV, and decannulation are feasible in CCHS children.
- The study offers a practical algorithm for selected centers to manage decannulation in CCHS patients.
Rationale:
Long-life ventilatory assistance is necessary for survival in pediatric patients with congenital central hypoventilation syndrome (CCHS). Invasive mechanical ventilation (IMV) through tracheostomy is the most used method, especially in the first years of life when the central nervous system is maturing. Nevertheless, IMV via tracheostomy is not ideal because tracheostomy in children is associated with impaired speech and language development, also frequent infections of the lower airway tract occur.
Objective:
Only few reports describe the transition from IMV to the noninvasive method, ending with decannulation in CCHS affected patients. We aim to provide our experience regarding decannulation program in CCHS affected children and to describe a proposal of an algorithm concerning transition from invasive to noninvasive ventilation (NIV) in CCHS patients.
Methods:
The study has been conducted retrospectively. Four children from two European centers underwent tracheostomy removal and decannulation, upon request of patients and their families.
Results:
All children were trained to carry out tracheostomy capping before decannulation and underwent endoscopic assessment of upper and lower airway. Subsequently they started training to NIV at mean age of 106.25 months (±40.7 SD). Decannulation occurred 12 months after and no patients needed the reintroduction of tracheal cannula in either short or long term follow up.
Conclusions:
our study shows that effective liberation from IMV, the transition to NIV and decannulation are possible in CCHS affected children and offers a proposal of an algorithm which can be applied in selected centers.
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