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.

Pediatric Pulmonology
|July 18, 2019
PubMed

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.
Abstract

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