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Airway Obstruction during Sleep due to Diaphragm Pacing Precludes Decannulation in Young Children with CCHS

Taher Valika1,2, Anthony C Chin3,4, Dana M Thompson5,4

  • 1Division of Otolaryngology-Head and Neck Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, USA, tvalika@luriechildrens.org.

Respiration; International Review of Thoracic Diseases
|July 10, 2019
PubMed

Insights

Children with CCHS may experience airway obstruction during sleep with diaphragm pacing (DP) after tracheal decannulation. Most patients require noninvasive mask ventilation for breathing support, highlighting the need for caution.

Area of Science:

  • Pediatric Pulmonology
  • Neuromuscular Disorders
  • Sleep Medicine

Background:

  • Congenital central hypoventilation syndrome (CCHS) results from PHOX2B mutations, causing breathing deficits.
  • Artificial ventilation is essential for life support in CCHS patients.
  • Phrenic nerve-diaphragm pacing (DP) is explored for tracheal decannulation in CCHS, but data in young children is limited.

Observation:

  • A case series of 3 children (ages 3.3-4.3 years) with CCHS who underwent tracheal decannulation while using DP for sleep support.
  • Sleep endoscopy revealed complete airway obstruction at various levels (oropharyngeal, supraglottic, glottic) during DP.
  • Inadequate gas exchange was confirmed by end-tidal CO2 and SpO2 monitoring.

Findings:

  • Despite DP adjustments, significant airway obstruction persisted during sleep in decannulated CCHS children.
  • All 3 patients required noninvasive mask ventilation for life support during sleep due to failed gas exchange with DP alone.
  • Families opted against re-tracheostomy, necessitating ongoing noninvasive ventilation.

Implications:

  • Extreme caution is advised for tracheal decannulation in young CCHS patients relying on DP for sleep.
  • Noninvasive mask ventilation is often necessary for life support post-decannulation, rather than solely DP.
  • This study highlights the need to manage parental and patient expectations regarding ventilation support after decannulation in CCHS.

Keywords:
Congenital central hypoventilation syndromeDecannulationDiaphragm pacingTracheostomyUpper airway obstruction

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