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

Research output: Contribution to journalArticle

Abstract

Children with congenital central hypoventilation syndrome (CCHS) have a PHOX2B mutation-induced control of breathing deficit necessitating artificial ventilation as life support. A subset of CCHS families seek phrenic nerve-diaphragm pacing (DP) during sleep with the goal of tracheal decannulation. Published data regarding DP during sleep as life support in the decannulated child with CCHS and related airway dynamics in young children are limited. We report a series of 3 children, ages 3.3-4.3 years, who underwent decannulation. Sleep endoscopy performed during DP revealed varied (oropharynx, supraglottic, glottic, etc.) levels of complete airway obstruction despite modification of pacer settings. Real-time analysis of end tidal CO2 and SpO2 confirmed inadequate gas exchange. Because the families declined re-tracheostomy, all 3 patients rely on noninvasive mask ventilation as a means of life support while asleep. These results emphasize the need for extreme caution in proceeding with tracheal decannulation in young children with CCHS who expect to use DP during sleep as life support. Parents and patients should anticipate that they will depend on noninvasive mask ventilation (rather than DP) during sleep after undergoing decannulation. This information may improve management and guide expectations regarding potential decannulation in young paced children with CCHS.

Original languageEnglish (US)
Pages (from-to)263-267
Number of pages5
JournalRespiration
Volume98
Issue number3
DOIs
StatePublished - Sep 1 2019

Fingerprint

Airway Obstruction
Diaphragm
Sleep
Noninvasive Ventilation
Masks
Phrenic Nerve
Oropharynx
Tracheostomy
Tongue
Endoscopy
Congenital central hypoventilation syndrome
Respiration
Gases
Parents
Mutation

Keywords

  • Congenital central hypoventilation syndrome
  • Decannulation
  • Diaphragm pacing
  • Tracheostomy
  • Upper airway obstruction

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

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title = "Airway Obstruction during Sleep due to Diaphragm Pacing Precludes Decannulation in Young Children with CCHS",
abstract = "Children with congenital central hypoventilation syndrome (CCHS) have a PHOX2B mutation-induced control of breathing deficit necessitating artificial ventilation as life support. A subset of CCHS families seek phrenic nerve-diaphragm pacing (DP) during sleep with the goal of tracheal decannulation. Published data regarding DP during sleep as life support in the decannulated child with CCHS and related airway dynamics in young children are limited. We report a series of 3 children, ages 3.3-4.3 years, who underwent decannulation. Sleep endoscopy performed during DP revealed varied (oropharynx, supraglottic, glottic, etc.) levels of complete airway obstruction despite modification of pacer settings. Real-time analysis of end tidal CO2 and SpO2 confirmed inadequate gas exchange. Because the families declined re-tracheostomy, all 3 patients rely on noninvasive mask ventilation as a means of life support while asleep. These results emphasize the need for extreme caution in proceeding with tracheal decannulation in young children with CCHS who expect to use DP during sleep as life support. Parents and patients should anticipate that they will depend on noninvasive mask ventilation (rather than DP) during sleep after undergoing decannulation. This information may improve management and guide expectations regarding potential decannulation in young paced children with CCHS.",
keywords = "Congenital central hypoventilation syndrome, Decannulation, Diaphragm pacing, Tracheostomy, Upper airway obstruction",
author = "Taher Valika and Chin, {Anthony C} and Thompson, {Dana M} and Rashmi Kabre and Lavin, {Jennifer M} and Neault, {Sarah H.} and Ballard, {Heather A} and Kenny, {Anna S.} and Weese-Mayer, {Debra E}",
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T1 - Airway Obstruction during Sleep due to Diaphragm Pacing Precludes Decannulation in Young Children with CCHS

AU - Valika, Taher

AU - Chin, Anthony C

AU - Thompson, Dana M

AU - Kabre, Rashmi

AU - Lavin, Jennifer M

AU - Neault, Sarah H.

AU - Ballard, Heather A

AU - Kenny, Anna S.

AU - Weese-Mayer, Debra E

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N2 - Children with congenital central hypoventilation syndrome (CCHS) have a PHOX2B mutation-induced control of breathing deficit necessitating artificial ventilation as life support. A subset of CCHS families seek phrenic nerve-diaphragm pacing (DP) during sleep with the goal of tracheal decannulation. Published data regarding DP during sleep as life support in the decannulated child with CCHS and related airway dynamics in young children are limited. We report a series of 3 children, ages 3.3-4.3 years, who underwent decannulation. Sleep endoscopy performed during DP revealed varied (oropharynx, supraglottic, glottic, etc.) levels of complete airway obstruction despite modification of pacer settings. Real-time analysis of end tidal CO2 and SpO2 confirmed inadequate gas exchange. Because the families declined re-tracheostomy, all 3 patients rely on noninvasive mask ventilation as a means of life support while asleep. These results emphasize the need for extreme caution in proceeding with tracheal decannulation in young children with CCHS who expect to use DP during sleep as life support. Parents and patients should anticipate that they will depend on noninvasive mask ventilation (rather than DP) during sleep after undergoing decannulation. This information may improve management and guide expectations regarding potential decannulation in young paced children with CCHS.

AB - Children with congenital central hypoventilation syndrome (CCHS) have a PHOX2B mutation-induced control of breathing deficit necessitating artificial ventilation as life support. A subset of CCHS families seek phrenic nerve-diaphragm pacing (DP) during sleep with the goal of tracheal decannulation. Published data regarding DP during sleep as life support in the decannulated child with CCHS and related airway dynamics in young children are limited. We report a series of 3 children, ages 3.3-4.3 years, who underwent decannulation. Sleep endoscopy performed during DP revealed varied (oropharynx, supraglottic, glottic, etc.) levels of complete airway obstruction despite modification of pacer settings. Real-time analysis of end tidal CO2 and SpO2 confirmed inadequate gas exchange. Because the families declined re-tracheostomy, all 3 patients rely on noninvasive mask ventilation as a means of life support while asleep. These results emphasize the need for extreme caution in proceeding with tracheal decannulation in young children with CCHS who expect to use DP during sleep as life support. Parents and patients should anticipate that they will depend on noninvasive mask ventilation (rather than DP) during sleep after undergoing decannulation. This information may improve management and guide expectations regarding potential decannulation in young paced children with CCHS.

KW - Congenital central hypoventilation syndrome

KW - Decannulation

KW - Diaphragm pacing

KW - Tracheostomy

KW - Upper airway obstruction

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