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- Title
Strategy of changing from tracheostomy and non‐invasive mechanical ventilation to diaphragm pacing in children with congenital central hypoventilation syndrome.
- Authors
Tsolakis, Nikolaos; Sindelar, Richard; Markström, Agneta; Nilsson, Pelle; Jonzon, Anders
- Abstract
In our experience, younger patients accept DP better and older patients need a longer adaptation period. We retrospectively studied 23 patients with central hypoventilation conditions who underwent DP implantation at the Hospital between 1 January 1980 and 31 December 2020. Abbreviations CCHS congenital central hypoventilation syndrome DP diaphragm pacing NIV non-invasive ventilation PAR polyalanine repeat expansion mutation Congenital central hypoventilation syndrome (CCHS) is a rare disorder that affects central control of breathing, and paediatric treatment varies worldwide.1 One approach is diaphragm pacing (DP), by phrenic nerve stimulation or direct diaphragm muscle stimulation, with or without a tracheostomy.2 In Sweden, non-invasive ventilation (NIV) has been the first-line ventilator support for patients with CCHS.3 However, disadvantages such as midface hypoplasia and unintentional leakage have required assessment over time.4 Diaphragm pacing implants are provided at the National Reference Center for Diaphragm Pacing at Uppsala University Hospital, Sweden, at 3-4 years of age, when the upper airways have become more stable. Strategy of changing from tracheostomy and non-invasive mechanical ventilation to diaphragm pacing in children with congenital central hypoventilation syndrome.
- Subjects
HYPOVENTILATION; ARTIFICIAL respiration; NONINVASIVE ventilation; TRACHEOTOMY; DIAPHRAGM (Anatomy)
- Publication
Acta Paediatrica, 2022, Vol 111, Issue 6, p1245
- ISSN
0803-5253
- Publication type
Article
- DOI
10.1111/apa.16256