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Tracheostomy decannulation to noninvasive positive pressure ventilation in congenital central hypoventilation syndrome
- Source :
- Sleep and Breathing. 26:133-139
- Publication Year :
- 2021
- Publisher :
- Springer Science and Business Media LLC, 2021.
-
Abstract
- Noninvasive positive pressure ventilation (NPPV) may permit tracheostomy decannulation (TD) in patients with congenital central hypoventilation syndrome (CCHS) requiring nocturnal positive pressure ventilation via tracheostomy (PPV-T). There is limited evidence on optimal strategies for transitioning patients from PPV-T to NPPV. This study aimed to describe the clinical course and outcome of children with CCHS who underwent TD and transitioned from PPV-T to NPPV. Retrospective review was conducted on patients with CCHS using nocturnal PPV-T who underwent TD to NPPV. The results of clinical evaluations, airway endoscopy, polysomnography, and clinical course leading to TD were analyzed. We identified 3 patients with CCHS aged 8–17 years who required PPV-T only during sleep. Patients underwent systematic multidisciplinary evaluations with a pediatric psychologist, pulmonologist, sleep physician, and otolaryngologist utilizing a TD algorithm. These included evaluation in the sleep clinic, NPPV mask fitting and desensitization, endoscopic airway evaluation, daytime tracheostomy capping, acclimatization to low-pressure NPPV, polysomnography with capped tracheostomy and NPPV titration, and if successful, TD. All patients underwent successful TD following optimal titration of NPPV during polysomnography. The duration to TD from decision to pursue NPPV was between 2.4 and 10.6 months, and the duration of hospitalization for TD was between 4 and 5 days. There were no NPPV-related complications; however, all patients required surgical closure of tracheocutaneous fistula. NPPV may be an effective and feasible option for patients with CCHS requiring PPV-T during sleep and permits TD. In patients with CCHS, a systematic multidisciplinary algorithm may optimize successful transition to NPPV and TD.
- Subjects :
- medicine.medical_specialty
Neurology
medicine.diagnostic_test
business.industry
Clinical course
Polysomnography
Congenital central hypoventilation syndrome
medicine.disease
Endoscopy
03 medical and health sciences
0302 clinical medicine
030228 respiratory system
Otorhinolaryngology
Anesthesia
medicine
Neurology (clinical)
Airway
Positive pressure ventilation
business
030217 neurology & neurosurgery
Subjects
Details
- ISSN :
- 15221709 and 15209512
- Volume :
- 26
- Database :
- OpenAIRE
- Journal :
- Sleep and Breathing
- Accession number :
- edsair.doi...........2135eb712eaf237f921baf7b993062ad