Diaphragm Pacing without Tracheostomy in Congenital Central Hypoventilation Syndrome Patients.

Abstract

BACKGROUND:

Congenital central hypoventilation syndrome (CCHS) is a rare disorder affecting central control of breathing. Thus, patients require lifelong assisted ventilation. Diaphragm pacing (DP) may permit decannulation in those who are ventilator dependent only during sleep.

OBJECTIVE:

The purpose of this study is to determine if patients with CCHS can be successfully ventilated by DP without tracheostomy.

METHODS:

We reviewed the records of 18 CCHS patients (mean age 19.5 ± 10.1 years; 44% female) who were ventilated by DP only during sleep.

RESULTS:

Prior to diaphragm pacer implantation surgery, 14 CCHS patients had been using home portable positive pressure ventilation (PPV) via tracheostomy, 1 had been on PPV via endotracheal tube, and 3 had been using noninvasive PPV (NPPV). Of the patients with tracheostomy prior to DP (n = 15), 11 (73%) were decannulated and ventilated successfully by DP without tracheostomy. Of all the patients reviewed (n = 18), 13 (72%) were successfully ventilated by DP without tracheostomy. Obesity prevented successful DP without tracheostomy in 1 patient, and upper airway obstruction prevented success in another patient. Snoring and/or obstructive apneas were present in some patients, but they were improved by diaphragm pacer changes, adenotonsillectomy, and/or use of nasal steroids.

CONCLUSIONS:

DP without tracheostomy can be successfully achieved in patients with CCHS. Snoring and obstructive apneas, when present, can be managed by diaphragm pacer changes and medical therapies. Obesity can pose a challenge to successful DP.

• Authors: Bonnie Diep, Annie Wang, Sheila Kun, J Gordon McComb, Donald B Shaul, Cathy E Shin, Thomas G Keens, Iris A Perez
• Journal: Respiration; international review of thoracic diseases (Respiration) Vol. 89 Issue 6 Pg. 534-8 ( 2015) ISSN: 1423-0356 [Electronic] Switzerland
• PMID: 25924848 (Publication Type: Clinical Study, Journal Article)
• Copyright: © 2015 S. Karger AG, Basel.

Topics

• Adolescent
• Adult
• Child
• Cohort Studies
• Diaphragm
• Electric Stimulation Therapy (methods)
• Female
• Humans
• Hypoventilation (complications, congenital, therapy)
• Male
• Noninvasive Ventilation
• Obesity (complications)
• Positive-Pressure Respiration
• Retrospective Studies
• Sleep Apnea, Central (complications, therapy)
• Sleep Apnea, Obstructive (complications)
• Tracheostomy
• Treatment Outcome
• Young Adult