The Science Journal of the American Association for Respiratory Care

2007 OPEN FORUM Abstracts

MASK VENTILATION IN THE EARLY MANAGEMENT OF CONGENITAL CENTRAL HYPOVENTILATION SYNDROME

P. Ramesh1, P. Boit2, M. Samuels3

Background: Congenital central hypoventilation syndrome (CCHS) is a rare genetic disorder of breathing due to abnormal autonomic control, which usually needs life long ventilatory support usually during sleep. Traditionally, infants receive positive pressure ventilation (PPV) via tracheostomy, with a majority being decannulated and transferred to non invasive PPV via face or nasal mask in later childhood. In the last few years, we have initiated non invasive ventilation via face/nasal mask from early infancy, thus avoiding the need for tracheostomy and the associated complications. This study aimed to review the introduction of mask ventilation in early infancy and to evaluate patient/parent preferences.

Methods: Periodic clinical and sleep study examination, case note review and prospective questionnaire to the patients/parents under follow up.

Results: Fifteen patients (10 girls) were identified with a median age 13 years (range 9 months – 21 years). Nine patients had received traditional ventilatory methods with either IPPV via tracheostomy or negative pressure ventilation before being weaned on to mask ventilation at a median age of 8 years (range 1.5 – 11 years). Six patients were established on mask ventilation in early infancy (median 8 weeks, range 5 - 26 weeks) and avoided or minimised the duration of tracheostomy. Mask ventilation provided effective ventilation in all six patients without major complications. Mid-face abnormalities were noted, but no patient needed maxillo-facial intervention. All the nine patients (and their parents) who had received ventilatory support via tracheostomy or negative pressure ventilation prior to the initiation of mask ventilation preferred mask ventilation and considered it a superior method of ventilatory support.

Conclusions: Mask ventilation can be safely commenced at an early age in children affected with CCHS. With appropriate management, mid-face problems, known to be a feature of the condition with all modes of ventilation, can be minimised. It is not only effective but is the preferred mode of ventilatory support by parents and patients.

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