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Congenital central hypoventilation syndrome A report of successful experience with bilateral diaphragmatic pacing

Abstract:

Bilateral diaphragmatic pacing was successfully performed in an 18-month-old child with congenital central hypoventilation syndrome (Ondine's curse) as an alternative to long-term mechanical ventilation. Subsequent complications were related more to cor pulmonale and tracheostomy care than to the pacing itself. Congenital central hypoventilation syndrome (CCHS), often referred to as' Ondine's curse', is a rare disorder of ventilatory control, characterised by hypoventilation during sleep. Patients with this disorder require long-term mechanical ventilation, and most eventually develop fatal cor pulmonale. Few paediatric cases have been reported. Hunt et al. 1 reported the successful treatment of 3 infants with this condition, using radiofrequency diaphragmatic pacing. This report describes another successful experience using bilateral diaphragmatic pacing in the treatment of an 18-month-old child with CCHS.