BI-LEVEL POSITIVE AIRWAY PRESSURE (BIPAP) VENTILATION IN AN INFANT WITH CENTRAL HYPOVENTILATION SYNDROME

A 4-month-old baby girl, after a period of apparent good health, began to have aphonia, dyspnea, difficulties with swallowing, cyanosis, apn ea, and hypopnea during sleep that resulted in admission to an intensi ve care unit for intubation and mechanical ventilation. At the age of 9 months she was admitted to our hospital with a possible diagnosis of central hypoventilation syndrome. A polysomnographic study showed apn ea and hypopnea (apnea + hypopnea index = 47.1), hypercapnia (mean end -tidal Pco(2) 89 +/- 15.0 mmHg), and arterial desaturation (mean Sao(2 ) 91 +/- 1.7%; lowest Sao(2) < 50%; 68% of total sleep time at Sao(2) below 93%); the study also showed an absent ventilatory response to CO 2, absent cardiac responses to apnea during sleep, and right ventricul ar hypertrophy. Nocturnal nasal bi-level positive airway pressure (BiP AP), applied initially at 6 cmH(2)O and gradually increased to 16 cmH( 2)O, caused the sleep-related abnormal respiratory events to disappear . End-tidal Pco(2) decreased to 39 mmHg, and Sao(2) increased to 94%. After 6 months of nocturnal BiPAP ventricular right hypertrophy revers ed and arrested growth and hypotonia normalized. The child has tolerat ed and has remained on BiPAP support up to her current age of 3 years and continues to use this form of ventilatory assistance without diffi culties. (C) 1997 Wiley-Liss, Inc.