TECHNICAL OBSERVATION Early use of Nasal-BiPAP in two infants with Congenital Central Hypoventilation syndrome C Migliori, A Cavazza, M Motta, R Bottino and G Chirico Division of Neonatology and Neonatal Intensive Care, Spedali Civili, Brescia, Italy Migliori C, Cavazza A, Motta M, Bottino R, Chirico G. Early use of Nasal-BiPAP in two infants with Congenital Central Hypoventilation syndrome. Acta Pædiatr 2003; 92: 823–826. Stockholm. ISSN 0803-5253 Aim: To reduce the problems caused by prolonged artificial ventilation in babies with Congenital Central Hypoventilation syndrome (CCHS). Methods: Two term infants with CCHS, weighing 4030 g and 3100 g, respectively, at the beginning of treatment and aged 53 and 31 d, respectively, were successfully ventilated with a Nasal Bilevel Positive Airway Pressure (N-BiPAP) device. Results: In the first patient the tcPO 2 recordings (mean  SD) during sleep were 46  12 mmHg before using N-BiPAP and 58  13 mmHg after using the device, while those for tcPCO 2 were 75  9 mmHg and 49  11 mmHg, respectively. In the second patient tcPO 2 during sleep was 42  3 mmHg before, and 55  5 after N-BiPAP, and for tcPCO 2 the recordings were 119  24 mmHg and 55  6 mmHg, respectively, showing a significant improvement. One infant had persistent gastro-oesophageal reflux, and frontal skin abrasion caused by the face mask. Never- theless, these complications did not necessitate the discontinuation of N-BiPAP ventilation, thus precluding prolonged use of intubation and tracheotomy. Conclusion: In infants with CCHS, early use of non-invasive, positive-pressure ventilation with N-BiPAP, in association with careful monitoring, can decrease problems caused by prolonged intubation and tracheotomy. Key words: BiPAP, Congenital Central Hypoventilation syndrome, infant, Ondine’s curse Claudio Migliori, Division of Neonatology and Neonatal Intensive Care, Spedali Civili, p. le Spedali Civili, IT-25123 Brescia, Italy (Tel. 39 030 3995 219, fax. 39 030 3700 817, e-mail. claudio.migliori@tin.it) Nasal Bilevel Positive Airway Pressure (N-BiPAP) is a non-invasive ventilation method used in adults (1) and children (2) for the treatment of respiratory failure. Air and oxygen are continuously sent to the upper respira- tory tract at two different levels of positive pressure, via a triangular face mask. The survival of subjects with Congenital Central Hypoventilation syndrome (CCHS), a rare idiopathic disease characterized by alveolar hypoventilation dur- ing sleep and adequate ventilation while the patient is awake, depends on ventilatory assistance with intuba- tion and tracheotomy. In these patients, BiPAP has improved quality of life by reducing the need for intubation and the associated complications (3). How- ever, only a small number of studies have been reported on the use of N-BiPAP in newborns and infants (4–6). We successfully used this ventilation method in two infants with CCHS, starting during the second month of life. Patients and methods Patient 1 A male, born by caesarean section at 38 wk of gestation, with a birthweight of 3580 g. Apgar scores were 9 at 1 and 5 min; clinical conditions were normal. On the second day of life, the patient was transferred to our Neonatal Intensive Care Unit (NICU) because of cyanosis and respiratory distress syndrome. After a short period of nasal continuous positive airways pressure (N-CPAP), the baby required intubation and artificial ventilation. Repeated attempts to wean the infant from mechanical ventilation failed because of episodes of hypercapnia (PaCO 2 > 90 mmHg) and hypoxia (PaO 2 < 50 mmHg) during sleep. Routine clinical investigations and brain magnetic resonance imaging (MRI) were normal. The polysomnography showed apnoea spells, with normal ECG and EEG tracings, allowing us to diagnose CCHS. On the 53rd day of life, when the patient’s weight was 4030 g, we started non-invasive ventilation with N-BiPAP. During the first 3 d, values of continuous tcPO 2 -tcPCO 2  2003 Taylor & Francis. ISSN 0803-5253 Acta Pñdiatr 92: 823±826. 2003 DOI 10.1080/08035250310002849