Early use of Nasal‐BiPAP in two infants with Congenital Central Hypoventilation syndrome

Abstract

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 tcPO2 recordings (mean ± SD) during sleep were 46 ± 12 mmHg before using N‐BiPAP and 58 ± 13 mmHg after using the device, while those for tcPCO2 were 75 ± 9 mmHg and 49 ±11 mmHg, respectively. In the second patient tcPO2 during sleep was 42 ± 3 mmHg before, and 55 ± 5 after N‐BiPAP, and for tcPCO2 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. Nevertheless, 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.

Nasal bi‐level positive airway pressure (BiPAP) versus nasal continuous positive airway pressure (CPAP) in preterm infants ≤32 weeks: A retrospective cohort study

“…BiPAP is a ventilator support that assists breathing alternating two level of positive pressure (peak inspiratory pressure (PIP) and positive end expiratory pressure (PEEP)) at preset intervals: T high (duration on PIP) and T low (duration on PEEP) administered through face mask or nasal prongs 8 . This technique is different from NIPPV as it does not use superimposed ventilator breath 9 . A randomised controlled trial showed that early extubation to NIPPV or BiPAP reduced the need for longer mechanical ventilation after administration at delivery 10 .…”

Congenital central hypoventilation syndrome; Safety of early transition to non-invasive ventilation

“…Establishing BiPAP has been done in infants with CCHS as young as one month of age. 7 Previous reports have stated that transition is typically not successful if the child is <6-7 years of age, 4,8 although there have been reports of success in children as young as 3 years. [9][10][11] Our patient was 3 years old at the time of transition.…”

“…Use of BiPAP comes with its own set of complications, including associated rhinitis, laryngitis, and gastroenteral reflux. 7 Chronic use of nasal mask ventilation may lead to skin breakdown, pressure-induced midface hypoplasia and dental malocclusion. 10 This can be avoided by altering nasal masks and taking regular photographs to monitor impairment of facial growth.…”

Cardiovascular Abnormalities and Arrhythmias in Patients with Ondine's Curse (Congenital Central Hypoventilation) Syndrome

“…Few patients have late onset of the disease. Noninvasive ventilation has been successful in some patients 12–14 . The course of this disease is variable.…”