title: Combined use of Neurally Adjusted Ventilatory Assist (NAVA) and Vertical Expandable Prostethic Titanium Rib (VEPTR) in a patient with Spondylocostal dysostosis and associated bronchomalacia.
abstract: Jarcho-Levin syndrome is a rare disorder characterised by defects in vertebral and costal segmentation of varying severity. Respiratory complications are the main cause of death or severe comorbidity due to a restrictive rib cage. A 3 months old infant with Spondylocostal dysostosis and associated bronchomalacia experiencing severe asynchrony during the weaning process is reported. The Neurally Adjusted Ventilatory Assist (NAVA) mode was used to improve adaptation to mechanical ventilation after Vertical Expandable Prosthetic Titanium Ribs (VEPTRs) were implanted. The synchrony achieved with the NAVA mode allowed a decrease of the sedoanalgesia he received. A follow-up CT scan showed a reduction in the volume of the posterobasal atelectasis. The evolution of this patient suggests that the combined use of VEPTR for thoracic expansion and ventilation using NAVA can favour the global improvement. This mode could be an option to consider in selected patients with difficult weaning from mechanical ventilation in paediatric intensive care units.
authors.affiliation: Pediatric Intensive Care Unit, Hospital Sant Joan de Deu, Esplugues De Llobregat, Spain.
authors.forename: Martí
authors.initials: M
authors.lastname: Pons-Odena
authors.affiliation: Pediatric Intensive Care Unit, Hospital Sant Joan de Deu, Esplugues De Llobregat, Spain.
authors.forename: Alba
authors.initials: A
authors.lastname: Verges
authors.affiliation: Pediatric Intensive Care Unit, Hospital Sant Joan de Deu, Esplugues De Llobregat, Spain.
authors.forename: Natalia
authors.initials: N
authors.lastname: Arza
authors.affiliation: Pediatric Intensive Care Unit, Hospital Sant Joan de Deu, Esplugues De Llobregat, Spain.
authors.forename: Francisco José
authors.initials: FJ
authors.lastname: Cambra
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery*; D055091:Bronchomalacia / Q000150:complications / Q000601:surgery*; D006548:Hernia, Diaphragmatic / Q000150:complications / Q000601:surgery*; D006801:Humans; D007223:Infant; D059725:Interactive Ventilatory Support; D008297:Male; D019736:Prostheses and Implants; D012272:Ribs; D014025:Titanium
chemical_list: D014025:Titanium