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Summary
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.
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Footnotes
Twitter Follow Martí Pons-Odena @Martí and Alba Verges @AVergés
Contributors MP-O conceived the case report and contributed to writing the manuscript. AV and NA were involved in acquisition of data and equally contributed to writing the manuscript. FJC contributed to writing the manuscript. All the authors read, reviewed and approved the final version of this case report.
Competing interests Although MP-O has been speaker for MAQUET and our institution received disposable material 6 years ago to start using this technique, we honestly consider that we are not promoting an specific product in order to favour commercial interests of the company. So, we declare this potential conflict of interest, nothing to hide, but we believe there is no a real conflict of the interest of the authors.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.