428 The use of non-invasive mechanical ventilation in the patient with deformity of the thoracic spine – case report

Abstract

<h3>Introduction</h3> Non-invasive ventilation (NIV) refers to the delivery of mechanical ventilation, without the need for an invasive artificial airway (endotracheal tube or tracheostomy tube). There are different types of interfaces available for use with NIV including: total face mask, oronasal mask, nasal mask, and nasal pillows. The use of NIV has been shown to be an effective in patients with acute or chronic respiratory failure. Scoliosis is a complex three dimensional deformity that leads to impaired chest wall mechanics that prevent normal inflation of the lungs resulting in decreased total lung capacity. Rotation of the chest can produce displacement of the intrathoracic structures and compression of the main bronchi, causing upper airway obstruction. As the disease progresses, due to chronic airway inflammation and and secretion retention, small airways also became obstructed. Over time, ventilation-to-perfusion mismatch leads to chronic respiratory failure. In this case, we emphasize the importance of NIV in the patient with chronic respiratory failure caused by thoracic spine deformities. <h3>Case Presentation</h3> A 17-year-old girl has been multidisciplinary followed at our hospital since early childhood due to progressive epileptic encephalopathy that led to severe cognitive impairment. The patient does not have major motor deficits and can walk or move unassisted. Due to recurrent respiratory infections associated with bronchial obstruction, at the age of 14 she underwent diagnostic testing at The Pulmonology, and Allergology division. Allergy tests were negative, and since the patient was uncooperative, pulmonary function tests were not performed. Chest radiograph showed significant progression of the idiopathic thoracolumbar scoliosis. Initially, the patient started therapy with inhaled corticosteroids, and later continued with combination therapy of inhaled steroids and long-acting beta2-agonists. Despite therapy, the patient developed severe acute respiratory failure during acute respiratory infections (most often viral infections with secondary bronchopneumonia). In February 2018, she developed severe acute respiratory failure, from which she was slowly recovering and was discharged home after 20 days of hospital treatment, dependent on oxygen. In September 2018 the patient started non-invasive positive pressure ventilation via oronasal mask using Trilogy100 ventilator. Despite severe cognitive impairment, the patient accepted the mask very well, and she continued to use NIV at home only during sleep, an average 6-8 hours per day. With the use of NIV, the patient achieved satisfactory oxygen saturation throughout the day. In the following two years after NIV introduction, the patient did not have any worsening of the respiratory status and her quality of life was significantly improved. <h3>Conclusion</h3> Deformities of the thoracic spine can lead to various pulmonary disorders. In patients with chronic respiratory failure, caused by deformity of the thoracic spine, non-invasive mechanical ventilation has proven to be an effective respiratory support.