View Abstract

A5630 - Non Invasive Resolution of a Complete Left Lung Atelectasis in a Child Affected by Spinal Muscular Atrophy Type 1
Author Block: C. Conti1, E. Falcier1, E. Roma1, B. Garabelli1, M. Iatomasi1, E. De Mattia1, S. Conforti2, K. Gorni1, F. Rao1, V. A. Sansone1; 1Centro Clinico NEMO-Niguarda Hospital, Milano, Italy, 2Department of Thoracic Surgery, Niguarda Hospital, Milano, Italy.
Spinal muscular atrophy (SMA) is a genetic neuromuscular disease causing a various spectrum of respiratory impairment, cough inefficacy and dysphagia. In particular, SMA I patients are those affected by the most severe alterations, and frequently need ventilatory support and gastrostomy to overcome them. Nevertheless, difficult secretion management remains an important issue. A 8 years old SMA I child, on nocturnal non invasive ventilation (NIV) since the first year of life, on regular treatment with cough assistant (CA) and feeded by gastrostomy for 6 years, was referred to our centre from another hospital, after the diagnosis of a subtotal atelectasis of the left lung following an upper respiratory tract infection. He was treated with amoxicilline/clavulanate, trimethoprim/sulfametoxazole and meropenem as a third line therapy. Furthermore, he underwent a first bronchoscopy with orotracheal intubation (OTI) and general anesthesia, where DNAase and surfactant were instilled in the left bronchial system. It gave no benefit but a worsening respiratory failure, leading to the need of continuous NIV support. After the arrival at our centre, a high resolution thoracic CT scan was obtained, confirming the presence of a complete atelectasis of the left lung and a triangle-shaped image of not clear significance at the left upper lobar bronchus. A second bronchoscopy was then performed, with deep sedation and oxygen support, avoiding OTI: the complete obstruction of the main left bronchus by thick secretions and bloody debris was treated with aspiration and bronchial toilette, with complete bronchial re-canalization. Clinically, it was not observed any improvement, and a chest X-ray obtained 2 days later showed the persistence of complete atelectasis. We then decided to add to CA, regularly practiced 3 times/day, the use of intrapulmonary percussive ventilation (IPV, Percussionaire) 15 minutes 3 times/day associated with nebulisations with saline and bronchodilators: 5 minutes with high, 5 with medium and 5 with low frequency/session. 7 days later, following an important improvement in clinical outcomes, such as NIV dependence for less than 12 hours/day and reduction of hypoxemia at blood gas analysis, we performed a new chest X-ray with a complete resolution of the atelectasis that was confirmed by a CT-scan performed 9 months after the discharge. The patient continued at home the clearance routine with IPV and CA. In this case, the combination of IPV and CA in secretion clearance has permitted to avoid the continuous resort to invasive manoeuvres in a child affected by severe neuromuscular respiratory failure.