PT - JOURNAL ARTICLE AU - Uros Krivec AU - Marina Praprotnik AU - Dusanka Lepej AU - Ana Kotnik Pirs AU - Jelena Berger TI - Flexible bronchoscopy in children with neuromuscular disease and acute hypoxemic respiratory failure requiring noninvasive ventilation DP - 2014 Sep 01 TA - European Respiratory Journal PG - 4656 VI - 44 IP - Suppl 58 4099 - http://erj.ersjournals.com/content/44/Suppl_58/4656.short 4100 - http://erj.ersjournals.com/content/44/Suppl_58/4656.full SO - Eur Respir J2014 Sep 01; 44 AB - IntroductionLower respiratory tract infections (LRTI) are frequently complicated by lung collapse in children with neuromuscular diseases (NMD). Both conditions predispose to acute respiratory failure and endotracheal intubation (EI). Bronchoscopic aspiration and noninvasive positive pressure ventilation (NIV) had been reported effective in adult patients.AimsTo evaluate feasibility & safety of flexible bronchoscopy (FB) and NIV in children with NMD with lung collapse and acute hypoxemic respiratory failure. The need for EI and atelecasts resolution were assessed.Methods8 consecutive patients with NMD, LRTI and atelectasis were included. Median age 3.2 years, range 2.4-16.5 years. One had spinal muscular atrophy (SMN) type 1, 4 had SMN type 2, 2 had Duchenne muscular dystrophy. 5 children were already on home NIV. 1 was hospitalized twice. Treatment protocol consisted of: admission to high dependency unit, FB aspiration under general anesthesia, NIV during and after FB, intensive respiratory physiotherapy (cough assist).ResultsAll patients required additional oxygen and had marked respiratory distress on admission. 6 were treated by the full protocol; in 2 cases FB was not performed. Mean hospital stay was 8.8 (± 1.9) days. None needed EI; one had a transient intensive care stay after FB. At discharge, re-expansion of collapsed lungs was seen in all cases with full protocol performed but not in the 2 patients without aspiration.ConclusionsFB aspiration in children with NMD requiring NIV for acute respiratory failure is feasible and safe in adequate settings. The protocol appears effective in promoting atelectasis re-expansion.