ABSTRACT
BACKGROUND: Spinal muscular atrophy (SMA) is a neurodegenerative disease that results in progressive muscular atrophy and weakness. The primary cause of morbidity and mortality in these children is pulmonary disease due to poor airway clearance that leads to acute respiratory failure. There is a paucity of literature on the treatment of children with SMA and acute respiratory failure. METHODS: We performed a retrospective chart review of pediatric patients with SMA type I or II admitted to the PICU in a tertiary-care children's hospital with acute respiratory failure who required mechanical ventilation and/or aggressive airway clearance. RESULTS: The study included 300 unique encounters among 137 unique subjects. All the subjects received airway clearance at home before admission, and 257 encounters (85.7%) were supported with noninvasive ventilation (NIV) before admission. Sixty-eight subjects (49.6%) required endotracheal intubation on admission or at some point during their PICU stay. The median (interquartile range [IQR]) time to intubation was 0 (0-20) h, and the median (IQR) duration of mechanical ventilation was 2 (1-5) d on invasive mechanical ventilation with no statistical difference between type I and type II (P = .89). Of those, 65 (91.1%) were successfully extubated to NIV on the first attempt, whereas 4 subjects (5.8%) required multiple extubation attempts and 3 subjects (4.4%) required subsequent tracheostomy. For the subjects who were intubated, both PICU and hospital length of stay were longer (P < .001) when compared with the subjects managed by NIV alone. The subjects with SMA type I had a longer PICU length of stay, with a median (IQR) of 5 (3-11) d versus 4 (2-7) d (P = .002). The hospital length of stay and duration of invasive mechanical ventilation were not statistically different (P = .055 and P = .068, respectively). CONCLUSIONS: The subjects with SMA types I or II can be treated successfully with NIV and aggressive airway clearance during acute respiratory failure. Similarly, when intubation is required, successful extubation can be achieved with NIV transitional support combined with aggressive airway clearance maneuvers.
ABSTRACT
There is evidence that reducing opioid exposure in children undergoing cardiac surgery may enhance postoperative recovery. We aimed to describe a minimal opioid postoperative management protocol in children undergoing cardiac surgery and our early outcomes with this strategy. We reviewed the medical records of children (6 months-18 years) who underwent elective cardiac surgery through a median sternotomy with cardiopulmonary bypass at our institution between 2016 and 2018. All patients were managed postoperatively using a standardized protocol. 101 children (median age 5 years) were included and 85% were extubated in the operating room. Although most patients (96%) received opioids postoperatively, opioid requirements decreased steadily over time, with 88%, 58%, and 18% of children receiving opioids on postoperative day 1, 2, and 3, respectively; 41% received no opioids after postoperative day 1. The median cumulative opioid exposure was 0.25 morphine milligram equivalents per kg (interquartile range, 0.10-0.75). Greater than mild pain was rare (<10%) at each time point. The rates of operative mortality and major complication were 0% and 3%, respectively. The median postoperative length of stay was 3 days, and 13% required readmission within 30 days. Age, cardiopulmonary bypass time, and number of benzodiazepine doses were independently associated with cumulative opioid exposure. Any complication, chest tube time, and higher STAT Category were independently associated with prolonged postoperative length of stay. A minimal opioid postoperative management protocol can be safe and effective in children undergoing cardiac surgery. Future prospective studies are needed to determine optimal practice and patient selection.
