Among infants with tracheostomy in this cohort, overall mortality rates were relatively low but not insignificant. CHD was associated with increased mortality; however, children with SGS showed more favorable outcomes. Other patient characteristics were not associated with differences in mortality. These data clarify outcomes in a group of infants with tracheostomy.

Non-invasive pneuRIP testing provided instantaneous PFT results. As compared to healthy subjects, NM patients showed abnormal results with increased markers of thoracoabdominal asynchrony, WOB indices, and biphasic breathing patterns likely resulting from NM weakness.

RationalePulmonary function testing (PFT) provides diagnostic information regarding respiratory physiology. However, many forms of PFT are time-intensive and require patient cooperation. Respiratory inductance plethysmography (RIP) provides thoracoabdominal asynchrony (TAA) and work of breathing (WOB) data. pneuRIP TM is a noninvasive, wireless analyzer that provides real-time assessment of RIP via an iPad. In this study, we show that pneuRIP TM can be used in a hospital clinic setting to differentiate WOB indices and breathing patterns in children with DMD as compared to age-matched healthy subjects. MethodsRIP using the pneuRIP TM was conducted on 9 healthy volunteers and 7 DMD participants (ages 5-18) recruited from the neuromuscular clinic, under normal resting conditions over 3-5 min during routine outpatient visits. The tests were completed in less than 10 minutes and did not add excessive time to the clinic visit. Variables recorded included laboredbreathing index (LBI), phase angle (Φ) between abdomen and rib cage, respiratory rate (RR), percentage of rib cage input (RC%), and heart rate (HR). The data were displayed in histogram plots to identify distribution patterns within the normal ranges. The percentages of data within the ranges (0� Φ �30 deg.; median RC %±10%; median RR±5%; 1�LBI�1.1) were compared. Unpaired t-tests determined significance of the data between groups. Results100% patient compliance demonstrates the feasibility of such testing in clinical settings. DMD patients showed a significant elevation in Φ, LBI, and HR averages (P<0.006, P<0.002, P<0.046, respectively). Healthy subjects and DMD patients had similar BPM and RC% averages. All DMD data distributions were statistically different from healthy subjects

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Objectives: There is a paucity of published literature identifying patients at higher risk of decannulation failure. The purpose of this study is to evaluate patient factors that may predict successful decannulation of pediatric tracheostomy patients and analyze factors contributing to tracheostomy decannulation failures.Methods: A retrospective chart review of tracheostomy outcomes was conducted at a pediatric referral hospital. Successful and failed decannulations were compared using the following patient variables: age at tracheostomy, sex, ethnicity, gestational age and weight, the primary indication for tracheostomy, comorbidities, age at decannulation attempt, polysomnography data, and status of airway before decannulation as assessed endoscopically by airway team.Results: Four hundred thirty-nine tracheostomies were performed over the 18-year period with 173 decannulation attempts. The overall rate of successful decannulation on the first attempt was 91.9% (159 of 173), with an eventual decannulation success rate of 97.1% (168 of 173). Compared with failed decannulations, the patients with successful decannulations had a shorter duration of tracheostomy and no medical comorbidities. Gestational age and weight approached, but did not achieve, statistical significance. After 25 months with a tracheostomy, approximately 50% of patients are decannulated with very few decannulations occurring after 75 months. The overall mortality rate in this cohort was 18.6% (78 of 420) with a tracheostomy-related mortality rate of 0.95% (4 of 420). Conclusions:The decannulation protocol at this institution is successful nearly 92% of the time. Fewer medical comorbidities, shorter duration of tracheostomy placement, and older gestational age may improve the likelihood of successful decannulation. Future studies are needed to determine the optimal timing and workup to evaluate patients for decannulation.