Detecting Ventilator-Induced Diaphragmatic Dysfunction Using Point-of-Care Ultrasound in Children With Long-term Mechanical Ventilation.

Long-term mechanical ventilation (MV) is defined as the use of MV for more than 6 hours per day for at least 3 weeks. Children requiring long-term MV include those with neuromuscular disease, central dysregulation, or lung dysfunction. Such children with medical complexity may be at risk for ventilator-induced diaphragmatic dysfunction. Ventilator-induced diaphragmatic dysfunction has been described in adult patients requiring acute MV with ultrasound (US). At this time, diaphragmatic US has not been evaluated in the pediatric post-acute care setting or incorporated into weaning strategies. We present 24 cases of children requiring long-term MV who underwent diaphragmatic US examinations to evaluate for ventilator-induced diaphragmatic dysfunction.

Longitudinal Analysis of Ventilation Perfusion Mismatch in Congenital Diaphragmatic Hernia Survivors.

OBJECTIVE: To determine the natural history of pulmonary function for survivors of congenital diaphragmatic hernia (CDH). STUDY DESIGN: This was a retrospective cohort study of survivors of CDH born during 1991-2016 and followed at our institution. A generalized linear model was fitted to assess the longitudinal trends of ventilation (V), perfusion (Q), and V/Q mismatch. The association between V/Q ratio and body mass index percentile as well as functional status was also assessed with a generalized linear model. RESULTS: During the study period, 212 patients had at least one V/Q study. The average ipsilateral V/Q of the cohort increased over time (P < .01), an effect driven by progressive reduction in relative perfusion (P = .012). A higher V/Q ratio was correlated with lower body mass index percentile (P < .001) and higher probability of poor functional status (New York Heart Association class III or IV) (P = .045). CONCLUSIONS: In this cohort of survivors of CDH with more severe disease characteristics, V/Q mismatch worsens over time, primarily because of progressive perfusion deficit of the ipsilateral side. V/Q scans may be useful in identifying patients with CDH who are at risk for poor growth and functional status.

Longitudinal Analysis of Pulmonary Function in Survivors of Congenital Diaphragmatic Hernia.

OBJECTIVE: To analyze longitudinal trends of pulmonary function testing in patients with congenital diaphragmatic hernia (CDH) followed in our multidisciplinary clinic. STUDY DESIGN: This was a retrospective cohort study of CDH patients born between 1991 and 2013. A linear mixed effects model was fitted to estimate the trends of percent predicted forced expiratory volume in 1 second (FEV1pp), percent predicted forced vital capacity (FVCpp), and FEV1/FVC over time. RESULTS: Of 268 patients with CDH who survived to discharge, 119 had at least 1 pulmonary function test study. The FEV1pp (P < .001), FVCpp (P = .017), and FEV1/FVC (P = .001) decreased with age. Compared with defect size A/B, those with defect size C/D had lower FEV1pp by an average of 11.5% (95% CI, 2.9%-20.1%; P = .010). A history of oxygen use at initial hospital discharge also correlated with decreased FEV1pp by an average of 8.0% (95% CI, 1.2%-15.0%; P = .023). CONCLUSIONS: In a select cohort of CDH survivors, average pulmonary function declines with age relative to expected population normative values. Those with severe CDH represent a population at risk for worsening pulmonary function test measurements who may benefit from recognition and monitoring for complications.

Respiratory failure.

On the basis of research evidence, (1)(2) numerous diseases and conditions can impair gas exchange, resulting in failure to meet the body's metabolic demands and leading to respiratory failure. On the basis of consensus, (1)(2)(7)(8)(9)(10) the clinical presentations of respiratory failure depend on the underlying cause and the level of hypoxemia and hypercapnia. Early diagnosis, close monitoring, and timely intervention are of utmost importance. On the basis of research evidence, (5)(14)(25) interventions range from noninvasive methods, such as close monitoring and supplemental oxygen, to full respiratory support with mechanical ventilation and in extreme cases even the use of extracorporeal membrane oxygenation.

Post-acute day and night non-invasive respiratory intervention use and outcome: A brief report.

OBJECTIVE: This study aimed to describe daytime and nighttime use and outcome of non-invasive respiratory intervention (NIRI) for infants born prematurely and for children with medical complexity (CMC) during a post-acute care hospital (PACH) admission. METHODS: Thirty-eight initial PACH admissions (October 2018 through September 2020) for premature infants (< 1 year; n = 19) and CMC (> 1 year; n = 19) requiring NIRI during the day and/or at night were retrospectively examined. Measures included: 1) daytime and nighttime NIRI use by type (supplemental oxygen therapy via low-flow nasal cannula or positive airway pressure [PAP] via high-flow nasal cannula, continuous positive airway pressure, or biphasic positive airway pressure at admission and discharge) and 2) daytime and nighttime NIRI outcome-reduction, increase, or no change from admission to discharge. RESULTS: For the total sample (n = 38), daytime vs nighttime NIRI use was significantly different (p < 0.001). At both admission and discharge, supplemental oxygen was the most common NIRI during the day, while PAP was most common at night. From admission to discharge, seven (18%) infants and children had a positive change (reduced NIRI) during the day, while nine (24%) had a positive change at night. At discharge, 11/38 (29%) infants and children required no daytime NIRI, while 4/38 (11%) required no day or night NIRI. CONCLUSION: NIRI use differs between day and night at PACH admission and discharge for CMC. Reductions in NIRI were achieved during the day and at night from PACH admission to discharge for both infants born prematurely and for children with varied congenital, neurological, or cardiac diagnoses.

Point-of-Care Ultrasound in Children With Medical Complexity.

BACKGROUND AND OBJECTIVES: Children with medical complexity (CMC) have significant chronic health conditions that involve congenital or acquired multisystem disease associated with medical fragility, functional limitations, dependence on technology, and high health care utilization. The objective of this study was to describe the indications, applications, and point-of-care ultrasound (POCUS) findings in this population. METHODS: A descriptive study of POCUS scans performed for clinical purposes in CMC admitted to a single pediatric postacute care hospital. All children for whom a POCUS was requested by a medical team provider were eligible for inclusion. RESULTS: One hundred and four POCUS evaluations were performed for 33 patients. Diagnostic groups for the 33 patients included multiple congenital anomalies (41%), neurologic or neuromuscular (31%), prematurity (25%) and cardiac (3%). Lung, cardiac, and diaphragmatic ultrasound accounted for 57% of POCUS requested. POCUS was abnormal in 82% of diaphragmatic, 73% of lung, and 11% of cardiac ultrasounds. Twenty-three percent of POCUS studies were requested to answer a specific clinical question, 56% for follow-up information, and 21% for baseline evaluation. CONCLUSIONS: Lung, diaphragmatic, and cardiac ultrasound were the most prevalent POCUS studies requested in a postacute care hospital. POCUS may offer an expanded role in such patients and settings by answering clinical questions and by providing baseline and follow-up information.

Ventilator weaning outcomes in chronic respiratory failure in children.

The purpose of this study was to describe mechanical ventilation weaning outcomes for children with chronic respiratory failure discharged from one of six post-acute rehabilitation facilities. Demographic, clinical and outcome data were collected from the medical record. Forty-four children were included in this prospective series; 20 (45%) were weaned off the ventilator at discharge. Children required significantly lower levels of ventilatory support at discharge than admission. Hourly use on the ventilator decreased from admission to discharge for the full cohort and for the subgroup who required a ventilator at discharge. Seventy-five percent of the children discharged with a ventilator had a portable unit. We conclude that nearly half of the children using mechanical ventilation achieve weaning during a postacute rehabilitation admission, whereas others have positive outcomes in severity, hours off the ventilator or portability of equipment.

Does the ex utero intrapartum treatment to extracorporeal membrane oxygenation procedure change morbidity outcomes for high-risk congenital diaphragmatic hernia survivors?

PURPOSE: In high-risk congenital diaphragmatic hernia (CDH), significant barotrauma or death can occur before extracorporeal membrane oxygenation (ECMO) can be initiated. We previously examined ex utero intrapartum treatment (EXIT)-to-ECMO in our most severe CDH patients, but demonstrated no survival advantage. We now report morbidity outcomes in survivors of this high-risk cohort to determine whether EXIT-to-ECMO conferred any benefit. METHODS: All CDH survivors with <15% predicted lung volume (PPLV) from September 1999 to December 2010 were included. We recorded prenatal imaging, defect size, and pulmonary, nutritional, cardiac, and neurodevelopmental outcomes. RESULTS: Seventeen survivors (8 EXIT-to-ECMO, 9 non-EXIT) had an average PPLV of 11.7%. Eight of 9 non-EXIT received ECMO within 2days. There were no significant defect size differences between groups, mostly left-sided (13/17) and type D (12/17). Average follow-up was 6.7years (0-13years). There were no statistically significant differences in outcomes, including supplemental oxygen, diuretics, gastrostomy, weight-for-age Z scores, fundoplication, pulmonary hypertension, stroke or intracranial hemorrhage rate, CDH recurrence, and reoperation. No survivor in our cohort was neurologically devastated. All had mild motor and/or speech delay, which improved in most. CONCLUSIONS: In this pilot series of severe CDH survivors, EXIT-to-ECMO confers neither significant survival nor long-term morbidity benefit. LEVEL OF EVIDENCE: Level III treatment study.

A fitness program for children with disabilities.

BACKGROUND AND PURPOSE: This case report describes a fitness program for children with disabilities and provides preliminary information about the safety and feasibility of the program. CASE DESCRIPTION: Nine children, 5 to 9 years of age with physical or other developmental disabilities, participated in a 14-week group exercise program held 2 times per week followed by a 12-week home exercise program. Energy expenditure index, leg strength (force-generating capacity of muscle), functional skills, fitness, self-perception, and safety were measured before intervention, after the group exercise program, and again after the home exercise program. OUTCOMES: No injuries occurred, and improvements in many of the outcome measures were observed. More improvements were observed after the group exercise program than after the home program, and adherence was better during the group exercise program. DISCUSSION: This case report demonstrates that a group exercise program of strength and endurance training may be a safe and feasible option for children with disabilities. Further research is needed to evaluate the effectiveness of a group fitness program and optimal training parameters.

Laughter is not always funny: breath-holding spells in familial dysautonomia.

Familial dysautonomia (FD) is a genetic disease characterized by primary autonomic dysfunction including parasympathetic hypersensitivity. Breath-holding spells (BHS) are believed to be caused by autonomic dysregulation mediated via the vagus nerve and increased in patients with a family history of BHS. Details and understanding of its pathophysiology are lacking. In this retrospective study of patients with FD, the incidence of BHS was higher at 53.3%, compared with previous studies in normal children. Laughter as a precipitating factor for BHS has not been previously reported in FD and occurred in 10% of patients in this study. Lower lung volumes, chronic lung disease, chronic CO2 retention, and inadequate autonomic compensation occur in those with FD leading to a higher incidence and severity of BHS when crying or laughing. Thus, FD may be a good model for understanding manifestations of the autonomic nervous system dysfunction and contribute to our knowledge of BHS mechanisms.

Oxygen and ventilator weaning during inpatient pediatric pulmonary rehabilitation.

Rates of oxygen and ventilator weaning, and factors related to successful weaning in inpatient pediatric pulmonary programs for infants and young children, have not been frequently reported in the literature. A retrospective review was conducted of 34 infants and toddlers with either a diagnostic condition of prematurity (PM) or congenital anomalies/neuromuscular disease (CA/NM) discharged from an inpatient pulmonary program. These cases represent 67 hospital admission-discharge episodes over a 6-year period. The rate of successful oxygen weaning (decrease to 0 hr per day) and ventilator weaning (decrease to <12 hr per day) and predictive factors related to successful ventilator weaning per admission-discharge episode were examined. Successful oxygen weaning was achieved during 24% and successful ventilator weaning was achieved during 30% of the admission-discharge episodes. No significant relationships were found between the selected demographic and clinical factors and oxygen weaning. Using a logistic regression model, the major variable associated with successful ventilator weaning per admission-discharge episode was diagnostic condition. Age at admission and the presence of comorbidities added slightly to the prediction model. The overall model yielded 86% accuracy for predicting a decrease in ventilator hours. However, projecting in which episodes children will not be weaned (negative predictive value = 88.9%) was more accurate than projecting in which episodes children will be weaned (positive predictive value = 73.3%). Although the program achieved a relatively low rate of successful ventilator weaning, children with a diagnostic condition of prematurity were more likely to be successfully weaned during inpatient pulmonary rehabilitation.

Clinical findings and resource use of infants and toddlers dependent on oxygen and ventilators.

Medical records were reviewed to describe characteristics, report clinical and resource measures, and determine if differences exist between the diagnostic groups of prematurity and multiple congenital anomalies/neurologic conditions for initial admissions of 37 infants and toddlers to an inpatient pulmonary rehabilitation program. More than 75% of the children had a tracheostomy at admission and discharge. Forty-six percent of the sample was admitted requiring only oxygen, whereas 51% were discharged requiring only oxygen and not mechanical ventilation. Thirty percent of the children weaned to a less invasive mode of ventilation while just under half of the children were discharged home. Between-group comparisons indicated statistically significant differences for nutritional support at discharge (p < or = 0.05) and discharge disposition (p = 0.04). Complete weaning of oxygen or ventilator support during an initial inpatient pulmonary rehabilitation admission occurred less frequently than weaning to a less invasive mode of ventilation. This is an important consideration for referring children to rehabilitation programs, for clinical program improvement activities, and for setting realistic expectations for referral sources, patients and families, clinical staff, and payers. Further study is recommended using clinical data in program planning, in program improvements, and for setting outcome expectations for infants and toddlers dependent on pulmonary technology.

Bronchoscopy findings in children and young adults with tracheostomy due to congenital anomalies and neurological impairment.

The aim of this study was to examine bronchoscopy findings for children and young adults with long-term tracheostomies due to congenital anomalies and neurological impairment and identify characteristics associated with abnormal bronchoscopic findings. We conducted a retrospective study of 128 bronchoscopy cases (81 children) at a pediatric rehabilitation center. Thirty-eight cases (30%) had normal findings and 14 children (17% of all children) were decannulated following bronchoscopy. Ninety cases (70% of cases) had abnormal findings (e.g. granulomas, airway inflammation, fixed obstruction). An acute indication for bronchoscopy was the strongest predictor of an abnormal finding, while age (younger) and diagnosis (multiple congenital anomalies (MCA)) also were associated with abnormalities. For a subsample of children undergoing bronchoscopy for routine surveillance (n= 90), underlying diagnosis (MCA) was the strongest predictor of an abnormal finding, while younger age contributed slightly. These findings add to the limited literature describing bronchoscopic findings in children and youth with tracheostomies due to congenital anomalies or neurological impairment. These findings may be useful for rehabilitation clinicians in determining care needs for children with long-term tracheostomy.

Predicting inadequate long-term lung development in children with congenital diaphragmatic hernia: an analysis of longitudinal changes in ventilation and perfusion.

PURPOSE: Infants born with congenital diaphragmatic hernia (CDH) demonstrate a wide variability in postnatal catch-up lung growth. The goals of this study were to assess the pulmonary development of children born with CDH using sequential ventilation-perfusion (V/Q) scintigraphy and to identify the perinatal factors that correspond to a progressive V/Q mismatch. METHODS: The records of 137 patients seen between 1990 and 2005 in a multidisciplinary CDH clinic were reviewed. Changes in the ipsilateral V/Q quotient were compared in 46 patients who had 2 or more studies with the following variables: sex, patch repair, laterality, gestational age, and use of extracorporeal membrane oxygenation. An abnormal V/Q quotient was defined as greater than 1.2 (reference range, 0.8-1.2). RESULTS: Abnormal V/Q scans were identified in 28 (61%) of the 46 patients at the time of the last V/Q study. Patients who underwent a patch repair had nearly 7 times the risk (P < .001) of developing an ipsilateral V/Q mismatch. The use of extracorporeal membrane oxygenation had a variable effect on the probability of an abnormal V/Q study finding. No other variable was significant. CONCLUSIONS: Many children with CDH develop significant and progressive V/Q mismatches. Although some perinatal variables appear to be predictive of this phenomenon, they may simply be surrogates for a greater degree of pulmonary hypoplasia present at birth. This subpopulation of CDH survivors is an identifiable group clearly at risk and thus requires long-term follow-up.

Outcomes of post-acute hospital episodes for young children requiring airway support.

In this descriptive study, we examined changes in invasive and non-invasive airway support; studied the rates of home discharge vs. long-term care or acute hospitalization; and examined the relationship between the level of airway support and discharge to home for 92 children (<3 years of age) with 104 admission-discharge episodes to a consortium of pediatric rehabilitation hospitals over a one-year period. We found a significant reduction (p < 0.001) in the level of airway support between admission and discharge. In 21 of 47 (45%) episodes, children weaned from mechanical ventilation to a less restrictive type of support. Sixty percent of the children had final discharges to home. There was a significant, though fair correlation (Spearman Rho = -0.344, p = 0.001) between home discharge and level of airway support. These outcomes data provide a multi-site baseline for understanding expected changes in airway support and home discharge rates of young children who are admitted to a post-acute inpatient program.

Weaning children from mechanical ventilation in a post-acute care setting.

As medical and technological advances have made it possible to prolong the life of children with chronic respiratory failure, children are being referred to post-acute inpatient rehabilitation programmes. In these settings, children can be weaned from their ventilators and receive medical and rehabilitative care in a developmentally supportive environment at a lower financial cost than in an intensive care unit. There is strong evidence that weaning children from mechanical ventilation has beneficial effects on their functionality, ease of care and quality of life. There is, however, little scientific evidence describing how often successful weaning is achieved or the most effective methods. The purpose of this article is to present a consensus report detailing a structured approach to weaning children from mechanical ventilation in a post-acute care setting. This study proposes a Weaning Severity Index and a Weaning Algorithm for use in the assessment and implementation of the weaning process in post-acute rehabilitation. Future clinical studies are needed to validate the suggested approach to ventilator weaning and to determine whether or not the weaning algorithm results in beneficial patient outcomes.

Tracheostomy speaking valves for children: tolerance and clinical benefits.

INTRODUCTION: Use of a tracheostomy speaking valve allows the expiratory flow of air to exit over the vocal folds promoting phonation. The purpose of this retrospective review was to determine: (1) what percentage of trial candidates tolerated a speaking valve; (2) whether candidates achieved phonation with a valve; and (3) which secondary benefits (coughing ability, secretion management, swallowing/feeding and oxygenation) could be clinically observed. METHODS: Twelve cases of children and youth (ages 8 months to 21 years) evaluated for a tracheostomy speaking valve at an inpatient rehabilitation hospital were reviewed. A speech-language pathologist and respiratory therapist evaluated the children for valve tolerance and candidacy for ongoing use. Clinical observations were used to determine phonation ability and to examine potential secondary benefits. RESULTS: All 10 subjects who tolerated the valve achieved phonation. Vocalizations included audible crying, non-specific vocalizations, word approximations, single words and short phrases. Minimal-to-no improvement was noted for coughing, secretion management, swallowing and oxygenation with clinical assessment. DISCUSSION: With supervision and training, speaking valves can enhance communication options for children and youth with tracheostomies and oxygen and ventilator dependence. Physiological and functional secondary benefits were observed but were more difficult to assess.

Head circumference and chronic positive pressure ventilation in children: a pilot study.

METHODS: We reviewed the cases of 11 children <12 years of age with complex medical conditions and respiratory failure requiring chronic positive pressure ventilation (CPPV). We conducted a within-group comparison of average head circumference (HC) percentiles with each child's age-expected 50th percentile value and a between-groups comparison with children with no history of ventilation. We examined the relationship between HC, peak levels of positive inspiratory pressure (PIP), and length of time on CPPV. RESULTS: We found that children on CPPV had an average HC value at the 71st percentile, significantly greater (p=0.009) than the age-expected 50th percentile; that children on CPPV had a greater (p=0.003) average HC percentile compared with children with complex medical conditions not on CPPV; and that peak levels of PIP had a moderately strong correlation to HC (r=0.689, p=0.019). CONCLUSION: We conclude that children on CPPV have larger than expected HC and HC appears related to the peak level of PIP. Research to further investigate the relationship between HC and CPPV in children appears to be warranted.