Emanuel syndrome and congenital diaphragmatic hernia: A systematic review.

BACKGROUND: Emanuel Syndrome (ES), a rare chromosomal disorder caused by a supernumerary chromosome 22 derivative (der(22)t(11;22)), was identified in a fetus with congenital diaphragmatic hernia (CDH) at our fetal center. We aimed to identify a precedent for clinical care and patient outcomes to guide family decision-making. METHODS: This non-funded and non-registered study queried the entire CDH Registry (CDHR) including >10,000 patients since 1995 and conducted a systematic literature review for patients with concomitant ES and CDH. RESULTS: Literature review captured 12 citations and identified 9 patients with CDH+ES from over 400 known ES cases. Given the rarity of the disease and to reduce bias, there were no exclusion criteria aside from non-English language. Of these 9, two underwent surgical CDH repair with neither surviving. The CDHR identified 6 patients with ES, all reported after 2013 and prenatally diagnosed. Median estimated gestational age was 39 weeks (range 37-40) and median birth weight was 2.72 kg (range 2.4-3.4 kg). 3 patients died within the first few postnatal days; surgical repair was not offered due to "anomalies" and "pulmonary hypertension" in two and one family chose comfort measures. The other 3 patients underwent surgical repair, and 2 were supported with ECMO. Two patients survived to discharge, incurring surgical comorbidities associated with severe CDH including gastrostomy dependence, tracheostomy, and CDH recurrence. CONCLUSIONS: ES patients with CDH have potential to tolerate repair and survive to discharge, however with significant additional morbidity combined with severe challenges inherent to ES. This represents the largest series of patients with CDH and ES to date. LEVEL OF EVIDENCE: IV (Case series with no comparison group).

VACTERL Association with Situs Inversus Totalis: A Unique Combination.

BACKGROUND: VACTERL association is a sporadic, nonrandom series of congenital malformations diagnosed by the presence of three or more of the following: vertebral malformations, anal atresia, cardiac defects, tracheoesophageal fistula, renal malformations, and limb malformations. Situs inversus totalis (SIT) and esophageal malformations are rarely associated. This is the first reported case in North America of VACTERL association with SIT. IMPLICATIONS FOR PRACTICE: Respiratory distress in the term infant requires full exploration of all possible causes because the etiology may be far more complex than routinely diagnosed respiratory distress syndrome. This particular case demonstrates physical exam findings and supportive imaging that would be observed in infants with VACTERL association and with SIT, highlighting considerations when, rarely, both occur simultaneously.

Early continuous renal replacement therapy during infant extracorporeal life support is associated with decreased lung opacification.

Lung opacification on chest radiography (CXR) is common during extracorporeal life support (ECLS), often resulting from pulmonary edema or inflammation. Concurrent use of continuous renal replacement therapy (CRRT) during ECLS is associated with improved fluid balance and cytokine filtration; through modification of these pathologic states, CRRT may modulate lung opacification observed on CXRs. We hypothesize that early CRRT use during infant ECLS decreases lung opacification on CXR. We conducted a retrospective cohort study comparing CXRs from infants receiving ECLS and early CRRT (n = 7) to matched infants who received ECLS alone (n = 7). The CXR obtained prior to ECLS, all CXRs obtained within the first 72 h of ECLS, and daily CXRs for the remainder of the ECLS course were analyzed. The outcome measure was the degree of opacification, determined by independent assessment of two, blinded pediatric radiologists using a modified Edwards et al.'s lung opacification scoring system (from Score 0: no opacification to Score 5: complete opacification). 220 CXRs were assessed (cases: 93, controls: 127). Inter-rater reliability was established (Cohen's weighted к = 0.74; p < 0.0001, good agreement). At baseline, the mean opacification score difference between cases and controls was 1 point (cases: 1.8, controls 2.8; p = 0.049). Using mixed modeling analysis for repeated measures accounting for differences at baseline, the average overall opacification score was 1.2 points lower in cases than controls (cases: 2.1, controls: 3.3; p < 0.0001). The overall distribution of scores was lower in cases than controls. Early CRRT utilization during infant ECLS was associated with decreased lung opacification on CXR.

Implementing a practice change: early initiation of continuous renal replacement therapy during neonatal extracorporeal life support standardizes care and improves short-term outcomes.

PURPOSE: We hypothesized that a standardized approach to early continuous renal replacement therapy (CRRT) during neonatal extracorporeal life support (ECLS) results in greater homogeneity of CRRT initiation times with improvements in fluid balance and outcomes. METHODS: Retrospective analysis of data (2007-2015) obtained from neonates treated prior to (E1; n = 32) and after (E2; n = 31) a 2011 practice change: CRRT initiation within 48 h of ECLS. RESULTS: Birthweight, gestational age, ECLS mode, and age at ECLS initiation were similar to each epoch. Survival [E1: median 75%, E2: 71%] and length of ECLS [E1: median 221 h, E2: 180 h] were comparable. During E2, 100% of infants received CRRT (vs. E1: 37%; p < 0.001) and 97% of infants initiated CRRT within 48 h of ECLS (vs. E1: 13%; p < 0.001). Control charts demonstrate reduced practice variation. Elapsed time from ECLS to CRRT differed between Epochs [E1: median 105 h, E2: 9 h; p < 0.001] as did weight at CRRT initiation [E1: 4.13 kg (29% above baseline), E2: 3.19 kg (0%); p < 0.001]. Significant differences in weight change were noted on days 6 and 7 (E1: 14%, E2: 2%; raw data comparison yielded p < 0.05) and curves were different (p < 0.05). CONCLUSIONS: We successfully implemented a practice change, initiating CRRT within 48 h of ECLS cannulation, leading to decreased practice variation and improved short-term outcomes including decreased weight gain at CRRT initiation and faster return to baseline weight during the first 7 days of ECLS. We did not demonstrate changes in duration of ECLS, invasive ventilation, or survival.

A new method for group decision making and its application in medical trainee selection.

CONTEXT: The problems associated with generating a collaborative ranked preference list represent a common source of dilemma in academic medicine and medical education. Such issues present during the process of choosing among applicants to medical schools, during the selection of postgraduate trainees, and in the course of performance assessments and the prioritising of financial expenditures. Currently, most institutions use pseudo-quantitative methods, such as the averaging of scores awarded on an arbitrary scale. These methods are mathematically problematic and may not accurately reflect group opinion. METHODS: The present authors developed a novel algorithm for creating a collaborative preference list that generates and sorts a matrix of pairwise comparisons between applicants or choices without placing any reliance on arbitrary Likert scale-type scores. This method achieves equality in influence across individual assessors, as well as transparency and reproducibility. The authors report a case study of their experience using this new algorithm in the 2013 neonatal-perinatal fellowship match. RESULTS: When used by this group in the selection of fellowship trainees, the method proposed here allowed for greater efficiency and created a rank-order list that did not require reshuffling or significant debate. A survey of faculty staff and fellows showed much higher levels of satisfaction with the new algorithm and a unanimous desire to use the new algorithm in the future, in preference to a score-based system. CONCLUSIONS: The algorithm developed and described here may reduce arbitrariness in processes that require the collaborative creation of a preference list. This method may have wide applicability in medical education and training, and beyond. The present authors' experience of using this algorithm during the National Resident Matching Program match showed improved perceptions of fairness, ease of use and efficiency.

NICU bedside caregivers sustain process improvement and decrease incidence of bronchopulmonary dysplasia in infants < 30 weeks gestation.

BACKGROUND: The objective of this study was to investigate whether a respiratory care bundle, implemented through participation in the Vermont Oxford Network-sponsored Neonatal Intensive Care Quality Improvement Collaborative (NIC/Q 2005) and primarily dependent on bedside caregivers, resulted in sustained decrease in the incidence of bronchopulmonary dysplasia (BPD) in infants < 30 wk gestation. METHODS: A retrospective cohort study was conducted. Infants inborn between 23 wk and 29 wk + 6 d of gestation were included. Patients with congenital heart disease, significant congenital or lung anomalies, or death before intubation were excluded. Four time periods (T1-T4) were identified: T1: September 1, 2002 to August 31, 2004; T2: September 1, 2004 to August 31, 2006; T3: September 1, 2006 to August 31, 2008; T4: September 1, 2008 to August 31, 2010. RESULTS: A total of 1,050 infants were included in the study. BPD decreased significantly in T3 post-implementation of the respiratory bundle compared with T1 (29.9% vs 51.2%, respectively; adjusted odds ratio [aOR] = 0.06 [95% CI 0.03-0.13], P = < .001). The decrease was not sustained into T4. There was a significant increase in the rate of BPD-free survival to discharge in T3 compared with T1 (53.1% vs 47%; aOR = 1.68 [95% CI 1.11-2.56], P = .01) that was also not sustained. The rate of infants requiring O2 at 28 d of life decreased significantly in T3 versus T1 (40.3% vs 69.9%, respectively; aOR = 0.12 [95% CI 0.07-0.20], P = < .001). Increases in the rate of surfactant administration by 1 h of life and rate of caffeine use were observed in T4 versus T1, respectively. There was a significant decrease in median ventilator days and a significant increase in the median number of noninvasive CPAP days throughout the study period. CONCLUSIONS: In this study, implementation of a respiratory bundle managed primarily by nurses and respiratory therapists was successful in increasing the use of less invasive respiratory support in a consistent manner among very low birthweight infants at a single institution. However, this study and others have failed to show sustained improvement in the incidence of BPD despite sustained process change.

Antibiotic timing in neonates with suspected hospital-acquired infections.

There exists general agreement within neonatology that antibiotics should be administered promptly to neonates with possible bacterial sepsis and meningitis. We initiated a series of quality improvement cycles designed to reduce delays in the initiation of antibiotic therapy to less than 2 hours when hospital-acquired infection (HAI) was suspected. All infants in this study were in neonatal intensive care (level II or III) who were started on antibiotics for a suspected HAI (defined as an infection that occurred 72 hours after admission to the NICU) were audited. Through a series of quality improvement cycles, we analyzed sources of delays in the initiation of antibiotic therapy from the time the order was written through administration. In subsequent cycles, we intervened to reduce delays through education, standardize the evaluation process, and develop an online ordering system that streamlined the workflow patterns in the nurseries and pharmacy. Using a prospective cohort design, we compared antibiotic delivery times after each process improvement cycle. Antibiotic delivery time was reduced from a median of 137.5 minutes to 75 minutes and variation of practice was reduced in terms of standard deviation and range (P < .001). The use of computerized physician order entry significantly improved the writing of STAT orders (P < .0001). A systematic analysis of workflow patterns and efficiencies, coupled with improvement cycles targeting delays and development of a computerized physician order entry system, allowed us to improve antibiotic delivery time in neonates with suspected HAI in an intensive care nursery system.

Thermoregulation in very low-birth-weight infants during the golden hour: results and implications.

The survival of very low-birth-weight (VLBW) infants has been shown to be effected by alterations in thermoregulation. Morbidity and mortality in these VLBW infants has remained higher than those in any other group of infants because of their innate vulnerability and because of exposure to risk factors in the environment. This leaves the premature infant vulnerable to cold stress especially in the first hours to weeks of life. At birth, the VLBW infant emerges from a warm, fluid environment and is thrust into a cold, abrasive environment before the protective layers of the epidermis have developed. Within minutes of birth, the core temperature begins to fall, particularly in infants whose birth weights are less than 1500 g. Hypothermia is a major cause of morbidity and mortality in infants; therefore, maintaining normal body temperatures in the delivery room is crucial. We reviewed evidence related to thermoregulation at birth in VLBW infants, including transepidermal water loss and temperature control in the delivery room, during stabilization and upon admission to the neonatal intensive care unit. Delivery room management that focuses on the adaptation of the infant as well as early interventions that improve long-term outcomes may emphasize the "golden hour" of care and improve outcomes in this extremely vulnerable population.

Simulation training in graduate medical education: a means of traversing a changed and changing landscape.

There have been significant changes in the graduate medical (resident) education in the United States over the last two decades. These changes have been the result of a wide range of societal, governmental, and regulatory alterations which have either directly or indirectly impacted today's physicians-in-training experiences and autonomy, raising concerns about their readiness for independent practice at the completion of training. This article reviews the evolution of these changes and the promise that simulation training holds as one of the keys to ensuring continuing excellence in the training of today's and tomorrow's physicians.

Parameter selection for and implementation of a web-based decision-support tool to predict extubation outcome in premature infants.

BACKGROUND: Approximately 30% of intubated preterm infants with respiratory distress syndrome (RDS) will fail attempted extubation, requiring reintubation and mechanical ventilation. Although ventilator technology and monitoring of premature infants have improved over time, optimal extubation remains challenging. Furthermore, extubation decisions for premature infants require complex informational processing, techniques implicitly learned through clinical practice. Computer-aided decision-support tools would benefit inexperienced clinicians, especially during peak neonatal intensive care unit (NICU) census. METHODS: A five-step procedure was developed to identify predictive variables. Clinical expert (CE) thought processes comprised one model. Variables from that model were used to develop two mathematical models for the decision-support tool: an artificial neural network (ANN) and a multivariate logistic regression model (MLR). The ranking of the variables in the three models was compared using the Wilcoxon Signed Rank Test. The best performing model was used in a web-based decision-support tool with a user interface implemented in Hypertext Markup Language (HTML) and the mathematical model employing the ANN. RESULTS: CEs identified 51 potentially predictive variables for extubation decisions for an infant on mechanical ventilation. Comparisons of the three models showed a significant difference between the ANN and the CE (p = 0.0006). Of the original 51 potentially predictive variables, the 13 most predictive variables were used to develop an ANN as a web-based decision-tool. The ANN processes user-provided data and returns the prediction 0-1 score and a novelty index. The user then selects the most appropriate threshold for categorizing the prediction as a success or failure. Furthermore, the novelty index, indicating the similarity of the test case to the training case, allows the user to assess the confidence level of the prediction with regard to how much the new data differ from the data originally used for the development of the prediction tool. CONCLUSION: State-of-the-art, machine-learning methods can be employed for the development of sophisticated tools to aid clinicians' decisions. We identified numerous variables considered relevant for extubation decisions for mechanically ventilated premature infants with RDS. We then developed a web-based decision-support tool for clinicians which can be made widely available and potentially improve patient care world wide.

Predicting extubation outcome in preterm newborns: a comparison of neural networks with clinical expertise and statistical modeling.

Even though ventilator technology and monitoring of premature infants has improved immensely over the past decades, there are still no standards for weaning and determining optimal extubation time for those infants. Approximately 30% of intubated preterm infants will fail attempted extubation, requiring reintubation and resuming of mechanical ventilation. A machine-learning approach using artificial neural networks (ANNs) to aid in extubation decision making is hereby proposed. Using expert opinion, 51 variables were identified as being relevant for the decision of whether to extubate an infant who is on mechanical ventilation. The data on 183 premature infants, born between 1999 and 2002, were collected by review of medical charts. The ANN extubation model was compared with alternative statistical modeling using multivariate logistic regression and also with the clinician's own predictive insight using sensitivity analysis and receiver operating characteristic curves. The optimal ANN model used 13 parameters and achieved an area under the receiver operating characteristic curve of 0.87 (out-of-sample validation), comparing favorably with multivariate logistic regression. It also compared well with the clinician's expertise, which raises the possibility of being useful as an automated alert tool. Because an ANN learns directly from previous data obtained in the institution where it is to be used, this makes it particularly amenable for application to evidence-based medicine. Given the variety of practices and equipment being used in different hospitals, this may be particularly relevant in the context of caring for preterm newborns who are on mechanical ventilation.

Web-based prediction of extubation outcome in premature infants on mechanical ventilation using an artificial neural network.

The web-based implementation of a decision-support tool for the prediction of extubation outcome in mechanically ventilated premature infants enables the integration of advanced and computationally intensive modeling approaches with easy-usage, no maintenance requirements and wide availability. Accordingly, the artificial neural network predictive tool developed provides decision-support in determining whether to extubate a premature infant to clinicians in NICUs anywhere with access to the Internet.

Breastfeeding rates at an urban medical university after initiation of an educational program.

BACKGROUND: Our objective was to improve breastfeeding initiation rates at an urban medical center. METHODS: A breastfeeding educational program for health care providers was developed and implemented in 1995. The outcome variable of interest was the change in breastfeeding initiation rate during 2 periods, 1993-1994 and 1996 to 1999, stratified by weight (> 2,000, 1,500 to 2,000, and < 1,500 g). RESULTS: The breastfeeding initiation rate in 1996 to 1999 for all mothers of newborns admitted to the hospital was 47.1% (4,107/8,724), compared with the 1993-1994 rate of 18.9% (816/4,315). During the second period, the breastfeeding rate among mothers of infants < 1,500 g was 60.8% (468/770), compared with 19.2% (56/293) during the earlier study period. Stratified by weight, the greatest improvement in rates of breastfeeding initiation and at discharge was seen with mothers of preterm infants. CONCLUSION: A breastfeeding educational program that interfaced with medical staff and mothers at an urban medical university was associated with increased rates of breastfeeding initiation.

Neonatal sepsis: evaluation and management.

Bacterial antigenic challenge presents a difficult fight for the neonatal immune system, and they have a smaller arsenal of weapons to fight bacterial infections than adults and older children. The baby's own systemic inflammatory response may have detrimental effects on several organs and longer lasting effects on the developing brain. Neurodevelopmental outcomes after maternal chorioamnionitis are worse than neonates without a contaminated intrauterine environment, regardless of gestation age and the baby's culture results. Successes with intrapartum antibiotic prophylaxis decreasing rates of GBS sepsis and maternal chorioamnionitis, have heartened care providers and parents. These results demonstrate the advances possible when specific diseases are made a national health priority, and good clinical trial work is applied to clinical practice.

Preparation of the critically ill neonate for transport.

Transport of a critically ill neonate is stressful for all involved. Adequate communication and stabilization will reduce stresses and improve outcomes. Periodic review of the stabilization and care provided to neonates prior to transport can help in further improving the process. Such reviews can be done in conjunction with the Regional Perinatal Center.

Discharge planning for very low birthweight infants.

As discharge approaches, usually indicated by feeding progression, thermoregulation and other events, it is important for the medical team to develop a plan of action and stick with it, assuming that nothing untoward happens. Experience tells us that families of infants with special needs at discharge cope better and maintain a more positive attitude if plans are clearly defined and followed consistently. A coordinated team approach is also helpful for personnel responsible for arranging equipment, training, and home health services.

Effects of changing health care financial policy on very low birthweight neonatal outcomes.

BACKGROUND: Our objective was to determine whether perinatal referral patterns and clinical outcomes for very low birthweight infants changed in relation to changing Medicaid financial policies in coastal South Carolina. METHODS: Referral patterns and outcome indicators for very low birthweight infants were compared during two periods in a cohort design. RESULTS: A total of 520 infants were identified over two funding periods. A decrease in the proportion of nonwhite very low birthweight infants was identified. There was an increase in very low birthweight infants with Medicaid funding born outside our level III center. CONCLUSIONS: Changes in financial public policy have been successful in the movement of low risk pregnancies into the private sector. However, an increased proportion of deliveries of very low birthweight infants occurred outside the level III center.