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To predict pulmonary hypertension (PH) therapy at discharge in a large multicenter cohort of infants with congenital diaphragmatic hernia (CDH). Six-year linked records from Children’s Hospitals Neonatal Database and Pediatric Health Information System were used; patients whose diaphragmatic hernia was repaired before admission or referral, who were previously home before admission or referral, and non-survivors were excluded. The primary outcome was the use of PH medications at discharge and the secondary outcome was an inter-center variation of therapies during inpatient utilization. Clinical factors were used to develop a multivariable equation randomly applied to 80% cohort; validated in the remaining 20% infants. A total of 831 infants with CDH from 23 centers were analyzed. Overall, 11.6% of survivors were discharged on PH medication. Center, duration of mechanical ventilation, and duration of inhaled nitric oxide were associated with the use of PH medication at discharge. This model performed well in the validation cohort area under the receiver operating characteristic curve of 0.9, goodness-of-fit χ2, p = 0.17. Clinical variables can predict the need for long-term PH medication after NICU hospitalization in surviving infants with CDH. This information may be useful to educate families and guide the development of clinical guidelines.

OBJECTIVE The aim of this study was to describe the use, duration, and intercenter variation of analgesia and sedation in infants with congenital diaphragmatic hernia (CDH). STUDY DESIGN This is a retrospective analysis of analgesia, sedation, and neuromuscular blockade use in neonates with CDH. Patient data from 2010 to 2016 were abstracted from the Children's Hospitals Neonatal Database and linked to the Pediatric Health Information System. Patients were excluded if they also had non-CDH conditions likely to affect the use of the study medications. RESULTS A total of 1,063 patients were identified, 81% survived, and 30% were treated with extracorporeal membrane oxygenation (ECMO). Opioid (99.8%), sedative (93.4%), and neuromuscular blockade (87.9%) use was common. Frequency of use was higher and duration was longer among CDH patients treated with ECMO. Unadjusted duration of use varied 5.6-fold for benzodiazepines (median: 14 days) and 7.4-fold for opioids (median: 16 days). Risk-adjusted duration of use varied among centers, and prolonged use of both opioids and benzodiazepines ≥5 days was associated with increased mortality (p

Our hypothesis was that among infants with hypoxic-ischemic encephalopathy (HIE), venoarterial (VA), compared to venovenous (VV), extracorporeal membrane oxygenation (ECMO) is associated with an increased risk of mortality or intracranial hemorrhage (ICH). Retrospective cohort analysis of infants in the Children’s Hospitals Neonatal Database from 2010 to 2016 with moderate or severe HIE, gestational age ≥36 weeks, and ECMO initiation

Objective Infants with congenital diaphragmatic hernia (CDH) require multiple invasive interventions carrying inherent risks, including central venous and arterial line placement. We hypothesized that specific clinical or catheter characteristics are associated with higher risk of nonelective removal (NER) due to complications and may be amenable to efforts to reduce patient harm. Study Design Infants with CDH were identified in the Children's Hospital's Neonatal Database (CHND) from 2010 to 2016. Central line use, duration, and complications resulting in NER are described and analyzed by extracorporeal membrane oxygenation (ECMO) use. Results A total of 1,106 CDH infants were included; nearly all (98%) had a central line placed, (average of three central lines) with a total dwell time of 22 days (interquartile range [IQR]: 14–39). Umbilical arterial and venous lines were most common, followed by extremity peripherally inserted central catheters (PICCs); 12% (361/3,027 central lines) were removed secondary to complications. Malposition was the most frequent indication for NER and was twice as likely in infants with intrathoracic liver position. One quarter of central lines in those receiving ECMO was placed while receiving this therapy. Conclusion Central lines are an important component of intensive care for infants with CDH. Careful selection of line type and location and understanding of common complications may attenuate the need for early removal and reduce risk of infection, obstruction, and malposition in this high-risk group of patients. Key Points

The management of infants with congenital diaphragmatic hernia (CDH) receiving extracorporeal life support (ECLS) is complex. Significant variability in both practice and prevalence of ECLS use exists among centers, given the lack of evidence to guide management decisions. The purpose of this report is to review existing evidence and develop management recommendations for CDH patients treated with ECLS. This article was developed by the Extracorporeal Life Support Organization CDH interest group in cooperation with members of the CDH Study Group and the Children's Hospitals Neonatal Consortium.

To determine practice variation in the utilization of neuromonitoring modalities in neonatal extracorporeal membrane oxygenation (ECMO) patients across Level IV neonatal intensive care units (NICUs). Cross-sectional survey design using electronic surveys sent to site sponsors of a multicenter collaborative of 34 Level IV NICUs of the Children’s Hospitals Neonatal Consortium (CHNC) from June to August 2018. We had 22 survey respondents from CHNC ECMO centers. Twenty-seven percent of respondents routinely monitored for seizures using electroencephalogram. Cerebral near infrared spectroscopy was used by 50%. Head ultrasound was performed by 95% but the frequency, duration, and type of views varied. Post ECMO screening brain MRI prior to hospital discharge was routinely performed by 77% of respondents. A majority of centers (95%) performed neurodevelopmental follow-up after hospital discharge. There is variation in neuromonitoring practices in Level IV NICUs performing ECMO. Lack of evidence and clear outcome benefits has contributed to practice variation across institutions.

Objective To evaluate how inotropic requirements in neonates with respiratory failure are affected by extracorporeal membrane oxygenation (ECMO) mode and whether high requirements predict mortality. Study design This retrospective chart review included all neonates undergoing ECMO for primary respiratory failure from 2010 to 2016 at a single institution. The vasoactive inotropy score (VIS) was calculated as described in the literature. Data were analyzed with descriptive statistics and univariate analyses. Results Of the 110 identified neonates, 96 underwent venovenous (VV) (87%), 11 (10%) venoarterial, and 3 (3%) converted from VV to venoarterial. The median precannulation VIS score was 33.02 for patients who underwent VV compared with 28.93 for venoarterial (P = .25) and 15 for infants converted. VIS decreased dramatically by 4 hours of ECMO in both groups. The VIS before cannulation was similar in survivors and nonsurvivors, but was significantly higher in nonsurvivors after 24 hours of ECMO (median VIS, 12 [IQR, 8-25] vs 8 [IQR, 3.0-14.5]; P = .035) and at decannulation (10 [IQR, 7-19] vs 3 [IQR, 0-7]; P Conclusions Neonates with respiratory failure can be successfully managed on VV ECMO even with considerable vasoactive requirements. Vasoactive requirement after 24 hours of ECMO was predictive of mortality.

Describe inpatient pulmonary hypertension (PH) treatment and factors associated with therapy at discharge in a multicenter cohort of infants with CDH. Six years linked records from Children’s Hospitals Neonatal Database and Pediatric Health Information System were used to describe associations between prenatal/perinatal factors, clinical outcomes, echocardiographic findings and PH medications (PHM), during hospitalization and at discharge. Of 1106 CDH infants from 23 centers, 62.8% of infants received PHM, and 11.6% of survivors were discharged on PHM. Survivors discharged on PHM more frequently had intrathoracic liver, small for gestational age, and low 5 min APGARs compared with those discharged without PHM (p

Prenatal diagnosis has changed perinatal medicine dramatically, allowing for additional fetal monitoring, referral and counseling, delivery planning, the option of fetal intervention, and targeted postnatal management. Teams participating in the delivery room care of infants with known anomalies should be knowledgeable about specific needs and expectations but also ready for unexpected complications. A small number of neonates will need rapid access to postnatal interventions, such as surgery, but most can be stabilized with appropriate neonatal care. These targeted perinatal interventions have been shown to improve outcome in selected diagnoses.

Thoracoscopic repair of congenital diaphragmatic hernia (CDH) has been associated with faster recovery, earlier extubation, and decreased morbidity. Nevertheless, thoracoscopic repair is rarely attempted in the post-extracorporeal membrane oxygenation (ECMO) patient. Commonly cited reasons for not attempting thoracoscopy include concerns that the patients' respiratory status is too tenuous to tolerate insufflation pressures or that presumed defect size is so large that it precludes thoracoscopic repair. Our purpose is to review our experience with post-ECMO thoracoscopic CDH repair and evaluate the success of this approach.We performed retrospective analysis of attempted thoracoscopic CDH repairs after ECMO decannulation at our institution from 2001 to 2015. Primary outcome was rate of conversion. Secondary outcomes were intraoperative end-tidal COWe identified 21 post-ECMO patients in whom thoracoscopic CDH repair was attempted. Thoracoscopic repair was successfully completed in 28%. No patients had reported intolerance to insufflation at 3-7 mmHg. Average end-tidal COThoracoscopic CDH repair is both safe and feasible after ECMO with no increase in operative morbidity or mortality. Insufflation pressures of 3-7 mmHg are well tolerated without undue increase in end-tidal CO

BACKGROUND In addition to substantial medical and surgical intervention, neonates with congenital diaphragmatic hernia often have concurrent concerns for acquired infection. However, few studies focus on infection and corresponding antimicrobial utilization in this population. METHODS The Children's Hospital Neonatal Database was queried for congenital diaphragmatic hernia infants hospitalized from January 2010 to February 2016. Patient charts were linked to the Pediatric Health Information Systems database. Descriptive clinical data including delivery history, cultures sent, diagnosed infection, antimicrobial use and outcomes were reported. RESULTS A total of 1085 unique patients were identified after data linkages; 275 (25.3%) were born at

Extracorporeal membrane oxygenation (ECMO), a life-saving therapy for respiratory and cardiac failure, was first used in neonates in the 1970s. The indications and criteria for ECMO have changed over the years, but it continues to be an important option for those who have failed other medical therapies. Since the Extracorporeal Life Support Organization (ELSO) Registry was established in 1989, more than 29,900 neonates have been placed on ECMO for respiratory failure, with 84% surviving their ECMO course, and 73% surviving to discharge or transfer. In this chapter, we will review the basics of ECMO, patient characteristics and criteria, patient management, ECMO complications, special uses of neonatal ECMO, and patient outcomes.

Background Congenital H-type tracheoesophageal fistula is a rare type of congenital tracheoesophageal malformation. In this malformation, the esophagus remains unobstructed, complicating and delaying the diagnosis. We present our experience with three infants with congenital H-type tracheoesophageal fistula who were initially treated by a minimally invasive approach via endoscopy with electrocautery. Results Two of the three patients ultimately required a surgical procedure after failure to close the fistula via electrocautery despite multiple attempts over several months. One of the three patients was successfully treated via electrocautery after the second procedure. Conclusions In our center, three infants with congenital H-type tracheoesophageal fistula were initially treated endoscopically with electrocautery. Two out of the three ultimately required a surgical procedure. If considering endoscopy with electrocautery, providers and families should recognize that the procedure may need to be performed several times before the fistula is successfully closed. Additionally, failed attempts may put patients at risk for complications such as respiratory decompensation, pneumonia and poor growth and may prolong hospitalization.

Appropriate post-natal growth remains a mainstay of therapeutic goals for infants with CDH, with the hypothesis that optimizing linear growth will improve survival through functional improvements in pulmonary hypoplasia. However, descriptions of growth and the effect on survival are limited in affected infants.Describe in-hospital weight gain related to survival among infants with CDH.Children's Hospitals Neonatal Database (CHND) identified infants with CDH born ≥34weeks' gestation (2010-14). Exclusion criteria were: admission age7days, death/discharge age14days, or surgical CDH repair prior to admission. Weight gain velocity (WGV: g/kg/day) was calculated using an established exponential approximation and the cohort stratified by Q1:25%ile, Q2-3: 25-75%ile, and Q4:75%ile. Descriptive measures and unadjusted Kaplan-Meier analyses describe the implications of WGV on mortality/discharge.In 630 eligible infants, median WGV was 4.6g/kg/day. After stratification by WGV [Q1: (n=156;3.1g/kg/day); Q2-3 (n=316; 3.1-5.9g/kg/day), and Q4 (n=158,5.9g/kg/day)] infants in Q1 had shortest median length of stay, less time on TPN and intervention for gastro-esophageal reflux relative to the other WGV strata (p0.01 for all). Unadjusted survival estimates revealed that Q1 [hazard ratio (HR)=9.5, 95% CI: 5.7, 15.8] and Q4 [HR=2.9, 95% CI: 1.7, 5.1, p0.001 for both] WGV were strongly associated with NICU mortality relative to Q2-3 WGV.Variable WGV is evident in infants with CDH. Highest and lowest WGV appear to be related to adverse outcomes. Efforts are needed to develop nutritional strategies targeting optimal growth.

Geotrichum candidum is a saprophytic yeast known to colonize the human skin, respiratory tract and gastrointestinal tract. It can cause local or disseminated disease (geotrichosis), mainly in the immunocompromised host. Trauma, indwelling catheter use, prolonged broad-spectrum antibiotic treatment and critical illness have also been implicated as risk factors. Here we report the first case, to our knowledge, of cutaneous G. candidum infection in a burn patient. The isolate had a high amphotericin B minimum inhibitory concentration (MIC) and the patient experienced concomitant Candida orthopsilosis fungaemia, and so was treated with a combination of voriconazole and micafungin. This case highlights the importance of source control, rapid identification of G. candidum infection and MIC determination to guide antifungal therapy, which typically consists of amphotericin B with or without flucytosine or voriconazole alone. Clinicians should be aware of geotrichosis as a clinical entity in burn patients as well as in the immunocompromised. Antifungal resistance and breakthrough disease are an ongoing concern due to the increasing number of immunocompromised at-risk patients and the use of routine mould prophylaxis.

To predict mortality or length of stay (LOS)109 days (90th percentile) among infants with congenital diaphragmatic hernia (CDH).We conducted a retrospective analysis using the Children's Hospital Neonatal Database during 2010 to 2014. Infants born34 weeks gestation with CDH admitted at 22 participating regional neonatal intensive care units were included; patients who were repaired or were at home before admission were excluded. The primary outcome was death before discharge or LOS109 days. Factors associated with this outcome were used to develop a multivariable equation using 80% of the cohort. Validation was performed in the remaining 20% of infants.The median gestation and age at referral in this cohort (n=677) were 38 weeks and 6 h, respectively. The primary outcome occurred in 242 (35.7%) infants, and was distributed between mortality (n=180, 27%) and LOS109 days (n=66, 10%). Regression analyses showed that small for gestational age (odds ratio (OR) 2.5, P=0.008), presence of major birth anomalies (OR 5.9, P0.0001), 5- min Apgar score ⩽3 (OR 7.0, P=0.0002), gradient of acidosis at the time of referral (P0.001), the receipt of extracorporeal support (OR 8.4, P0.0001) and bloodstream infections (OR 2.2, P=0.004) were independently associated with death or LOS109 days. This model performed well in the validation cohort (area under curve (AUC)=0.856, goodness-of-fit (GF) χ(2), P=0.16) and acted similarly even after omitting extracorporeal support (AUC=0.82, GF χ(2), P=0.05).Six variables predicted death or LOS ⩾109 days in this large, contemporary cohort with CDH. These results can assist in risk adjustment for comparative benchmarking and for counseling affected families.

OBJECTIVE To describe the use of double-lumen venovenous (VVDL) extracorporeal membrane oxygenation (ECMO) with cephalic draining cannula (VVDL+V) as a primary approach for all neonatal respiratory diagnoses and to compare our single-center experience with data as collected in the Extracorporeal Life Support Organization (ELSO) database. STUDY DESIGN We retrospectively reviewed all cases of ECMO for neonatal respiratory failure performed in the neonatal intensive-care unit at a large referral children's hospital, the Children's Healthcare of Atlanta at Egleston (CHOA-E). Comparisons were then made to neonatal respiratory ECMO data retrieved from the ELSO database. RESULTS At CHOA-E 162 of 189 cases were completed with the VVDL+V approach. Survival in the VVDL+V cohort was 89.1% versus 68.7% from ELSO, P

To predict incident bloodstream infection and urinary tract infection (UTI) in infants with congenital diaphragmatic hernia (CDH).We conducted a retrospective analysis using the Children's Hospital Neonatal Database during 2010-2016. Infants with CDH admitted at 22 participating regional neonatal intensive care units were included; patients repaired or discharged to home prior to admission/referral were excluded. The primary outcome was death or the occurrence of bloodstream infection or UTI prior to discharge. Factors associated with this outcome were used to develop a multivariable equation using 80% of the cohort. Validation was performed in the remaining 20% of infants.Median gestation and postnatal age at referral in this cohort (n = 1085) were 38 weeks and 3.1 hours, respectively. The primary outcome occurred in 395 patients (36%); and was associated with low birth weight, low Apgar, low admission pH, renal and associated anomalies, patch repair, and extracorporeal membrane oxygenation (P .001 for all; area under receiver operating curve = 0.824; goodness of fit χInfants with CDH are at high risk of infection which was predicted by clinical factors. Early identification and low threshold for sepsis evaluations in high-risk infants may attenuate acquisition and the consequences of these infections.

Background: Necrotizing enterocolitis (NEC) is a leading contributor of neonatal morbidity and mortality. Few risk-prediction models exist to inform discussions with families before surgery or guide risk-adjustment for between center comparisons of surgical outcomes. Useful prediction models should be parsimonious, validated, well-calibrated, and include inputs using readily accessible pre-operative data. In addition, the performance of currently available prediction tools, including the Score for Neonatal Acute Physiology Perinatal Extension (SNAPPE-II), Vermont Oxford Risk Adjustment Tool (VON) and American College …

The aim of this study is to characterize medical and surgical therapies and short-term outcomes in infants with congenital diaphragmatic hernia (CDH).Retrospective analysis of CDH infants admitted to 27 children's hospitals submitting data to Children's Hospital Neonatal Database (CHND) from 2010 to 2013, stratified by gestational age, birth weight, and survival.A total of 572 infants were identified, 508 (89%) born ≥ 34 weeks' gestation and ≥ 2 kg. More mature infants had higher APGAR scores, shorter duration of mechanical ventilation, and were more likely to receive extracorporeal membrane oxygenation (ECMO). Overall, mortality for the cohort was 29%, with mortality lower in infants born ≥ 34 weeks' gestation and ≥ 2 kg (26 vs. 50%, p 0.01). Nonsurvivors were more likely to receive treatment with high-frequency oscillatory ventilation (HFOV), vasopressors, pulmonary vasodilators, and ECMO, and to have associated major congenital anomalies than survivors. In hospital morbidity and complications were relatively uncommon among survivors.Infants with CDH have a high risk of morbidity and mortality, and for preterm infants with CDH those risks are amplified. Patterns of respiratory and circulatory support appeared to be different for survivors. In addition to established data registries, this consortium of regional neonatal intensive care units provides a new collaborative effort to describe short-term outcomes for infants referred with CDH.

Two cases of devastating fetal malformations associated with vanished monochorionic twins were identified upon review of pathology files. A 35-year-old G1P0 woman and 36-year-old G3P1 woman were both diagnosed with an intrauterine twin gestation via transvaginal ultrasound at 10 weeks. The spectrum of fetal anomalies ranged from omphalocele, bilateral upper extremity, and unilateral lower extremity hypoplasia, to craniofacial malformation with diaphragmatic hernia. On histopathologic examination, the placentas demonstrated vascular anastomoses between the surviving co-twin and the “vanished” fetal sac. We propose anastomotic placental vasculature as a contributing factor to the observed fetal malformations. Additionally, genetic or teratogenic factors may have been attributed to the demise of the first twin and the anomalies seen in the other twin. While such instances are rare, they are important to consider when counseling patients regarding outcomes associated with a monochorionic vanished twin.

Objective To compare existing outcome prediction models and create a novel model to predict death or intestinal failure (IF) in infants with surgical necrotizing enterocolitis (NEC). Study design A retrospective, observational cohort study conducted in a 2-campus health system in Atlanta, Georgia, from September 2009 to May 2015. Participants included all infants ≤37 weeks of gestation with surgical NEC. Logistic regression was used to model the probability of death or IF, as a composite outcome, using preoperative variables defined by specifications from 3 existing prediction models: American College of Surgeons National Surgical Quality Improvement Program Pediatric, Score for Neonatal Acute Physiology Perinatal Extension, and Vermont Oxford Risk Adjustment Tool. A novel preoperative hybrid prediction model was also derived and validated against a patient cohort from a separate campus. Results Among 147 patients with surgical NEC, discrimination in predicting death or IF was greatest with American College of Surgeons National Surgical Quality Improvement Program Pediatric (area under the receiver operating characteristic curve [AUC], 0.84; 95% CI, 0.77-0.91) when compared with the Score for Neonatal Acute Physiology Perinatal Extension II (AUC, 0.60; 95% CI, 0.48-0.72) and Vermont Oxford Risk Adjustment Tool (AUC, 0.74; 95% CI, 0.65-0.83). A hybrid model was developed using 4 preoperative variables: the 1-minute Apgar score, inotrope use, mean blood pressure, and sepsis. The hybrid model AUC was 0.85 (95% CI, 0.78-0.92) in the derivation cohort and 0.77 (95% CI, 0.66-0.86) in the validation cohort. Conclusions Preoperative prediction of death or IF among infants with surgical NEC is possible using existing prediction tools and, to a greater extent, using a newly proposed 4-variable hybrid model.

We report a case of identical twins with lethal congenital pulmonary airway malformation (CPAM) type 0. Twin A expired several hours after birth, and twin B was sustained by extra-corporeal membrane oxygenation (ECMO) support; however, care was withdrawn from twin B following the autopsy of twin A, which revealed a diagnosis of CPAM type 0. Both twins showed pulmonary hypoplasia, histologically consistent with CPAM type 0 and pulmonary hypertension. Furthermore, the family also had a previous male who presented with pulmonary hypoplasia and respiratory failure and died shortly after birth; however, no autopsy was performed to confirm a diagnosis of CPAM. Here, in discussing our case, as well as previously reported cases, we demonstrate CPAM type 0's high prevalence among females (9:1 ratio). From the reported cases, it appears that CPAM type 0's tendency to recur in families is up to 40%, suggesting an autosomal recessive inheritance pattern. However, the actual tendency of familial recurrence is hard to assess due to the rarity of the disease and the potential lack of reporting CPAM type 0 cases. To our knowledge, our report represents the first description of CPAM type 0 in identical twins.

Importance Data regarding the contribution of red blood cell (RBC) transfusion and anemia to necrotizing enterocolitis (NEC) are conflicting. These associations have not been prospectively evaluated, accounting for repeated, time-varying exposures. Objective To determine the relationship between RBC transfusion, severe anemia, and NEC. Design, Setting, and Participants In a secondary, prospective, multicenter observational cohort study from January 2010 to February 2014, very low-birth-weight (VLBW, ≤1500 g) infants, within 5 days of birth, were enrolled at 3 level III neonatal intensive care units in Atlanta, Georgia. Two hospitals were academically affiliated and 1 was a community hospital. Infants received follow-up until 90 days, hospital discharge, transfer to a non–study-affiliated hospital, or death (whichever came first). Multivariable competing-risks Cox regression was used, including adjustment for birth weight, center, breastfeeding, illness severity, and duration of initial antibiotic treatment, to evaluate the association between RBC transfusion, severe anemia, and NEC. Exposures The primary exposure was RBC transfusion. The secondary exposure was severe anemia, defined a priori as a hemoglobin level of 8 g/dL or less. Both exposures were evaluated as time-varying covariates at weekly intervals. Main Outcomes and Measures Necrotizing enterocolitis, defined as Bell stage 2 or greater by preplanned adjudication. Mortality was evaluated as a competing risk. Results Of 600 VLBW infants enrolled, 598 were evaluated. Forty-four (7.4%) infants developed NEC. Thirty-two (5.4%) infants died (all cause). Fifty-three percent of infants (319) received a total of 1430 RBC transfusion exposures. The unadjusted cumulative incidence of NEC at week 8 among RBC transfusion-exposed infants was 9.9% (95% CI, 6.9%-14.2%) vs 4.6% (95% CI, 2.6%-8.0%) among those who were unexposed. In multivariable analysis, RBC transfusion in a given week was not significantly related to the rate of NEC (adjusted cause-specific hazard ratio, 0.44 [95% CI, 0.17-1.12]; P = .09). Based on evaluation of 4565 longitudinal measurements of hemoglobin (median, 7 per infant), the rate of NEC was significantly increased among VLBW infants with severe anemia in a given week compared with those who did not have severe anemia (adjusted cause-specific hazard ratio, 5.99 [95% CI, 2.00-18.0]; P = .001). Conclusions and Relevance Among VLBW infants, severe anemia, but not RBC transfusion, was associated with an increased risk of NEC. Further studies are needed to evaluate whether preventing severe anemia is more important than minimizing RBC transfusion.