Search

Introduction: Congenital diaphragmatic hernia is a complex disease associated with pulmonary hypoplasia and hypertension. Fetoscopic endoluminal tracheal occlusion (FETO) has been shown to improve survival and pulmonary hypertension, however, is associated with tracheomegaly. We aim to describe neonatal tracheomegaly, airway management, and outcomes following FETO., Methods: A single-center retrospective cohort review was performed for congenital diaphragmatic hernia patients who received FETO at our institution (4/12-6/22). Those with fetal demise, death at delivery, and those awaiting delivery were excluded. Demographics and perinatal outcomes were collected. Tracheal measurements were collected from initial postnatal chest x-ray by a single radiologist. Data were analyzed with descriptive analysis., Results: 34 patients underwent FETO with a median gestational age at a diagnosis of 23 wk [IQR 20-26] and at delivery of 36 wk [IQR 34-37]. Tracheomegaly was noted in 24 patients. The median maximum tracheal diameter was 9.9 mm [IQR 8.7-10.5]. All patients were intubated at birth. Ten (29%) eventually received a cuffed endotracheal tube (ETT), with 7/10 switched from an uncuffed ETT due to clinical concerns (i.e., large air leak) and 3/10 initially intubated with cuffed ETT. Nine (26%) patients underwent airway endoscopy, with two initially intubated with cuffed ETT compared to 7 with uncuffed ETT. A maximum of five endoscopies were performed on a single patient initially intubated with an uncuffed ETT, compared to 2 with cuffed ETT., Conclusions: Given the need for multiple reintubations and use of airway endoscopies following FETO in patients with and without tracheomegaly, the placement of larger or cuffed ETT may be considered in initial resuscitation of FETO patients., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Introduction: Necrotizing enterocolitis (NEC) is a severe inflammatory disease of the gastrointestinal tract and one of the most common life-threatening emergencies affecting newborns. Intestinal fatty acid-binding protein (I-FABP) has been used as a possible marker of intestinal damage in NEC. We aimed to carry out a scoping review of all publications that explore the role of I-FABP in NEC to inspire new research into the potential utility of I-FABP as a marker of NEC., Methods: We searched for relevant publications using the keywords "necrotizing enterocolitis," "intestinal fatty acid binding protein," "NEC," and "I-FABP" in the National Library of Medicine (PubMed/MEDLINE), Embase, SCOPUS, and Web of Science. Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews was used for reporting findings., Results: We identified 61 relevant articles, which we divided into clinical (n = 47) and experimental (n = 14) groups., Conclusions: I-FABP is a promising marker of NEC, especially for NEC stage 2 and 3. Urinary I-FABP follows the same patterns as serum and plasma I-FABP. The definitive roles of I-FABP in early diagnosis of NEC, differential diagnosis in breast feeding, alimentary intolerance, and screening of surgical NEC need clarification and remain a challenge to investigators., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Introduction: Traditionally, gestational age <34 wk and weight <2 kg are considered relative contraindications to extracorporeal membrane oxygenation (ECMO). There is a paucity of information that explains the outcomes in this unique population of premature neonates. The purpose of this study is to examine outcomes of patients who undergo ECMO at <34 wk at a single institution., Methods: A single-center retrospective review was performed for neonates managed with ECMO in the neonatal intensive care unit from January 2012 to April 2022. Characteristics and outcome data were collected. The primary outcome studied was survival at discharge. Secondary outcomes were intraventricular hemorrhage, ischemic brain injury, and thrombosis. Data were analyzed with descriptive statistics., Results: Following exclusion, 107 patients were included with eight having initiating ECMO at <34 wk. Three (38%) patients, who received ECMO at <34 wk, incurred intraventricular hemorrhages compared to 14 (14%) in the ≥34-wk cohort. Two (25%), who underwent ECMO at <34 wk, exhibited signs of brain ischemia on imaging compared to 9 (9%) in those ≥34 wk, and 3 (38%) patients <34 wk experienced thrombosis compared to 31 (31%) in the ≥34-wk cohort. Five (63%) of those in the <34-wk cohort survived to discharge, similar to 61 (61%) in the ≥34 wk cohort., Conclusions: Our data suggest that EGA <34 wk may not be a contraindication for ECMO, with appropriate counseling of potential risks., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Introduction: Congenital diaphragmatic hernia (CDH) is a complex pathology with severe pulmonary morbidity. Administration of surfactant in CDH is controversial, and the advent of fetoscopic endoluminal tracheal occlusion (FETO) has added further complexity. While FETO has been shown to improve survival outcomes, there are risks of prematurity and potential surfactant deficiency. We aim to evaluate the characteristics and outcomes of surfactant administration for CDH infants and elucidate potential benefits or risks in this unique population., Methods: A single-center retrospective cohort review of patients with unilateral CDH from September 2015 to July 2022 was performed. Demographics, prognostic perinatal imaging features, and outcomes were collected. Patients were stratified by surfactant administration and history of FETO. Data were analyzed with descriptive statistics, two-sample t-tests, chi-squared analyses, and logistic regression., Results: Of 105 included patients, 19 (18%) underwent FETO and 25 (24%) received surfactant. Overall, surfactant recipients were born at earlier gestational ages and lower birthweights regardless of FETO history. Surfactant recipients possessed significantly worse prenatal prognostic features such as observed to expected total fetal lung volume, observed to expected lung to head ratio, and percent liver herniation. In CDH patients without FETO history, surfactant recipients demonstrated worse outcomes than nonrecipients. This association is notably absent in the FETO population, where surfactant recipients have more favorable survival and comparable outcomes. When controlling for defect severity or surfactant usage, as a proxy for respiratory status, surfactant recipients that underwent FETO trended toward improved survival and decreased ECMO use., Conclusions: Surfactant administration is not associated with increased morbidity and mortality and may be beneficial in CDH patients that have undergone FETO., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: We compared strategy outcomes and financial impact over the first two years of life (F2YOL) for patients with giant omphaloceles undergoing early repair (ER) (primary or staged) versus delayed repair (DR)., Methods: A retrospective review of giant omphaloceles (fascial defect > 5 cm/> 50% liver herniation) at a tertiary children's hospital between 1/1/2010 and 12/31/2019 was performed. Survival, length of stay, age at repair, ventilation days (VD), time to full enteral feeds, readmissions during the F2YOL, incidence of major associated anomalies, and total hospitalization charges during the F2YOL were compared. A subanalysis removing potential confounders and only including patients who underwent fascial closure within the F2YOL was also conducted., Results: Thirty four giant omphaloceles (23DR and 11ER) were identified. The median age (days) at repair was 289 [148, 399] DR versus 10 [5, 21] ER, P < 0.001. Total cohort two-year survival was significantly higher in the DR group (95.7% versus 63.6%, P = 0.03). Including patients with a tracheostomy there was no significant difference in VD during the index hospitalization. Excluding tracheostomy patients, the DR group had significantly fewer VD during the index hospitalization, 15 [0, 15] versus 18 [10, 54], P = 0.02 and over the F2YOL 6.5 [ 0, 21] versus 18 [14, 43], P = 0.03. There were no significant differences in the incidence/type of major associated anomalies, time to full enteral feeds, index length of stay, total hospital days, total admissions, or associated hospital charges. On subanalysis, there was no significant difference in VD or survival at any time., Conclusions: Delayed and early repair strategies for giant omphaloceles have equivalent outcomes in the index hospitalization and over the course of the first two years of life. These findings are useful for family counseling and expectation setting., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: In pediatric and neonatal populations, the carotid artery is commonly cannulated for venoarterial (VA) extracorporeal membrane oxygenation (ECMO). The decision to ligate (carotid artery ligation [CAL]) versus reconstruct (carotid artery reconstruction [CAR]) the artery at decannulation remains controversial as long-term neurologic outcomes remain unknown. The objective of this study was to summarize current literature on clinical outcomes following CAL and CAR after Venoarterial Extracorporeal Membrane Oxygenation (VA-ECMO)., Methods: PubMed (MEDLINE), Embase, Web of Science, and Cochrane databases were searched using keywords from January 1950 to October 2020. Studies examining clinical outcomes following CAL and CAR for VA-ECMO in patients <18 y of age were included. Prospective and retrospective cohort studies, case series, case-control studies, and case reports were included. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were performed independently by two reviewers. Assessment of risk of bias was performed., Results: Eighty studies were included and classified into four categories: noncomparative clinical outcomes after CAL (n = 23, 28.8%), noncomparative clinical outcomes after CAR (n = 12, 15.0%), comparative clinical outcomes after CAL and/or CAR (n = 28, 35.0%), and case reports of clinical outcomes after CAL and/or CAR (n = 17, 21.3%). Follow-up ranged from 0 to 11 y. CAR patency rates ranged from 44 to 100%. There was no substantial evidence supporting an association between CAL versus CAR and short-term neurologic outcomes., Conclusions: Studies evaluating outcomes after CAL versus CAR for VA-ECMO are heterogeneous with limited generalizability. Further studies are needed to evaluate long-term consequences of CAL versus CAR, especially as the first survivors of pediatric/neonatal ECMO approach an age of increased risk of carotid stenosis and stroke., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: It has been reported that pediatric patients experienced a delay in treatment for acute appendicitis during the pandemic, resulting in increased rates of complicated appendicitis. We investigated the association of the COVID-19 pandemic and the incidence and severity of acute appendicitis among pediatric Medicaid patients using a population-based approach., Methods: The claims database of Partners For Kids, a pediatric Medicaid accountable care organization (ACO) in Ohio, was queried for cases of acute appendicitis from April to August 2017-2020. The monthly rate of acute appendicitis/100,000 covered lives was calculated each year and compared over time. Rates of complicated appendicitis were also compared. Diagnosis code validation for classification as complicated or uncomplicated appendicitis was performed for patients treated at our hospital., Results: During the study period, 465 unique cases of acute appendicitis were identified. Forty percent (186/465) were coded as complicated. No significant difference in the incidence of acute appendicitis cases was observed across the 4 y, either in an overall comparison or in pairwise comparisons (P > 0.15 for all). The proportion of acute appendicitis cases that were coded as complicated did vary significantly over the 4-year study period (P = 0.005); this was due to this proportion being significantly higher in 2018 than in either 2019 (P = 0.005 versus 2018) or 2020 (P = 0.03 versus 2018)., Conclusions: The COVID-19 pandemic was not associated with reduced access to treatment for acute appendicitis among patients in a pediatric Medicaid ACO. This suggests that an ACO may promote continued healthcare access for their covered population during an unexpected crisis., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: This study's purpose was to (1)determine the effect of locally administered serum amyloid P (SAP) on the development of hypertrophic scars (HTS) in porcine and rabbit HTS models and (2)determine the pharmacokinetics of systemically administered SAP and its effect on circulating fibrocyte quantities., Methods: Two large animal (New Zealand White Rabbit and Female Red Duroc Pigs) HTS models were utilized to study the effects of daily local injections of SAP immediately post wounding (x5 d in rabbits; x7 d in pigs) on HTS development as measured by scar elevation index , scar area, wound closure, and molecular expression studies of scar components. For SAP pharmacokinetics, total and human SAP levels in porcine blood were measured at regular intervals following intravenous administration of human SAP. Fibrocyte quantities were determined prior to and 1 h following human SAP intravenous administration., Results: In the rabbit model, local SAP significantly decreased the level of tissue inhibitor of metalloproteinases-1 mRNA expression and maintained matrix mettaloproteinase-9 expression, while control and vehicle groups significantly declined. In the pig model, there was a significant decrease in the trend of scar elevation indexes treated with local SAP versus controls over the study period. This decrease was statistically significant at days 14 and 84. Human SAP administered intravenously is degraded within 24 h and does not influence circulating fibrocyte quantities., Conclusions: This is the first study to demonstrate attenuation of HTS formation using locally administered SAP in large animal HTS models. Local SAP administration reduces HTS formation by maintaining matrix mettaloproteinase-9 and decreasing tissue inhibitor of metalloproteinases-1. Intravenous administration of SAP is not as effective., (Copyright © 2023. Published by Elsevier Inc.)

Introduction: Certain congenital cardiac lesions are at increased risk for the development of necrotizing enterocolitis (NEC). These patients are often reliant on pulmonary and systemic vasomodulators to maintain adequate perfusion and oxygenation. This study sought to determine whether pulmonary or systemic vasodilator treatment is protective against the development of NEC in this population., Methods: We utilized International Classification of Diseases (ICD) codes to identify high risk congenital cardiac disease patients ≤6 mo of age, cared for at a tertiary children's hospital between January 2011 and January 2021. Cardiac anomalies were stratified into ductal dependent (pulmonary DD-P or systemic DD-S) or independent lesions. The rate of NEC development in those who received vasodilators (inhaled nitric oxide [iNO], pulmonary vasodilators, systemic vasodilators) was compared to controls in a multivariate analysis., Results: Of the 352 patients, who met inclusion criteria, 77.6% had ductal dependent lesions (DD-S 41.9%, DD-P 35.7%), 19.5% received iNO, and 37.5% received other vasodilatory drugs. The overall NEC rate was 15.1%. On univariate analysis, DD-S, iNO use, and systemic vasodilators was associated with a significantly higher risk of NEC, while DD-P was associated with lower NEC risk. On multivariate analysis, only iNO (odds ratio 2.725, confidence interval [1.36-5.44]) and DD-S (odds ratio 2.279, confidence interval [1.02-5.11]) were independent risk factors for NEC., Conclusions: In patients with at-risk congenital cardiac disease lesions, a ductus dependent systemic circulation or iNO treatment is associated with an increased risk of developing NEC. The presence of iNO or DD-S should be utilized as markers of increased risk both in the prevention and workup of suspected NEC., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: Interleukin-10 (IL-10) is essential in fetal regenerative wound healing and likewise promotes a regenerative phenotype in adult dermal wounds. However, the role of endogenous IL-10 in postnatal dermal wound healing is not well-established. We sought to determine the function of endogenous IL-10 in murine full thickness excisional wounds that are splinted to prevent contracture and mimic human patterns of wound closure., Methods: Full-thickness excisional wounds were made in wildtype (WT) and IL-10 -/- mice on a C57BL/6J background (F/M, 8 wk old). In a subset of wounds, contraction was prevented by splinting with silicone stents (stenting) and maintaining a moist wound microenvironment using a semiocclusive dressing. Wounds were examined for re-epithelialization, granulation tissue deposition, and inflammatory cell infiltrate at day 7 and fibrosis and scarring at day 30 postwounding., Results: We observed no difference in wound healing rate between WT and IL-10 -/- mice in either the stented or unstented group. At day 7, unstented IL-10 -/- wounds had a larger granulation tissue area and more inflammatory infiltrate than their WT counterparts. However, we did observe more F4/80 + cell infiltrate in stented IL-10 -/- wounds at day 7. At day 30, stented wounds had increased scar area and epithelial thickness compared to unstented wounds., Conclusions: These data suggest that endogenous IL-10 expression does not alter closure of full thickness excisional wounds when wound hydration and excessive contraction of murine skin are controlled. However, the loss of IL-10 leads to increased inflammatory cell infiltration and scarring. These new findings suggest that IL-10 contributes to the regulation of inflammation without compromising the healing response. These data combined with previous reports of increased rates of healing in IL-10 -/- mice wounds not controlled for hydration and contraction suggest an important role for murine wound healing models used in research studies of molecular mechanisms that regulate healing., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Introduction: Covered abdominal wall defects (CAWD) can be categorized into giant omphaloceles (GOs), nongiant omphaloceles (NGOs), and umbilical cord hernias (UCHs). We sought to evaluate differences in management and outcomes of the different CAWD, treated at a large tertiary children's hospital, with regards to survival and association with other major congenital anomalies., Methods: A retrospective review of CAWD patients between January 2010 and January 2021 was conducted. GO was defined as a fascial defect >5 cm or >50% liver herniation. UCH were defined as fascial defects ≤ 2 cm. All others were classified as NGO. Type of repair, time to fascial closure, index hospitalization length of stay (LOS), and survival rates were compared. Four major anomaly categories were identified: cardiac, midline, Beckwith-Weidemann Syndrome, and other genetic anomalies., Results: We identified 105 CAWD patients (UCH n = 40; GO n = 34; and NGO n = 31). Ninety percent of UCH underwent primary repair, 10% were never repaired. NGOs were repaired by primary or staged methods in 92.9% of cases and 7.1% by delayed repair. Primary or staged repair occurred in 32.4% of GOs and delayed repair occurred in 67.6%. The median days to repair was 181 [24,427] GO, 1 [1,3] NGO, and 1 [0,1] UCHs (P < 0.01). Index hospitalization median LOS (days) was 90 [55,157] GO, 23 [10,48] NGO, 9 [5,22] UCH, (P < 0.01). There were no statistical differences in survival rates, number of patients with major anomalies (GO 35.4%, NGO 51.5%, UCH 50%), or types of anomalies., Conclusions: UCHs and omphaloceles have similar incidences of major associated anomalies. Thus, all patients with a covered abdominal wall defect should undergo workup for associated anomalies., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Introduction: We sought to investigate whether hydroxyurea therapy is associated with the need for surgical splenectomy among patients with sickle cell disease (SCD). We hypothesized that as hydroxyurea gained widespread use, surgical splenectomy among pediatric patients with SCD occurred at a higher rate and older age among those taking hydroxyurea., Methods: In this retrospective cross-sectional study, the Pediatric Health Information System was queried for all SCD International Classification of Diseases 9/10 diagnosis codes and splenectomy procedure codes from January 1, 2005, to December 31, 2020. Hydroxyurea use was defined as at least one hospital admission with hydroxyurea listed as a medication. The rates of surgical splenectomy, age at splenectomy, hospital length of stay, and incidence of blood transfusion during the splenectomy admission were compared among patients receiving hydroxyurea versus those not receiving hydroxyurea. Additional subanalysis was performed in the Hemoglobin-SS, Hemoglobin-SC, and Other cohorts separately., Results: During the study period, 28,520 patients were identified. All patients with SCD receiving hydroxyurea had a significantly higher rate of surgical splenectomy compared with the nontreatment group (7.2% versus 3.2%, P = 0.01). The age at surgical splenectomy was significantly younger among Hemoglobin-SS patients receiving hydroxyurea (5.7 [5.1, 6.4] y versus 6.6 [5.8, 7.4] y; P < 0.01). There were no significant differences in length of stay or incidence of blood transfusion during the surgical splenectomy admission between treatment groups., Conclusions: Hydroxyurea use in children is associated with higher rates of surgical splenectomy and occurs at a younger age in the Hemoglobin-SS population. Although these findings warrant further investigation for causality, it provides useful information to clinicians and patients alike, allowing for more informed decision-making., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Introduction: Congenital diaphragmatic hernia is associated with pulmonary hypoplasia, pulmonary hypertension, and significant neonatal morbidity. Although intrathoracic liver herniation (LH) >20% is associated with adverse outcomes, the relationship between LH <20% and outcomes is poorly characterized., Methods: A single-center retrospective cohort study was performed from 2011 to 2020 of 80 fetuses with left-sided congenital diaphragmatic hernia that were delivered and repaired at our institution. Perinatal, perioperative, and postoperative data were collected. We evaluated the association of %LH with outcomes as a stratified ordinal variable (0%-10% LH, 10%-19% LH, and >20% LH) and as a continuous variable. Data were analyzed by analysis of variance with Bonferroni post hoc analysis, chi-square analyses, and univariate logistic regression., Results: Extracorporeal membrane oxygenation (ECMO) (P < 0.001), repair on ECMO (P = 0.002), repair with patch (P < 0.001), length of stay (P = 0.002), inhaled nitric oxide use (P < 0.001), and sildenafil use at discharge (P < 0.001), showed significant differences among LH groups. There were no differences among the groups concerning survival (at discharge, 6 mo, and 1 y) and tracheostomy. On further analysis there was no difference between 10% and 19% LH and ≥20% LH patients concerning ECMO (P = 0.55), repair on ECMO (P = 0.54), repair with patch (P = 1.00), length of stay (P = 1.00), and inhaled nitric oxide use (P = 0.33). Logistic regression analysis displayed a significant association with LH and ECMO, repair on ECMO, repair with patch, inhaled nitric oxide use, and sildenafil use., Conclusions: Our analysis displays no significant difference in perinatal management between patients with 10%-19% and ≥20% LH. These findings suggest that the historical cutoff of ≥20% LH may not be sufficient alone to guide perinatal counseling and decision-making., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Introduction: The purpose of the present study is to evaluate our institutional management of high-risk congenital lung malformations (CLM) with particular consideration of the use of multiple maternal steroid courses and maternal steroids in CLMs with pathologies other than congenital pulmonary airway malformation (CPAM)., Methods: A single-center retrospective review was performed for all fetuses evaluated for CLM who received maternal steroids and/or had a CLM volume ratio (CVR) ≥ 1.6 (2015-2020). Fetuses were categorized as receiving no steroids, single steroid, or multiple steroid courses. Outcomes evaluated included CVR growth rate, resolution of early hydrops, and resolution of hydrops. Results are reported with a descriptive analysis., Results: Nineteen patients were identified who had CVR ≥ 1.6 (single steroid course 9/19, multiple steroid courses 6/19, and no steroids 4/19). A majority (n = 13, 68%) of all lesions had a reduction or no change in CVR between initial and final measurements (single steroid course 7/9, 78%; multiple steroid courses 4/6, 67%). When evaluating by pathology, ≥ 50% of each classification had reduction or no growth of CVR (CPAM 7/11, bronchial atresia 2/4, sequestration 3/3, congenital lobar emphysema 1/1). Seventy five percent (3/4) of lesions with early hydrops had resolution following steroid treatment (single steroid course 1, multiple steroid courses 2). Of the four lesions that had hydrops, only one had resolution after receiving multiple steroid courses., Conclusions: Our institutional experience reports the majority of CLM (including pathologies other than CPAM) who received steroids had reduction or no change in CVR. Given the low risk-benefit ratio of maternal steroids, physicians could consider use of multiple steroid courses for CLM refractory to a single course., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Congenital lung malformations (CLMs) have a variable natural history: some patients require urgent perinatal surgical intervention (UPSI) and others remain asymptomatic. These lesions have potential growth until 26-28 wk gestation. CLM volume ratio (CVR) has been shown to predict the risk of hydrops in CLMs. However, no criteria exist to delineate lesions requiring urgent surgical intervention in the perinatal period. Our goal was to determine prenatal diagnostic features that predict the need for UPSI in patients diagnosed with CLM., Methods: Records and imaging features of all fetuses evaluated by our fetal center between May 2015 and December 2018 were retrospectively reviewed. Data included demographics, fetal ultrasound and magnetic resonance imaging, CVR, surgical treatment, and outcome. Features were analyzed for their ability to predict the need for UPSI., Results: Sixty-four patients were referred for CLM, with 48 patients serially followed. Nine (18.8%) patients were followed nonoperatively, 35 (72.9%) underwent resection, and four (8.3%) were lost to follow-up. Of the patients who underwent resection, 24 (68.5%) were electively resected and 11 were urgently resected. Five (14.3%) patients underwent ex utero intrapartum treatment resection, and six (17.1%) were urgently resected for symptomatic CLM. There were no cases of UPSI with final CVR <1.1. Of the patients with final CVR 1.1-1.7, 43% required urgent resection. CVR ≥1.1 has 100% sensitivity and 87.8% specificity to predict patients requiring UPSI (area under the curve of 0.98)., Conclusions: A final CVR ≥1.1 is highly predictive for UPSI. Patients with a final CVR ≥1.1 should be referred for delivery at centers with pediatric surgeons equipped for potential UPSI for CLM., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: The purpose of this study was to evaluate our experience with pentalogy of Cantrell and the various embryologic variants., Materials and Methods: Patient charts and diagnostic imaging studies of all fetuses evaluated at Texas Children's Fetal Center for pentalogy of Cantrell between April 2004 and June 2014 were reviewed retrospectively. Data collected from patient charts included demographic information, clinical presentation, fetal and postnatal imaging findings, operative treatment, pathologic evaluation, and outcomes., Results: There were 10 patients who presented with embryologic variants of pentalogy of Cantrell over a 6-y period. Two cases displayed the full range of embryologic defects observed, and eight cases exhibited variants of the classic pentalogy. Sternal and pericardial defects were each present in 40% of patients. Additional anomalies present included pulmonary hypoplasia, pulmonary artery stenosis, and chromosomal abnormalities. Four patients presented with diaphragmatic defects but no defect in the pericardium, and one patient presented with a defective pericardium but no associated diaphragmatic defect, suggesting highly specific losses of somatic mesoderm during embryologic development. One patient was lost to follow-up, and a second patient underwent termination of pregnancy. Five of the remaining eight patients survived, one of which had the full range of embryologic defects and now attends preschool but requires speech and occupational therapy. The remaining surviving patients have developed without serious sequelae., Conclusions: This report highlights the spectrum of anomalies observed in the pentalogy of Cantrell and demonstrates that these fetuses can survive but with substantial morbidity., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: Studies comparing outcomes of right- and left-sided congenital diaphragmatic hernia (R-CDH and L-CDH) have yielded conflicting results. We hypothesized that R-CDH is associated with higher short-term pulmonary morbidity than L-CDH., Methods: We reviewed all CDH patients at a tertiary children's hospital over 10 y. In prenatally diagnosed CDH, the observed-to-expected total fetal lung volume and percentage liver herniation (%LH) were calculated using fetal magnetic resonance imaging-based measurements. Outcomes were compared in patients with isolated CDH. Patients were subsequently matched by %LH to compare outcomes., Results: Of 189 CDH patients, 37 (20.1 %) were R-CDH and 147 (79.9%) were L-CDH. Those with R-CDH were prenatally diagnosed at a significantly lower rate (40.5% versus 73.5%; P < 0.001) and later gestational age (26.5 ± 7.7 versus 22.6 ± 5.65 wk; P = 0.062). There was no difference in observed-to-expected total fetal lung volume between those with R-CDH and L-CDH (30.2 ± 11.1% versus 33.1 ± 14.2%; P = 0.471). Fetuses with R-CDH had a higher %LH than those with L-CDH (37.5 ± 14.1% versus 18.6 ± 12.2%; P < 0.001). Patients with isolated R-CDH had a higher need for extracorporeal membrane oxygenation than L-CDH (48% versus 27%; P = 0.055). There was no difference in duration of tracheal intubation, hospital stay, need for supplemental oxygen at 30-d of life or 6-mo mortality between groups. There was no difference in mortality and pulmonary morbidity when patients were matched by %LH., Conclusions: Compared to those with L-CDH, fetuses with R-CDH are less likely to be diagnosed prenatally and have a higher need for extracorporeal membrane oxygenation. The sidedness of the hernia defect was not associated with differences in short-term pulmonary morbidity in this large, contemporary single-institution experience of neonates with CDH., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: The purpose of this study was to describe the current management and outcomes of infants with omphalocele., Methods: The medical records of all patients treated for omphalocele at a large children's hospital from January, 2003-February, 2014 were reviewed. Patients were classified as having an isolated omphalocele or omphalocele with minor or major associated anomalies. Prenatal data collected included fetal magnetic resonance imaging-based observed-to-expected total fetal lung volumes. Giant omphalocele (GO) was defined as >50% of liver in the omphalocele sac., Results: Of 95 patients, 59 presented prenatally and had comprehensive fetal center evaluation. Of 82 live-born infants, 21 had chromosomal and 25 had major associated anomalies. No live-born baby with an isolated defect (n = 19) died, whereas mortality was 41% and 17% for those with major and minor anomalies, respectively (P = 0.006). Infants with major anomalies had significantly longer median length of intubation (36 versus 0 versus 0 d; P = 0.04) and hospital stay (157 versus 28.5 versus 18 d; P < 0.001) compared with those with minor or no anomalies. Of 40 infants with GO, the majority (85%) were managed surgically by delayed closure with a median age at repair of 10 mo (range, 3.4-23.6 mo). Six-month survival was 80%. None of the delayed repair patients required a later operative revision, whereas 2 of 5 with early repair did., Conclusions: The presence of associated anomalies is the strongest predictor of morbidity and mortality in fetuses or neonates with omphalocele. In patients with GO, delayed closure is associated with good outcomes, but larger, prospective studies comparing delayed to early closure are needed to determine the optimal timing of repair., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: Previous studies of infants with esophageal atresia (EA) suggest those diagnosed prenatally have worse outcomes because of a higher incidence of associated anomalies. The purpose of this study was to compare characteristics and outcomes of infants with EA diagnosed after fetal center evaluation to those diagnosed postnatally., Methods: The records of all neonates treated for EA at our institution from 2002-2012 were reviewed. Infants with a prenatal diagnosis of EA were compared with those postnatally diagnosed using chi-square and Student t-test as appropriate., Results: Of 91 patients treated with EA during the study period, 15 (16%) were diagnosed prenatally at our fetal center. Although those prenatally diagnosed had a higher incidence of pure EA and polyhydramnios, the gestational age and birth weight in that group were similar to those diagnosed postnatally. There were no differences in outcomes between groups with regard to the incidence of major cardiac anomalies, surgical complications, hospital length of stay, and survival., Conclusions: Treatment at a tertiary care center provides excellent outcomes for all infants with EA, despite an 80% frequency of concurrent anomalies. Prenatal diagnosis of EA and attentive obstetric management of polyhydramnios decrease the risk for prematurity and prematurity-associated morbidity., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Purpose: Despite improvements in the management of persistent pulmonary hypertension of the newborn (PPHN), a number of infants with inadequate gas exchange are treated with extracorporeal membrane oxygenation (ECMO). The objectives of this study were to use the Extracorporeal Life Support Organization Registry to review the outcomes of neonates with PPHN receiving ECMO, and to identify pre-ECMO variables that may be associated with increased mortality., Materials and Methods: The study is a retrospective analysis of all patients with PPHN supported with ECMO and reported to the Extracorporeal Life Support Organization registry from 2000 to 2010. Prematurity was defined as <37 wk gestation. Univariate analysis was performed using Student's t-test or Fisher's exact test. Variables found to be statistically significant underwent multivariate analysis by logistic regression. Kaplan-Meier survival curves were generated to analyze the relationship between duration of ECMO support and patient survival., Results: A total of 1569 neonates with PPHN received ECMO support during the study period, at an average age of 3.1 d of life and for a duration of 6.9 d. Survival among neonates with PPHN receiving ECMO support was 81%, and those receiving support for 7, 10, 14, and 21 d survived at rates of 88%, 78%, 55%, and 25%, respectively. By logistic regression, prematurity (P < 0.01), pre-ECMO pH ≤7.2 (P = 0.02), pre-ECMO SaO(2) ≤65% (P = 0.01), and duration of ECMO ≥7 d (P < 0.001) were independent predictors of death in this group. An average of 2.2 complications occurred per patient, with cardiovascular, mechanical, and renal complications being the most common., Conclusions: Neonates with PPHN have high survival rates with ECMO support. Prematurity, acidosis, and profound hypoxemia are independently associated with increased mortality. Furthermore, prolonged ECMO support (>7 d) is associated with a higher risk of mortality in this cohort than in patients supported for <1 wk., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: Intercellular adhesion molecule-1 (ICAM-1) permits leukocyte-endothelial adhesion and transmigration during inflammation. Membrane-bound ICAM-1 knockout mice have been used to understand this molecule's role in wound-healing, but expressed spliced isoforms of ICAM-1 that may have impacted results. We aimed to characterize wound-healing in an ICAM-1 null model devoid of all ICAM-1 isoforms., Methods: Full-thickness 8-mm wounds were created on C57/BL6 wild-type (n = 24) and ICAM-1 null (n = 24) mice. Wound area was calculated using daily photographs. Histologic samples were harvested on postoperative Days 1, 3, 7, and 14. Wound margins were evaluated for mRNA expression of 13 inflammatory cytokines. A separate group of wild-type and ICAM-1 null mice (n = 24) received full-thickness incisions with tensiometry measured at Day 14. Separately, complete blood counts were measured in unwounded wild-type (n = 4) and ICAM-1 null mice (n = 4)., Results: Wound-closure was significantly delayed in ICAM-1 null mice through Day 7 by gross and histologic measurement. mRNA expression of VEGF-A was increased in ICAM-1 null mice on Day 3, although no increase in VEGF-A was observed in the wound bed by immunohistochemistry. ICAM-1 null wounds demonstrated higher stiffness by tensiometry on Day 14 compared to the wild-type (1880 ± 926 kPa versus 478 ± 117 kPa; P < 0.01), and had higher counts of white blood cells (10,009 versus 5720 cells/μL, P < 0.05), neutrophils (2130 versus 630 cells/μL, P < 0.01), and lymphocytes (7130 versus 4,740 cells/μL, P < 0.05)., Conclusions: ICAM-1 null mice demonstrate delayed wound-healing and decreased wound elasticity compared to wild-type controls. This lag, however, was less than observed in earlier membrane-bound ICAM-1 knockouts, suggesting that other ICAM-1 isoforms may promote delayed wound-healing., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: As the Fundamentals of Research and Career Development Course (FRCDC) is conducted internationally, questions have arisen regarding the cultural appropriateness of the United States (US) course. We therefore assessed the US-based teaching methodology during the FRCDC in Abuja, Nigeria. We hypothesized that the US-based instructional methods would be effective., Methods: Twenty questions were distributed to attendees of the FRCDC prior to commencement. The same 20 questions were administered at the conclusion of the course after random reordering. Differences between the pre- and post-test results were assessed for normalcy and compared using the paired t-test., Results: There were 89 attendees, of whom 60 completed the pre-test and 77 completed the post-test. The pre-test group answered 12.3 ± 2.6 questions correctly, which improved to 15.0 ± 2.6 in the post-test group (P < 0.001). On the pre-test, the least common correct answers were for questions regarding type 1 and 2 error (16.7% correct), the definition of health services and outcomes research (26.7%), and how to best address missing data (26.7%). On the post-test, the questions with the least common correct answers were regarding the definition of health services and outcomes research (35%), and the components of an NIH grant (37.7%)., Conclusions: Our results suggest that the FRCDC in Nigeria as given by US faculty has short-term efficacy. Attendees were able to improve their scores despite the cultural differences between them and the lecturers. Our next goal will be to demonstrate long-term efficacy at future courses in the region using similar questionnaire strategies., (Copyright © 2010 Elsevier Inc. All rights reserved.)