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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: 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: 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: 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.)

Objective: To study the role of fetal endoscopic tracheal occlusion (FETO) on resolution of pulmonary hypertension (PH) in fetuses with isolated moderate left-sided diaphragmatic hernia (CDH)., Methods: This retrospective study included fetuses with CDH evaluated between February 2004 and July 2017. Using the tracheal occlusion to accelerate lung growth (TOTAL) trial definition, we classified fetuses into moderate left CDH if O/E-LHR (observed/expected-lung head ratio) was 25-34.9% regardless of liver position or O/E-LHR of 35-44.9% if liver was in the chest. Postnatal echocardiograms were used to diagnose PH. Logistic regression analyses were performed to determine the relationship of FETO with study outcomes., Results: Of 184 cases with no other major anomalies, 30 (16%) met criteria. There were nine FETO and 21 non-FETO cases. By hospital discharge, a higher proportion of infants in the FETO group had resolution of PH (87.5 (7/8) vs. 40% (8/20); p =.013). FETO was associated with adjusted odds ratio of 17.3 (95% CI: 1.75-171; p =.015) to resolve PH by hospital discharge. No significant differences were noted in need for ECMO or survival to discharge between groups., Conclusions: Infants with moderate left-sided CDH according to O/E-LHR, FETO is associated with resolution of PH by the time of hospital discharge.

Significance: This body of work gives a concise and comprehensive overview for the clinician and scientist on the latest treatment modalities for hypertrophic scars (HTS) and keloids in the pediatric population, as well as the most promising methods of prevention currently being investigated. This review will serve as a guide to the clinician for treatment selection and as an efficient tool for the scientist to achieve a comprehensive overview of the scientific literature to guide their future experiments aimed at pathologic scar prevention. Recent Advances: Current studies in the literature suggest carbon dioxide (CO 2 ) laser and E-light (bipolar radiofrequency, intense pulsed light, and cooling) are two of the most effective treatment modalities for HTS, while surgical excision+CO 2 laser+triamcinolone injection was one of the most successful treatments for keloids. In animal models, drug impregnated electrospun nanofiber dressings offer encouraging results for HTS prevention, while Kelulut honey showed promising results for keloid prevention. Critical Issues: Treatment outcome reproducibility is hindered by small cohorts of patients, inadequate-follow up, and variability in assessment tools. Prevention studies show multiple ways of achieving the same result, yet fall short of complete prevention. Furthermore, some studies that have purported full prevention have not been validated. Future Directions: To establish a standard of care, large clinical trials of the most successful modalities in small cohorts are needed. The key for prevention will be validation in animal models of the most successful methods, followed by translational and clinical studies.

Purpose: To develop a quantifiable approach to identify a dome shape of the repaired diaphragm using post-operative chest radiograph and to determine if a dome-shaped prosthetic patch repair is associated with a decreased rate of CDH recurrence., Methods: We conducted a retrospective review of all neonates evaluated at our institution from January 2004 to August 2017 with left- and right-sided CDH with at least 6 months of follow-up after CDH repair. Patch use, post-operative imaging and postnatal outcomes were analyzed. Neonates with patch repair were divided into two groups based on the presence of a dome. Using postoperative chest radiographs, the presence of a dome was classified as having a vertical-horizontal diaphragm ratio (VHDR) greater than 20%. Primary outcome was CDH recurrence after repair., Results: We identified 192 neonates who met our inclusion criteria. Cohort survival was 96%, recurrence rate was 15%, 78% had a left-sided CDH; 54% received a patch repair, of which 58% had a type C defect. Of the 104 infants with patch repairs, the CDH recurrence rate was 22% (n = 23) at a median age of 8.5 months (3.8, 20.1). Although neonates with a dome repair had more ECMO use and longer hospital stay, their recurrence rate was about half of those with a non-dome repair (14% vs 28%, p = 0.07)., Conclusions: A dome-shaped repair may reduce recurrence following prosthetic patch repair of congenital diaphragmatic hernia. A larger, multi-institutional study is needed to statistically validate this clinically significant observation., Type of Study: Retrospective review., Level of Evidence: III., (Copyright © 2021. Published by Elsevier Inc.)

Optimizing enteral nutrition for premature infants may help mitigate extrauterine growth restriction and adverse chronic health outcomes. Previously, we showed in neonatal pigs born at term that lean growth is enhanced by intermittent bolus compared with continuous feeding. The objective was to determine if prematurity impacts how body composition, muscle protein synthesis, and myonuclear accretion respond to feeding modality. Following preterm delivery, pigs were fed equivalent amounts of formula delivered either as intermittent boluses (INT; n = 30) or continuously (CONT; n = 14) for 21 days. Body composition was measured by dual-energy X-ray absorptiometry (DXA) and muscle growth was assessed by morphometry, myonuclear accretion, and satellite cell abundance. Tissue anabolic signaling and fractional protein synthesis rates were determined in INT pigs in postabsorptive (INT-PA) and postprandial (INT-PP) states and in CONT pigs. Body weight gain and composition did not differ between INT and CONT pigs. Longissimus dorsi (LD) protein synthesis was 34% greater in INT-PP than INT-PA pigs ( P < 0.05) but was not different between INT-PP and CONT pigs. Phosphorylation of 4EBP1 and S6K1 and eIF4E·eIF4G abundance in LD paralleled changes in LD protein synthesis. Satellite cell abundance, myonuclear accretion, and fiber cross-sectional area in LD did not differ between groups. These results suggest that, unlike pigs born at term, intermittent bolus feeding does not enhance lean growth more than continuous feeding in pigs born preterm. Premature birth attenuates the capacity of skeletal muscle to respond to cyclical surges in insulin and amino acids with intermittent feeding in early postnatal life. NEW & NOTEWORTHY Extrauterine growth restriction often occurs in premature infants but may be mitigated by optimizing enteral feeding strategies. We show that intermittent bolus feeding does not increase skeletal muscle protein synthesis, myonuclear accretion, or lean growth more than continuous feeding in preterm pigs. This attenuated anabolic response of muscle to intermittent bolus feeding, compared with previous observations in pigs born at term, may contribute to deficits in lean mass that many premature infants exhibit into adulthood.

Extrauterine growth restriction in premature infants is largely attributed to reduced lean mass accretion and is associated with long-term morbidities. Previously, we demonstrated that prematurity blunts the feeding-induced stimulation of translation initiation signaling and protein synthesis in skeletal muscle of neonatal pigs. The objective of the current study was to determine whether the blunted feeding response is mediated by reduced responsiveness to insulin, amino acids, or both. Pigs delivered by cesarean section preterm (PT; 103 days, n = 25) or at term (T; 112 days, n = 26) were subject to euinsulinemic-euaminoacidemic-euglycemic (FAST), hyperinsulinemic-euaminoacidemic-euglycemic (INS), or euinsulinemic-hyperaminoacidemic-euglycemic (AA) clamps four days after delivery. Indices of mechanistic target of rapamycin complex 1 (mTORC1) signaling and fractional protein synthesis rates were measured after 2 h. Although longissimus dorsi (LD) muscle protein synthesis increased in response to both INS and AA, the increase was 28% lower in PT than in T. Upstream of mTORC1, Akt phosphorylation, an index of insulin signaling, was increased with INS but was 40% less in PT than in T. The abundances of mTOR·RagA and mTOR·RagC, indices of amino acid signaling, increased with AA but were 25% less in PT than in T. Downstream of mTORC1, eIF4E·eIF4G abundance was increased by both INS and AA but attenuated by prematurity. These results suggest that preterm birth blunts both insulin- and amino acid-induced activation of mTORC1 and protein synthesis in skeletal muscle, thereby limiting the anabolic response to feeding. This anabolic resistance likely contributes to the high prevalence of extrauterine growth restriction in prematurity. NEW & NOTEWORTHY Extrauterine growth faltering is a major complication of premature birth, but the underlying cause is poorly understood. Our results demonstrate that preterm birth blunts both the insulin-and amino acid-induced activation of mTORC1-dependent translation initiation and protein synthesis in skeletal muscle, thereby limiting the anabolic response to feeding. This anabolic resistance likely contributes to the reduced accretion of lean mass and extrauterine growth restriction of premature infants.

Introduction: Pediatric patients with complex colorectal and genitourinary conditions often require coordinated multidisciplinary care; however, this coordinated care can be hard to structure and deliver. The purpose of this paper is to review the development and implementation of a multidisciplinary colorectal and pelvic health program, one year after the program's initiation., Methods: This is an observational retrospective 1-year study (10/1/2017 to 9/30/2018). In fiscal year (FY) 2018, a multidisciplinary colorectal and pelvic health program was initiated. The program development incorporated bimonthly team meetings, educational conferences, and initiation of three clinics: a complex colorectal and genitourinary reconstruction clinic, a bowel management clinic, and a colonic motility clinic. Conditions treated included complex anorectal and cloacal malformations, Hirschsprung disease, and idiopathic constipation. The fiscal year was selected to provide comparative administrative data after program implementation., Results: During the study period, 121 patients underwent comprehensive collaborative evaluation of which 58 (47%) were new to the institution compared to 12 (19%) new patients in the previous year (p < 0.001). In FY 2018, there were 130 procedures performed and 512 collaborative visits with an average of 47 visits per month. This was a 3.4-fold increase in visits compared to FY2017 (171 visits). Of the new patients, 60% (35/58), traveled a median of 181 miles, representing 33 statewide counties, and 4 states compared to a median of 93 miles in the previous fiscal year (p = 0.004)., Conclusion: The development of a colorectal and pelvic health program is feasible and requires a collaborative approach, necessitating multiple service lines within an institution. Program creation and implementation can result in rapid institutional clinical growth by filling a local and regional need through coordinated multidisciplinary care., Level of Evidence: IV., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to compare the accuracy of prenatal and postnatal imaging modalities for evaluation and management of congenital lung malformations (CLMs)., Methods: A retrospective review was performed of all fetuses evaluated for a CLM between December 2001 and January 2018. Pre and postnatal imaging findings, operative treatment, and patient outcomes were collected. Patients were included in analysis if they had fetal imaging (US and/or fetal MRI), a postnatal chest CT, and surgical pathology., Results: Over the study period, we identified 157 patients with prenatal imaging that also had a follow-up with postnatal chest CT at a median age of 2.1 [1.4, 3.2] months. Of these, 75% (n = 117) had surgical resection. Diagnostic accuracy (DA) for localization of unilobar lesions was 100% for pre- and postnatal imaging and 97% vs 98% for multilobar disease, respectively. On comparison for identification of aberrant vasculature and pathology prediction, pre- and postnatal imaging DAs were similar. However, postnatal CT had the highest specificity for diagnosing lesions overall (p < 0.05)., Conclusion: Prenatal imaging provides valuable information for counseling and possible fetal intervention. However, this study suggests that postnatal CT scan continues to provide important information for preoperative counseling and surgical management., Type of Study: Retrospective study., Level of Evidence: Level IV., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Introduction: Fetal magnetic resonance imaging (MRI) has been used to stratify severity of congenital diaphragmatic hernia (CDH) after ultrasound diagnosis. The purpose of this study was to determine if timing of MRI influenced prediction of severity of outcome in CDH., Methods: A single institution retrospective review of all CDH referred to our institution from February 2004 to May 2017 was performed. Patients were included if they underwent at least 2 fetal MRIs prior to delivery. Prenatal MRI indices including observed-to-expected total fetal lung volume (o/e TFLV) were evaluated. Indices were categorized by trimester, either 2nd (20-27 weeks gestation) or 3rd trimester (>28 weeks gestation) and further analyzed for outcome predictability. Primary outcomes were survival, extracorporeal membrane oxygenation (ECMO), and pulmonary hypertension (PAH). Student t test and logistic and linear regression were used for data analyses., Results: Of 256 fetuses evaluated for CDH, 197 were further characterized by MRI with 57 having both an MRI in the 2nd and 3rd trimesters. There was an average of 9.95 weeks (±4.3) between the 1st and 2nd MRI. Second trimester o/e TFLV was the only independent predictor of survival by logistic regression (OR 0.890, p < 0.01). Third trimester MRI derived lung volumes were associated with, and independent predictors of, severity of PAH and need for ECMO. Interval TFLV growth was a strong predictor of PAH postnatally (OR 0.361, p < 0.01). Overall cohort survival was 79%., Conclusion: Accuracy of MRI lung volumes to predict outcomes is dependent on the -gestational age at the time of exam. While MRI lung volumes at either the 2nd or 3rd trimester are predictive of morbidity, 2nd trimester lung volumes strongly correlated with mortality., (© 2019 S. Karger AG, Basel.)

Background: To perform a comprehensive assessment of postnatal gastrointestinal (GI) morbidity and determine the prenatal imaging features and postnatal factors associated with its development in patients with congenital diaphragmatic hernia (CDH)., Materials and Methods: A retrospective review was conducted of all infants evaluated for CDH at a quaternary fetal center from February 2004 to May 2017. Prenatal imaging features and postnatal variables were analyzed. GI morbidity was the primary outcome. The Mann-Whitney U test, the Kruskal-Wallis test with Dunnett's T3 post hoc analysis and logistic regression, and the χ2 test were performed when appropriate., Results: We evaluated 256 infants; 191 (75%) underwent CDH repair and had at least 6 months of follow-up. Of this cohort, 60% had gastroesophageal reflux disease (GERD), 13% had gastroparesis, 32% received a gastrostomy tube (G-tube), and 17% needed a fundoplication. Large defect, patch repair, extracorporeal membrane oxygenation (ECMO), and prolonged use of mechanical ventilation were significantly associated with having GERD, gastroparesis, G-tube placement, and fundoplication (p < 0.05). Fetuses with stomach grades 3 and 4 were most likely to have GERD, a G-tube, and a long-term need for supplemental nutrition than fetuses with stomach grades 1 and 2 (p < 0.05)., Conclusion: Survivors of CDH with large defects, prolonged use of mechanical ventilation, or that have received ECMO may be at an increased risk for having GERD, gastroparesis, and major GI surgery. Marked stomach displacement on prenatal imaging is significantly associated with GI morbidity in left-sided CDH., (© 2019 S. Karger AG, Basel.)

Objective: Fetal endoscopic tracheal occlusion (FETO) is associated with increased perinatal survival and reduced need for extracorporeal membrane oxygenation (ECMO) in fetuses with severe congenital diaphragmatic hernia (CDH). This study evaluates the impact of FETO on the resolution of pulmonary hypertension (PH) in fetuses with isolated CDH., Methods: We reviewed retrospectively the medical records of all fetuses evaluated for CDH between January 2004 and July 2017 at a single institution. Fetuses with additional major structural or chromosomal abnormalities were excluded. CDH cases were classified retrospectively into mild, moderate and severe groups based on prenatal magnetic resonance imaging indices (observed-to-expected total fetal lung volume and percentage of intrathoracic liver herniation). Presence of PH was determined based on postnatal echocardiograms. Logistic regression analyses were performed to evaluate the relationship between FETO and resolution of PH by 1 year of age while controlling for side of the CDH, use of ECMO, gestational age at diagnosis, gestational age at delivery, fetal gender, sildenafil use at discharge and CDH severity. Resolution of PH by 1 year of age was compared between a cohort of fetuses with severe CDH that underwent FETO and a cohort that did not have the procedure (non-FETO). A subanalysis was performed restricting the analysis to isolated left CDH. Parametric and non-parametric tests were used for comparisons., Results: Of 257 CDH cases evaluated, 72% (n = 184) had no major structural or chromosomal anomalies of which 58% (n = 107) met the study inclusion criteria. The FETO cohort consisted of 19 CDH cases and the non-FETO cohort (n = 88) consisted of 31 (35%) mild, 32 (36%) moderate and 25 (28%) severe CDH cases. All infants with severe CDH, regardless of whether they underwent FETO, had evidence of neonatal PH. FETO (OR, 3.57; 95% CI, 1.05-12.10; P = 0.041) and ECMO (OR, 5.01; 95% CI, 2.10-11.96; P < 0.001) were independent predictors of resolution of PH by 1 year of age. A higher proportion of infants with severe CDH that underwent FETO had resolution of PH by 1 year after birth compared with infants with severe CDH in the non-FETO cohort (69% (11/16) vs 28% (7/25); P = 0.017). Similar results were observed when the analysis was restricted to cases with left-sided CDH (PH resolution in 69% (11/16) vs 28% (5/18); P = 0.032)., Conclusion: In infants with severe CDH, FETO and ECMO are independently associated with increased resolution of PH by 1 year of age. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd., (Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.)

Purpose: The purpose of this study was to determine the accuracy of fetal echocardiogram (ECHO) for detecting cardiac structural anomalies that may impact Extracorporeal Membrane Oxygenation (ECMO) candidacy in infants with Congenital Diaphragmatic Hernia (CDH)., Methods: A retrospective review was performed on fetuses with CDH (January 2007-June 2017). Inclusion criteria were inborn and at least one prenatal and postnatal ECHO. ECHOs were evaluated for structural heart defects. Primary outcomes were accuracy of prenatal fetal ECHO and identify differences between prenatal and postnatal ECHO. Descriptive statistics and Chi-square analysis were performed., Results: 131 inborn patients were identified. Mean gestational age of fetal ECHO was 26.6 ± 5.5 weeks. The median time to postnatal ECHO was DOL 1 [0-30]. Fetal ECHO had 92% accuracy, 83% sensitivity, 93% specificity, PPV of 95%, NPV of 92%, and a 90% accuracy for visualization of at least one pulmonary vein into the left atrium on the contralateral (non-CDH) side. Thirty-five percent of patients received ECMO, and 26% had an associated cardiac anomaly. All ECMO patients had an accurate structural fetal ECHO., Conclusion: Fetal ECHO is sufficient for identifying major structural heart defects at large volume centers with trained pediatric cardiologists and may be used to guide clinical management, particularly in regards to ECMO candidacy., Level of Evidence: Level III., (Copyright © 2019. Published by Elsevier Inc.)

Omphalocele (exomphalos) is one of the most common abdominal wall defects. The size of the defect and the severity of the associated anomalies determine the overall morbidity and mortality. Routine prenatal screening and diagnosis of the abdominal wall defect and concurrent anomalies is important as it allows for effective prenatal counseling and optimal perinatal management. The purpose of this article is to discuss the approach to prenatal diagnosis and management of omphalocele., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: The benefits to early repair (<72 h postcannulation) of infants with congenital diaphragmatic hernia (CDH) on extracorporeal membrane oxygenation (ECMO) are increasingly recognized. Yet it is not known if even earlier repair (<24 h) results in comparable or improved patient outcomes. The goal of this study was to compare "super-early" (<24 h) to early repair (24-72 h) of CDH patients on ECMO., Methods: A retrospective review of infants with CDH placed on ECMO (2004-2017; n = 72) was performed. Data collected on the patients repaired while on ECMO within 72 h of cannulation (n = 33) included pre- and postnatal disease severity stratification variables and postnatal outcomes. Comparison groups were those patients repaired within 24 h of cannulation (n = 14) and those repaired between 24 and 72 h postcannulation (n = 19)., Results: Patients undergoing "super-early" (<24 h) repair had an average survival of 71.4% compared to the average survival of 59.7% in the early repair group. Pre- and postnatal variables predicting disease severity were not significantly different between the groups. Mean hospital stays, ventilator days, and cannulation days were statistically similar between the groups., Conclusions: Repair of patients with CDH patients on ECMO at less than 24 h postcannulation achieves outcomes that are comparable to those of repair between 24 and 72 h. While the present data suggest that there is not a "too early" time point for CDH repair on ECMO, larger multicenter studies are needed to validate our findings and determine the overall benefits., Type of Study: Retrospective comparative study., Level of Evidence: Level III., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: To examine postsurgical outcomes of a consecutive series of children treated with elective operations for congenital lung malformations (CLM)., Methods: A retrospective review was performed on a prospectively collected dataset of all fetuses evaluated for a CLM between July 2001 and June 2016. Prenatal findings, operative treatment and postnatal outcomes were collected. Children having elective operations were divided in two groups based on age at time of surgery., Results: Of 220 fetuses, 143 had operations and follow-up at our center. Six had open fetal lobectomy, 17 had EXIT-to-resection, 16 infants had urgent resection for symptoms and 110 with asymptomatic lesions had elective resection. Of these 110, the median fetal maximum CVR was 0.8 [range 0.1-2.2], and median age at operation was 4 (1.5-60) months (58% had resection at ≤4 months). Overall complication rate, including air-leak and pleural effusion, was 15%. When comparing those having resection at ≤4 months to those >4 months, there were no significant differences in complication rates or length of stay. Operative time was shorter for patients with early resection (154 ± 59 vs 181 ± 89, p = 0.05). No infant having resection at ≤4 months was readmitted. Overall, children survived with good recovery., Conclusion: Early elective resection of congenital lung malformations prior to 4 months of age is feasible and not associated with increased operative risk., Type of Study: Restropective study., Level of Evidence: Level III., (Copyright © 2018. Published by Elsevier Inc.)

Congenital diaphragmatic hernia (CDH) is the result of incomplete formation of the diaphragm that occurs during embryogenesis. The defect in the diaphragm permits the herniation of abdominal organs into the thoracic cavity contributing to the impairment of normal growth and development of the fetal lung. In addition to the hypoplastic lung, anomalies of the pulmonary arterioles worsen the pulmonary hypertension that can have detrimental effects in severe cases. Most cases of CDH can be effectively managed postnatally. Advances in neonatal and surgical care have resulted in improved outcomes over the years. When available, extracorporeal membrane oxygenation can provide temporary cardiorespiratory support for those not effectively supported by mechanical ventilation. In spite of these advances, very severe cases of CDH still carry a very high mortality and morbidity rate. Advances in imaging and evaluation now allow for early and accurate prenatal diagnosis of CDH, thereby identifying those at greatest risk who may benefit from prenatal intervention. This review article discusses some of the surgical and non-surgical prenatal interventions in the management of isolated severe congenital diaphragmatic hernia.

Purpose: The purpose of this study was to evaluate treatment and surgical outcomes of patients of Beckwith-Wiedemann Syndrome (BWS) treated at a tertiary children's hospital., Methods: A retrospective review of infants evaluated at Texas Children's Hospital for BWS from August 2000 to December 2016 was performed. Data collected included demographic information, clinical presentation, genetic evaluation, fetal imaging, operative treatment, and outcomes., Results: Forty-seven children with a diagnosis of BWS were identified. Sixty-four percent (n=30) had a genetic mutation in an imprinting domain of chromosome 11p15. Thirty-two patients (68%) underwent at least one operation related to BWS with a median of 2 [range: 0-8] surgical procedures per patient. Sixteen underwent omphalocele repair, 12 had partial glossectomies-, 7 underwent surgeries related to hemihypertrophy, and 6 had resection of an embryonal tumor (two adrenal cortical adenoma, one Wilms' tumor, two hepatoblastoma). Overall, survival was 100% with feeding difficulty (47%) being the most frequent complication., Conclusion: A substantial number of patients with Beckwith-Wiedemann Syndrome will require surgery. However, overall outcomes are similar between those that require surgery and those that do not., Level of Evidence: Level III., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to evaluate the characteristics of patients with congenital heart disease (CHD) who developed necrotizing enterocolitis (NEC)., Methods: A retrospective review of neonates with CHD at a tertiary care center between January 2006 and January 2016 was performed. Diagnosis of NEC was based on modified Bell's criteria. Patients were grouped by Risk Adjustment for Congenital Heart Surgery (RACHS-1) or by ductal-dependent (DD) lesions that require a patent ductus arteriosus to supply pulmonary or systemic circulation., Results: Of 1811 neonates with CHD, 3.4% (n=61) developed NEC. Eighteen (30%) of these required surgical management. The rate of NEC among DD patients was 5% (n=33/653), compared to 2.4% (n=28/1158) in the non-DD group (p=0.003). RACHS-1 score>2 had a higher rate of NEC 6.2% (41/658) compared to RACHS-1≤2 cases, 1.7% (20/1153) (p=0.005). DD patients and complex patients with RACHS-1>2 were more likely to develop NEC after cardiac surgery. Hypoplastic left heart syndrome patients had a rate of 9% (n=16/185). Surgical NEC was more prevalent in the non-DD group. Mortality was similar among groups., Conclusion: CHD patients with ductal-dependent lesions or complex cases (RACHS-1 score>2) have higher rates of NEC than non-ductal-dependent patients or RACHS-1 score of 2 or less. Mortality is similar regardless of ductal dependence, but surgical NEC was more prevalent in non-DD patients., Level of Evidence: Level IIb., (Copyright © 2018. Published by Elsevier Inc.)

Background/purpose: The purpose of this study was to develop a computational algorithm that would predict the need for ECMO in neonates with congenital diaphragmatic hernia (CDH)., Methods: CDH patients from August 2010 to 2016 were enrolled in a study to continuously measure cerebral tissue oxygen saturation (cStO2) of left and right cerebral hemispheres. NIRS devices utilized were FORE-SIGHT, CASMED and INVOS 5100, Somanetics. Using MATLAB©, a data randomization function was used to deidentify and blindly group patient's data files as follows: 12 for the computational model development phase (6 ECMO and 6 non-ECMO) and the remaining patients for the validation phase., Results: Of the 56 CDH patients enrolled, 22 (39%) required ECMO. During development of the algorithm, a difference between right and left hemispheric cerebral oxygenation via NIRS (ΔHCO) was noted in CDH patients that required ECMO. Using ROC analysis, a ΔHCO cutoff >10% was predictive of needing ECMO (AUC: 0.92; sensitivity: 85%; and specificity: 100%). The algorithm predicted need for ECMO within the first 12h of life and at least 6h prior to the clinical decision for ECMO with 88% sensitivity and 100% specificity., Conclusion: This computational algorithm of cerebral NIRS predicts the need for ECMO in neonates with CDH., Level of Evidence: II., (Copyright © 2017 Elsevier Inc. All rights reserved.)