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We sought to elucidate the degree of variation across centers by calculating center-specific standardized mortality ratios (SMRs) for infants with congenital diaphragmatic hernia (CDH) requiring extracorporeal life support (ECLS).The Extracorporeal Life Support Organization (ELSO) registry data (2000-2019) were used to estimate SMRs. Center-specific SMRs and their 95% confidence intervals (CIs) were used to identify centers with mortality as significantly worse (SW), significantly better (SB), or not different (ND) relative to the median standardized mortality rate.We identified 4,223 neonates with CDH from 109 centers. SMRs were risk-adjusted for pre-ECLS case-mix (birthweight, sex, race, 5 min Apgar, blood gases, gestational age, hernia side, prenatal diagnosis, pre-ECLS arrest, and comorbidities). Observed (unadjusted) mortality rates across centers varied substantially (range: 14.3%-90.9%; interquartile range [IQR]: 42.9%-62.1%). Thirteen centers (11.9%) had SB SMRs1 (SMR 0.52 to 0.84), 7 centers (6.4%) had SW SMRs1 (SMR 1.25 to 1.43), and 89 centers (81.7%) had SMRs ND relative to the median SMR rate across all centers (i.e., SMR not different from one). Descriptive analyses demonstrated that SB centers had a lower proportion of cases with renal complications, infectious complications and discontinuation of ECLS owing to complications, as well as differences in pre-ECLS treatments and timing of CDH repair, compared to SW and ND centers.This study specifically identified ECLS centers with higher and lower survival for patients with CDH, which may serve as a benchmark for institutional quality improvement. Future studies are needed to identify those specific processes at those centers that leads to favorable outcomes with the goal of improving care globally.Level III.

Upper gastrointestinal (UGI) pathologies are common in adolescents with obesity. This study aims to determine the prevalence of UGI inflammation on preoperative esophagogastroduodenoscopy (EGD) in adolescents undergoing sleeve gastrectomy (SG) and to assess weight loss outcomes.This is a retrospective analysis of pathology reports from EGD biopsies performed prior to SG from September 2017 to August 2020. Percentage weight loss was measured at 3, 6, and 12 mo after surgery. Percent total body weight loss (TBWL) was compared between patients with and without UGI inflammation.Thirty adolescents underwent laparoscopic SG. Mean TBWL was 22% of total body weight 12 mo after surgery. Preoperative EGD identified 9 (30%) patients with esophagitis, 10 (33%) with gastritis, and 9 (30%) with duodenitis. Twenty-one patients (70%) had inflammation of at least one area, 5 (17%) were Helicobacter pylori positive, and 1 (3%) had a gastric ulcer that delayed surgery. Five (17%) patients were taking antacids prior to EGD. Patients with preoperative gastric or duodenal inflammation had significantly less TBWL 12 mo after SG compared to patients without gastric (24.6% versus 16.7%, P = 0.04) or duodenal inflammation (25.7% versus 14.1%, P = 0.02).There is a high prevalence of UGI inflammation in adolescents undergoing SG. Gastric and duodenal inflammation is associated with less TBWL after SG.

Congenital diaphragmatic hernia (CDH) is a challenging surgical disease that requires complex preoperative, perioperative, and postoperative care. Survival depends on successful reduction and repair of the defect, and numerous complex decisions must be made regarding timing and preparation for surgery. This review describes the challenges and controversies inherent to surgical CDH care and provides recommendations for management based on the most recent evidence.

Anorectal malformations (ARMs) have a wide spectrum of presentation ranging from mild defects with perineal fistulas to more severe defects requiring complex management. A primary repair of ARMs with perineal or rectovestibular fistulas has been shown to have good outcomes. However, the timing of the reconstruction is still debated. The aim of this study is to investigate the safety of early versus delayed repair.This study was performed using data from the National Surgical Quality Improvement Program-Pediatric (NSQIP-P) from 2012 to 2017. Patients who underwent repair of anorectal malformation with perineal or vestibular fistula were included in the study. Patients with associated diagnosis for Hirschsprung disease, cloaca, rectal prolapse or stenosis, bladder exstrophy, and tracheoesophageal fistula were excluded. 30-day postoperative outcomes included wound and nonwound complications, readmissions, and reoperations. Outcomes were compared by early (≤7 days of age) versus delayed repair (6 weeks to 8 months).A total of 291 patients were included, with 66 in the early and 231 in the delayed group. Patients in the early group were more likely to be male (68.2% vs 31.8%; p 0.01) and have cardiac risk factors (71.2% vs 49.4%, p 0.01). The mean operative time was significantly shorter in the early group (90.1 vs 129.6 min; p 0.01). 30-day complications were not statistically significant between the two groups (p = 0.76). After multivariate analysis, timing of repair did not affect 30-day complications (p = 0.15).Our study shows that early repair of low anorectal malformations with a perineal or vestibular fistula appears to be associated with no increase in risk of postoperative complications as compared to delayed repair. At present, the decision remains dependent on the surgeon's experience and judgment.Level III. Retrospective comparative study.

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.

1. Sara A. Mansfield, MD, MS* 2. Tim Jancelewicz, MD, MA, MS* 1. *Division of Pediatric Surgery, Le Bonheur Children’s Hospital, University of Tennessee Health Science Center, Memphis, TN Clinicians should be aware of the strategies for prenatal and postnatal management of infants with omphalocele and gastroschisis. After completing this article, readers should be able to: 1. Recognize the implications and differences in management between gastroschisis and omphalocele. 2. Review the initial stabilization and management of infants with omphalocele and gastroschisis. 3. Review the surgical management options and rationale for infants with ventral abdominal wall defects. Abdominal wall defects represent a wide spectrum of congenital anomalies. These can range from lethal limb-body wall syndrome to benign umbilical cord hernias. Gastroschisis and omphalocele are the 2 most common defects and are the focus of this review. Although both of these diseases affect the umbilical area, they differ widely in their underlying pathogenesis, genetics, and associated disorders. Consequently, their management techniques and outcomes are quite different. This article reviews the pathogenesis, genetics, diagnostics, and outcomes for each disease, followed by an in-depth review of recent management updates. ### Pathogenesis Omphalocele (or exomphalos) is a herniation of the abdominal viscera through a midline abdominal wall defect (Fig 1). This defect is located at the base of the umbilical stalk, and herniated viscera are covered by a 3-layer membrane of peritoneum, Wharton jelly, and amnion. This contrasts with umbilical cord hernias, which are covered by intact skin and contain only a small protrusion of abdominal contents, and gastroschisis, which has no covering at all and occurs to the right of midline (Table). Omphaloceles are present in approximately 1 per 1,100 pregnancies. However, there is a high rate of spontaneous abortion, making the incidence of omphalocele per live birth approximately 1 per 4,000 to 1 per 6,000. (1) …

Purpose The American Pediatric Surgical Association (APSA) guidelines for the treatment of isolated solid organ injury (SOI) in children were published in 2000 and have been widely adopted. The aim of this systematic review by the APSA Outcomes and Evidence Based Practice Committee was to evaluate the published evidence regarding treatment of solid organ injuries in children. Methods A comprehensive search strategy was crafted and the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines were utilized to identify, review, and report salient articles. Four principal questions were examined based upon the previously published consensus APSA guidelines regarding length of stay (LOS), activity level, interventional radiologic procedures, and follow-up imaging. A literature search was performed including multiple databases from 1996 to 2016. Results LOS for children with isolated solid organ injuries should be based upon clinical findings and may not be related to grade of injury. Total LOS may be less than recommended by the previously published APSA guidelines. Restricting activity to grade of injury plus two weeks is safe but shorter periods of activity restriction have not been adequately studied. Prophylactic embolization of SOI in stable patients with image-confirmed arterial extravasation is not indicated and should be reserved for patients with evidence of ongoing bleeding. Routine follow-up imaging for asymptomatic, uncomplicated, low-grade injured children with abdominal blunt trauma is not warranted. Limited data are available to support the need for follow-up imaging for high grade injuries. Conclusion Based upon review of the recent literature, we recommend an update to the current APSA guidelines that includes: hospital length of stay based on physiology, shorter activity restrictions may be safe, minimizing post-injury imaging for lower injury grades and embolization only in patients with evidence of ongoing hemorrhage. Type of Study Systematic Review. Levels of Evidence Levels 2–4.

Studying the timing of repair in CDH is prone to confounding factors, including variability in disease severity and management. We hypothesized that delaying repair until post-ECMO would confer a survival benefit.Neonates who underwent CDH repair were identified within the ELSO Registry. Patients were then divided into on-ECMO versus post-ECMO repair. Patients were 1:1 matched for severity based on pre-ECMO covariates using the propensity score (PS) for the timing of repair. Outcomes examined included mortality and severe neurologic injury (SNI).After matching, 2,224 infants were included. On-ECMO repair was associated with greater than 3-fold higher odds of mortality (OR 3.41, 95% CI: 2.84-4.09, p0.01). The odds of SNI was also higher for on-ECMO repair (OR 1.49, 95% CI: 1.13-1.96, p0.01). A sensitivity analysis was performed by including the length of ECMO as an additional matching variable. On-ECMO repair was still associated with higher odds of mortality (OR 2.38, 95% CI: 1.96-2.89, p0.01). Results for SNI were similar but were no longer statistically significant (OR 1.33, 95% CI: 0.99-1.79, p=0.06).Of the infants who can be liberated from ECMO and undergo CDH repair, there is a potential survival benefit for delaying CDH repair until after decannulation.Treatment Study LEVEL OF EVIDENCE: III.

INTRODUCTION: Biliary dyskinesia (BD) is a common indication for pediatric cholecystectomy. While diagnosis is primarily based on diminished gallbladder ejection fraction (GB-EF), work-up and management in pediatrics is controversial. METHODS: We conducted a multi-institutional retrospective review of children undergoing cholecystectomy for BD to compare perioperative work-up and outcomes. RESULTS: Six hundred seventy-eight patients across 16 institutions were included. There was no significant difference in gender, age, or BMI between institutions. Most patients were white (86.3%), non-Hispanic (79.9%), and had private insurance (55.2%). Gallbladder ejection fraction (EF) was reported in 84.5% of patients, and 44.8% had an EF < 15%. 30.7% of patients were initially seen by pediatric surgeons, 31.3% by pediatric gastroenterologists, and 23.4% by the emergency department with significant variability between institutions (p < 0.001). Symptoms persisted in 35.3% of patients post-operatively with a median follow-up of 21 days (IQR 13, 34). On multivariate analysis, only non-white race and the presence of psychiatric comorbidities were associated with increased risk of post-operative symptoms. CONCLUSION: There is significant variability in evaluation and follow-up both before and after cholecystectomy for BD. Prospective research with standardized data collection and follow-up is needed to develop and validate optimal care pathways for pediatric patients with suspected BD. STUDY TYPE: Case Series, Retrospective Review. LEVEL OF EVIDENCE: Level IV.

Purpose The purpose of this study was to identify management preferences that may exist in the care of infants with CDH receiving ECMO with emphasis on VV-ECMO. Methods A survey was created to measure treatment preferences regarding ECMO use in CDH. The survey was distributed to all APSA and ELSO/Euro-ELSO members via e-mail. Survey results were summarized using descriptive statistics. Results The survey had 230 respondents. The survey participants were surgeons (75%), neonatologists/intensivists (23%), and “other” (2%). The mean annual center volume was 11.6(± 9.6) CDH cases, and the average number treated with ECMO was 4.5 (± 6.4) cases/yr. The most agreed upon criteria for ECMO initiation were preductal O2 saturation 40 (80%), severe air-leak (79%), and mixed acidosis (75%). Over 60% of respondents agreed the VV-ECMO would be optimum for average risk neonates. However, this preference diminished as the pre-ECMO level of cardiac support increased. When asked about why each respondent would choose VA-ECMO over VV-ECMO, the responses varied significantly between surgeons and non-surgeons. Conclusion While there seem to be areas of consensus among practitioners, such as criteria for initiation of ECMO, this survey revealed substantial variation in individual practice patterns regarding the use of ECMO for CDH. Type of Study Qualitative, Survey. Level of Evidence IV.

Background Treatment of the neonate with long gap esophageal atresia (LGEA) is one of the most challenging scenarios facing pediatric surgeons today. Contributing to this challenge is the variability in case definition, multiple approaches to management, and heterogeneity of the reported outcomes. This necessitates a clear summary of existing evidence and delineation of treatment controversies. Methods The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee drafted four consensus-based questions regarding LGEA. These questions concerned the definition and determination of LGEA, the optimal method of surgical management, expected long-term outcomes, and novel therapeutic techniques. A comprehensive search strategy was crafted and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were utilized to identify, review and report salient articles. Results More than 3000 publications were reviewed, with 178 influencing final recommendations. In total, 18 recommendations are provided, primarily based on level 4–5 evidence. These recommendations provide detailed descriptions of the definition of LGEA, treatment techniques, outcomes and future directions of research. Conclusions Evidence supporting best practices for LGEA is currently low quality. This review provides best recommendations based on a critical evaluation of the available literature. Based on the lack of strong evidence, prospective and comparative research is clearly needed. Type of study Treatment study, prognosis study and study of diagnostic test. Level of evidence Level II–V.

Background Congenital diaphragmatic hernia (CDH) is a morbid and potentially fatal condition that challenges providers. The aim of this study is to compare outcomes in neonates with prenatally diagnosed CDH that are inborn (delivered in the institution where definitive care for CDH is provided) versus outborn. Methods Prenatally diagnosed CDH cases were identified from the Congenital Diaphragmatic Hernia Study Group (CDHSG) database between 2007 and 2019. Using risk adjustment based on disease severity, we compared inborn versus outborn status using baseline risk and multivariable logistic regression models. The primary endpoint was mortality and the secondary endpoint was need for extracorporeal life support (ECLS). Results Of 4195 neonates with prenatally diagnosed CDH, 3087 (73.6%) were inborn and 1108 (26.4%) were outborn. There was no significant difference in birth weight, gestational age, or presence of additional congenital anomalies. There was no difference in mortality between inborn and outborn infants (32.6% versus 33.8%, P = 0.44) or ECLS requirement (30.9% versus 31.5%, P = 0.73). Among neonates requiring ECLS, outborn status was a risk factor for mortality (OR 1.51, 95% CI 1.13-2.01, P = 0.006). After adjusting for post-surgical defect size, which is not known prenatally, outborn status was no longer a risk factor for mortality for infants requiring ECLS. Conclusions Risk of mortality and need for ECLS for inborn CDH patients is not different to outborn infants. Future studies should be directed to establishing whether highest risk infants are at risk for worse outcomes based on center of birth.

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.

Background Neonates receiving extracorporeal life support (ECLS) for congenital diaphragmatic hernia (CDH) require prolonged support compared with neonates with other forms of respiratory failure. Hemolysis is a complication that can be seen during ECLS and can lead to renal failure and potentially to worse outcomes. The purpose of this study was to identify risk factors for the development of hemolysis in CDH patients treated with ECLS. Methods The Extracorporeal Life Support Organization database was used to identify infants with CDH (2000-2015). The primary outcome was hemolysis (plasma-free hemoglobin >50 mg/dL). Potentially associated variables were identified in the data set. Descriptive statistics and a series of nested multivariable logistic regression models were used to identify associations between hemolysis and demographic, pre-ECLS, and on-ECLS factors. Results There were 4576 infants with a mortality of 52.5%. The overall mean rate of hemolysis was 10.5% during the study period. In earlier years (2000-2005), the hemolysis rates were 6.3% and 52.7% for roller versus centrifugal pumps, whereas in later years (2010-2015), they were 2.9% and 26.5%, respectively. The fully adjusted model demonstrated that the use of centrifugal pumps was a strong predictor of hemolysis (odds ratio: 6.67, 95% confidence interval: 5.14-8.67). In addition, other risk factors for hemolysis included low 5-min Apgar score, on-ECLS complications (renal, metabolic, and cardiovascular), and duration of ECLS. Conclusions In our cohort of CDH patients receiving ECLS over 15 y, the use of centrifugal pumps increased over time, along with the rate of hemolysis. Patient- and treatment-level risk factors were identified contributing to the development of hemolysis.

OBJECTIVE To measure the survival among comparable neonates with CDH supported with and without ECLS. SUMMARY OF BACKGROUND DATA Despite widespread use in the management of newborns with CDH, ECLS has not been consistently associated with improved survival. METHODS A retrospective cohort study was performed using ECLS-eligible CDH Study Group registry patients born between 2007 and 2019. The primary outcome was in-hospital mortality. Neonates who did and did not receive ECLS were matched based on variables affecting risk for the primary outcome. Iterative propensity score-matched, survival (Cox regression and Kaplan-Meier), and center effects analyses were performed to examine the association of ECLS use and mortality. RESULTS Of 5855 ECLS-eligible CDH patients, 1701 (29.1%) received ECLS. "High-risk" patients were best defined as those with a lowest achievable first-day arterial partial pressure of CO2 of ≥60 mm Hg. After propensity score matching, mortality was higher with ECLS (47.8% vs 21.8%, odds ratio 3.3, 95% confidence interval 2.7-4.0, hazard ratio 2.3, P < 0.0001). For the subgroup of high-risk patients, there was lower mortality observed with ECLS (64.2% vs 84.4%, odds ratio 0.33, 95% confidence interval 0.17-0.65, hazard ratio 0.33, P = 0.001). This survival advantage was persistent using multiple matching approaches. However, this ECLS survival advantage was found to occur primarily at high CDH volume centers that offer frequent ECLS for the high-risk subgroup. CONCLUSIONS Use of ECLS is associated with excess mortality for low- and intermediate-risk neonates with CDH. It is associated with a significant survival advantage among high-risk infants, and this advantage is strongly influenced by center CDH volume and ECLS experience.

PURPOSE Enormous variability in management and cost occurs in CDH care. The purpose of this study was to identify regional mortality and cost patterns underlying this variability. METHODS This is a retrospective study of neonatal CDH patients at U.S. hospitals using data from the Pediatric Health Information System (PHIS) database (2015-2018). Patients were risk-stratified using CDH Study Group predicted survival (CDHSG-PS), and mortality and costs were assessed by region (East, West, Mid-West, and South) and center. RESULTS Higher mortality and extracorporeal life support (ECLS) rates were found in the Mid-West and South (p

Although longer ECMO run times for patients with congenital diaphragmatic hernia (CDH) have been associated with worse outcomes, a large study has not been conducted to examine the risk factors for long ECMO runs.The Extracorporeal Life Support Organization (ELSO) Registry from 2000 to 2015 was used to identify predictors of long ECMO runs in CDH patients. A long run was any duration of ≥14 days. Multivariable logistic regression models were used to examine the association between demographics, pre-ECMO blood gas/ventilator settings, comorbid conditions, and therapies on long ECMO runs.There were 4730 CDH-infants examined. The largest association with long ECMO runs was on-ECMO repair (OR: 3.72, 95% CI: 3.013-4.602, p 0.001) and the use of THAM (OR: 1.463, 95% CI: 1.062-2.016, p = 0.02). Each drop in pH quartile was associated with an increased risk of long ECMO run: pH ≥ 7.3 (reference), pH 7.2-7.9 (OR 1.24, 95% CI: 0.98-1.57, p = 0.07), pH 7.08-7.19 (OR 1.46, 95% CI: 1.17-1.84, p = 0.001), pH ≤ 7.07 (OR 1.64, 95% CI: 1.29-2.07, p 0.001).We found a correlation between both pre-ECMO demographics/timing of repair and the subsequent risk of long ECMO runs, providing insight for both providers and parents about the risk factors for longer runs.Treatment Study.Level III.

Purpose Congenital diaphragmatic hernia (CDH) is the most common indication for neonatal extracorporeal membrane oxygenation (ECMO), but mortality remains at 50%. Multiorgan failure can occur in 25% and has been linked to worse outcomes. We sought to examine the factors that would increase the risk of multiorgan dysfunction (MOD). Methods The Extracorporeal Life Support Organization (ELSO) database was used to identify infants with CDH (2000–2015). The primary outcome was MOD, which was defined as the presence of organ failure in ≥ 2 organ systems. We used a multivariable logistic regression to examine the effect of demographics, pre-ECMO respiratory status, comorbidities, and therapies on MOD. Results There were a total of 4374 CDH infants who were treated with ECMO. Overall mortality was 52.4%. The risk models demonstrated that pre-ECMO cardiac arrest (OR 1.458, CI: 1.146–1.861, p = 0.002) and hand-bagging (OR 1.461, CI: 1.094–1.963, p = 0.032) had the strongest association with MOD. In addition, other pre-ECMO indicators of disease severity (pH, HFOV, MAP, 5-min APGAR) and pre-ECMO therapies (bicarb, neuromuscular [NM] blockers) were also associated with MOD. Conclusions The level of pre-ECMO support has a significant association with the development of MOD, and initiation of ECMO prior to arrest seems to be critical to avoid complications. Type of study Treatment study. Level of evidence Level III.

Variation in management characterizes treatment of infants with a congenital pulmonary airway malformation (CPAM). This review addresses six clinically applicable questions using available evidence to provide recommendations for the treatment of these patients. Questions regarding the management of a pediatric patient with a CPAM were generated. English language articles published between 1960 and 2014 were compiled after searching Medline and OvidSP. The articles were divided by subject area and by the question asked, then reviewed and included if they specifically addressed the proposed question. 1040 articles were identified on initial search. After screening abstracts per eligibility criteria, 130 articles were used to answer the proposed questions. Based on the available literature, resection of an asymptomatic CPAM is controversial, and when performed is usually completed within the first six months of life. Lobectomy remains the standard resection method for CPAM, and can be performed thoracoscopically or via thoracotomy. There is no consensus regarding a monitoring protocol for observing asymptomatic lesions, although at least one chest computerized tomogram (CT) should be performed postnatally for lesion characterization. An antenatally identified CPAM can be evaluated with MRI if fetal intervention is being considered, but is not required for the fetus with a lesion not at risk for hydrops. Prenatal consultation should be offered for infants with CPAM and encouraged for those infants in whom characteristics indicate risk of hydrops. Very few articles provided definitive recommendations for care of the patient with a CPAM and none reported Level I or II evidence. Based on available information, CPAMs are usually resected early in life if at all. A prenatally diagnosed congenital lung lesion should be evaluated postnatally with CT, and prenatal counseling should be undertaken in patients at risk for hydrops.

Congenital diaphragmatic hernia occurs when a portion of the fetal diaphragm is absent, allowing abdominal contents to enter the thorax, and is associated with impaired pulmonary development. Although overall mortality is near 30%, a mortality rate less than 15% may be possible by following a standardized multidisciplinary care plan. Fetal diagnosis and evaluation can improve coordination of care, but there is no clear role for fetal intervention. After birth, gentle ventilation with permissive hypercapnia supports the infant while minimizing lung injury. Appropriate cardiovascular support, treatment of pulmonary hypertension, and extracorporeal membrane oxygenation may benefit some patients. Timing of surgical repair depends on disease severity. All patients should have close follow-up after discharge.

Background Standardized care may improve outcomes in many diseases including congenital diaphragmatic hernia (CDH). Our study assesses the variability of CDH clinical practice guidelines (CPG) among North American centers. Methods North American member institutions of the CDH Study Group and the Pediatric Surgical Research Collaborative were solicited to submit their CDH CPG. Elements from each CPG were collected and classified according to therapeutic purpose. Elements were assigned to umbrella topics of prenatal assessment, delivery plus initial resuscitation, ventilatory and cardiovascular management, therapeutic targets, analgesia, and criteria for transitions in care. Descriptive analyses were performed to characterize the scope and variability of CPGs. Results Sixty-eight centers provided 40 responses (59%). Of these, 29 (73%) had a CDH CPG, of which 27 were obtained for review. All CPGs had a primary focus of preoperative care. Conventional ventilation was the first-line strategy in all CPGs. Ninety-three percent reported a peak inspiratory pressure limit (mean: 25.2 ± 2 cm H2O). Target oxygenation and ventilatory variables had low coefficients of variation. Two-thirds of CPGs discussed echocardiography, with indications for inhaled nitric oxide, sildenafil, and prostaglandins detailed in 81%, 30%, and 22% of CPGs, respectively. Extracorporeal life support and operative indications were specified in 93% and 59%, respectively, although specific targets for each were highly variable. Conclusions This synthesis of North American CDH CPGs identifies areas of both alignment and variability and provides objective data about individual institutional guidelines in CDH care. These data may inform the development of a consensus-based, multi-institutional approach to standardized CDH management in North America.

Background Management of CDH is highly variable from center to center, as are patient outcomes. The purpose of this study was to examine risk-stratified survival and extracorporeal membrane oxygenation (ECMO) rates at a single center, and to determine whether adverse outcomes are related to patient characteristics or management. Methods A retrospective single-center review of CDH patients was performed, and outcomes compared to those reported by the CDH Study Group (CDHSG) registry. Patient demographics, disparities, and clinical characteristics were examined to identify unique features of the cohort. A model derived using the registry that estimates probability of ECMO use or death in CDH newborns was used to risk-stratify patients and assess mortality rates. Observed over expected (O/E) ECMO use rates were calculated to measure whether “excess” or “appropriate” ECMO use was occurring. Results There were 81 CDH patients treated between 2004–2017, and 5034 in the CDHSG registry. Mortality in ECMO-treated patients was higher than the registry. Socioeconomic variables were not significantly associated with outcomes. The strongest predictors of mortality were ECMO use and early blood gas variables. The risk model accurately predicted ECMO use with a c-statistic of 0.79. Compared with the registry, the disparity in mortality rates was greatest for moderate-risk patients. O/E ECMO use was highest in low and moderate-risk patients. Conclusions ECMO use is a more consistent predictor of mortality than CDH severity at a single center, and there is relative overuse of ECMO in lower-risk patients. Risk stratification allows for more accurate institutional assessment of mortality and ECMO use, and other centers could consider such an adjusted analysis to identify opportunities for outcomes improvement. Level of Evidence III.

Objective The goal of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee was to derive recommendations from the medical literature regarding the management of pilonidal disease. Methods The PubMed, Cochrane, Embase, Web of Science, and Scopus databases from 1965 through June 2017 were queried for any papers addressing operative or non-operative management of pilonidal disease. The Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines were followed. Consensus recommendations were derived for three questions based on the best available evidence, and a clinical practice guideline was constructed. Results A total of 193 articles were fully analyzed. Some non-operative and minimally invasive techniques have outcomes at least equivalent to operative management. Minimal surgical procedures (Gips procedure, sinusectomy) may be more appropriate as first-line treatment than radical excision due to faster recovery and patient preference, with acceptable recurrence rates. Excision with midline closure should be avoided. For recurrent or persistent disease, any type of flap repair is acceptable and preferred by patients over healing by secondary intention. There is a lack of literature dedicated to the pediatric patient. Conclusions There is a definitive trend towards less invasive procedures for the treatment of pilonidal disease, with equivalent or better outcomes compared with classic excision. Midline closure should no longer be the standard surgical approach. Type of study Systematic review of level 1–4 studies. Level of evidence Level 1–4 (mainly level 3–4).

Because congenital diaphragmatic hernia (CDH) is characterized by a spectrum of severity, risk stratification is an essential component of care. In both the prenatal and postnatal periods, accurate prediction of outcomes may inform clinical decision-making, care planning, and resource allocation. This review examines the history and utility of the most well-established risk prediction tools currently available, and provides recommendations for their optimal use in the management of CDH patients.

Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival rate of neonates with CDH and HLHS is only 1%-5%. We review our experience with CDH and HLHS and compare our outcomes with published literature. Retrospective review of all neonates with CDH and HLHS at our institution over a 10-year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies, and outcomes were reviewed, and an algorithmic approach was proposed. Five patients with CDH and HLHS were treated between 2006 and 2016. All had mitral stenosis with aortic stenosis. Four patients had a left-sided Bochdalek diaphragmatic hernia and 1 patient had a large bilateral Morgagni hernia. Two (2/4) of the Bochdalek hernias were associated with significant pulmonary hypoplasia and required patch closure of the CDH; both were palliated with percutaneous ductal stents and both died. Three patients underwent primary Norwood operation followed by repair of less severe CDH defect. All 3 patients are currently well and have survived bidirectional Glenn anastomosis; one patient is well after Fontan operation. Successful palliation of neonates with HLHS and associated CDH is possible in the current era. Outcome is determined primarily by the severity of the CDH and the degree of associated pulmonary hypoplasia. An algorithmic team approach is helpful in management of this difficult group of patients.

We sought to determine optimal timing for CPAM resection within the first year of life.We queried the National Surgical Quality Improvement Program pediatric database from 2012 to 2015 for elective CPAM resections on patients less than 1year of age. Patients were divided by age in months: 1-3 (n=57), 4-6 (n=135), and 6-12 (n=214). Patient operative variables and 30-day postoperative outcomes were compared.A total of 406 patients were included with no differences in demographics or comorbidities. Median operative time increased with each older age category (115min, 152min, 163min, respectively; p0.01). Thoracoscopic approach was less utilized in 1-3months (40.4%) compared to the older two age categories (65.9% and 69.6%, respectively; p0.01). There were no differences by age in major complications, conversion to open, or readmissions. On multivariate analysis, ASA class≥3 (p0.01) and prolonged operative time (p0.01) were associated with a major complication. Furthermore, operations on patients aged 6-12months were associated with increased operative time (p0.01) regardless of operative approach.Elective CPAM resections are equally safe in patients 1-12months of age. Earlier resection including both open and thoracoscopic resection is associated with decreased operative time.IIc, Outcomes Research.

Pediatric scald burns result in frequent emergency room visits and hospitalizations. We investigated whether cooking-related burns produce greater morbidity requiring more extensive care than noncooking burns.We performed a 6-y review at our free-standing children's hospital. Children aged18 y admitted for accidental scald burns were included. Demographics, injury pattern, treatment, and outcome (contractures and/or limited mobility and nonhealing and/or infected wounds) data were analyzed comparing cooking versus noncooking burns. The Mann-Whitney U test, a chi-square test, and the negative binomial were used to compare continuous, categorical, and count data between groups. Bivariate analysis was performed to identify risk factors among patients with adverse outcomes.We identified 308 patients; 262 (85%) cooking and 46 (15%) noncooking burns. Most patients were African-American males, with public insurance, and a median age of 2 y. Cooking burns preferentially occurred over the head, neck, and upper body; noncooking burns were distributed over the lower body (P 0.02). Median total body surface area was equal for both groups (P 0.11). In subgroup analysis, semisolid and grease burns resulted in increased rates of wound contractures and/or limited mobility when compared with noncooking burns (P = 0.05 and P = 0.008, respectively). Patients with complications were more likely to have third degree burns and required more consults, longer hospitalization, and more surgical debridements and clinic visits.Most accidental scald burns occurred in young children during food preparation. Greater long-term morbidity was found in patients with semisolid and grease burns. This subset of children has a higher injury burden and requires extensive care in the acute and long-term setting.

A means for early postnatal stratification of ECMO risk in CDH newborns could be used to comparatively assess the utilization and outcomes of ECMO use between centers. While multiple CDH mortality risk calculators are available, no validated tool exists specifically for prediction of ECMO use. The purpose of this study was to derive and validate an ECMO risk stratification model.The study population was obtained from CDH Study Group registry for the period between 2007 and 2016. Only centers offering ECMO were included. The cohort was restricted to ECMO candidates and then divided into derivation and validation sets. Using all relevant perinatal predictors in the registry, univariate analysis was performed for the composite outcome of ECMO use or death without ECMO use. The model was derived using the derivation cohort with multivariable logistic regression and automatic stepwise forward selection (P 0.05 for qualifying variables), and a c-statistic was obtained. The model was then tested on the validation cohort. Sample reuse validation and bootstrap validation were performed. The validated model was then tested for accuracy on CDH subgroups.There were 1992 patients in the derivation cohort. Four significant variables were identified in the final ECMO risk model: 1-min and 5-min Apgar scores and highest and lowest post-ductal partial pressure of CO2 during the first 24 h of life. The model c-statistic was 0.824 which was confirmed with cross-validation and bootstrap optimism correction. The validation cohort c-statistic was 0.823 (N = 993). The model had good discrimination for left and right CDH, inborn and outborn patients, patients born before and after 2011, and high and low volume centers. The model performed significantly better for postnatally diagnosed patients.This study represents proof-of-concept that a risk model can accurately estimate the probability of ECMO use in CDH newborns. This stratification could assist centers as a metric for assessment of ECMO usage and outcomes. Refinement and prospective validation of this model should be carried out prior to clinical application.3.

Objective Ovarian torsion in pediatric patients is a rare event and is primarily managed by pediatric general surgeons. Torsion can be treated with detorsion of the ovary or oopherectomy. Oopherectomy is the most common procedure performed by pediatric general surgeons for ovarian torsion. The purpose of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee was to examine evidence from the medical literature and provide recommendations regarding the optimal treatment of ovarian torsion. Methods Using PRISMA guidelines, six questions were addressed by searching Medline, Cochrane, Embase Central and National clearing house databases using relevant search terms. Risks of ovarian detorsion including thromboembolism and malignancy, indications for oophoropexy, benefits of detorsion including recovery of function and subsequent fertility, and recommended surveillance after detorsion were evaluated. Consensus recommendations were derived for each question based on the best available evidence. Results Ninety-six studies were included. Risks of ovarian detorsion such as thromboembolism and malignancy were reviewed, demonstrating minimal evidence for unknowingly leaving a malignancy behind in the salvaged ovary and no evidence in the literature of thromboembolic events after detorsion of a torsed ovary. There is no clear evidence supporting the benefit of oophoropexy after a single episode of ovarian torsion. The gross appearance of the ovary does not correlate with long-term ovarian viability or function. Pregnancies have occurred in patients after detorsion of an ovary both spontaneously and with harvested oocytes from previously torsed ovaries. The consensus recommendation for imaging surveillance following ovarian detorsion is an ultrasound at 3months postprocedure but sooner if there is a concern for malignancy. Conclusion There appears to be overwhelming evidence supporting ovarian detorsion rather than oopherectomy for the management of ovarian torsion in pediatric patients. Ovarian salvage is safe and is the preferred treatment for ovarian torsion. Most salvaged ovaries will maintain viability after detorsion. Type of study Systematic review of level 3–4 studies. Level of evidence 3–4

Objective The goal of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee was to develop recommendations regarding time to appendectomy for acute appendicitis in children within the context of preventing adverse events, reducing cost, and optimizing patient/parent satisfaction. Methods The committee selected three questions that were addressed by searching MEDLINE, Embase, and the Cochrane Library databases for English language articles published between January 1, 1970 and November 3, 2016. Consensus recommendations for each question were made based on the best available evidence for both children and adults. Results Based on level 3–4 evidence, appendectomy performed within 24h of admission in patients with acute appendicitis does not appear to be associated with increased perforation rates or other adverse events. Based on level 4 evidence, time from admission to appendectomy within 24h does not increase hospital cost or length of stay (LOS). Data are currently limited to determine an association between the timing of appendectomy and parent/patient satisfaction. Conclusions There is a paucity of high-quality evidence in the literature regarding timing of appendectomy for patients with acute appendicitis and its association with adverse events or resource utilization. Based on available evidence, appendectomy performed within the first 24h from presentation is not associated with an increased risk of perforation or adverse outcomes. Type of study Systematic Review of Level 1–4 studies

The minimally invasive surgery (MIS) approach for congenital diaphragmatic hernia (CDH) repair remains controversial. Our objective was to compare outcomes and complications of the MIS and open approaches, with risk-stratification of patients based on defect size and key patient characteristics.The multinational CDH Study Group (CDHSG) registry was queried for the period from 2007 to 2015. Patient demographics and operative details, including the CDHSG Staging System defect size (A to D), were reviewed. Open cases consisted of laparotomy and thoracotomy; MIS repairs included laparoscopy and thoracoscopy. Outcomes included length of stay (LOS) for patients surviving to discharge, hernia recurrence, and adhesive small bowel obstruction (SBO) requiring surgery. Regression analyses were performed. Odds ratios (ORs) with 95% CIs were derived.A total of 3,067 CDH patients underwent open (n = 2,579; 84%) or MIS (n = 488; 16%) repair. Patients undergoing open repair were more likely to be diagnosed prenatally, be premature, have lower 5-minute Apgar scores, and have major cardiac anomalies (all p0.001). Among MIS repairs, 79% were low risk (size A and B) defects vs 50% among open repairs (p 0.001). Patients undergoing MIS repair experienced shorter overall median LOS, higher recurrence rates, and fewer SBO. With multivariable regression adjusting for defect size and key patient characteristics, an MIS approach was significantly associated with decreased LOS (mean -13.4 days; 95% CI -18 to -8.8 days), increased recurrences (OR 3.10; 95% CI 1.91 to 5.04), and decreased SBO (OR 0.19; 95% CI 0.06 to 0.60).After risk-stratification of CDH patients, an MIS approach was independently associated with decreased LOS and SBO, but higher recurrence rates.

Objective The goal of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee was to derive recommendations from the medical literature regarding the surgical treatment of pediatric gastroesophageal reflux disease (GERD). Methods Five questions were addressed by searching the MEDLINE, Cochrane, Embase, Central, and National Guideline Clearinghouse databases using relevant search terms. Consensus recommendations were derived for each question based on the best available evidence. Results There was insufficient evidence to formulate recommendations for all questions. Fundoplication does not affect the rate of hospitalization for aspiration pneumonia, apnea, or reflux-related symptoms. Fundoplication is effective in reducing all parameters of esophageal acid exposure without altering esophageal motility. Laparoscopic fundoplication may be comparable to open fundoplication with regard to short-term clinical outcomes. Partial fundoplication and complete fundoplication are comparable in effectiveness for subjective control of GERD. Fundoplication may benefit GERD patients with asthma, but may not improve outcomes in patients with neurologic impairment or esophageal atresia. Overall GERD recurrence rates are likely below 20%. Conclusions High-quality evidence is lacking regarding the surgical management of GERD in the pediatric population. Definitive conclusions regarding the effectiveness of fundoplication are limited by patient heterogeneity and lack of a standardized outcomes reporting framework. Type of study Systematic review of level 1–4 studies. Level of evidence Level 1–4 (mainly level 3–4)

Vascular endothelial growth factor (VEGF) is required for blood vessel formation during lung growth and repair. Alteration of VEGF isoform expression has been demonstrated in response to fetal tracheal occlusion and in models of lung injury. The purpose of this study was to investigate VEGF expression during compensatory lung growth in the mouse.Under general anesthesia, adult mice underwent left thoracotomy with (n = 5) or without (sham, n = 5) pneumonectomy. The right lungs were harvested at 1, 3, and 7 d after the operation. Lung-to-body weight ratio as well as total DNA and protein content were measured. VEGF protein expression was analyzed by Western blot and ELISA. VEGF isoform expression was evaluated using semi-quantitative PCR followed by Imagequant optical densitometry. Values were compared by Student's t-test and ANOVA using Fisher's protected least significant difference post-hoc test where appropriate.Compensatory lung growth was observed as measured by increases in right lung-to-body weight ratio and in DNA and protein content. Total VEGF RNA and protein expression did not change after pneumonectomy. However, on post-operative day 1, there was a decrease in the relative percentage of VEGF188 mRNA (P0.01), and an increase in the relative percentage of VEGF164 mRNA (P = 0.05). At 3 d postpneumonectomy, low relative VEGF188 expression persisted (P0.05), VEGF164 expression normalized, and relative VEGF120 expression increased (P0.01). Isoform expression in the pneumonectomy animals was identical to sham animals by the seventh d. There were no differences observed in VEGF receptor expression.During compensatory lung growth, we have observed an early postoperative reversion of VEGF isoform expression to the pattern seen during fetal lung development and in lung injury models.

Acute neonatal appendicitis is a rare condition associated with significant morbidity and mortality. The severity of this disease is caused by its tendency to occur more frequently in premature infants, an increased perforation rate with rapid progression to peritonitis, and delay in diagnosis and intervention. Although appendicitis in the perinatal period may occur as an isolated event, in many cases it occurs in association with other pathologic states, including prematurity, inguinal hernia, and others. The presentation of neonatal appendicitis can be identical to necrotizing enterocolitis, leading to misdiagnosis. Here we report a case of perforated appendicitis in a 9-day-old boy with tetralogy of Fallot and esophageal atresia with tracheoesophageal fistula. In addition, we present a review of the clinical features of neonatal appendicitis and a discussion of previously described, associated conditions.

Diagnosing intestinal strangulation complicating a small bowel obstruction (SBO) remains a considerable challenge. Despite decades of experience and numerous studies, no clinical indicators have been identified that reliably predict this life-threatening condition. Our goal was to determine which clinical indicators in patients with SBO can be used to independently predict the presence of strangulated intestine. Medical records were reviewed for 192 adult patients operated on for acute SBO over an 11-year period (1996–2006). Seventy-two preoperative clinical, laboratory, and radiologic findings at admission were examined. Data from patients with strangulated intestine were compared to data from patients without bowel compromise. Likelihood ratios were generated for each significant parameter in a multivariate logistic regression analysis. Forty-four patients had bowel strangulation requiring bowel resection, and 148 had no strangulation. The most significant independent predictor of bowel strangulation was the computed tomography (CT) finding of reduced wall enhancement, with a sensitivity and specificity of 56% and 94% [likelihood ratio (LR) 9.3]. Elevated white blood cell (WBC) count and guarding were moderately predictive (LR 1.7 and 2.8). Regression analysis of multiple preoperative criteria demonstrates that reduced wall enhancement on CT, peritoneal signs, and elevated WBC are the only variables independently predictive of bowel strangulation in patients with SBO.

Neonates with cholestasis may undergo many tests before biliary atresia (BA) or an alternative diagnosis is reached, and delayed intervention may worsen outcomes. An optimal diagnostic approach to reduce risk, cost, and delay has yet to be defined. The purpose of this study was to develop an algorithm that rapidly and accurately excludes BA for infants with cholestatic jaundice.A single-center retrospective comparison of diagnostic workup was made between cholestatic infants with BA, and those without BA who underwent hepatobiliary iminodiacetic acid (HIDA) scan during admission. Patients were born between 2000 and 2010 and those older than 100days at assessment were excluded. Sensitivity and specificity analysis of predictive variables was performed and an algorithm constructed.There were 45 BA and 167 non-BA patients. Some variables were 100% sensitive for the exclusion of BA: conjugated bilirubin2.5mg/dL, gamma-glutamyl transpeptidase150U/L, excretion on HIDA, or a normal percutaneous cholangiogram. Clinical variables and ultrasound were less useful as screening tests owing to low specificity and sensitivity, respectively. Liver biopsy was 98% sensitive and 84% specific in the diagnosis of BA. An algorithm was constructed that rules out BA with a negative laparotomy rate of 3-22%.We propose a screening algorithm for infants with conjugated hyperbilirubinemia that permits efficient exclusion of BA with minimal invasive testing and with a low risk of negative laparotomy. This algorithm now requires prospective evaluation to determine its diagnostic accuracy and its ability to reduce hospital costs, patient morbidity, and time to Kasai portoenterostomy in patients with BA.

Alimentary tract duplications (ATDs) are rare congenital anomalies, with an incidence of 1 in 4500 individuals. Patients with these lesions present in a variety of ways, and although histologically benign, ATDs can lead to lethal complications including volvulus, intussusception, or bowel obstruction. Irrespective of anatomical location, the epithelial lining of ATDs contains ectopic acid-secreting mucosa in more than 50% of cases, which can lead to ulceration, bleeding, or transmural erosion and perforation. We report an unusual case of a child who presented with small bowel obstruction caused by an ileal duplication cyst that had become infected with Salmonella. Although reports of infected mesenteric cysts and duodenal duplication cysts have been published, this is the first reported case of an infected ileal duplication cyst. We also present a review of the literature regarding these interesting lesions.

Background Congenital ultra-short bowel syndrome (USBS) is a challenging problem with a poor outcome. We report a new management approach for USBS infants that attempts to optimize gut growth potential. Methods We report five neonates with USBS in whom no correction was performed at primary surgery except placement of a gastrostomy (G) tube. Sham feeds were started with intermittent G-tube clamping to induce bowel dilatation/growth. Serial fluoroscopy was done until bowel caliber reached 5 cm. STEP was performed and continuity established to the colonic remnant. Small bowel length (SBL) and enteral caloric intake were tabulated. Results Patients were born with a mean residual SBL of 19 ± 7.6 cm (14.8% of expected). Median duration of sham feeds prior to STEP was 108 (range 27–232) days. Mean SBL at STEP was 47 ± 12.1 cm, which increased post-STEP to 70 ± 12.7 cm (a mean increase of 296% from birth, representing 36.4% ± 13.1% of expected gut length). With a median follow-up time of 20 months (range 8–28), 4/5 achieved > 50% enteral calories and have normal liver function. One has undergone liver transplantation. Conclusions In USBS patients, delayed surgical correction with sham feeds accelerates gut growth, optimizing potential for autologous reconstruction. This approach may offer greater opportunity for intestinal adaptation than traditional options.

Background CDH patients experience multi-system morbidity. Despite apparent health, late childhood complications do occur. We reviewed the long-term surgical morbidity of our CDH patients to determine whether protracted clinical surveillance is warranted. Methods A single-institution retrospective chart review of all CDH survivors treated from 1999 to 2011 who are followed at our CDH multidisciplinary clinic was performed. Descriptive and statistical analyses were performed to show risk of surgical complications over time. Results A total of 187 CDH patients were treated with 160 surviving to discharge (86%). Primary repair was performed in 115 (73%), and 42 (27%) underwent patch repair. CDH recurrence occurred in 23 (15%) at a median time of 0.7 (range 0–8.5) years (65% asymptomatic). Seventy percent of recurrences occurred before 2years and 17% after 4years. Bowel obstruction occurred in 12 (8%) at a median time of 0.7 (range 0.2–7.2) years post-repair, and chest deformity occurred in 13 (8%) at a median of 5 (range 1.1–6.8) years. For patch repairs, scoliosis occurred in 4 (10%) patients at a median age of 3 (range 0.6–5) years. Conclusion Surgical complications in CDH survivors are common, can occur many years later, and are frequently asymptomatic. Long-term surveillance of CDH patients is recommended for early identification and treatment of complications.

Background Higher recurrence rates have been reported for thoracoscopic repair (TR) of neonatal congenital diaphragmatic hernia (CDH) compared to open repair. Our centre initiated changes in surgical management following a quality review in order to improve outcome. Methods A retrospective review of TR patients from 2000 to 2011 at a single institution was performed. A review was done in 2007, and changes were implemented to decrease recurrence rates. These included use of pledgets, an extracorporeal corner stitch, liberal prosthetic patch use, lower insufflation pressures, and TR was limited to two experienced surgeons. Outcome data before and after this quality improvement process were compared. Non-TR patients from the same time period served as controls. Data are quoted as median (range) and non-parametric tests used to compare. P Results There were 23 neonatal TR patients with median follow-up of 3.6 (range 0.4–7) years. Median age at repair was 2 (range 0–21) days. There were 5 patch repairs (22%), all after 2008. There were 9 recurrences (39%) at a median time of 162 days after TR, compared to 13 (10%) recurrences in the control cohort. For primary TR, there was a trend towards a decreased recurrence rate from 50% prior to 2008 to 25% after 2008 (P=0.26). Conclusions Systematic quality review was modestly effective in decreasing the recurrence rate for neonatal TR, but further outcome data are required.

Background/purpose The prognosis for multiple vs singleton pregnancies affected by congenital diaphragmatic hernia (CDH) is not known. To improve the counseling of families with multiple gestation pregnancies complicated by CDH, we examined outcomes of a consecutive series of CDH cases occurring in multiple gestation pregnancy referrals. Methods Clinical characteristics and morbidity and mortality data were gathered for a consecutive series of infants with CDH from 16 multiple gestation pregnancies. Outcomes were compared to a cohort of 91 patients with CDH from singleton pregnancies. Multivariate regression was also used in an attempt to determine whether multiple gestation pregnancy was independently predictive of subsequent long-term adverse outcomes. Results Four pregnancies were lost to follow-up, and 1 underwent selective reduction. Overall mortality for live-born multiple gestation fetuses affected by CDH was 30% and was 8% for unaffected siblings. No pregnancy was concordant. Clinical features were not different between the case series and control infants, except median gestational age at delivery, which was significantly lower for the multigestational infants (34 [range, 32-36] vs 38 [range, 28-41] weeks) ( P = .02). Long-term morbidity was comparable between cases and controls. Conclusions In terms of mortality, outcomes of multigestational pregnancies affected by CDH are no worse than for CDH pregnancies in general. Long-term risk may depend more on CDH severity rather than the presence of multiple fetuses.

Surgical complications are common in survivors of congenital diaphragmatic hernia (CDH), but little is known about long-term incidence patterns and associated predictors.A cohort of 99 CDH survivors was prospectively followed at a single-institution multidisciplinary clinic. Data were gathered regarding the adverse surgical outcomes of hernia recurrence, chest and spinal deformity, and operative small bowel obstruction (SBO), and then were retrospectively analyzed in relation to perinatal and perioperative markers of disease severity to determine significant predictors. Statistical methods used included univariate and multivariate regression analysis, hazard modeling, and Kaplan-Meier analysis.At a median cohort age of 4.7 (range, 0.2-10.6) years, 46% of patients with patch repairs and 10% of those with primary repairs had a hernia recurrence at a median time of 0.9 (range, 0.1-7.3) years after repair. Chest deformity was detected in 47%. Small bowel obstruction and scoliosis occurred in 13%. Recurrence and chest deformity were significantly more common with patch repair, liver herniation, age at neonatal extubation greater than 16 days, oxygen requirement at discharge, and prematurity. The strongest predictor of SBO was patch repair. Multivariate analysis showed that patch repair was independently predictive of recurrence and early chest deformity (odds ratios of 5.0 and 4.8, confidence intervals of 1-24 and 1-21, P.05). Use of an absorbable patch was associated with the highest risk of surgical complications.For long-term survivors of CDH, specific perinatal and operative variables, particularly patch repair, are associated with subsequent adverse surgical outcomes.

Objective Gastroschisis is associated with inflammatory changes in the exposed bowel which leads to intestinal dysmotility after postnatal repair. The insult is a combined effect of amniotic fluid exposure and mechanical constriction. We hypothesized that in utero anatomic repair is possible in a sheep model, and that it may halt the inflammatory damage caused by both mechanisms. Methods Gastroschisis was surgically created in mid-gestation (day 75) in 8 sheep fetuses. On gestational day 100, 2 fetuses underwent open fetal gastroschisis repair, where the eviscerated bowel was returned to the peritoneal cavity, and the abdominal wall was primarily closed. All fetuses were harvested at 135 days of gestation. Results Six fetuses survived the initial operation, and both fetuses that underwent gastroschisis repair survived to term. At 100 and 135 days of gestation, the eviscerated bowel showed progressive signs of inflammation and peel development. The gross and microscopic inflammatory changes in the gastroschisis bowel at 100 days of gestation were completely resolved at term following in utero repair. Conclusion In utero anatomic repair of gastroschisis is possible in mid-gestation in the fetal lamb model, and it appears to ameliorate the inflammatory process.

Congenital cystic adenomatoid malformation (CCAM) of the lung may result from a localized aberrant epithelial-mesenchymal interaction during lung development. We used laser microdissection (LMD) to isolate the epithelium and mesenchyme of CCAM, and studied candidate gene expression in these pure cell populations.Congenital cystic adenomatoid malformation tissue was obtained from fetal (n = 5) and postnatal (n = 5) surgical specimens. Normal fetal lung (n = 10) was obtained from abortus material, and normal postnatal lung (n = 5) was identified from surgical specimens. Whole tissue was analyzed using immunohistochemistry and reverse transcriptase polymerase chain reaction (RT-PCR). Using LMD, columnar bronchiolar type epithelium and underlying mesenchyme were isolated. Multiplex nested RT-PCR was then used to detect message levels of candidate genes.Reverse transcriptase polymerase chain reaction performed on LMD-isolated tissue, but not whole tissue homogenate, revealed differences between CCAM and normal lung. In this report, we focus on the fibroblast growth factor (FGF) family. By RT-PCR, there was 4-fold more epithelial expression of FGF9 in fetal CCAM vs normal fetal lung (P.07). This was qualitatively confirmed by immunohistochemistry. We also detected decreased FGF7 expression in CCAM mesenchyme (P.05) but no significant differences in FGF10 or FGFR2.LMD may be used to overcome the limitations of tissue heterogeneity in the study of CCAM. Abnormal growth factor expression may play a role in the etiology of this lesion.

Na+/H+exchanger (NHE) activity is exquisitely dependent on the intra- and extracellular concentrations of Na+and H+. In addition, Cl−ions have been suggested to modulate NHE activity, but little is known about the underlying mechanism, and the Cl−sensitivity of the individual isoforms has not been established. To explore their Cl−sensitivity, types 1, 2, and 3 Na+/H+exchangers (NHE1, NHE2, and NHE3) were heterologously expressed in antiport-deficient cells. Bilateral replacement of Cl−with nitrate or thiocyanate inhibited the activity of all isoforms. Cl−depletion did not affect cell volume or the cellular ATP content, which could have indirectly altered NHE activity. The number of plasmalemmal exchangers was unaffected by Cl−removal, implying that inhibition was due to a decrease in the intrinsic activity of individual exchangers. Analysis of truncated mutants of NHE1 revealed that the anion sensitivity resides, at least in part, in the COOH-terminal domain of the exchanger. Moreover, readdition of Cl−into the extracellular medium failed to restore normal transport, suggesting that intracellular Cl−is critical for activity. Thus interaction of intracellular Cl−with the COOH terminus of NHE1 or with an associated protein is essential for optimal activity.