Gastroschisis is the most common congenital abdominal wall defect, with an increasing incidence. It results in extrusion of abdominal contents with associated delayed intestinal motility. Abnormal heart rate characteristics (HRCs) such as decreased variability occur due to the inflammatory response to sepsis in preterm infants. This study aimed to test the hypothesis that infants with gastroschisis have decreased heart rate variability (HRV) after birth and that this physiomarker may predict outcomes.

 We analyzed heart rate data from and clinical variables for all infants admitted with gastroschisis from 2009 to 2020.

 Forty-seven infants were admitted during the study period and had available data. Complex gastroschisis infants had reduced HRV after birth. For those with sepsis and necrotizing enterocolitis, abnormal HRCs occurred early in the course of illness.

 Decreased HRV was associated with complex gastroschisis. Infants in this group experienced complications that prolonged time to full enteral feeding and time on total parenteral nutrition.

· Infants with gastroschisis can be classified into two subcategories, simple and complex disease.. · Those with complex disease often require prolonged stays in the neonatal intensive care unit and costly hospitalizations. We hypothesized that infants with complex gastroschisis are more likely to have abnormal HRC due to intestinal inflammation.. · In this study, we identified associations between abnormal HRV, heart rate characteristicHRC, and the development of gastroschisis complications. Additionally, we described differences in clinical characteristics between infants with complex versus simple gastroschisis..

Omphalocele, a congenital anomaly characterized by the protrusion of abdominal viscera through the umbilical ring, often presents challenges in surgical management, especially when concurrent with other anomalies such as intestinal atresia. We presented a case of a female infant weighing 2.6 kg born with omphalocele and concurrent ileal atresia. The child was successfully managed through prompt surgical intervention. Preoperative investigations revealed signs suggestive of intestinal obstruction, necessitating immediate surgical exploration. Intraoperatively, meticulous reduction of the omphalocele sac and resection of the atretic segment were performed. Postoperative care in the neonatal intensive care unit ensured optimal recovery. This case underscored the importance of timely intervention and multidisciplinary collaboration in managing complex congenital anomalies in neonates.

The aim of this study was to compare perinatal outcomes between patients with and without prenatal ultrasound markers predictive of complex gastroschisis.

A prospective cohort of 98 patients with isolated fetal gastroschisis underwent antenatal ultrasound and delivered in a tertiary referral center. Patients were classified according to eight ultrasonographic markers predictive of complexity, and perinatal outcomes were assessed accordingly. The primary outcome was the presence of fetal growth restriction and staged SILO reduction postnatally. RESULTS:  Of all fetuses, 54.1% (n = 53) displayed ultrasonographic markers predictive of complexity at 32.7 ± 4.3 weeks of gestation. Gastric dilatation was the most frequent marker followed by extra-abdominal bowel dilatation. The presence of ultrasound markers predictive of complexity, was not associated with fetal growth restriction but its absence was less associated with staged SILO reduction of the abdominal wall postnatally with a relative risk of 0.79 (CI 95% 0.17-0.53).

Fetuses with ultrasound markers that predict complexity were not associated with fetal growth restriction, but its absence was less associated with staged SILO reduction of the abdominal wall postnatally. It is necessary to unify criteria, establish cut-off points, and the optimal moment to measure these markers.

· The association between ultrasound markers and adverse perinatal outcomes in fetuses with gastroschisis remain controversial.. · The absence of ultrasound markers that predict complexity was less associated with staged SILO reduction postnatally.. · It is necessary to unify criteria, establish cut-off points, and the optimal moment to measure these markers..

To describe the clinical and feeding findings of premature infants with gastroschisis (GTQ) in a neonatal intensive care unit and compare them to preterm infants (NBs) without congenital anomalies.

A retrospective case-control study was conducted with 50 premature NBs (25 with GTQ and 25 without comorbidities - control group). The NBs were compared regarding demographic and clinical data: risk of mortality and speech-language assessment (nonnutritive and nutritive sucking). Subsequently, a multiple logistic regression model was applied to determine the variables associated with the negative speech therapy outcome (speech therapy discharge after more than 7 days considering the first speech therapy evaluation).

The results of the first analysis indicated that there was a difference between the GTQ and the CG for the following variables: total time in days of hospitalization; use of mechanical ventilation (invasive x noninvasive); days of life on the date of the first speech-language assessment; use of alternative feeding route; and the GTQ group had worse results. The results of the multiple logistic regression indicated that the diagnosis of GTQ, the use of invasive mechanical ventilation, and the absence of adequate intraoral pressure during the first speech-language evaluation were risk factors for a negative speech-language outcome.

The feeding skills of preterm infants with QTG are related to the severity of the condition (gastrointestinal tract complications), requiring longer hospitalization, use of invasive mechanical ventilation, prolonged use of alternative feeding route and requiring more speech therapy to start oral feeding.

To describe the incidence and to analyze risk factors associated with cholestasis in neonates with gastroschisis.

This is a retrospective cohort study in a tertiary single center analyzing 181 newborns with gastroschisis between 2009 and 2020. The following risk factors associated with cholestasis were analyzed: gestational age, birth weight, type of gastroschisis, silo closure or immediate closure, days of parenteral nutrition, type of lipid emulsion, days of fasting, days to reach a full diet, days with central venous catheter, presence of infections, and outcomes.

Among the 176 patients evaluated, 41 (23.3%) evolved with cholestasis. In the univariate analysis, low birth weight (p=0.023), prematurity (p<0.001), lipid emulsion with medium-chain triglycerides and long-chain triglycerides (p=0.001) and death (p<0.001) were associated with cholestasis. In the multivariate analysis, patients who received lipid emulsion with fish oil instead of medium chain triglycerides/long chain triglycerides (MCT/LCT) emulsion had a lower risk of cholestasis.

Our study shows that lipid emulsion with fish oil is associated with a lower risk of cholestasis in neonates with gastroschisis. However, this is a retrospective study and a prospective study should be performed to confirm the results.

Newborns with gastroschisis need surgery to reduce intestines into the abdominal cavity and to close the abdominal wall. Due to an existing volume-outcome relationship for other high-risk, low-volume procedures, we aimed at examining the relationship between hospital or surgeon volume and outcomes for gastroschisis.

We conducted a systematic literature search in Medline, Embase, CENTRAL, CINAHL and Biosis Previews in June 2021 and searched for additional literature. We included (cluster-) randomized controlled trials (RCTs) and prospective or retrospective cohort studies analyzing the relationship between hospital or surgeon volume and mortality, morbidity or quality of life. We assessed risk of bias of included studies using ROBINS-I and performed a systematic synthesis without meta-analysis and used GRADE for assessing the certainty of the evidence.

We included 12 cohort studies on hospital volume. Higher hospital volume may reduce in-hospital mortality of neonates with gastroschisis, while the evidence is very uncertain for other outcomes. Findings are based on a low certainty of the evidence for in-hospital mortality and a very low certainty of the evidence for all other analyzed outcomes, mainly due to risk of bias and imprecision. We did not identify any study on surgeon volume.

The evidence suggests that higher hospital volume reduces in-hospital mortality of newborns with gastroschisis. However, the magnitude of this effect seems to be heterogeneous and results should be interpreted with caution. There is no evidence on the relationship between surgeon volume and outcomes.

Compare in-hospital outcomes in gastroschisis with intestinal atresia versus simple gastroschisis (GS) using a national database.

The Children's Hospitals Neonatal Database identified infants with gastroschisis from 2010 to 2016.

2078 patients with gastroschisis were included: 183 (8.8%) with co-existing intestinal atresia, 1713 (82.4%) with simple gastroschisis, the remainder with complex gastroschisis without atresia. Length of hospitalization was longer for those with atresia, and yielded higher rates of mortality, medical NEC, and intestinal perforation. They began enteral feedings later, were less likely to initiate feeds orally, and reached full feedings later. They were less likely to be receiving any maternal breast milk or breastfeeding at discharge and more likely than simple gastroschisis to be discharged with a feeding tube.

A large multicenter cohort showed gastroschisis with atresia results in worse outcomes and complications, including necrotizing enterocolitis, feeding delays, and enteral feeding tube dependence.

To analyze gastroschisis annual incidence, mortality rates, and trends in the Brazilian state of Rio Grande do Sul from the year 2000 to the year 2017.

Population-based study with the analysis of the temporal trend of gastroschisis annual incidence and mortality rates. Data were obtained from the Live Birth Information System and the Mortality Information System, with the analysis performed by polynomial regression modeling.

There were 2,612,532 live births, 705 hospitalizations, and 233 deaths due to gastroschisis. The annual incidence of gastroschisis was 2.69 per 10,000 live births. The annual incidence rate increased by 85% in the total period (p = 0.003), and mortality was 33% in the 2000-2017 period. Maternal age < 25 years was a risk factor for gastroschisis (p < 0.001). Children were more likely to be born weighing < 2,500 g (p < 0.001) and with a gestational age < 37 weeks (p < 0.001). The annual incidence trend was to increase, and the mortality trend was to decrease.

Similar to what has been described in several regions/countries, there was a trend showing an 85% increase in the annual incidence of gastroschisis (p = 0.003) and the mortality was 33% with a trend of decreasing (p = 0.002).

This study aims to define the extent of additional surgical procedures after abdominal wall closure (AWC) in patients with gastroschisis (GS) and omphalocele (OC) with special focus on gastrointestinal related operations.

A retrospective chart review was performed including all operations in GS and OC patients in the first year after AWC (2010-2019). The risk for surgery was calculated using the one-year cumulative incidence (CI).

33 GS patients (18 simple GS, 15 complex) and 24 OC patients (12 without (= OCL), 12 OC patients with liver protrusion (= OCL +)) were eligible for analysis. 43 secondary operations (23 in GS, 20 in OC patients) occurred after a median time of 84 days (16-824) in GS and 114.5 days (12-4368) in OC. Patients with complex versus simple GS had a significantly higher risk of undergoing a secondary operation (one-year CI 64.3% vs. 24.4%; p = 0.05). 86.5% of surgical procedures in complex GS and 36.3% in OCL + were related to gastrointestinal complications. Complex GS had a significantly higher risk for GI-related surgery than simple GS. Bowel obstruction was a risk factor for surgery in complex GS (one-year CI 35.7%).

Complex GS and OCL + patients had the highest risk of undergoing secondary operations, especially those with gastrointestinal complications.

Gastroschisis is an index neonatal malformation with an increasing incidence from earlier decades. The aim of this study was to investigate regional variations in patient demographics, outcomes, and costs in gastroschisis.

The 2016 kid's inpatient database (KID) obtained from the Healthcare Cost and Utilization Project (HCUP) was used to obtain gastroschisis birth, demographic, and outcome data across four U.S. geographical regions: Northeast, Midwest, South, and West. Categorical variables were analyzed with Pearson's Chi-squared test. Odds ratios (OR) with 95% confidence intervals (CI) were reported for variables with significance (p < 0.05). Appropriate logistic regressions were used for comparing categorical, cardinal, and continuous data.

2043 gastroschisis cases were obtained from the 2016 KID database with a weighted incidence of 5.1 per 10,000 live births. Incidence of gastroschisis across regions was similar when controlling for a number of hospitals reporting to KID. Prematurity was common, with 59% (1204) of neonates with gastroschisis born at less than 37 weeks. 52% (N = 1067) of gastroschisis infants were male. 46% (955) were White, 9% (178) African American, 22% (450) Hispanic, and 8% (155) other. The overall mortality of gastroschisis in this database was 3.6% (75). The average length of stay was 39 days and average cost of treatment was $357,436. Though without significant differences in mortality, there were significant differences in demographics, costs, and lengths of stay between regions.

There has been a significant increase in gastroschisis incidence over the last three decades. This significant increase appears to be related to an increase in socioeconomic inequality and an alteration in the population demographics in the United States. Further study of regional variations will facilitate the development of best practice guidelines that benefit all.

This study aimed to characterize risk factors for inpatient mortality in patients born with gastroschisis in a contemporary cohort.

This was a retrospective cohort study of infants born with gastroschisis using the Kids' Inpatient Database 2016. Simple descriptive statistics were used to characterize the patients by demographics, and illness severity was estimated using the All-Patient Refined Diagnosis-Related Groups classification. Variables associated with an increased risk of mortality on univariate analysis were incorporated into a multivariable logistic regression model to generate adjusted odds ratios (aORs) for mortality.

An estimated 1,990 patient with gastroschisis were born in 2016, with a 3.7% mortality rate during the initial hospitalization. Multivariable logistic regression demonstrated the following variables to be associated with an increased risk of inpatient mortality: black or Asian race compared with white (aOR: 2.6, 95% confidence interval [CI]: 1.1-6.1, p = 0.03 and aOR: 4.1, 95% CI: 1.3-13.3, p = 0.02, respectively), whereas private health insurance compared with government (aOR: 0.2; 95% CI: 0.2-0.8; p = 0.007) and exurban domicile compared with urban (aOR: 0.5; 95% CI: 0.2-0.9; p = 0.04) appeared to be associated with a decreased risk of inpatient mortality.

Inpatient mortality for neonates with gastroschisis is relatively low. Even after correcting for illness severity, race, health insurance status, and domicile appear to play a role in mortality disparities. Opportunities may exist to further decrease mortality in at-risk populations.

Background: Gastroschisis management remains a controversy. Most surgeons prefer reduction and fascial closure. Others advise staged reduction to avoid a sudden rise in intra-abdominal pressure (IAP). This study aims to evaluate the feasibility of using the umbilical cord as a flap (without skin on the top) for tension-free repair of gastroschisis. Methods: In a prospective study of neonates with gastroschisis repaired between January 2018 to October 2020 in Tanta University Hospital, we used the umbilical cord as a flap after the evacuation of all its blood vessels and suturing the edges of the cord with the skin edges of the defect. They were guided by monitoring abdominal perfusion pressure (APP), peak inspiratory pressure (PIP), central venous pressure (CVP), and urine output during 24 and 48 h postoperatively. The umbilical cord flap is used for tension-free closure of gastroschisis if PIP > 24 mmHg, IAP > 20 cmH₂O (15 mmHg), APP <50 mmHg, and CVP > 15cmH₂O. Results: In 20 cases that had gastroschisis with a median age of 24 h, we applied the umbilical cord flap in all cases and then purse string (Prolene Zero) with daily tightening till complete closure in seven cases, secondary suturing after 10 days in four cases, and leaving skin creeping until complete closure in nine cases. During the trials of closure, the range of APP was 49-52 mmHg. The range of IAP (IVP) was 15-20 cmH₂O (11-15 mmHg), the range of PIP was 22-25 cmH₂O, the range of CVP was 13-15 cmH₂O, and the range of urine output was 1-1.5 ml/kg/h. Conclusion: The umbilical cord flap is an easy, feasible, and cheap method for tension-free closure of gastroschisis with limiting the PIP ≤ 24 mmHg, IAP ≤ 20 cmH₂O (15 mmHg), APP > 50 mmHg, and CVP ≤ 15cmH₂O.

 The study aims to assess the changes in prevalence and mortality of gastroschisis, and to identify associated anomalies.

 It is a population-based nationwide study. All gastroschisis cases were identified in the Finnish Register of Congenital Malformations and the Care Register for Health Care from 1993 to 2014 including live births, stillbirths, and terminations of pregnancy due to fetal anomalies. Associated anomalies were recorded, and analyzed, and prevalence and infant mortality were calculated.

 There were 320 cases of gastroschisis; 235 (73%) live births, 16 (5%) stillbirths, and 69 (22%) terminations of pregnancy. Live birth prevalence of gastroschisis in Finland was lower than generally reported (1.73 in 10,000). However, due to relatively high rates of abortion, our total prevalence of 2.57/10,000 was similar with other reports. The most common risk factor was young maternal age. Babies with gastroschisis were born prematurely, on average on the 36th week and most are delivered by caesarean section. There was a significant increasing trend in live birth prevalence (p = 0.0018). Overall infant mortality was 7.7% (18/235), 7.2% (16/222) in simple gastroschisis and 15% (2/13) in complex gastroschisis. Associated anomalies were rare both in aborted fetuses and neonates, and there was only one case with a chromosomal abnormality.

 Gastroschisis is usually an isolated anomaly with increasing birth prevalence and excellent survival rates. Regardless of the good prognosis, the abortion rates in Finland are higher than previously reported, and we hypothesize this to be due to lack of appropriate antenatal counselling.

Gastroschisis is a congenital anomaly that needs surgical management for repositioning intestines into the abdominal cavity and for abdominal closure. Higher hospital or surgeon volume has previously been found to be associated with better clinical outcomes for different especially high-risk, low volume procedures. Therefore, we aim to examine the relationship between hospital or surgeon volume and outcomes for gastroschisis.

We will perform a systematic literature search from inception onwards in Medline, Embase, CENTRAL, CINAHL, and Biosis Previews without applying any limitations. In addition, we will search trial registries and relevant conference proceedings. We will include (cluster-) randomized controlled trials (RCTs) and prospective or retrospective cohort studies analyzing the relationship between hospital or surgeon volume and clinical outcomes. The primary outcomes will be survival and mortality. Secondary outcomes will be different measures of morbidity (e.g., severe gastrointestinal complications, gastrointestinal dysfunctions, and sepsis), quality of life, and length of stay. We will systematically assess risk of bias of included studies using RoB 2 for individually or cluster-randomized trials and ROBINS-I for cohort studies, and extract data on the study design, patient characteristics, case-mix adjustments, statistical methods, hospital and surgeon volume, and outcomes into standardized tables. Title and abstract screening, full text screening, critical appraisal, and data extraction of results will be conducted by two reviewers independently. Other data will be extracted by one reviewer and checked for accuracy by a second one. Any disagreements will be resolved by discussion. We will not pool results statistically as we expect included studies to be clinically and methodologically very diverse. We will conduct a systematic synthesis without meta-analysis and use GRADE for assessing the certainty of the evidence.

Given the lack of a comprehensive summary of findings on the relationship between hospital or surgeon volume and outcomes for gastroschisis, this systematic review will put things right. Results can be used to inform decision makers or clinicians and to adapt medical care.

Open Science Framework (DOI: https://doi.org/10.17605/OSF.IO/EX34M ; https://doi.org/10.17605/OSF.IO/HGPZ2 ).

Neonatal surgery for abdominal wall defects is not performed in a centralized manner in Germany. The aim of this study was to investigate whether treatment for abdominal wall defects in Germany is equally effective compared to international results despite the decentralized care.

All newborn patients who were clients of the major statutory health insurance company in Germany between 2009 and 2013 and who had a diagnosis of gastroschisis or omphalocele were included. Mortality during the first year of life was analysed.

The 316 patients with gastroschisis were classified as simple (82%) or complex (18%) cases. The main associated anomalies in the 197 patients with omphalocele were trisomy 18/21 (8%), cardiac anomalies (32%) and anomalies of the urinary tract (10%). Overall mortality was 4% for gastroschisis and 16% for omphalocele. Significant factors for non-survival were birth weight below 1500 g for both groups, complex gastroschisis, volvulus and anomalies of the blood supply to the intestine in gastroschisis, and female gender, trisomy 18/21 and lung hypoplasia in omphalocele.

Despite the fact that paediatric surgical care is organized in a decentralized manner in Germany, the mortality rates for gastroschisis and omphalocele are equal to those reported in international data.

The aim of this study was to determine the risk of central line associated blood-stream infections (CLABSI) in neonatal gastroschisis patients, risk factors, outcomes, and financial implications.

The 2016 Healthcare Cost and Utilization Project (HCUP)'s kid's inpatient database (KID), a national database of pediatric inpatient admissions across the United States, was used to obtain a large sample of gastroschisis admissions. Incidence of CLABSI in the gastroschisis patient population was compared to the incidence of CLABSI in the database. To further study the factors influencing CLABSI in gastroschisis, demographic and clinical features of patients were analyzed. Categorical variables were analyzed using Fisher's exact test or Pearson's chi-squared test. Odds ratios (OR) with 95% confidence intervals (CI) for variables found to have significance (p < 0.05) were calculated.

Incidence of CLABSI in this database for pediatric inpatients was 4449 out of 298,862 central line insertions [1.48%] and was 81 out of 2032 [3.9%] (OR 2.83, 95% CI 2.26-3.54, p < 0.001) in the gastroschisis cohort. African American neonates had a significantly higher risk of CLABSI with gastroschisis. Prematurity and low birth-weight in gastroschisis were protective from CLABSI, along with patients from suburban areas or admitted in the Southern USA. Average costs were greater in gastroschisis patients with CLABSI, increasing from $281,779 to $421,970 (p = 0.008). The average length of stay increased from 31 days to 38 days with a CLABSI (p < 0.001).

In gastroschisis patients, CLABSI incidence is high and adds great morbidity and expense. For uncertain reasons, premature and low birth weight babies appear to be protected.

Level III.

To determine if mother's own milk (MOM) dose after gastroschisis repair is associated with time from feeding initiation to discharge. Secondary outcomes included parenteral nutrition (PN) duration and length of stay (LOS).

Retrospective study of 44 infants with gastroschisis examined demographics, gastroschisis type, PN days, timing of nutrition milestones, feeding composition, and LOS.

MOM dose was significantly associated with shorter time to discharge from feeding initiation (adjusted hazard ratio [HR] for discharge per 10% increase in MOM dose, 1.111; 95% CI, 1.011-1.220, p = 0.029). MOM dose was also significantly associated with shorter LOS (adjusted HR for discharge per 10% increase in MOM dose, 1.130; 95% CI, 1.028-1.242, p = 0.011).

MOM dose was significantly associated with a decrease in time to discharge from feeding initiation and LOS in a dose-dependent manner. Mothers of gastroschisis patients should receive education and proactive lactation support to optimize MOM volume for feedings.

To evaluate the incidence of respiratory failure requiring ECMO in newborns with gastroschisis (GC), compare it to the incidence in the general population, review the surgical outcomes of newborns with GC requiring ECMO and compare them to newborns with GC not requiring ECMO.

This is a retrospective review of all neonatal admissions for GC from December 2010 to September 2015.

110 newborns with GC were admitted to our NICU between 12/2010 and 9/2015; 36 were term. Four cases, all term, all prenatally diagnosed, all outborn, developed respiratory failure requiring ECMO secondary to meconium aspiration syndrome (MAS). This 11% (4/36 term GC) represents a 300-fold increase in the incidence of MAS-associated respiratory failure requiring ECMO compared to the general population of term newborns (0.037%). Median time on ECMO was 12 (9-20) days. The time to achieve full enteral feedings in the GC/ECMO group was twice the time of the 106 newborns in the GC/non-ECMO group [median: 70 (48-77) vs. 35 (16-270) days, respectively]. Time to hospital discharge was three times longer in the GC/ECMO group compared to the GC/non-ECMO group (median: 42 [20-282] versus 125 [69-223] days, respectively). All patients survived.

The incidence of respiratory failure requiring ECMO is remarkably higher in patients with GC than in the general population and much higher in the subgroup of term GC. While infrequent, the possibility of this event supports the concept that fetuses with GC benefit from being delivered at tertiary centers with immediate pediatric surgery and ECMO capabilities.

Gastroschisis is a severe congenital anomaly associated with a significant morbidity and mortality. There are limited temporal trend data on incidence, mortality, length of stay, and hospital cost of gastroschisis. Our aim was to study these temporal trends using the National Inpatient Sample (NIS).

We identified all neonatal admissions with a diagnosis of gastroschisis within the NIS from 2010 through 2014. We limited admission age to ≤ 28 days and excluded all those transferred to other hospitals. We estimated gastroschisis incidence, mortality, length of hospital stay, and cost of hospitalization. For continuous variables, trends were analyzed using survey regression. Cochrane-Armitage trend test was used to analyze trends for categorical variables. P < 0.05 was considered as significant.

The incidence of gastroschisis increased from 4.5 to 4.9/10,000 live births from 2010 through 2014 (P = 0.01). Overall mortality was 3.5%, median length of stay was 35 days (95% CI 26-55 days), and median cost of hospitalization was $75,859 (95% CI $50,231-$122,000). After adjusting for covariates, there was no statistically significant change in mortality (OR = 1.13; 95% CI 0.87-1.48), LOS (β = - 2.1 ± 3.5; 95% CI - 9.0 to 4.8) and hospital cost (β = - 2.137 ± 10.813; 95% CI - 23,331 to 19,056) with each calendar year increase on multivariate logistic regression analysis.

The incidence of neonates with gastroschisis increased between 2010 and 2014. Incidence was highest in the West. No difference in mortality and resource utilization was observed.

The number of infants with gastroschisis is increasing worldwide, but advances in neonatal intensive care and parenteral nutrition have reduced gastroschisis mortality. Recent clinical data on gastroschisis are often from Western nations. This study aimed to examine clinical features and practice patterns of gastroschisis in Japan.

We examined treatment options, outcomes, and discharge status among inpatients with simple gastroschisis (SG) and complex gastroschisis (CG), 2010-2016, using a national inpatient database in Japan.

The 247 eligible patients (222 with SG) had average birth weight of 2102 g and average gestational age of 34 weeks; 30% had other congenital anomalies. Digestive anomalies were most common, followed by circulatory anomalies. In-hospital mortality was 8.1%. The median age at start of full enteral feeding was 30 days. The median length of stay was 46 days. There were no significant differences in outcomes except for length of stay, starting full enteral feeding and total hospitalization costs between the SG and CG groups. About 80% of patients were discharged to home without home medical care. The readmission rate was 28%.

This study's findings on the clinical characteristics and outcomes of gastroschisis are useful for the clinical management of gastroschisis.

In patients undergoing gastroschisis closure, the effects of timing of closure and patient and hospital-level characteristics on length of stay (LOS) and time to enteral autonomy are unknown.

Using the Pediatric Health Information System, we compared neonates who underwent early (within 1day of birth) versus delayed (>1day after birth) gastroschisis closure from 2005 to 2013. We evaluated the relationship between time to closure and both LOS and days on total parenteral nutrition (TPN).

Of 4459 neonates with gastroschisis, 43.9% underwent early closure and 56.1% underwent delayed closure. Delayed closure, complicated gastroschisis, government insurance, lower birth weight, older age at closure, and complex chronic conditions were associated with longer LOS and days on TPN (all p<0.05). There was significant inter-hospital variability in both outcomes, after adjusting for patient- and hospital-level characteristics, including hospitals' gastroschisis and neonatal volumes, median age at closure, and percentages of complicated and delayed gastroschisis patients, (p<0.01).

Delayed gastroschisis closure is associated with longer LOS and duration of TPN, even after excluding complicated cases. Furthermore, after controlling for hospital volume, rate of complicated gastroschisis, and timing of closure, the persistent inter-hospital variability suggests that practice variability is partially responsible for these differences.

Retrospective study.

III.

The incidence of gastroschisis has increased 30% between the periods 1995-2005 and 2006-2012, with the largest increase in Black neonates born to Black mothers younger than 20years old.

Racial disparities in peri- and post-operative outcomes have been previously identified in several types of adult and pediatric surgical patients. Is there an association between race and clinical outcomes and healthcare resource utilization in neonates with gastroschisis?

Retrospective study using national administrative data from the Kid's Inpatient Database (KID) from 2006, 2009, and 2012 for neonates (age<28days) with gastroschisis. Multivariable logistic regression was constructed to determine the association of race and socioeconomic characteristics with complications and mortality; linear regression was used for length of stay and hospital charges.

We identified 3846 neonates with gastroschisis that underwent surgical repair, including 676 patients with complex gastroschisis. When controlling for birth weight, payer status, socioeconomic status, and hospital characteristics, Black neonates had increased odds of having complex gastroschisis and associated atresias. Mortality was higher in patients with complex gastroschisis, patients from the lowest income quartiles, and patients with Medicaid as primary payer (compared to those with private insurance). Length of stay (LOS) was increased in patients with complex gastroschisis, birth weight <2500g, and Medicaid patients. Hospital charges were higher in complex gastroschisis, Black and Hispanic neonates (as compared to Whites), males, birth weight <2500g, and Medicaid patients.

There is an association between race and complex gastroschisis, associated intestinal atresias, and total charges in neonates with gastroschisis. In addition, income status is associated with mortality and hospital charges while payer status is associated with complications, mortality, LOS, and hospital charges. Public health and prenatal interventions should target at-risk populations to improve clinical outcomes.

Level of Evidence: II.

During the last decades neonatal outcomes for children born with gastroschisis have improved significantly. Survival rates >90% have been reported. Early prenatal diagnosis and increased survival enforce the need for valid data for long-term outcome in the pre- and postnatal counseling of parents with a child with gastroschisis.

Long-term follow-up on all newborns with gastroschisis at Odense University Hospital (OUH) from January 1 1997-December 31 2009. Follow-up included neonatal chart review for neonatal background factors, including whether a GORE®DUALMESH was used for staged closure, electronic questionnaires, interview and laboratory investigations. Cases were divided into complex and simple cases according to the definition by Molik et al. (2001). Survival status was determined by the national personal identification number registry. Because of the consistency of the registration, survival status was obtained from all children participating in the study.

A total of 71 infants (7 complex and 64 simple) were included. Overall seven out of the 71 children (9.9%, median age: 52days (25-75% percentile 0-978days) had died at the time of follow-up. Three died during the neonatal period and four died after the neonatal period. Parenteral nutrition (PN) induced liver failure and suspected adhesive small bowel obstruction were the causes of deaths after the neonatal period. Overall mortality was high in the "complex" group compared to the simple group (3/7 (42.9%) vs 4/64 (6.3%), p = 0.04). Forty (62.5%) of the surviving children consented to participate in the follow-up. A total of 12 children had had suspected adhesive small bowel obstruction. Prevalence of small bowel obstruction was not related to the number of operations needed for neonatal closure of the defect. Staged closure was done in 5/12 (41.7%) who developed small bowel obstruction vs 11/35 (31.43%) without small bowel obstruction, p=0.518. A GORE®DUALMESH was used in 16 children (22.5%). Of these 2 were complex and 14 were simple cases. Prevalence of recurrent abdominal pain was 22.5% (9/40) among children with gastroschisis compared to 12% in a study on Danish school children, p=0.068. Gastrointestinal symptoms had led to hospital admission after primary discharge in significantly more children with gastroschisis 16 (40.0%) than children younger than 16years old in the general Danish population 129.419/1.081.542 (12.0%), p=0.000. Fecal calprotectin level was above the reference level (>50mg/kg) in 6/16 (37.5%) children >8years old with gastroschisis compared to 1/7 (14.3%) healthy children. (Fisher's exact=0.366). Only 8/38 (21.1%) children with gastroschisis reported to have an umbilicus.

Mortality among children with gastroschisis is still significant with the highest risk among complicated cases. The majority of the deaths is potentially preventable as PN-related causes and suspected adhesive small bowel obstruction counted for five of seven deaths. Neither categorization upon method of abdominal wall closure nor categorization into simple and complex cases can predict the risk of adhesive small bowel obstruction. With improved administration of PN and timely information and attention to the risk of the small bowel obstruction there is good possibility that the associated mortality could decrease. Type of study and level of evidence: Prognosis study, level II.

Gastroschisis research is limited in quality by the presence of significant heterogeneity in outcome measure reporting (PloS One 10(1):e0116908, 2015). Using core outcome sets in research is one proposed method for addressing this problem (Trials 13:103, 2012; Clin Rheumatol 33(9):1313-1322, 2014; Health Serv Res Policy 17(1):1-2, 2012). Ultimately, standardising outcome measure reporting will improve research quality and translate into improvements in patient care.

Candidate outcome measures have been identified through systematic reviews. These outcome measures will form the starting point for an online, three-phase Delphi process that will be carried out in parallel by three panels of experts. Panel 1 is a neonatal panel, panel 2 is a non-neonatal panel and panel 3 is a lay panel. In round 1, experts will be asked to score the previously identified outcome measures from 1-9 based on how important they think the measures are in determining the overall success of their/their child's/their patient's gastroschisis treatment. In round 2, experts will be presented with the same list of outcome measures and with graphical representations of how their panel scored that outcome in round 1. They will be asked to re-score the outcome measure taking into account how important other members of their panel felt it to be. In round 3, experts will again be asked to re-score each outcome measure, but this time they will receive a graphical representation of the distribution of scores from all three panels which they should take into account when re-scoring. Following round 3 of the Delphi process, 40 experts will be invited to attend a face-to-face consensus meeting. Participants will be invited in a purposive manner to obtain balance between the different panels. The results of the Delphi process will be discussed, and outcomes re-scored. Outcome measures where > 70 % of the participants at the meeting scored them as 7-9 and < 15 % scored them as 1-3 will form the core outcome set.

Development of a core outcome set will help to reduce the heterogeneity of the outcome measure reporting in gastroschisis. This will increase the quality of research taking place and ultimately improve care provided to infants with gastroschisis.

To determine the most cost-effective timing of delivery in pregnancies complicated by gastroschisis, using a decision-analytic model.

We created a decision-analytic model to compare planned delivery at 35, 36, 37, 38 and 39 weeks' gestation. Outcomes considered were stillbirth, death within 1 year of birth and respiratory distress syndrome (RDS). Probability estimates of events (stillbirth, complex gastroschisis and RDS for each gestational age at delivery and risk of death with simple and complex gastroschisis), utilities and costs assigned to the outcomes were obtained from the published literature. Cost analysis was assessed from a societal perspective, using a willingness-to-pay threshold of $100,000 per surviving infant. Outcomes and costs were considered throughout 1 year of postnatal life. Multiway sensitivity analysis was performed to address uncertainties in baseline assumptions.

In the base-case analysis, delivery at 38 weeks' gestation was the most cost-effective strategy. Planned delivery at 35 weeks was associated with the fewest stillbirths and deaths within 1 year of delivery, owing largely to a lower ongoing risk of stillbirth. In Monte Carlo simulation when every variable was varied over its entire range, delivery at 38 weeks was cost-effective compared to delivery at 39 weeks in 76% of trials and delivery at 37 weeks was cost-effective in 69% of trials. Delivery at 38 weeks resulted in three additional cases of RDS for every 100 stillbirths or deaths within 1 year that were prevented.

For pregnancies complicated by gastroschisis, the most cost-effective timing of delivery is at 38 weeks. Few additional cases of RDS are caused for every one stillbirth or death within 1 year that was prevented with delivery at 37-38 weeks compared with at 39 weeks.

The abdominal wall is an integral component of the chest wall. Defects in the ventral abdominal wall alter respiratory mechanics and can impair diaphragm function. Congenital abdominal wall defects also are associated with abnormalities in lung growth and development that lead to pulmonary hypoplasia, pulmonary hypertension, and alterations in thoracic cage formation. Although infants with ventral abdominal wall defects can experience life-threatening pulmonary complications, older children typically experience a more benign respiratory course. Studies of lung and chest wall function in older children and adolescents with congenital abdominal wall defects are few; such investigations could provide strategies for improved respiratory performance, avoidance of respiratory morbidity, and enhanced exercise ability for these children.

The optimal surgical approach in infants with gastroschisis (GS) is unknown. The purpose of this study was to estimate the association between staged closure and length of stay (LOS) in infants with GS.

We used the Children's Hospital Neonatal Database to identify surviving infants with GS born ≥34 weeks' gestation referred to participating NICUs. Infants with complex GS, bowel atresia, or referred after 2 days of age were excluded. The primary outcome was LOS; multivariable linear regression was used to quantify the relationship between staged closure and LOS.

Among 442 eligible infants, staged closure occurred in 68.1% and was associated with an increased median LOS relative to odds ration (OR):primary closure (37 vs. 28 days, p<0.001). This association persisted in the multivariable equation (β=1.35, 95% CI: 1.21, 1.52, p<0.001) after adjusting for the presence of necrotizing enterocolitis, short bowel syndrome, and central-line associated bloodstream infections.

In this large, multicenter cohort of infants with GS, staged closure was independently associated with increased LOS. These data can be used to enhance antenatal and pre-operative counseling and also suggest that some infants who receive staged closure may benefit from primary repair.

To describe the outcomes of gastroschisis (GS) patients managed with and without a multidisciplinary team during the postoperative period in Canada.

The Canadian Pediatric Surgery Network (CAPSNet) collects patient data from all Canadian tertiary perinatal centres. The outcomes of 396 GS patients born between 2005 and 2009 who were managed with and without a multidisciplinary team were analyzed.

Significantly more parenteral nutrition (PN) days (P=0.003) and longer lengths of stay (P=0.018) were observed among patients in centres with multidisciplinary teams. Higher mortality rate and earlier death were observed in centres without multidisciplinary teams, especially for high-risk patients, although this was not statistically significant. With regard to low-risk patients, those in centres with multidisciplinary teams had significantly more PN days (P=0.019).

GS patients managed by multidisciplinary teams had significantly more PN days and longer lengths of stay compared with patients who were not managed by multidisciplinary teams. This difference may be due to improved survival of high-risk patients in centres with multidisciplinary teams, and a uniform feeding approach for all patients.

A relationship between gastroschisis-associated gastroesophageal reflux (GER) and hiatal hernia (HH) has not been previously reported. In reviewing our experience with gastroschisis-related GER, we noted a surprising incidence of associated HH in patients requiring antireflux procedures.

A single center retrospective chart review focused on GER in all gastroschisis patients repaired between January 1, 2000 and December 31, 2012 was performed.

Of the 141 patients surviving initial gastroschisis repair and hospitalization, 16 (11.3%) were noted to have an associated HH (12 Type I, 3 Type II, 1 Type III) on upper gastrointestinal series for severe reflux. Ten of the 13 (76.9%) patients who required an antireflux procedure had an associated HH. The time to initiation of feeds was similar in all patients, 19 and 23 days. However, time to full feedings and discharge was delayed until a median of 80 and 96 days, respectively, in HH patients.

This study describes a high incidence of associated HH in gastroschisis patients. The presence of large associated HH correlated with severe GER, delayed feeding, requirement for antireflux surgery, and a prolonged hospital stay. Patients with gastroschisis and clinically severe GER should undergo early assessment for associated HH.

Gastroschisis may be complicated by intestinal atresia, necrosis, and/or perforation. In the absence of an urgent indication, intestinal procedures are often delayed to allow for bowel recovery. This practice has not been evaluated.

We queried a prospective Canadian database of all patients with gastroschisis born between 2005 and 2011. Patients with intestinal atresia who underwent an intestinal operation during the first 21 days of life (EARLY GROUP) were compared with those who underwent operations later (LATE GROUP).

Of 629 gastroschisis patients, 78 (12.4%) had intestinal complications; 27 patients (4.3%) had intestinal operations for atresia without necrosis or perforation - 14 EARLY and 13 LATE. Baseline clinical parameters were similar between the two groups. There was a decreased incidence of the following complications in the EARLY group but none reached statistical significance: post-operative bowel obstruction (28.6% vs. 61.5%, p = 0.1); line sepsis (14.3% vs. 30.8 %, p = 0.4); and wound infection (14.3% vs. 46.1%, p = 0.1). Earlier tolerance of enteral feeding in the EARLY group was manifested by younger age at first enteral feeding (14.8 + 2.6 vs. 44.7 + 7.4 days, p = 0.002) and higher tolerance of enteral feeding at 28 days of life [less patients exclusively on TPN (28.6% vs. 61.5%, p = 0.06), and more patients on more than 50 cc kg(-1)day(-1) of enteral feeding (42.9% vs. 7.7%, p = 0.08)].

Early intestinal operations in patients with gastroschisis and intestinal atresia are not associated with increased complications, and allow patients to receive and tolerate enteral feeding earlier.

To determine if the choice of enteral feeds after gastroschisis repair relates to the time to achieve full feeds and time to discharge.

A retrospective study of infants with gastroschisis from 2000 to 2010 examined demographics, days at closure, days at initiation of feeds, days to full feeds, time to discharge and length of stay.

Ninety infants were identified, 22 received (human milk) HM exclusively, 15 were fed >50% HM, 16 were fed <50% HM and 26 were fed only cow milk-based formulas. Infants fed exclusively HM had significantly shorter times to full enteral feedings (median 5 days versus 7 days, P=0.03). The time from initiation of feedings to hospital discharge, which accounts for initiation age, significantly favored the exclusively HM-fed infants (median 7 days versus 10 days, P=0.01).

Exclusive HM feeding after gastroschisis repair decreases time to achieve full enteral feeds and time to discharge.

The aim of the study was to describe the physical growth and developmental outcomes of babies born with gastroschisis.

We retrospectively reviewed all cases of gastroschisis in Western Australia born between 1997 and 2010.

In the 128 pregnancies with fetal gastroschisis, 117 babies were live born. 112 (95.7%) survived to one year. 19% had z scores of<-1.28 for weight at birth (<10th centiles) compared with 30% at one year. Neurodevelopmental data were available in 88/112 (79%) of survivors (Griffiths scores in 67; reports of ages and stages questionnaire (ASQ) in 21). The mean GQ at 12 months was 99 (SD 9.8). Suboptimal neurodevelopmental outcomes were noted in eight. Complex gastroschisis (present at birth) and acquired gut related complications were associated with adverse long term outcomes. The incidence of acquired gut complications was least (5%) in those who underwent silo reduction as the primary management. However, on univariate and multivariate analysis, the type of primary reduction did not significantly influence the outcome.

A large proportion of infants with gastroschisis exhibit suboptimal weight gain during the first year. The incidence of adverse developmental outcomes appears to be low.

Gastroschisis is the most common congenital abdominal wall defect. Despite advances in the surgical closure of gastroschisis, consensus is lacking as to which method results in the best patient outcomes. The purpose of this meta-analysis was to compare short-term outcomes associated with primary fascial closure and staged repair with a silo in patients with gastroschisis.

We reviewed Medline citations, as well as the Cochrane Database of Systematic Reviews, between January 1, 1996 and June 1, 2012. Articles were identified using the search term "gastroschisis" and [("treatment outcome" or "prognosis") or randomized controlled trials]. Case reports, reviews, letters, abstracts only, non-English abstracts, and studies that did not address at least one of the outcomes of interest were excluded from the meta-analysis. Two independent reviewers identified relevant articles for final inclusion. A standard data collection form created by the authors was used to extract study information, including study design, patient characteristics, and reported patient outcomes. The data were analyzed using standard meta-analytic techniques.

Twenty studies were included in the meta-analysis. In the five studies that selected closure method randomly or as a temporal shift in practice, silo was associated with better outcomes, with a significant reduction in ventilator days (p<0.0001), time to first feed (p=0.04), and infection rates (p=0.03). When all studies were included, primary closure was associated with improved outcomes.

Silo closure is associated with better clinical outcomes in the studies with the least selection bias. Larger prospective studies are needed to definitively determine the best closure technique.

Our goal is to identify the impact of time to surgical intervention on the outcomes of infants with gastroschisis.

After institutional review board approval, we performed a retrospective review of the medical records of all infants admitted to our institution from 2001 to 2010. Transport, bowel stabilization, and closure times were defined as the time from birth to admission, admission to the first-documented operative intervention, and first operative intervention to abdominal closure, respectively. Outcomes included age at full enteral feeds, total parental nutrition days, ventilator days, and hospital length of stay. Multivariate analysis was used to identify independent predictors of the outcomes.

One hundred eighteen infants with gastroschisis were included in our study. Transport and bowel stabilization times were not predictive of any outcome. However, the time to abdominal wall closure and postnatal gastrointestinal complications were independently predictive of age at full enteral feeds, total parenteral nutrition days, and hospital length of stay.

Time to surgical evaluation/bowel stabilization was not predictive of any clinically relevant outcomes in infants with gastroschisis. These data demonstrate that potential benefits from prenatal regionalization of infants with gastroschisis are not supported by decreased time to operative intervention.

Infants with gastroschisis (GS) have significant morbidity from dysmotility, feeding intolerance, and are at increased risk of developing intestinal failure. Although the molecular mechanisms regulating GS-related intestinal dysfunction (GRID) are largely unknown, we hypothesized that mechanical constriction (nonocclusive mesenteric hypertension (NMH)) from the abdominal wall defect acts as a stimulus for GRID. The purpose of this study was to determine the effect of NMH on intestinal function and inflammation.

Neonatal rats had placement of a silastic disk to the base of the mesentery (NMH) or no disk placement (Sham). At 24 and 72 h, mesenteric venous pressures (MVPs), intestinal transit, electric impedance, permeability, length, and tissue water content were measured.

After placement of the silastic disk, there was a significant increase in MVP at both time points. There was also decreased intestinal transit. As compared to Sham animals, NMH animals had significant changes in bowel impedance without an increase in tissue water, suggesting significant intestinal remodeling. NMH rats had significantly increased smooth-muscle thickness and loss of intestinal length as compared with Sham rats.

NMH may be an initiating factor for GRID. Measurement of MVP and/or bowel impedance may be a way to assess severity and monitor progression and/or resolution of GRID.

Correction of gastroschisis may be accomplished by either primary or staged closure or even delayed primary closure after the use of a preformed silo. However, there is neither a consensus on the best approach nor established criteria to favor one method over the other. The aim of this paper was to investigate the role of intravesical pressure (IVP) as a tool to prevent abdominal compartment syndrome in newborns undergoing correction of abdominal wall defects.

We retrospectively analyzed 45 newborns with gastroschisis in whom trans-operative intravesical pressure was used to choose between primary or staged closure. A threshold of 20 cm H(2)O was used and the outcomes between the two methods were compared.

In 24 children delayed primary closure was achieved while the remaining 21 underwent staged reduction and closure. There was no difference in the frequency of complications, time to begin oral feeding, length of parenteral nutrition or length of hospital stay between the children of the two groups. The incidence of temporary oliguria or anuria, averaged 33% and it was similar in both groups of children.

The data here presented suggests that monitoring intraoperative IVP during correction of gastroschisis may help to select children in whom staged closure is necessary, keeping their complication rate and overall outcome similar to that of children undergoing delayed primary closure. Further prospective studies should investigate more deeply the correlation between type of closure and the development of a compartment syndrome.

To describe one year outcomes for a national cohort of infants with gastroschisis.

Population based cohort study of all liveborn infants with gastroschisis born in the United Kingdom and Ireland from October 2006 to March 2008.

All 28 paediatric surgical centres in the UK and Ireland.

301 infants (77%) from an original cohort of 393.

Duration of parenteral nutrition and stay in hospital; time to establish full enteral feeding; rates of intestinal failure, liver disease associated with intestinal failure, unplanned reoperation; case fatality.

Compared with infants with simple gastroschisis (intact, uncompromised, continuous bowel), those with complex gastroschisis (bowel perforation, necrosis, or atresia) took longer to reach full enteral feeding (median difference 21 days, 95% confidence interval 9 to 39 days); required a longer duration of parenteral nutrition (median difference 25 days, 9 to 46 days) and a longer stay in hospital (median difference 57 days, 29 to 95 days); were more likely to develop intestinal failure (81% (25 infants) v 41% (102); relative risk 1.96, 1.56 to 2.46) and liver disease associated with intestinal failure (23% (7) v 4% (11); 5.13, 2.15 to 12.3); and were more likely to require unplanned reoperation (42% (13) v 10% (24); 4.39, 2.50 to 7.70). Compared with infants managed with primary fascial closure, those managed with preformed silos took longer to reach full enteral feeding (median difference 5 days, 1 to 9) and had an increased risk of intestinal failure (52% (50) v 32% (38); 1.61, 1.17 to 2.24). Event rates for the other outcomes were low, and there were no other significant differences between these management groups. Twelve infants died (4%).

This nationally representative study provides a benchmark against which individual centres can measure outcome and performance. Stratifying neonates with gastroschisis into simple and complex groups reliably predicts outcome at one year. There is sufficient clinical equipoise concerning the initial management strategy to embark on a multicentre randomised controlled trial comparing primary fascial closure with preformed silos in infants suitable at presentation for either treatment to determine the optimal initial management strategy and define algorithms of care.

This study attempted to evaluate the association of early hypoalbuminemia with the risk of intestinal failure in gastroschisis patients.

Neonates with gastroschisis treated at a tertiary children's hospital over a 10-year period were initially categorized into groups based on the lowest serum albumin measurement during the first 7 days of life. Based on preliminary analysis, patients with serum albumin <1.5 g/dL were considered to have early severe hypoalbuminemia. Intestinal failure was defined as inability of the patient to wean from parenteral nutrition (PN) during the initial hospital admission, thus requiring home PN. Logistic regression modeling was performed to adjust for sex, gestational age, birth weight, and concomitant intestinal complications.

One hundred and thirty-five gastroschisis patients were included, of whom 21% had early severe hypoalbuminemia. Patients with early severe hypoalbuminemia had a significantly higher risk of intestinal failure compared to those with higher albumin levels (26 vs. 8%, p = 0.015). On multivariable logistic regression modeling, early severe hypoalbuminemia was strongly associated with intestinal failure (OR 6.4, 95% CI 1.8-23.3, p = 0.005).

Early severe hypoalbuminemia appears to be an independent risk factor for long-term intestinal compromise rather than merely an indicator of overall illness. Further interventional studies are needed to determine whether clinical protocols utilizing judicious fluid administration, exogenous albumin, and early enteral feeding can improve clinical outcomes in gastroschisis.

The optimal management of neonates with gastroschisis is unclear, and there is a significant morbidity. We performed a review of neonates with gastroschisis treated at our center of pediatric surgery over the last 21 years to determine predictive factors of outcome.

Single-center retrospective analysis of 79 neonates with gastroschisis (1989-2009) was done. Length of hospital stay (LOS), days of parenteral nutrition (PN), and survival were outcome measures. Univariate and multiple regression analyses were used.

Overall survival was 92%, and primary closure was achieved in 80%. Median LOS was 25 days, and median duration on PN, 17 days. Intestinal atresia, closed gastroschisis, secondary closure, and sepsis were the primary variables associated with poor outcome independent of other variables, but prematurity also affected outcome. Route of delivery and associated malformations were not related to poorer outcome. Necrotizing enterocolitis did not occur in any of our patients.

Outcome in our patients was favorable as measured by survival, LOS, and days on PN. Primary predictors of poor outcome were factors related to short bowel syndrome and secondary closure, indicating a need to further improve treatment of short bowel syndrome.

Babies with gastroschisis (G) have high morbidity rate and long hospital stay due to bowel hypomotility caused by chronic exposure of the bowel to the amniotic fluid. Our aim was to evaluate the reactivity of isolated ileum in fetal rats selected for experimental gastroschisis.

G was surgically created at 18.5 days of gestation (term = 22 days). Concentration-dependent curve to the muscarinic agonist methacholine (1-30 μM) and contractions induced by electrical field stimulation (EFS, 1-16 Hz, 50 V, 1 ms) were carried out in isolated ileum of groups control (C), sham (S) and gastroschisis (G) (n = 30). Protein expression for M(3) was assessed by western blot analysis.

The frequency and amplitude of spontaneous contractions were decreased in G (p < 0.001). Methacholine produced concentration-dependent contractions being the maximal response values higher in G (p < 0.01). EFS-induced frequency-dependent contractions showed 1.8 times higher in G as well as an increase of M(3) expression.

The frequency and the amplitude of rhythmic contractions were reduced along with an increase in the contraction induced by mucarinic agonist and by EFS in G. These results suggest the occurrence of an adaptative supersensitivity to cholinergic response via increases in the protein expression for M(3) receptor.

To compare perinatal and infant surgical outcomes in fetuses with gastroschisis with and without gastric dilation in a single-center cohort.

This was a retrospective study of all singleton pregnancies with a prenatal diagnosis of gastroschisis managed at University of Toronto perinatal centers between January 2001 and February 2010. Digital prenatal ultrasound images were reviewed to determine fetal gastric size within 2 weeks of delivery. Perinatal and surgical outcomes were compared in fetuses with and without gastric dilation including: gestational age at delivery, mode of delivery, indication for Cesarean section, meconium-stained amniotic fluid, birth weight percentile, Apgar scores at 1 and 5 min, umbilical artery pH, time to full enteral feeding, length of hospital stay, bowel atresia or necrosis and need for bowel resection.

Ninety-eight fetuses with prenatally diagnosed gastroschisis managed at our center were included in the study, of which 32 (32.7%) were found to have gastric dilation. Gastric dilation predicted meconium-stained amniotic fluid at delivery (53% vs. 24%; P = 0.017), but no other adverse perinatal outcome. Surgical morbidity rates (bowel atresia, bowel necrosis, perforation diagnosed postnatally, need for bowel resection, total time to full enteral feeding and length of hospital stay) were unaffected by gastric dilation.

In gastroschisis, fetal gastric dilation is associated with meconium-stained amniotic fluid at delivery, but is not predictive of any serious perinatal or postnatal complications. Fetal growth and well-being should be serially evaluated on ultrasound using biophysical and Doppler assessment to decide on the optimal timing and mode of delivery.

The pathogenesis of gastroschisis is unknown. It may be helpful in understanding its pathogenesis to know the structural relationships among umbilical components including umbilical vessels, urachus, and vitelline structures, and thus, the authors investigated the remnants of vitelline structures in a series of cases of gastroschisis.

Medical records of 41 cases with gastroschisis treated in our institute from 1979 to 2009 were retrospectively reviewed.

Paraumbilical bands, possible remnants of vitelline structures, were observed in 4 cases (9.8%). All 4 bands were attached to the skin edge of the abdominal defect without incorporation into the umbilical cord. The band ended at the mesentery in 3 cases and at the antimesenteric site of the ileum in the remaining case. Histologic findings showed fibrous tissues in all cases. One was possibly associated with the development of colonic atresia. Another was noticed after silo reduction when herniated bowels became strangulated by the band. The other 2 cases were uncomplicated.

Our findings may support the recently proposed hypothesis that the developmental failure of the yolk sac and related vitelline structures to merge with or to be incorporated into the umbilical stalk might be associated with the pathogenesis of the abdominal wall defect in gastroschisis. Paraumbilical bands derived from vitelline structures may possibly cause intestinal ischemia prenatally or postnatally.