Search

Objectives: In gastroschisis, the intestines are exposed to amniotic fluid during pregnancy. The defect in the abdominal wall may also compress the mesentery and impair the intestinal blood supply. There is a varying degree of intestinal damage at birth. Complex gastroschisis is defined as gastroschisis with severe complications such as intestinal atresia, volvulus, necrosis and perforation. The aim of this study was to investigate long-term gastrointestinal morbidity and compare simple and complex cases., Methods: A nation-wide retrospective cohort study with data from national registers was conducted. All children born with gastroschisis in Sweden from 1 January 1997 to 31 December 2016 were included in the study. Exposure was complex gastroschisis and the primary outcomes were intestinal failure and bowel obstruction., Results: The study included 315 cases with gastroschisis, 260 classifieds as simple gastroschisis and 55 as complex. The median time to follow was 8 years. A significantly higher risk of developing intestinal failure (hazard ratio [HR]: 11.7) was found in complex cases. Nine percent of the complex cases underwent autologous gastrointestinal reconstructive surgery for intestinal failure, none of the simple cases did. The complex cases had a higher risk for bowel obstruction (HR: 4.3) with a higher proportion requiring surgery (18.2% vs. 6.9%) compared to simple cases., Conclusions: This nationwide study showed that the risk for intestinal failure and bowel obstruction is significantly higher for children with complex gastroschisis compared to simple gastroschisis. Most of the events occurred during the first 2 years of life., (© 2024 The Author(s). Journal of Pediatric Gastroenterology and Nutrition published by Wiley Periodicals LLC on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Omphalocele and gastroschisis are the most common types of abdominal wall defects. Comprehensive local experience helps parents to make decisions on the pregnancy and foresee the disease journey. A retrospective review of abdominal wall defect patients in all three pediatric surgical centers in Hong Kong between January 2003 and February 2023 was conducted. All patients consecutively diagnosed with omphalocele and gastroschisis were included, excluding other forms. Data of demographics and short- and long-term outcome parameters were collected. A total of 99 cases were reviewed and 85 patients met the inclusion criteria. Diagnoses include omphalocele major (n = 49, 57.6%), omphalocele minor (n = 22, 25.9%) and gastroschisis (n = 14, 16.5%), with mean gestational age 37 weeks (SD 2.2) and birth weight 2.7 kg (SD 0.6). Omphalocele is most commonly associated with cardiovascular (n = 28, 39.4%) and chromosomal defects (n = 11, 15.5%). Surgical procedures including primary repair (n = 38, 53.5%), staged closure (n = 30, 42.3%) with average 8.6 days (SD 4.7) of silo reduction, and conservative management (n = 3, 4.2%) were performed. The mortality rate was 14.1% (n = 10) and the complication rate was 36.6% (n = 26). The majority of patients had normal intellectual development (92.5%) and growth (79.2%) on the latest follow-up. For gastroschisis, one patient (7.1%) had intestinal atresia. Surgical procedures included primary repair (n = 9, 64.3%) and staged closure (n = 5, 35.7%) with average 8 days (SD 3.5) of silo reduction. Complication rate was 21.4% (n = 3), with one mortality (7.1%). All patients had normal intellectual development and growth. The mean follow-up time of this series is 76.9 months (SD 62.9). Most abdominal wall defects in our series were managed surgically with a good overall survival rate and long-term outcome. This information is essential during antenatal and postnatal counseling for parents., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Neonates with intestinal failure (IF) are at risk for infection due to central venous access, and intestinal surgery. Infection can cause systemic inflammation and sepsis, potentially affecting growth. The purpose of this study was to identify risk factors for, and the potential impact of infection to help with preventative strategies., Methods: A retrospective review of infants with IF, at a single centre from 2018 to 2022 was conducted. Clinical characteristics, intestinal pathology, nutritional intake, and growth were compared among infants with bloodstream infection (BSI), other infection (OI) (urinary, respiratory, or wound), or no infection (NI) within 2 months of diagnosis. Mann-Whitney and Kruskal-Wallis tests were used for comparisons with p-values <0.05 considered significant., Results: Eighty-six infants were included, with gastroschisis (41%) and necrotizing enterocolitis (26%) the most common diagnoses. Fifty-nine % of infants developed infection (22% BSI and 37% OI). Those with BSI or OI had a lower gestational age and birthweight, and were more likely to have a stoma. All infants with complex gastroschisis developed infection compared to 38% of infants with simple gastroschisis. Median daily weight gain was suboptimal across all groups and did not differ over 6 weeks following infection., Conclusion: Most infants with IF develop infection shortly after diagnosis. Risk factors include prematurity, complex gastroschisis, and the presence of a stoma. Growth was suboptimal but did not differ among infants with or without infection., Type of Study: Retrospective Review., Level of Evidence: Level III Retrospective Comparative Study., Competing Interests: Conflicts of interest The authors have no conflicts of interest to declare., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objectives: Infants with anterior abdominal wall defects (AWD) can suffer from pulmonary complications. Our aims were to determine if the chest radiographic thoracic areas (CRTAs) on day one differed between infants with exomphalos or gastroschisis, whether this related to differing severity of outcomes and if they were lower than those of controls indicating abnormal antenatal lung growth., Methods: A review of infants with exomphalos or gastroschisis born between January 2004 and January 2023 was conducted. The control group was term, newborn infants ventilated for poor respiratory drive at birth. Chest radiographs on day one were analysed and the highest CRTA in the first 24 h after birth for each infant included in the analysis., Results: The 127 infants with gastroschisis had a lower gestational age and birthweight than the 62 exomphalos infants and 130 controls (all p<0.001) The CRTAs of the controls were greater than the CRTAs of the exomphalos and the gastroschisis infants (p = 0.001). The median CRTA corrected for birthweight was lower in the exomphalos infants [688, IQR 568-875 mm 2 /kg] than the gastroschisis infants [813, IQE 695-915 mm 2 /kg] No gastroschisis infant developed bronchopulmonary dysplasia (BPD). A CRTA of 1759 mm 2 had a sensitivity of 81 % and specificity of 71 % in predicting BPD in infants with exomphalos., Conclusions: Infants with gastroschisis or exomphalos had lower CRTAs than controls suggesting both groups had abnormal antenatal lung development. The CRTA was lower in the exomphalos infants who also had worse respiratory outcomes, hence CRTA assessment may a useful prognostic aid., (© 2024 the author(s), published by De Gruyter, Berlin/Boston.)

Introduction:  The purpose of this study is to describe the long-term growth and nutrition outcomes of sutureless versus sutured gastroschisis repair. We hypothesized that weight z-score at 1 year would be affected by social determinants of health measured by the U.S. Centers for Disease Control Social Vulnerability Index (SVI)., Materials and Methods:  We conducted a single-center retrospective review of patients who underwent gastroschisis repair ( n  = 97) from 2007 to 2018. Growth z-scores collected through 5 years of age and long-term clinical outcomes were compared based on the closure method and the type of gastroschisis (simple vs. complicated). Multiple regression analysis was performed to identify the impact of SVI themes and other covariates on weight for age z-score at 1 year., Results:  In total, 46 patients underwent sutureless repair and 51 underwent sutured repair with median follow-up duration of 2.5 and 1.9 years, respectively. Weight and length z-scores decreased after birth but normalized within the first year of life. Growth and long-term clinical outcomes were similar regardless of the closure method, while patients with complicated gastroschisis had higher rates of hospitalizations, small bowel obstructions, and additional abdominal surgeries. Using multiple regression, both low discharge weight and high SVI in the "minority status and language" theme were associated with lower weight for age z-scores at 1 year ( p  = 0.003 and p  = 0.03)., Conclusion:  Sutureless and sutured gastroschisis repairs result in similar growth and long-term outcomes. Patients living in areas with greater social vulnerability may be at increased risk of poor weight gain. Patients should be followed at least through their first year to ensure appropriate growth., Competing Interests: None declared., (Thieme. All rights reserved.)

Objective: Gastroschisis (GS) is a congenital anomaly in the abdominal wall with the intestinal loops exiting laterally to the umbilicus. The contact of the loops with Amniotic Fluid (AF) causes an inflammatory process in the exposed part, leading to an extended hospital stay and an increased risk of morbidity due to alterations related to intestinal motility. The authors aimed to evaluate the time of exposure to the AF in the experimental GS and to search for potential biomarkers of intestinal inflammation by measuring microRNAs., Methods: Rat fetuses were divided into three groups: a) CONTROL, b) GS reared on day 18 (GS = 18), and c) GS reared on day 19.5 (GS = 19) (term = 22 days). On day 21.5, the fetuses were removed for biometric parameters and biochemical analyses: 1) Biometrics: Body and Intestinal Weight (BW, IW), and intestinal-body weight ratio (IW/BW); 2) Descriptive histopathology and 3) miR-143 quantification by real-time Polymerase Chain Reaction (PCR)., Results: BW was higher in CONTROL than GS 18 and G19 (p < 0.05). IW, IW/BW, intestinal water, and mRNA-143 were higher in GS 18 and GS 19 than in CONTROL, and GS 18 was higher than GS 19 (p < 0.05). The average of the inflammation score from the intestinal wall with mucosal inflammation and intra-epithelial lymphocytes shows worst in GS 18 and GS 19 vs. CONTROL (p < 0.05)., Conclusions: The tissue expression of mRNA-143 and the morphological changes in the intestine of GS worsened according to the time of exposure to AF, which could be a possible marker of fetal intestinal damage., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest., (Copyright © 2023 HCFMUSP. Published by Elsevier España, S.L.U. All rights reserved.)

Background: Two strong risk factors for gastroschisis are young maternal age (<20 years) and low/normal pre-pregnancy body mass index (BMI), yet the reasons remain unknown. We explored whether neighborhood-level socioeconomic position (nSEP) during pregnancy modified these associations., Methods: We analyzed data from 1269 gastroschisis cases and 10,217 controls in the National Birth Defects Prevention Study (1997-2011). To characterize nSEP, we applied the neighborhood deprivation index and used generalized estimating equations to calculate odds ratios and relative excess risk due to interaction., Results: Elevated odds of gastroschisis were consistently associated with young maternal age and low/normal BMI, regardless of nSEP. High-deprivation neighborhoods modified the association with young maternal age. Infants of young mothers in high-deprivation areas had lower odds of gastroschisis (adjusted odds ratio [aOR]: 3.1, 95% confidence interval [CI]: 2.6, 3.8) than young mothers in low-deprivation areas (aOR: 6.6; 95% CI: 4.6, 9.4). Mothers of low/normal BMI had approximately twice the odds of having an infant with gastroschisis compared to mothers with overweight/obese BMI, regardless of nSEP (aOR range: 1.5-2.3)., Conclusion: Our findings suggest nSEP modified the association between gastroschisis and maternal age, but not BMI. Further research could clarify whether the modification is due to unidentified biologic and/or non-biologic factors., (© 2023 Wiley Periodicals LLC.)

Objective: To describe the incidence and to analyze risk factors associated with cholestasis in neonates with gastroschisis., Methods: 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., Results: 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., Conclusions: 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.

Gastroschisis mortality is 75-100% in low-resource settings. In Rwanda, late deaths are often due to sepsis. We aimed to understand the effect of antimicrobial use on survival. We conducted a retrospective review of gastroschisis patients at a tertiary hospital in Kigali, Rwanda between January 2016-June 2019. Demographics, antimicrobial use, microbiology, and outcomes were abstracted. Descriptive and univariate analyses were conducted to assess factors associated with improved survival. Among 92 gastroschisis patients, mortality was 77%(n = 71); 23%(n = 21) died within 48 h. 98%(n = 90) of patients received antibiotics on arrival. Positive blood cultures were obtained in 41%(n = 38). Patients spent 86%(SD = 20%) of their hospital stay on antibiotics and 38%(n = 35) received second-line agents. There was no difference in age at arrival, birth weight, gestational age, silo complications, or antimicrobial selection between survivors and non-survivors. Late death patients spent more total hospital days and post-abdominal closure days on antibiotics (p < 0.001) compared to survivors. There was no difference in the proportion of hospital stay on second-line antibiotics (p = 0.1)., Conclusion: We identified frequent late deaths, prolonged antibiotic courses, and regular use of second-line antibiotic agents in this retrospective cohort of Rwandan gastroschisis patients. Future studies are needed to evaluate antimicrobial resistance in pediatric surgical patients in Rwanda., What Is Known: • Global disparities in gastroschisis outcomes are extreme, with <4% mortality in high-income settings and 75-100% mortality in low-income settings. • Antimicrobial surveillance data is sparse across Africa, but existing evidence suggests high levels of resistance to first-line antibiotics in Rwanda., What Is New: • In-hospital survival for gastroschisis was 23% from 2016-2019 and most deaths occurred late (>48hrs after admission) due to sepsis. • Rwandan gastroschisis patients received prolonged courses of antibiotics and second-line antibiotics were frequently used without culture data, raising concern for antimicrobial resistance., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Gastroschisis with colonic atresia is a rare association. Due to its rarity and variation in presentation, no standardized surgical treatment option exists. Complicated gastroschisis is associated with a higher morbidity and mortality than intestinal atresia or gastroschisis alone., Methods: This is a case report of a neonate with congenital gastroschisis. On day of life 1, a silo was placed. On day of life 4, the upper portion of silo contents appeared more congested with dusky discoloration and the patient was oliguric. She was taken to the operating room for exploration., Results: The patient required resection of the terminal ileum and an atretic portion of the cecum due to necrosis. Four days later, primary closure of the gastroschisis defect was performed with creation of an end ileostomy. Elective ileostomy takedown was performed 5 months later. She returned to the operating room for anastomotic revision and gastrostomy tube placement for intestinal dysmotility and prolonged ileus. Tube feedings were discontinued 3 months post-operatively., Conclusions: Gastroschisis with intestinal atresia is associated with increased hospital length of stay, longer duration of parenteral nutrition, more severe intestinal dysfunction, increased surgical complications, and higher mortality than gastroschisis or intestinal atresia alone. With associated colonic atresia, loss of a significant length of bowel due to necrosis, including the ileocecal region, is almost unavoidable. An already compromised blood supply to the atretic bowel is further impaired by a tight abdominal wall defect and postnatal increases in gravitational force. Complicated gastroschisis remains a complex surgical challenge requiring further discussion.

Background: Gastroschisis is a challenging neonatal condition often with prolonged hospitalizations, need for parenteral nutrition, infectious complications, and can even result in death. Infection is reported to occur in up to two-thirds of patients with gastroschisis and is a strong risk factor for increased morbidity and mortality. Increased days with a central venous catheter, complex gastroschisis, and delayed abdominal wall closure have been consistently found to be associated with increased risk of infection, whereas sutureless gastroschisis closure has been associated with fewer infections. Although one of the most common complications of gastroschisis is infection, the use of antibiotic agents varies widely with variability in the literature to guide management. Antibiotic usage should be selective and short-term, especially in neonates with simple gastroschisis regardless of method for abdominal wall closure. Conclusions: Future initiatives should focus on development of evidence-based guidelines on the care of these patients with the goal of reducing variability and improve outcomes within and across institutions.

Objective: This study aimed to verify risk factors associated with gastroschisis mortality in three neonatal intensive care units located in the state of Espírito Santo, Brazil., Methods: A retrospective cohort study of neonates with gastroschisis was performed between 2000 and 2018. Prenatal, perinatal, and postsurgical variables of survival or nonsurvival groups were compared using chi-square statistical test, t-test, Mann-Whitney U test, and logistic regression. Tests with p<0.05 were considered statistically determined., Results: A total of 142 newborns were investigated. Mean maternal age, gestational age, and birth weight were lower in the group of nonsurvival (p<0.05). Poor clinical conditions during admission, complex gastroschisis, closure with silo placement, the use of blood products, surgical complications, and short bowel syndrome were more frequent in the nonsurvival group (p<0.05). Complex gastroschisis [adjusted odds ratio (OR) 3.74, 95% confidence interval (95%CI) 1.274-11.019] and short bowel syndrome (adjusted OR 7.55, 95%CI 2.177-26.225) increased the risk of death. Higher birth weight inversely reduced the risk for mortality (adjusted OR 0.99, 95%CI 0.997-1.000)., Conclusion: Complex gastroschisis and short bowel syndrome increased the risk of death, with greater birth weight being inversely correlated with the risk of mortality. The findings of this research can contribute to the formulation of protocols to improve the quality and safety of care in order to reduce neonatal mortality associated with gastroschisis.

Importance: Psychiatric disorders are common among female individuals of reproductive age. While antipsychotic medication use is increasing, the safety of such medications in pregnancy is an area with large evidence gaps., Objective: To evaluate the risk of first-trimester antipsychotic exposure with respect to congenital malformations, focusing on individual drugs and specific malformation subtypes., Design, Setting, and Participants: This cohort study used data from nationwide health registers from the 5 Nordic countries and the US and spanned 1996 to 2018. The Nordic cohort included all pregnancies resulting in singleton live-born infants, and the US cohort consisted of publicly insured mothers linked to their live-born infants nested in the nationwide Medicaid Analytic eXtract. Data were analyzed from November 2020 to April 2022., Exposures: One or more first-trimester dispensing of any atypical, any typical, and individual antipsychotic drugs., Main Outcomes and Measures: Any major congenital malformation and specific malformation subtypes previously suggested to be associated with antipsychotic exposure in utero: cardiovascular malformations, oral clefts, neural tube defects, hip dysplasia, limb reduction defects, anorectal atresia/stenosis, gastroschisis, hydrocephalus, other specific brain anomalies, and esophageal disorders. Propensity score stratification was used to control for potential confounders. Pooled adjusted estimates were calculated using indirect standardization., Results: A total of 6 455 324 unexposed mothers (mean maternal age range across countries: 24-31 years), 21 751 mothers exposed to atypical antipsychotic drugs (mean age range, 26-31 years), and 6371 mothers exposed to typical antipsychotic drugs (mean age range, 27-32 years) were included in the study cohort. Prevalence of any major malformation was 2.7% (95% CI, 2.7%-2.8%) in unexposed infants, 4.3% (95% CI, 4.1%-4.6%) in infants with atypical antipsychotic drug exposure, and 3.1% (95% CI, 2.7%-3.5%) in infants with typical antipsychotic drug exposure in utero. Among the most prevalent exposure-outcome combinations, adjusted relative risks (aRR) were generally close to the null. One exception was olanzapine exposure and oral cleft (aRR, 2.1 [95% CI, 1.1-4.3]); however, estimates varied across sensitivity analyses. Among moderately prevalent combinations, increased risks were observed for gastroschisis and other specific brain anomalies after atypical antipsychotic exposure (aRR, 1.5 [95% CI, 0.8-2.6] and 1.9 [95% CI, 1.1-3.0]) and for cardiac malformations after chlorprothixene exposure (aRR, 1.6 [95% CI, 1.0-2.7]). While the association direction was consistent across sensitivity analyses, confidence intervals were wide, prohibiting firm conclusions., Conclusions and Relevance: In this study, considering the evidence from primary and sensitivity analyses and inevitable statistical noise for very rare exposure-outcome combinations, in utero antipsychotic exposure generally was not meaningfully associated with an increased risk of malformations. The observed increased risks of oral clefts associated with olanzapine, gastroschisis, and other specific brain anomalies with atypical antipsychotics and cardiac malformations with chlorprothixene requires confirmation as evidence continues to accumulate.

Introduction:  The optimal timing of delivery for pregnancies complicated by prenatally diagnosed gastroschisis remains controversial. Therefore, the aim of this study was to find whether elective or expectant delivery is associated with improved neonatal outcome., Materials and Methods:  MEDLINE and Embase databases were searched for studies up to 2021 that reported timing of delivery for prenatally diagnosed gastroschisis. A systematic review and meta-analysis were then performed in group 1: moderately preterm (gestational age [GA]: 34-35 weeks) elective delivery versus expectant management after GA 34-35 weeks; and group 2: near-term (GA: 36-37 weeks) elective delivery versus expectant management after GA 36-37 weeks. The following clinical outcomes were evaluated: length of stay (LOS), total parenteral nutrition (TPN) days, bowel morbidity (atresia, perforation, and volvulus), sepsis, time of first feeding, short gut syndrome and respirator days, and mortality., Results:  Two randomized controlled trials (RCT)s and eight retrospective cohort studies were included, comprising 629 participants. Moderately preterm elective delivery failed to improve clinical outcomes. However, near-term elective delivery significantly reduced bowel morbidity (7.4 vs. 15.4%, relative risk = 0.37; 95% confidence interval [CI]: 0.18, 0.74; p  = 0.005; I2 = 0%) and TPN days (mean difference =-13.44 days; 95% CI: -26.68, -0.20; p  = 0.05; I2 = 45%) compared to expectant delivery. The mean LOS was 39.2 days after near-term delivery and 48.7 days in the expectant group ( p  = 0.06)., Conclusion:  Based on the data analyzed, near-term elective delivery (GA 36-37 weeks) appears to be the optimal timing for delivery of pregnancies complicated by gastroschisis as it is associated with less bowel morbidity and shorter TPN days. However, more RCTs are necessary to better validate these findings., Competing Interests: None declared., (Thieme. All rights reserved.)

BACKGROUND The purpose of this study is to discuss a patient with a history of conditions, including arthrogryposis, gastroschisis, and malignant hyperthermia, who presented with cecal volvulus requiring urgent surgical intervention. CASE REPORT A 29-year-old woman with a history of arthrogryposis, gastroschisis, malignant hyperthermia, and multiple childhood abdominal surgeries presents to the Emergency Department (ED) with 2 days of abdominal pain and bloody diarrhea. A CT abdomen/pelvis revealed findings concerning for a cecal volvulus. The patient was premedicated and monitored closely by the anesthesia team due to her history of malignant hyperthermia. She underwent an exploratory laparotomy, where a dilated cecum and proximal ascending colon were found to be completely volvulized around the mesentery. Manual bowel detorsion was performed, which resulted in reperfusion of the ischemic-appearing bowel, which then appeared viable. She recovered well after the procedure and was discharged on postoperative day 5. CONCLUSIONS This case highlights a patient who presented with a combination of 4 findings: arthrogryposis, gastroschisis, malignant hyperthermia, and cecal volvulus. With arthrogryposis reported to be associated with gastroschisis and malignant hyperthermia, this report not only corroborates this association, but also aims to draw attention to the fact that these conditions have potential to occur jointly with cecal volvulus. Given the patient's history of gastroschisis requiring extensive abdominal surgeries that contribute as risk factors for cecal volvulus, it is possible there may be other arthrogryposis patients who present with cecal volvulus similar to that seen in this patient.

Introduction: Abdominal wall defects (AWDs) interfere with postnatal respiratory parameters. We aimed to evaluate lung volume (LV) in fetuses with AWD using three-dimensional (3D) ultrasound (US) and to correlate AWD with the type (omphalocele and gastroschisis) and size of the defect and neonatal morbidity and mortality., Methods: This prospective observational study included 72 pregnant women with fetuses with AWD and a gestational age &lt;25 weeks. The data on abdominal volume, 3D US LV, and herniated volume were acquired every 4 weeks up to 33 weeks. LV was compared with normal reference curves and correlated with abdominal and herniated volumes., Results: Omphalocele (p &lt; 0.001) and gastroschisis (p &lt; 0.001) fetuses had smaller LV than normal fetuses. LV was positively correlated with abdominal volume (omphalocele, r = 0.86; gastroschisis, r = 0.88), whereas LV was negatively correlated with omphalocele-herniated volume/abdominal volume (p &lt; 0.001, r = -0.51). LV was smaller in omphalocele fetuses that died (p = 0.002), were intubated (p = 0.02), or had secondary closure (p &lt; 0.001). In gastroschisis, a smaller LV was observed in fetuses discharged using oxygen (p = 0.002)., Conclusion: Fetuses with AWD had smaller 3D LV than normal fetuses. Fetal abdominal volume was inversely correlated with LV. In omphalocele fetuses, a smaller LV was associated with neonatal mortality and morbidity., (© 2023 S. Karger AG, Basel.)

Background: About 20%-30% of children with birth defects have multiple major birth defects in more than one organ system, often referred to as multiple congenital anomalies (MCAs). Evaluating the patterns of MCAs can provide clues to the underlying causes, pathogenic mechanisms, and developmental pathways. We sought to explore selected patterns of MCAs within the National Birth Defects Prevention Study (NBDPS), a population-based, case-control study that excluded cases attributed to known chromosomal or single-gene abnormalities., Methods: We defined MCAs as having two or more NBDPS-eligible birth defects and calculated the adjusted observed-to-expected ratio for all observed MCA patterns using co-occurring defect analysis., Results: Of the 50,186 case infants eligible for NBDPS, 2,734 (3.7%) had at least two eligible birth defects. We observed 209 distinct 2-way combinations of birth defects, 297 distinct 3-way combinations, 179 distinct 4-way combinations, and 69 distinct 5-way combinations. Sacral agenesis had the largest proportion of cases with MCAs (70%), whereas gastroschisis had the lowest (3%). Among the cases with MCAs, 63% had a heart defect, 23% had an oral cleft, and 21% had anorectal atresia/stenosis. Of the patterns with adjusted observed-to-expected ratios in the top 20%, most were consistent with the known associations or syndromes, including VATER/VACTERL association and CHARGE syndrome., Conclusions: Most but not all patterns that had the highest adjusted observed-to-expected ratios were instances of known syndromes or associations. These findings highlight the importance of considering birth defect combinations that suggest syndromic patterns in the absence of a formal syndromic diagnosis. New approaches for screening for sequences and associations, and VATER/VACTERL in particular, in surveillance systems with limited resources for manual review may be valuable for improving surveillance system quality. The observed MCA patterns within NBDPS may help focus future genetic studies by generating case groups of higher yield., (© 2022 Wiley Periodicals LLC.)

Objective: Prenatal ultrasound (US) has been shown to overestimate the incidence of suspected fetal growth restriction (FGR) in gastroschisis cases. This is largely because of altered sonographic abdominal circumference (AC) measurements when comparing gastroschisis cases with population nomograms. Individualized Growth Assessment (IGA) evaluates fetal growth using serial US measurements that allow consideration of the growth potential for a given case. Our goal was to assess the utility of IGA for distinguishing normal and pathological fetal growth in gastroschisis cases., Study Design: Pregnancies with prenatally diagnosed fetal gastroschisis were managed and delivered at a single academic medical center. US fetal biometry including head circumference (HC), abdominal circumference (AC), and femur diaphysis length (FDL), and neonatal measurements including birthweight and HC were collected and analyzed for 32 consecutive fetal gastroschisis cases with at least two 2nd and two 3rd trimester measurements. Second trimester growth velocities were compared to a group of 118 non-anomalous fetuses with normal neonatal growth outcomes. Gastroschisis cases were classified into groups based on fetal growth pathology score (FGPS9) patterns. Agreement between IGA (FGPS9) and serial conventional estimated fetal weight (EFW) measurements for determining growth pathology was evaluated. Neonatal size outcomes were compared between conventional birthweight classifications for determining small for gestational age (SGA) and IGA Growth Potential Realization Index (GPRI) for weight and head circumference measurements., Results: Fetal growth pathology score (FGPS9) measurements identified three in-utero growth patterns: no growth pathology, growth restriction and recovery, and progressive growth restriction. In the no growth pathology group ( n  = 19), there was 84% agreement between IGA and conventional methods in determining pathological growth in both the 3rd trimester and at birth. In the growth restriction and recovery group ( n  = 7), there was 71% agreement both in the 3rd trimester and at birth between IGA and conventional methods. In the progressive growth restriction group ( n  = 5), there was 100% agreement in the 3rd trimester and 60% agreement at birth between IGA and conventional methods., Conclusion: We present the first study using IGA to evaluate normal and pathological fetal growth in prenatally diagnosed gastroschisis cases. IGA was able to delineate two 3rd trimester growth pathology patterns - one with persistent growth restriction and another with in-utero growth recovery. Further validation of these initial findings with larger cohorts is warranted.

Background: Management of gastroschisis involves either primary or staged closure. Bowel ischemia and abdominal compartment syndrome (ACS) are possible complications that can be related to a method of treatment. NIRS monitoring has never been applied in this group of patients and may allow for earlier detection of complications., Objective: To assess near-infrared spectroscopy (NIRS) monitoring in neonates with gastroschisis for detecting changes in tissue oxygenation (rSO 2 ) related to bowel reductions or height of bowel in the silo and for detecting tissue ischemia., Methods: Patients with gastroschisis and controls underwent continuous multi-channel assessment of oxygenation of the brain (CrSO 2 ), kidney (RrSO 2 ) and bowel (GrSO 2 ) in a prospective pilot study., Results: Fifteen neonates were treated with primary closure ( n  = 3) or staged closure ( n  = 12); two had confirmed bowel ischemia, none developed ACS.There was no significant correlation between height of the bowel and GrSO 2 at apex ( p  = .72) or base ( p  = .54) within the silo. During staged reductions there was a clinically non-significant change in RrSO 2 (Δ-2.5%, p  = .04), but no significant changes in CrSO 2 ( p  = .11), and GrSO 2 of apex ( p  = .97) and base ( p  = .31). Patients with confirmed ischemia had GrSO 2 that were lower than controls., Conclusions: Measuring GrSO 2 through a silo is feasible. Staged reduction seems safe based on NIRS measurements, with minimal effect of hydrostatic pressure on bowel oxygenation. NIRS was able to detect subtle changes in intra-abdominal renal perfusion during reduction and could differentiate healthy and ischemic bowel.

Abdominal wall defects are one of the most frequently encountered human congenital anomalies. They are seen in as many as 1 in 2,000 live births with evidence to suggest that their incidence is increasing. While often discussed together abdominal wall defects consist mainly of two entities namely gastroschisis and omphalocele. There are marked differences in their theories of embryo-pathogenesis, clinical presentation/anatomy and overall outcomes. There is no clear consensus explaining the precise embryological mechanisms leading to the development of abdominal wall defects. Many clinicians and embryologists have attempted to explain the genesis of congenital abdominal wall defects because of failure of progression of various phases of normal embryonic development. This review summarizes the mechanisms involved in normal and abnormal development of the ventral abdominal wall leading to the development of gastroschisis and omphalocele., (Copyright © 2022. Published by Elsevier Inc.)

Introduction: We evaluate the incidence, outcomes, and management of peri‑umbilical hernias after sutured or sutureless gastroschisis closure., Methods: A retrospective, longitudinal follow-up of neonates with gastroschisis who underwent closure at 11 children's hospitals from 2013 to 2016 was performed. Patient encounters were reviewed through 2019 to identify the presence of a peri‑umbilical hernia, time to spontaneous closure or repair, and associated complications., Results: Of 397 patients, 375 had follow-up data. Sutured closure was performed in 305 (81.3%). A total of 310 (82.7%) infants had uncomplicated gastroschisis. Peri-umbilical hernia incidence after gastroschisis closure was 22.7% overall within a median follow-up of 2.5 years [IQR 1.3,3.9], and higher in those with uncomplicated gastroschisis who underwent primary vs. silo assisted closure (53.0% vs. 17.2%, p< 0.001). At follow-up, 50.0% of sutureless closures had a persistent hernia, while 16.4% of sutured closures had a postoperative hernia of the fascial defect (50.0% vs. 16.4%, p< 0.001). Spontaneous closure was observed in 38.8% of patients within a median of 17 months [9,26] and most frequently observed in those who underwent a sutureless primary closure (52.2%). Twenty-seven patients (31.8%) underwent operative repair within a median of 13 months [7,23.5]. Rate and interval of spontaneous closure or repair were similar between the sutured and sutureless closure groups, with no difference between those who underwent primary vs. silo assisted closure., Conclusion: Peri-umbilical hernias after sutured or sutureless gastroschisis closure may be safely observed similar to congenital umbilical hernias as spontaneous closure occurs, with minimal complications and no additional risk with either closure approach., Levels of Evidence: Level II., Competing Interests: Declarations of Competing Interest None., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Omphalocele and gastroschisis are the two most common congenital abdominal wall defects; however, no previous study has focused on gastrointestinal and hepatobiliary tract malformations in these two conditions. This study aimed to investigate the demographic characteristics, coexisting congenital gastrointestinal and hepatobiliary tract anomalies, hospital course, and outcomes of patients with gastroschisis and omphalocele., Methods: This is retrospective chart review of all patients admitted to one tertiary medical center in Taiwan between January 1, 2000 and June 30, 2020 with a diagnosis of gastroschisis or omphalocele. The medical records were reviewed to obtain demographic data regarding coexisting gastrointestinal and hepatobiliary tract anomalies and outcomes., Results: Of the 51 patients included, 21 had gastroschisis and 30 had omphalocele. Gastroschisis was associated with a significantly younger maternal age and a higher incidence of small for gestational age. Of the 30 patients with omphalocele, twelve had associated gastrointestinal and hepatobiliary anomalies. Seven of the 21 patients with gastroschisis had gastrointestinal anomalies, and none had hepatobiliary anomalies. Among the omphalocele patients, three (10%) had documented malrotation, and one developed midgut volvulus. Among gastroschisis patients, four patients (19%) had malrotation, and two developed midgut volvulus. There were no statistically significant differences in postoperative complications or mortality rates between those with and without gastrointestinal/hepatobiliary tract anomalies., Conclusion: The diversity of coexisting gastrointestinal and hepatobiliary tract anomalies is higher in the omphalocele than in gastroschisis. In addition, we demonstrate that patients with gastroschisis or omphalocele have a higher rate of intestinal malrotation and midgut volvulus., (Copyright © 2022 Taiwan Pediatric Association. Published by Elsevier B.V. All rights reserved.)

Purpose: We compared cases of anemia in gastroschisis versus omphalocele and investigated this clinical question., Methods: A multicenter study of five pediatric surgery departments in southern Japan was planned. Sixty patients were collected between 2011 and 2020, with 33 (gastroschisis: n = 19, omphalocele: n = 14) who met the selection criteria ultimately being enrolled. Anemia was evaluated before discharge and at the first outpatient visit., Results: Despite gastroschisis cases showed more frequent iron administration during hospitalization than omphalocele (p = 0.015), gastroschisis cases tended to show lower hemoglobin values at the first outpatient visit than omphalocele cases (gastroschisis: 9.9 g/dL, omphalocele: 11.2 g/dL). Gastroschisis and the gestational age at birth were significant independent predictors of anemia at the first outpatient visit, (gastroschisis: adjusted odds ratio [OR] 19.00, p = 0.036; gestational age at birth: adjusted OR 0.341, p = 0.028). A subgroup analysis for gastroschisis showed that the ratio of anemia in the 35-36 weeks group (8/10, 80.0%) and the > 37 weeks group (6/6, 100%) was more than in the < 34 weeks group (0/3, 0.0%)., Conclusions: Gastroschisis may carry an increased risk of developing anemia compared with omphalocele due to the difference of direct intestinal exposure of amnion fluid in utero., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: An accurate estimated fetal weight (EFW) calculated with traditional formulae in cases of abdominal wall defects (AWDs) can be challenging. As a result of reduced abdominal circumference, fetal weight may be underestimated, which could affect prenatal management. Siemer et al. proposed a formula without the use of abdominal circumference, but it is not used in our protocols yet., Objectives: Our aim was to evaluate the correlation of EFW and birth weight in fetuses with AWD by using Hadlock 1, Hadlock 2, and Siemer et al.'s formulae. Our secondary goal was to evaluate how often fetuses classified as small for gestational age (SGA) were in fact SGA at birth., Study Design: This was a retrospective cohort study of gestations complicated by gastroschisis and omphalocele at two tertiary-care centers in Brazil and Italy during an 8-year period. Of a total of 114 cases, 85 (44 cases of gastroschisis and 41 cases of omphalocele) met our criteria., Results: The last prenatal scan was performed 5.2 (±4.1) days before birth. The mean gestational age at birth was 37.2 (±1.8) weeks. Correlation of EFW with birth weight was calculated with the three formulae with and without adjustment for weight gain between scan and birth, with the use of the Spearman coefficient. The correlation between EFW and weight at birth was positive according to all three formulae for the infants with gastroschisis. This finding was not confirmed in the infants with omphalocele. All formulae overestimated the number of SGA cases: although only 17.6% of fetuses were actually SGA at birth, the Hadlock formulae had classified nearly 35% of them as SGA, and Siemer et al.'s formula, 15.3%., Conclusion: All three formulae yielded a good correlation between EFW in the last scan and birth weight in the infants with gastroschisis but not for those with omphalocele. Cases of SGA were overestimated.

Objective: We sought to perform a meta-analysis of the predictive value of antenatal ultrasonographic markers of bowel dilation, gastric dilation, polyhydramnios and abdominal circumference that predict complex gastroschisis and adverse perinatal outcomes DATA SOURCES: PubMed, Web of Science, Scopus and Embase were searched for relevant articles up to December 2020. Studies reporting prenatal ultrasonographic markers including intra-abdominal bowel dilation (IABD), extra-abdominal bowel dilation (EABD), bowel wall thickness, polyhydramnios, abdominal circumference <5th percentile, gastric dilation (GD) and bowel dilation not otherwise specified (BD-NOS) were included. The primary outcome was prediction of complex gastroschisis; secondary outcomes were length of hospital stay for newborn, time to full enteral feeding, postnatal mortality rate, incidence of necrotising enterocolitis and short bowel syndrome., Results: Thirty-six studies were included in this meta-analysis. We found significant associations between complex gastroschisis and IABD (OR=5.42; 95% CI 3.24 to 9.06), EABD (OR=2.27; 95% CI 1.40 to 3.66), BD-NOS (OR=6.27; 95% CI 1.97 to 19.97), GD (OR=1.88; 95% CI 1.22 to 2.92) and polyhydramnios (OR=6.93; 95% CI 3.39 to 14.18). Second trimester IABD and EABD have greater specificity for the prediction of complex gastroschisis than third trimester values with specificity of 95.6% (95% CI 58.1 to 99.7) and 94.6% (95% CI 86.7 to 97.9) for the second trimester IABD and EABD, respectively., Conclusion: Prenatal ultrasonographic markers, especially the second trimester IABD and EABD, can identify fetuses that develop complex gastroschisis. Furthermore, these specific ultrasonographic markers can identify those babies at the highest risk for severe complications of this congenital anomaly and hence selected for future antenatal interventions., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: Cryptorchidism associated with gastroschisis has been poorly investigated. We aimed to assess the prevalence of this association, and to address the management and outcome of cryptorchidism in this context., Materials and Methods: In this systematic review, we searched electronic databases (PubMed, Web of Science, and Google Scholar), without language restrictions from inception to March 31, 2021, for studies on cryptorchidism and gastroschisis co-occurrence. Random effects meta-analysis was used to calculate pooled prevalence estimates of cryptorchidism in gastroschisis and spontaneous testicular descent in this population., Results: Twenty-five studies were included for the systematic review. All were retrospective case reports or series for a total of 175 patients. Pooled prevalence estimate for cryptorchidism in gastroschisis was 19% (95% CI 13-26). Complete data sets for management and outcome analysis were available in 94 patients, involving 120 undescended testes [63 (52.5%) nonpalpable, 34 (28.3%) prolapsed outside the abdominal wall defect, and 23 (19.2%) palpable]. Fifty-five descended spontaneously, with a pooled prevalence estimate of 50% (95% CI 31-69). Twelve testes/remnants were primarily removed or absent, while 49 testes underwent orchiopexy (success rate, 63%). Excluding 4 testes still awaiting orchiopexy, 30 of the remaining 116 (25.8%) testes had a dismal outcome, including testicular loss (19), hypoplasia (8) or recurrent cryptorchidism (3)., Conclusion: Cryptorchidism in gastroschisis appears to occur more frequently than in the normal population. The overall mediocre testicular outcome seems to reflect the most unfavorable presentation of cryptorchidism in gastroschisis, which is represented by originally intrabdominal or prolapsed testis in the majority of cases., Level of Evidence: IV., Competing Interests: Declaration of Competing Interest None., (Copyright © 2021. Published by Elsevier Inc.)

Objectives: Hereditary hemolytic anemia (HHA) results from genetic mutations that cause red blood cell abnormalities. Little research exists on the relationship between HHA and birth defects. Using data from the National Birth Defects Prevention Study (NBDPS), we described characteristics of HHA-exposed women and estimated associations between HHA during pregnancy and specific birth defects., Methods: The NBDPS was a population-based, case-control study of major birth defects and included pregnancies with estimated delivery dates from October 1997 through December 2011. Participants were ascertained from hospital discharge lists or birth defect registries at 10 sites. Trained interviewers collected information about pregnancy exposures via telephone questionnaire. We described characteristics among HHA-exposed women and calculated crude odds ratios and exact 95% confidence intervals for defects with ≥3 exposed cases., Results: Among 31 HHA-exposed women (28 cases/3 controls), 13 (42%) reported sickle cell anemia, 17 (55%) reported thalassemia, and one (3%) reported hereditary spherocytosis. The average age at delivery for HHA-exposed case women was 27.3 years (range: 17-38). The majority (82%) of HHA-exposed case women reported additional conditions during pregnancy, including hypertension, genitourinary infections, and respiratory illnesses. Additionally, 93% of case women reported using medication during pregnancy. Among the 28 cases, 18 (64%) had isolated birth defects. The defects with ≥3 exposed cases were anencephaly, atrial septal defect, gastroschisis, and cleft palate. Except for anencephaly, the 95% confidence intervals for all estimates were close to or included the null., Conclusion: This hypothesis-generating study adds to the sparse literature on the association between HHA and birth defects., (© 2022 Wiley Periodicals LLC.)

Introduction: Neonatal surgical diseases are prime examples of the global disparity in surgical access and outcomes, with survival for conditions like gastroschisis reaching above 95% in high-income settings but usually fatal in low-income settings. This study aims to examine outcomes and predictors of mortality in patients with two specific neonatal surgical conditions that often require early transfer and prolonged inpatient care (gastroschisis and intestinal atresia) at Rwanda's main pediatric referral hospital., Methods: A single-institution retrospective chart review of neonates with gastroschisis and intestinal atresia was conducted between January 2016 and June 2019. Abstracted data included demographics, referral history, admission interventions, operative details, in-hospital complications, nutrition patterns, length of stay, and mortality. Daily logs were created to evaluate feeding status, infection status, and antibiotic usage. Descriptive and univariate analysis was conducted, with the primary outcome being survival to hospital discharge., Results: A total of 112 patients met inclusion criteria (82% gastroschisis [n = 92] and 18% intestinal atresia [n = 20]). Median age at arrival was 0 d (GS) [IQR 0-1 d] and 8.5 d (IA) [IQR 4-10 d] (P < 0.0001). Survival to discharge was 22.8% (GS) (n = 21) and 60% (IA) (n = 12) with a mean length of stay of 28.3 d (GS) and 18.4 d (IA). The median number of days to initiation of oral feeds was 8.5 d [IQR 7-11] for gastroschisis survivors., Conclusions: Neonatal surgical conditions that require early transfer and prolonged nutritional intervention are challenging in low-resource settings, but through treatment by a comprehensive pediatric surgical service, improving survival is possible., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: To identify prenatal and neonatal predictors of short bowel syndrome-related intestinal failure (SBS-IF) in gastroschisis., Study Design: This retrospective study included all patients with gastroschisis born between 2000 and 2017 who were enrolled in our home parenteral nutrition program, and all patients with gastroschisis born in our institution who survived 2 weeks, during the same time period. Prenatal ultrasound features, neonatal status, anatomic features, oral feeding, and parenteral nutrition dependency were analyzed., Results: Among 180 patients, 35 required long-term parenteral nutrition (SBS-IF group) and 145 acquired full oral feeding within 6 months (oral feeding group). The mean follow-up was 7.9 years (IQR, 1.6-17.5 years) and 5.0 years (IQR, 0.1-18.2 years), respectively. Both bowel matting (OR, 14.23; 1.07-16.7; P = .039) and secondarily diagnosed atresia or stenosis (OR, 17.78; 3.13-100.98; P = .001) were independent postnatal predictors of SBS-IF. Eighteen children (51% of the SBS-IF group) were still dependent on artificial nutrition at the last follow-up. patients with SBS-IF who achieved full oral feeding had a median residual small-bowel length of 74 cm (IQR, 51-160 cm) vs 44 cm (IQR, 10-105 cm) for those still dependent on artificial nutrition (P = .02). An initial residual small bowel length of more than 50 cm was the best predictive cut-off for nutritional autonomy, with a sensitivity of 67% and a specificity of 100%., Conclusions: Bowel matting, complex gastroschisis, and secondary intestinal obstruction were associated with SBS-IF in gastroschisis. For patients with SBS-IF, a small bowel length of more than 50 cm was predictive of secondary nutritional autonomy., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objectives: To analyze late-onset sepsis and to describe the etiological agents in newborns with gastroschisis., Methods: A retrospective cohort, including newborns with gastroschisis whose admissions occurred in the period between January 2012 to December 2018 in a tertiary referral center. Maternal and newborn characteristics, surgical procedures and evolution in hospitalization were verified. A bivariate analysis was performed with patients with proven late-onset neonatal sepsis and according to the simple or complex gastroschisis category, the prevalent microorganisms in positive cultures were identified, statistical tests were carried out and the significance level adopted was p < 0,05. Results are presented in proportions, averages and standard deviation or medians. The level of significance adopted was p < 0.05., Results: 101 newborns were analyzed, 45 (44.5%) were confirmed late-onset sepsis. The median birth weight was 2285+498 grams, and the gestational age was 35.9 +1.74weeks. The incidence of complex gastroschisis was 17.8%, the hospitalization time was 48.2+29.67 days and mortality was 9.9%. The newborns were divided into 2 groups: Group 1: late-onset sepsis (44.6%), and Group 2: no late-onset sepsis. The presence of complex gastroschisis was a factor associated with infection (p < 0.009). Fasting time (p < 0.001), parenteral nutrition time (p < 0.001), time to achieve full diet (p < 0.001), and hospitalization stay (p < 0.001) were higher in group 2. Gram-positive were the most frequent (51.1%), followed by Gram-negative (20%), and fungi (4.4%)., Conclusions: Newborns with gastroschisis have a higher risk of evolving with late-onset sepsis, despite this study did not calculate the risk of sepsis statistically, and the main germs detected by cultures were gram-positive bacteria, specifically Staphylococcus epidermidis., Competing Interests: Conflicts of interest The authors declare no conflicts of interest., (Copyright © 2021 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

Objective: Compare in-hospital outcomes in gastroschisis with intestinal atresia versus simple gastroschisis (GS) using a national database., Study Design: The Children's Hospitals Neonatal Database identified infants with gastroschisis from 2010 to 2016., Results: 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., Conclusion: A large multicenter cohort showed gastroschisis with atresia results in worse outcomes and complications, including necrotizing enterocolitis, feeding delays, and enteral feeding tube dependence., (© 2021. The Author(s), under exclusive licence to Springer Nature America, Inc.)

Introduction: Prior data suggest that infants with gastroschisis are at high risk for hypothermia and infectious complications (ICs). This study evaluated the associations between perioperative hypothermia (PH) and ICs in gastroschisis using a multi-institutional cohort., Methods: Retrospective review of infants with gastroschisis who underwent abdominal closure from 2013-2017 was performed at 7 children's hospitals. Any-IC and surgical site infection (SSI) were stratified against the presence or absence of PH, and perioperative characteristics associated with PH and SSI were determined using multivariable logistic regression., Results: Of 256 gastroschisis neonates, 42% developed PH, with 18% classified as mild hypothermia (35.5-35.9 °C), 10.5% as moderate (35.0-35.4 °C), and 13% severe (<35 °C). There were 82 (32%) ICs with 50 (19.5%) being SSIs. No associations between PH and any-IC (p = 0.7) or SSI (p = 0.98) were found. Pulmonary comorbidities (odds ratio (OR)=3.76, 95%CI:1.42-10, p = 0.008) and primary closure (OR=0.21, 95%CI:0.12-0.39, p<0.001) were associated with PH, while silo placement (OR=2.62, 95%CI:1.1-6.3, p = 0.03) and prosthetic patch (OR=3.42, 95%CI:1.4-8.3, p = 0.007) were associated with SSI on multivariable logistic regression., Conclusions: Primary abdominal closure and pulmonary comorbidities are associated with PH in gastroschisis, however PH was not associated with increased risk of ICs. Independent risk factors for SSI include silo placement and prosthetic patch closure., Competing Interests: Declaration of Competing Interest None., (Copyright © 2021. Published by Elsevier Inc.)

The pathogenesis of omphalocele and gastroschisis is not obvious. Their etiology is disputed. The prevalence and the types of anomalies co-occurring with omphalocele and gastroschisis are variable in the different series published. The aim of this study was to estimate the frequency and the types of co-occurring anomalies in cases with gastroschisis and omphalocele. This study was performed in a well-described population of 387,067 consecutive births between 1979 and 2007. Hundred-one cases with omphalocele were registered (2.61 per 10,000), 75 (74.3%) had co-occurring anomalies comprising chromosomal anomalies (28 cases, 27.7%, including 18 trisomy 18), non-chromosomal syndromes (16 cases, 15.8%, including 3 cases with Beckwith-Wiedemann syndrome, 2 cases with the OEIS sequence, and one case with the Pentalogy of Cantrell complex), and 31 cases, 30.7% with MCA (multiple congenital anomalies). The most common MCA were musculoskeletal (23.5%), urogenital (20.4%), cardiovascular (15.1%), and central nervous (9.1%). Seventy-one cases of gastroschisis were ascertained (1.83 per 10,000). However, the prevalence increased during the study period. The frequency was highest in the mothers 15-19 years old. Sixteen out of the 71 cases with gastroschisis, (22.5%) had co-occurring anomalies including 11 cases of MCA and 5 cases with syndromes. To conclude, the frequency and the types of anomalies co-occurring with omphalocele and gastroschisis are peculiar. Therefore, cases with gastroschisis and omphalocele need to be screened for co-occurring anomalies., (© 2021 Wiley Periodicals LLC.)

Gastroschisis is an uncommon congenital defect of the abdominal wall resulting in intestinal prolapse, most commonly associated with short gut syndrome or bowel obstruction. Wandering spleen, movement of the spleen due to the underdevelopment of splenic ligaments, has a prevalence of 0.25% and is asymptomatic in 15% of paediatric cases. An 11-year-old patient, admitted with a history of gastroschisis repaired at birth, presents with 18 months of intermittent, worsening abdominal pain. Imaging demonstrated splenomegaly and tortuosity of the splenic vein with abnormal positioning of the superior mesenteric artery and vein. The patient was found to have a wandering spleen with subacute splenic infarct secondary to splenic torsion, necessitating emergent surgical intervention. This patient experienced an extremely rare complication of gastroschisis that has not previously been reported. This complication is caused by a lack of appropriate abdominal fixation points for the spleen., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

The World Health Summit 2011 confirmed the epidemic-like occurrence of diabetes mellitus and obesity. In Germany, 62.7 % and 21.9 % of the population have a BMI of more than 25 kg/m² and more than 30 kg/m 2 , respectively. Currently, 10.5 obese people per 100 000 German inhabitants undergo bariatric surgery, while 86 and 114.8 per 100 000 in France and in Sweden, respectively, favor bariatric surgical interventions., Aim: By means of a scientific case report, the instructive case of a young patient with morbid obesity is illustrated based on 1) selective references from the medical literature and 2) insights from the daily clinical practice in the case-specific medical and perioperative management after successful surgery for malformation in his childhood and, thus, the limited therapeutic options of metabolic surgery., Case Report (case-, Diagnostic-, and Treatment-Specific Aspects): 35-year-old patient with morbid obesity. Medical history : Status after surgical intervention for gastroschisis as a newborn (surgery report not available). Clinical findings : Super obesity characterized by 234 kg and 174 cm (→ BMI: 77.3 kg/m²), hypogonadotrophic hypogonadism. Approach & course : · Initial treatment with gastric balloon followed by a weight reduction of 46 kg within the first 6 months; however, despite weight reduction, development of an insulin-dependent diabetes with insulin resistance from a diet-based diabetes;. · Repeat gastric balloon therapy for "bridging" but with no further weight reduction despite additional administration of GLP-1 analogues.. · Surgical intervention: Removal of the balloon - termination because of excessive adhesions to the liver and spleen as well as filiforme hepatic lesions (histopathology: liver hamartoma). Open surgery: extensive adhesiolysis because of previous pediatric surgery for gastroschisis, including associated non-rotation of the intestine with complete right-sided position of the intestine (left side: colon; right flexure: at infralienal position) prompting single-anastomosis duodeno-ileostomy (SADI)-procedure, leaving the stomach in situ with simultaneous cholecystectomy and herniotomy in sublay technique.. Outcome (early postoperative and mid- to long-term): The patient tolerated the intervention well. Postoperative course was uneventful with regard to mobilization, beginning of oral nutrition, and wound healing; there was a subsequent weight reduction due to a "common channel" of 250 cm., Conclusion: While the increase of obesity prevalence in adults has currently stopped, incidence in children and teenagers is rapidly rising. The consequence might be that children and young adults who have undergone bariatric surgery in childhood and adolescence can develop complications from these former interventions as adults. Therefore, it is reasonable to recommend follow-up investigations within specialized centers according to well-established standards. On the other hand, the increasing prevalence of obesity in childhood leads to the possibility that adults who underwent pediatric surgery because of embryonal malformations may require an appointment with a bariatric surgeon at some point. For these patients (as a representative example of the transition of care phenomenon), the risk of metabolic surgical intervention is increased; such operations require the appropriate knowledge and expertise of the bariatric surgeon on embryonal malformations and their approach by pediatric surgery., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

Background: Gastroschisis occurs in one of 2000 births with survival rates partially contingent on intestinal complications and time to establishing feeding. Enhancements in prenatal imaging have given better insight into postnatal outcomes. The goal of this study was to examine the gastroschisis patient population at a single children's hospital in the modern era and to use prenatal ultrasound (US) to develop new prenatal prognostic indicators., Methods: We performed a retrospective review of gastroschisis patients at a quaternary-care referral children's hospital from 2010 through 2018. We recorded demographics, prenatal data and imaging, early postnatal data, operative data, and patient outcomes. We compared patients within our cohort born with complex gastroschisis (bowel atresia/perforation) to uncomplicated gastroschisis patients. Second trimester and third trimester prenatal US were evaluated for changes in amniotic fluid level, amount of external bowel, bowel dilatation, and bowel wall edema to identify prognostic indicators of the status of the bowel at birth. For categorical variables, chi-square tests were used to assess for significance. Univariate and multivariable analyses were used to assess significance between categorical and continuous variables using medians and interquartile ranges or means., Results: A total of 134 patients were included in the study: complex (n = 24), uncomplicated (n = 110). Compared with uncomplicated gastroschisis, complex patients required longer median days to feeding initiation (44 versus 10; P < 0.001), full feeding (80 versus 23; P < 0.001), length of stay (83 versus 33; P < 0.001), and total parenteral nutrition at discharge (P = 0.004). Full US data were available on 81% of patients, and partial data were identified on 19%. Prenatal US analysis showed significantly more complex patients had polyhydramnios on third trimester US (23.5%-4.3%; P = 0.018). US analysis showed these additional factors to be most associated with complex gastroschisis: large amount of external bowel on third trimester US, increase in bowel edema on third trimester US, and increase in external bowel dilation on third trimester US. Multivariable logistic regression analyses revealed amniotic fluid on third trimester US to be the most significant predictor of complex gastroschisis (P = 0.01). Polyhydramnios in combination with two-thirds of the other US factors had both sensitivity and positive predictive value for predicting complex gastroschisis of 75%. Patients with two or less of these positive US factors had high specificity (96.8%) and negative predictive value (87.5%), suggesting uncomplicated disease. There were no differences in perioperative or long-term complications in the complex group when compared with the group with uncomplicated gastroschisis., Conclusions: Polyhydramnios on third trimester prenatal US on babies with gastroschisis can predict complex gastroschisis at birth, whereas the absence of markers on prenatal US can suggest uncomplicated disease. Complex gastroschisis is associated with increased time to feeds and length of stay., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Limited guidance exists regarding appropriate timing for feed initiation and advancement in gastroschisis. We hypothesized that implementation of a gastroschisis management protocol would allow for standardization of antibiotic and nutritional treatment for these patients., Methods: We conducted a retrospective comparison of patients with simple gastroschisis at two pediatric hospitals before and after initiation of our gastroschisis care protocol. Complicated gastroschisis and early mortality were excluded. The control group extended from January 2012 to January 2014 and the protocol group from July 2014 to July 2016. Variables of interest included time to feed initiation, time to goal feeds, length of stay, and National Surgical Quality Improvement Program-defined complications. We performed a subgroup analysis for primary versus delayed gastroschisis closure. Statistical analyses, including F-tests for variance, were conducted in Prism., Results: Forty-seven patients with simple gastroschisis were included (control = 22, protocol = 25). Protocol compliance was 76% with no increase in complication rates. There was no difference in length of stay or time from initiation to full feeds overall between the control and protocol groups. However, neonates who underwent delayed closure reached full feeds significantly earlier, averaging 9 d versus 15 d previously (P = 0.04)., Conclusions: For infants undergoing delayed closure, the time to full feeds in this group now appears to match that of patients undergoing primary closure, indicating that delayed closure should not be a reason for slower advancement. Additional studies are needed to assess the impact of earlier full enteral nutrition on rare complications and rates of necrotizing enterocolitis., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: This study aimed to investigate factors that influence growth in infants with gastroschisis., Study Design: Growth parameters at birth, discharge, 6, 12, and 18 months of age were collected from 42 infants with gastroschisis., Results: The mean z-scores for weight, length, and head circumference were below normal at birth and decreased between birth and discharge. Lower gestational age correlated with a worsening change in weight z-score from birth to discharge (rho 0.38, p  = 0.01), but not with the change in weight z-score from discharge to 18 months (rho 0.04, p  = 0.81). There was no correlation between the day of life when the enteral feeds were started and the change in weight z-score from birth to discharge (rho 0.12, p  = 0.44) or discharge to 18 months (rho -0.15, p  = 0.41)., Conclusion: Our study demonstrates that infants with gastroschisis experience a significant decline in weight z-score between birth and discharge, and start to catch up on all growth parameters after discharge. Prematurity in gastroschisis infants is associated with a greater risk for weight loss during this time. This information emphasizes the importance of minimizing weight loss prior to discharge in premature infants with gastroschisis and highlights the need for optimal management strategies for these infants., Competing Interests: None declared., (Thieme. All rights reserved.)

Gastroschisis and omphalocele are the two most common abdominal wall birth defects, and epidemiologic characteristics and frequency of occurrence as part of a syndromic condition suggest distinct etiologies between the two defects. We assessed complex patterns of defect co-occurrence with these defects separately using the Texas Birth Defects Registry. We used co-occurring defect analysis (CODA) to compute adjusted observed-to-expected (O/E) ratios for all observed birth defect patterns. There were 2,998 non-syndromic (i.e., no documented syndrome diagnosis identified) cases with gastroschisis and 789 (26%) of these had additional co-occurring defects. There were 720 non-syndromic cases with omphalocele, and 404 (56%) had additional co-occurring defects. Among the top 30 adjusted O/E ratios for gastroschisis, most of the co-occurring defects were related to the gastrointestinal system, though cardiovascular and kidney anomalies were also present. Several of the top 30 combinations co-occurring with omphalocele appeared suggestive of OEIS (omphalocele, exstrophy of cloaca, imperforate anus, spinal defects) complex. After the exclusion of additional cases with features suggestive of OEIS in a post-hoc sensitivity analysis, the top combinations involving defects associated with OEIS (e.g., spina bifida) were no longer present. The remaining top combinations involving omphalocele included cardiovascular, gastrointestinal, and urogenital defects. In summary, we identified complex patterns of defects that co-occurred more frequently than expected with gastroschisis and omphalocele using a novel software platform. Better understanding differences in the patterns between gastroschisis and omphalocele could lead to additional etiologic insights., (© 2020 Wiley Periodicals LLC.)

The testis is rarely encountered within the organs prolapsed outside the abdominal wall defect of patients with gastroschisis. Optimal treatment strategy of this unusual type of cryptorchidism remains undefined, with less than 30 cases reported to date. We describe a new case where simple relocation of the testis into the abdomen was followed by spontaneous testicular descent. Additionally, he developed a urinary calculus impacted in the navicular fossa 4 years later. Given the rarity of the 2 conditions, the probability of their co-occurrence is exceptionally rare, especially considering that they seem to be causally and temporally unrelated to one another., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Competing Interests: Competing interests: None declared.

Aim: Mortality in infants born with gastroschisis (GS) in low-to-middle-income countries (LMICs) is high. This study aimed to assess factors which might affect outcome in Egypt in order to improve survival., Methods: A prospective study over a 15-month duration was completed. Variables assessed covered patient, maternal, antenatal, treatment, and complications. The Gastroschisis Prognostic Score (GPS) was used to predict outcome. A validated questionnaire was used to assess socioeconomic status. The main outcome was mortality., Results: Twenty-four cases were studied. Median gestational age was 37 (26-40) weeks, and 9 (38%) were preterm. Mortality occurred in 15 (62%) infants. Median transfer time was 8 (1.5-35) hours, and 64% survived if transferred before 8 h. Median maternal age was 20 (16-27) years. All families were of a low or very-low socioeconomic level. Only 25% had antenatal scans. Most cases were simple GS, and only 3 (12.5%) were complex GS. Median length of stay was 14 (1-52) days, TPN duration was 12 (0-49) days, and days to full feeds was 5 (3-11) days. The GPS score ranged from 0 to 6 in the studied cases and negatively correlated with outcome (r S  = -0.98; p = 0.03)., Conclusion: The mortality of GS in Egypt is very high, mainly due to sepsis and prematurity. Young maternal age and poor socioeconomic status are linked to GS. The GPS is a good indicator of morbidity and mortality in a LMIC setting. Survival improved with better resuscitation and strict management protocols. More effort is needed to improve antenatal detection, and transfer time should be ideally below 8 h., Level of Evidence: Level IV., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background and Aims: Studies on long-term sequelae of gastroschisis are scarce. The limited data suggest increased abdominal complaints in young children. To provide proper counseling for both parents and patients, more information on long-term outcome is needed. This study aims to evaluate long-term outcome regarding GI function, gastroesophageal reflux (GER), health-related quality of life (HRQoL) and cosmetic satisfaction., Methods: An observational longitudinal cohort study was performed. All patients (N = 43) born between 1982 and 2008 with gastroschisis that were admitted to the University Medical Centre Utrecht, Wilhelmina Children's Hospital were invited to fill in a survey. Data of included patients were compared to validated Dutch reference standards., Results: Fourteen patients responded to the survey. The median follow-up was 18 years. Abdominal pain on weekly basis was present in two patients (14%) and feeding difficulties were present in one patient. Presence of a complication during gastroschisis treatment led to more GI symptoms (80% versus 22%). One patient experienced moderate complaints of regurgitation or dyspepsia. Although the overall HRQoL was lower in teenage gastroschisis patients as compared to healthy controls (73/100 versus 83/100, respectively), we found no relevant difference in overall HRQoL in the other age groups. Seven patients (50%) were satisfied with the cosmetic result of the scar., Conclusions: GI function and HRQoL in gastroschisis patients seem similar to healthy controls at adolescent and adult age. Complications during gastroschisis treatment led to an increase of abdominal complaints later in life. The surgical technique had no significant effect on the cosmetic results., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to disclose, (Copyright © 2019 The Authors. Published by Elsevier B.V. All rights reserved.)

Purpose: 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., Methods: 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., Findings: 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)., Conclusions: 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 of Evidence: Level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Gastroschisis is an increasingly common congenital abdominal wall defect. Due to advances in neonatal critical care and early surgical management, mortality from gastroschisis and associated complications has decreased to less than 10% in most series. However, it has been recognized that the outcome of gastroschisis has a spectrum and that the disorder affects a heterogeneous cohort of neonates. The goal of this study is to predict morbidity and mortality in neonates with gastroschisis using clinically relevant variables., Methods: A multicenter, retrospective observational study of neonates born with gastroschisis was conducted. Neonatal characteristics and outcomes were collected and compared. Prediction of morbidity and mortality was performed using multivariate clinical models., Results: Five hundred and sixty-six neonates with gastroschisis were identified. Overall survival was 95%. Median hospital length of stay was 37 d. Sepsis was diagnosed in 107 neonates. Days on parenteral nutrition and mechanical ventilation were considerable with a median of 27 and 5 d, respectively. Complex gastroschisis (atresia, perforation, volvulus), preterm delivery (<37 wk), and very low birth weight (<1500 g) were associated with worse clinical outcomes including increased sepsis, short bowel syndrome, parenteral nutrition days, and length of stay. The composite metric of birth weight, Apgar score at 5 min, and complex gastroschisis was able to successfully predict mortality (area under the curve, 0.81)., Conclusions: Clinical variables can be used in gastroschisis to distinguish those who will survive from nonsurvivors. Although these findings need to be validated in other large multicenter data sets, this prognostic score may aid practitioners in the identification and management of at-risk neonates., (Copyright © 2019 Elsevier Inc. All rights reserved.)