European reference network for rare inherited congenital anomalies (ERNICA) evidence based guideline on the management of gastroschisis.

BACKGROUND: The European Reference Network for rare Inherited Congenital Anomalies, ERNICA, guidelines for gastroschisis cover perinatal period to help teams to improve care. METHOD: A systematic literature search including 136 publications was conducted. Research findings were assessed following the GRADE methodology. The evidence to decision framework was used to determine the strength and direction of recommendations. RESULTS: The mode or timing of delivery do not impact neonatal mortality, risk of NEC or time on parenteral nutrition (PN). Intra or extra abdominal bowel dilatation predict complex gastroschisis and longer length of hospital stay but not increased perinatal mortality. Outcomes after Bianchi procedure and primary fascia closure under anesthesia are similar. Sutureless closure decreases the rate of surgical site infections and duration of ventilation compared to surgical closure. Silo-staged closure with or without intubation results in similar outcomes. Outcomes of complex gastroschisis (CG) undergoing early or delayed surgical repair are similar. Early enteral feeds starting within 14 days is associated with lower risk of surgical site infection. RECOMMENDATIONS: The panel suggests vaginal birth between 37 and 39 w in cases of uncomplicated gastroschisis. Bianchi's approach is an option in simple gastroschisis. Sutureless closure is suggested when general anesthesia can be avoided, sutured closure. If anesthesia is required. Silo treatment without ventilation and general anesthesia can be considered. In CG with atresia primary intestinal repair can be attempted if the condition of patient and intestine allows. Enteral feeds for simple gastroschisis should start within 14 days.

Risk Factors for Infection in Neonates With Intestinal Failure.

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.

Association of Exclusive Breast Milk Intake and Outcomes in Infants With Uncomplicated Gastroschisis: A National Cohort Study.

BACKGROUND: Enteral feeding is an essential part of the management of infants with gastroschisis. We hypothesized that exclusive breast milk is associated with improved neonatal outcomes. METHODS: We conducted a retrospective review of infants with uncomplicated gastroschisis through the Canadian Pediatric Surgery Network (CAPSNet) and Canadian Neonatal Network (CNN). The primary outcome was time to full enteral feeds. RESULTS: We identified 411 infants with gastroschisis treated at CAPSNet centres from 2014 to 2022. 144 patients were excluded due to gestational age <32 weeks, birth weight <1500 g, other congenital anomalies, or complicated gastroschisis. Of the remaining 267 participants, 78% (n = 209) received exclusive breast milk diet in the first 28 days of life, whereas 22% (n = 58) received supplemental or exclusive formula. Infants who received exclusive breast milk experienced higher time to reach full enteral feeding (median 24 vs 22 days, p = 0.047) but were more likely to have undergone delayed abdominal closure (32% vs 17%, p = 0.03). After adjustment, there were no significant differences between groups in time to reach full enteral feeds, duration of parenteral nutrition, or length of stay. Infants who received supplemental or exclusive formula had a similar risk of necrotizing enterocolitis (4% vs 3%) but were less likely to transition to exclusive breast milk at discharge (73% vs 11%, p < 0.001). CONCLUSION: Early use of exclusive breast milk in infants with uncomplicated gastroschisis is associated with similar outcomes compared to supplemental or exclusive formula. Patients who received supplemental or exclusive formula were unlikely to transition to exclusive breastfeeding by discharge. LEVEL OF EVIDENCE: Level IIb (Individual Cohort Study).

Trends in Attempted Vaginal Delivery among Pregnancies Complicated by Gastroschisis, 2014 to 2020.

OBJECTIVE: Gastroschisis is a full-thickness congenital defect of the abdominal wall through which intestines and other organs may herniate. In a prior analysis, attempted vaginal delivery with fetal gastroschisis appeared to increase through 2013, although cesarean delivery remained common. The objective of this analysis was to update current trends in attempted vaginal birth among pregnancies complicated by gastroschisis. STUDY DESIGN: We performed an updated cross-sectional analysis of live births from 2014 and 2020 using data from the U.S. National Vital Statistics System and evaluated trends in attempted vaginal deliveries among births with gastroschisis. Trends were evaluated using joinpoint regression. We constructed logistic regression models to evaluate the association between demographic and clinical variables and attempted vaginal delivery in the setting of gastroschisis. RESULTS: Among 5,355 deliveries with gastroschisis meeting inclusion criteria, attempted vaginal delivery increased significantly from 68.9% to 75.1%, an average annual percent change of 1.7% (95% confidence interval [CI], 0.8-2.5). Among gastroschisis-complicated pregnancies, patients 35 to 39 years old (adjusted odds ratio [aOR], 0.53; 95% CI, 0.37-0.79) and Hispanic race/ethnicity (aOR, 0.69; 95% CI, 0.58-0.62) were at lower likelihood of attempted vaginal delivery in adjusted analyses. CONCLUSION: These findings suggest that vaginal delivery continues to increase in the setting of gastroschisis. Further reduction of surgical delivery for this fetal defect may be possible. KEY POINTS: · Vaginal deliveries increased among gastroschisis pregnancies.. · Hispanic patients were less likely to attempt vaginal delivery.. · Some gastroschisis pregnancies still deliver surgically..

Impact of Bowel Coverage and Resuscitation Protocol on Gastroschisis Mortality in Low-Income Countries: Experience and Lessons From Uganda.

BACKGROUND: Gastroschisis causes near complete mortality in low-income countries (LICs). This study seeks to understand the impact of bedside bowel reduction and silo placement, and protocolized resuscitation on gastroschisis outcomes in LICs. METHODS: We conducted a retrospective cohort study of gastroschisis patients at a tertiary referral center in Kampala, Uganda. Multiple approaches for bedside application of bowel coverage devices and delayed closure were used: sutured urine bags (2017-2018), improvised silos using wound protectors (2020-2021), and spring-loaded silos (2022). Total parental nutrition (TPN) was not available; however, with the use of improvised silos, a protocol was implemented to include protocolized resuscitation and early enteral feeding. Risk ratios (RR) for mortality were calculated in comparison to historic controls from 2014. RESULTS: 368 patients were included: 42 historic controls, 7 primary closures, 81 sutured urine bags, 133 improvised silos and 105 spring-loaded silos. No differences were found in sex (p = 0.31), days to presentation (p = 0.84), and distance traveled to the tertiary hospital (p = 0.16). Following the introduction of bowel coverage methods, the proportion of infants that survived to discharge increased from 2% to 16-29%. In comparison to historic controls, the risk of mortality significantly decreased: sutured urine bags 0.65 (95%CI: 0.52-0.80), improvised silo 0.76 (0.66-0.87), and spring-loaded silo 0.65 (0.56-0.76). CONCLUSION: Bedside application of bowel coverage and protocolization decreases the risk of death for infants with gastroschisis, even in the absence of TPN. Further efforts to expand supply of low-cost silos in LICs would significantly decrease the mortality associated with gastroschisis in this setting. TYPE OF STUDY: Treatment Study. LEVEL OF EVIDENCE: III (Retrospective Comparative Study).

Gastroschisis in Sub-Saharan Africa: a scoping review of the prevalence, management practices, and associated outcomes.

Gastroschisis is a congenital defect of the anterior abdominal wall characterized by a periumbilical abdominal wall defect with associated bowel protrusion. Limitations in the diagnosis and management of gastroschisis in Sub-Saharan African countries contribute to the high mortality rate. Few studies have been published despite its significant contribution to neonatal mortality in Sub-Saharan Africa. This review study explores the prevalence of gastroschisis, likewise the management and clinical outcomes. Full-text articles reporting the prevalence, management, and associated outcomes of gastroschisis in Sub-Saharan Africa were included. Data were extracted from databases such as PubMed, Google Scholar, and Ajol following a systematic search. The study was reported following the PRISMA-ScR guideline. A total of ten articles which included studies conducted from 1999 to 2022 fulfilled the criteria. The prevalence of gastroschisis varied widely, ranging from 0.026 to 1.75 with an overall mortality rate of 62.48%. Young maternal age is strongly associated with the incidence of gastroschisis. The study showed a slight male preponderance with a M: F ratio of 1.12:1. Staged closure with silos is the preferred method of management, it is explicitly linked to improved clinical outcomes. The prevalence rate and associated mortality of gastroschisis remain alarmingly high in most of the studies. There is a need for advanced diagnostic and management practices as well as increased awareness of gastroschisis to reduce mortality and improve survival outcomes.

Evaluation of quality of care in neonatal anesthesia using a bundle of intraoperative parameters.

BACKGROUND: Maintenance of physiological homeostasis is key in the safe conduct of pediatric anesthesia. Achieving this goal is especially difficult in neonatal surgery. AIMS: The first aim was to document the absolute number of seven intraoperative parameters monitored during anesthesia in neonates undergoing gastroschisis surgery. The second aims were to determine the frequency of monitoring of each of these intraoperative parameters as well as the proportion of cases in which each parameter was both monitored and maintained within a pre-defined range. METHODS: This retrospective observational analysis includes data from 53 gastroschisis surgeries performed at Caen University Hospital (2009-2020). Seven intraoperative parameters were analyzed. First, we assessed if the intraoperative parameters were monitored or not. Second, when monitored, we assessed if these parameters were maintained within a pre-defined range, based on the current literature and on local agreement. RESULTS: The median [first-third Q], range (min-max) number of intraoperative parameters monitored during the 53 gastroschisis surgeries was 6 [5-6], range (4-7). There were no missing data for the automatically recorded ones such as arterial blood pressure, heart rate, end-tidal CO2, and oxygen saturation. Temperature was monitored in 38% of the patients, glycemia in 66%, and natremia in 68% of the cases. Oxygen saturation and heart rate were maintained within the pre-defined range in 96% and 81% of the cases respectively. The blood pressure (28%) and temperature (30%) were instead the least often maintained within the pre-defined range. CONCLUSION: Although a median of six out of the seven selected intraoperative parameters were monitored during gastroschisis repair, only two of them (oxygen saturation and heart rate) were maintained within the pre-defined range more than 80% of the time. It might be of interest to extend physiologic age- and procedure-based approach to the development of specific preoperative anesthetic planning.

Association between birth location and short-term outcomes for babies with gastroschisis, congenital diaphragmatic hernia and oesophageal fistula: a systematic review.

BACKGROUND: Neonatal care is commonly regionalised, meaning specialist services are only available at certain units. Consequently, infants with surgical conditions needing specialist care who are born in non-surgical centres require postnatal transfer. Best practice models advocate for colocated maternity and surgical services as the place of birth for infants with antenatally diagnosed congenital conditions to avoid postnatal transfers. We conducted a systematic review to explore the association between location of birth and short-term outcomes of babies with gastroschisis, congenital diaphragmatic hernia (CDH) and oesophageal atresia with or without tracheo-oesophageal fistula (TOF/OA). METHODS: We searched MEDLINE, CINAHL, Web of Science and SCOPUS databases for studies from high income countries comparing outcomes for infants with gastroschisis, CDH or TOF/OA based on their place of delivery. Outcomes of interest included mortality, length of stay, age at first feed, comorbidities and duration of parenteral nutrition. We assessed study quality using the Newcastle-Ottawa Scale. We present a narrative synthesis of our findings. RESULTS: Nineteen cohort studies compared outcomes of babies with one of gastroschisis, CDH or TOF/OA. Heterogeneity across the studies precluded meta-analysis. Eight studies carried out case-mix adjustments. Overall, we found conflicting evidence. There is limited evidence to suggest that birth in a maternity unit with a colocated surgical centre was associated with a reduction in mortality for CDH and decreased length of stay for gastroschisis. CONCLUSIONS: There is little evidence to suggest that delivery in colocated maternity-surgical services may be associated with shortened length of stay and reduced mortality. Our findings are limited by significant heterogeneity, potential for bias and paucity of strong evidence. This supports the need for further research to investigate the impact of birth location on outcomes for babies with congenital surgical conditions and inform future design of neonatal care systems. PROSPERO REGISTRATION NUMBER: CRD42022329090.

Bedside reduction of gastroschisis: A preliminary experience in yaounde-cameroon.

Background: Gastroschisis denotes a congenital or sporadic malformation of the anterior abdominal wall, which is rarely associated with other anomalies. The mortality in African countries is still high almost 100%. Objective: The aim was to determine the feasibility and safety of bedside reduction of gastroschisis and factors affecting the outcome in low-income setting. Methodology: This was a retrospective, descriptive and analytic study over a period of 6 years conducted in the Pediatric Surgery Service of the Yaoundé Gyneco-Obstetric and Pediatric Hospital. Only neonates with gastroschisis seen within 6 h of life without bowel necrosis and in whom bedside reduction was attempted in the neonatology unit under sedation (with 0.5 mg/kg of diazepam intra-rectally and 0.5-1 mg of atropine intravenously) were included in this study. Ethical clearance was obtained for the Ethical Committee of the Yaoundé Gyneco-Obstetric and Pediatric Hospital and a signed consent form was required from the parents of the children prior to the procedure. Results: Twelve neonates with a mean age of 16.8 h (0 and 24 h) and mean birth weight of 2245 g (1860-3600 g) were enrolled. The mean time to presentation at hospital was 3.5 h (2-9 h). Bedside closure was successful in 10 patients. Two patients underwent primary closure in the theatre after failure of bedside reduction due to the volume of contents of gastroschisis. Mortality rate in our study was 33.3% and the morbidity was dominated by compartment syndrome and malnutrition. Conclusion: Bedside reduction of gastroschisis under sedation in Yaoundé seems to be way to reduce the mortality.

Prenatal repair of gastroschisis using partial carbon dioxide insufflation fetoscopy: lessons learned.

We report the long-term outcomes of a case of prenatal gastroschisis repair using a fully percutaneous fetoscopic approach with partial carbon dioxide insufflation. Surgery was performed as an experimental procedure before the scheduled elective birth. The fetal intestines were successfully returned to the abdominal cavity without any fetal or maternal complications. Ultrasonography performed 24 hours later revealed bowel peristalsis and no signs of fetal distress. After 48 hours, partial extrusion of the small bowel was observed, and the fetus was delivered. Gastroschisis repair was immediately performed upon delivery using the EXIT-like procedure as per our institutional protocol. The newborn did not require assisted mechanical ventilation, was discharged at 14 days of age and was then exclusively breastfed. At 3-year follow-up, the patient had no associated gastroschisis-related complications. This is the first case of prenatal repair of gastroschisis, which provides baseline knowledge for future researchers on the potential hurdles and management of prenatal repair.

Infectious Complications and Antibiotic Use in Gastroschisis.

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.

Prevalence of gastroschisis and its neonatal mortality in the Eastern Cape Province tertiary institutions.

Context: Gastroschisis is a common abdominal wall defect faced by paediatric surgeons worldwide. Early gastroschisis detection, access to improved neonatal intensive care, parenteral nutrition and surgical techniques have led to a reported improvement in mortality of between 4% and 8% in high-income countries. In low to middle income countries, such as in Southern Africa, however, there is as much as 84% mortality among patients with gastroschisis. This is thought to be due to factors such as lack of antenatal screening, access to neonatal intensive care services and parenteral nutrition. Aims: The purpose of this study was to calculate the prevalence of gastroschisis and report on its neonatal mortality in the Eastern Cape Province of South Africa. Settings and Design: A retrospective observational study on all neonates with gastroschisis, presenting to a tertiary facility offering paediatric surgical services within the Eastern Cape Province from 1 January 2016 to 31 December 2018. Subjects and Methods: A convenience sampling method was used in retrieving patient files for the study period. Statistical analysis used: Stata version 13. Results: Thirty-seven neonates were included in the study. The prevalence of gastroschisis ranged from 0.07% to 0.18% throughout the 3-year study. The majority (81%) of the neonates were outborn and delivered by mode of caesarean section. Nearly 60% (n = 22) were female. 54% (n = 20) of neonates died within the neonatal period. Conclusions: The majority of the neonates in this study were outborn and female. Although their mortality rate was higher than reported in high-income countries, it was much improved from what is reported in the low to middle income countries.

Predicting mortality in neonates with gastroschisis in a Southeastern state of Brazil.

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.

Assessment of the neonatal referral and transport system for patients with gastroschisis in Kenya.

PURPOSE: To assess the neonatal referral and transport system for gastroschisis patients referred to a tertiary level hospital in Kenya. METHODS: This was a prospective cross-sectional study carried out at Kenyatta National Hospital (KNH) which recruited patients with gastroschisis using consecutive sampling approach. Data were collected on pre-, intra-transit factors, time and distance covered. Assessment was done using pre and intra transit factors as per the standard transport protocols in literature. RESULTS: Twenty-nine patients presented with gastroschisis during the eight month study period. Mean age was 7.07 h. There were 16 (55.2%) males and 13 (44.8%) females. Mean birthweight was 2020 g, and a mean gestational age of 36.5 weeks. Mean duration of transit was 5 h. Mean distance from referring facility was 153.1 km. Most affected factors in the pre-transit protocol were lack of monitoring chart (0%), comment on blood investigations (0%), gastric decompression (3.4%), and prenatal obstetric scan (44.8%). For intra-transit scores, most affected were incubator use (0%), bowel monitoring (0%), functioning nasogastric tube (13.8%), and adequate bowel cover (34.5%). CONCLUSION: This study demonstrates that pre-transit and transit care of neonates with gastroschisis is inadequate in Kenya. Interventions needed, as identified by this study, to promote care of neonates with gastroschisis are advised.

Surgical management of gastroschisis with the use of primary and staged closure based on the department's 20-year experience.

ABSTRACTBACKGROUND: Gastroschisis is a common developmental anomaly of the abdominal front wall. The aim of surgical management is to restore the integrity of the abdominal wall and to insert the bowel into the abdominal cavity with the use of the primary or staged closure technique.The objective of this paper is to analyze our 20-year experience with surgical treatment of gastroschisis with primary and staged closure, to compare the postoperative course for the said techniques as well as to identify factors influencing the course and early results of treatment. METHODS: The research materials consist of a retrospective analysis of medical history of patients treated at the Pediatric Surgery Clinic in Poznan over 20 years period from 2000 to 2019. 59 patients were operated on: 30 girls and 29 boys. RESULTS: Surgical treatment was performed in all the cases. Primary closure was performed in 32% of the cases, whereas staged silo closure was performed in 68% of the cases. Postoperative analgosedation was used for 6 days on average after primary closures, and 13 days on average after staged closures. Generalized bacterial infection was present in 21% of patients treated with primary closures and 37% for staged closures. Infants treated with staged closure began enteral feeding considerably later (day 22) than those treated with primary closure (day 12). CONCLUSIONS: It is not possible to indicate clearly which surgical technique is superior to the other based on the results obtained. When choosing the treatment method, the patient's clinical condition, associated anomalies, and the medical team's experience must be taken into consideration.

Surgical neonates in a low-resource setting: Baseline nutrition and outcome assessment.

INTRODUCTION: Congenital anomalies necessitating prolonged fasting have a high mortality in low-income settings, partially due to malnutrition and electrolyte disturbances in the absence of parenteral nutrition (PN). Interventions to address these problems require an accurate baseline quantification of the morbidity and mortality of this population. This prospective study aimed to determine peri-operative morbidity, fluid and electrolyte disturbance, growth, and mortality in neonates with gastroschisis (GS), intestinal atresia (IA), and esophageal atresia (EA) in Uganda. METHODS: Standardized patient care of 45 neonates treated in Uganda from Oct 2021 to March 2022 with protocolized fluid and nutrition, vital signs, and routine laboratory measurements. Patient demographics, admission and hospital characteristics are described with mean ± SD. Characteristics of survivors and non-survivors were compared with Fischer's exact tests, logrank tests, and CoX Ph model. RESULTS: Twenty-eight (62.2%) patients had GS, 4 (8.9%) EA, and 13 (28.9%) IA. Thirty-six percent (16/44) of patients survived to discharge (26% GS, 50% EA, 54% IA) with an average length of stay of 17.3 days ( ± 2.2) (survivors) and 9 days ( ± 1.7) (non-survivors). Average weight was 2.21 kg ( ± 0.62) at presentation, with no significant weight change during the study. Abnormal serum sodium in 64%, phosphate 36.5%, and magnesium 20.8% of measurements. Mortality did not correlate with diagnosis (p = 0.47), electrolyte derangement, or weight change. CONCLUSION: Mortality of neonates born with GS, EA, and IA is high in Uganda. Malnutrition and fluid/electrolyte derangements are common and may affect mortality. This study provides a comparison group for studying interventions to improve outcomes for these populations. TYPE OF STUDY: Prospective cohort. LEVEL OF EVIDENCE: Level 3.