Expansion of the prenatal phenotype of Baraitser-Winter syndrome: Presentation of two cases of multiple congenital anomaly syndrome.

Baraitser-Winter cerebrofrontofacial syndrome (BWCFF) is a variable multiple congenital anomaly condition, typically presenting postnatally with neurocognitive delays, distinctive facial features, cortical brain malformations, and in some, a variety of additional congenital malformations. However, only a few cases have reported the prenatal presentation of this syndrome. Here, we report two cases of BWCFF and their associated prenatal findings. One case presented with non-immune hydrops fetalis and a horseshoe kidney and was found to have a de novo heterozygous variant in ACTB (c.158A>G). The second case presented with gastroschisis, bilateral cleft lip and palate, and oligohydramnios, and was found to harbor a different de novo variant in ACTB (c.826G>A). Limited reports exist describing prenatally identified anomalies that include fetal growth restriction, increased nuchal fold, bilateral hydronephrosis, rocker bottom foot, talipes, cystic hygroma, omphalocele, and hydrops fetalis. In addition, only three of these cases have included detailed prenatal imaging findings. The two prenatal cases presented here demonstrate an expansion of the prenatal phenotype of BWCFF to include gastroschisis, lymphatic involvement, and oligohydramnios, which should each warrant consideration of this diagnosis in the setting of additional anomalies.

Descriptive epidemiology of gastroschisis in China from 2007 to 2020: a nationwide surveillance-based study.

BACKGROUND: Gastroschisis is a common abdominal wall defect that increases infant mortality risk and health care costs. However, recent epidemiological data on gastroschisis in China is limited. METHODS: Using 2007-2020 data from the Chinese Birth Defects Monitoring Network (CBDMN), we analyzed gastroschisis prevalence rates stratified by birth year, maternal age group, residence area, geographical region, and infant sex. We also examined the temporal variations in prevalence, pregnancy outcomes of affected infants, prenatal diagnoses, and co-occurring anomalies. RESULTS: From 2007 to 2020, a total of 6,813 cases of gastroschisis were identified among 25,909,000 births, comprising 4,675 isolated and 2,138 non-isolated cases. Prevalence rates per 10,000 live and still births were 2.63, 1.80, and 0.83 for the overall, isolated, and non-isolated gastroschisis, respectively, all showing a decreasing trend over the study period. The prevalence of overall gastroschisis varied significantly by maternal age (< 20 years, 9.88/10,000; 20-24 years, 4.17/10,000; 25-29 year, 2.08/10,000; 30-34 years, 1.88/10,000;≥35 years, 2.24/10,000), maternal residence (urban, 2.45/10,000; rural, 2.85/10,000), geographic region (central, 2.54/10,000; east, 2.57/10,000; west, 2.80/10,000), and infant sex (male, 2.13/10,000; female, 1.79/10,000). Non-isolated gastroschisis cases had a higher early neonatal mortality rate than isolated cases (41.91% vs. 28.10%) and frequently co-occurred with musculoskeletal anomalies. CONCLUSIONS: This study highlights a declining trend in gastroschisis prevalence in Chinese population, a contrast to previous studies, and underscores the need for improved perinatal management due to adverse pregnancy outcomes associated with this condition.

Evaluation of gastroschisis feeding protocol: A retrospective cohort study.

AIM: The primary objective of this study is to determine the impact of a standardised feeding protocol for infants with gastroschisis on early enteral feeds, suck feeds, management of gastric residuals and breastfeeding at discharge. Secondary objectives were evaluation of growth, length of stay in neonatal intensive care unit (NICU), the duration of total parental nutrition (TPN), blood-culture confirmed sepsis and serum bilirubin level (SBR). METHODS: This single-centre retrospective quality improvement project included infants admitted to a quaternary care NICU for management of gastroschisis from 2010 to 2021. The Gastroschisis feeding protocol, a standardised pathway for managing the feeding of infants with gastroschisis, was implemented in this NICU in 2016. The outcomes of infants in the pre-feeding protocol and post-feeding protocol groups were compared. RESULTS: Of the 100 infants included in the study, 49 were in the post-feeding protocol group. The baseline characteristics of the study population did not differ statistically in both groups. In post-feeding protocol group, there was significant reduction in the time to the first enteral feed (P value <0.0001) and first suck feed (P value = 0.002). The median length of stay in the post-feeding protocol group was significantly lower by 11 days (P value = 0.001). Duration of TPN was 241 h higher in the pre-feeding protocol group (P value 0.0007). CONCLUSIONS: Implementation of a feeding protocol in infants with gastroschisis led to earlier initiation of enteral feeds and suck feeds. There is a likelihood of reduction in the use of TPN and duration of admission in NICU.

Gastroschisis and septo-optic-pituitary dysplasia: Is there an association?

AIM: There are several case reports describing patients with both optic nerve hypoplasia/septo-optic-pituitary dysplasia (ONH/SOD) and gastroschisis (GS). Our aim was to investigate whether ONH/SOD is associated with GS. METHODS: A retrospective population-based study was undertaken using the Population Research Data Repository at the Manitoba Center for Health Policy in Manitoba, Canada to investigate if any patient with ONH/SOD also had GS. In addition, Winnipeg's Surgical Database of Outcomes and Management (WiSDOM), a hospital-based paediatric surgical database, was searched to ascertain if any of the patients with GS also have ONH/SOD. RESULTS: Cases were 124 patients with ONH/SOD diagnosed during 1990-2019. None had GS. The surgical database had 188 patients from Manitoba with GS during 1991-2019. None had ONH/SOD. CONCLUSION: There does not appear to be an association between ONH/SOD and GS in our cohorts of patients with these two disorders.

The Association of Prenatal Diagnoses with Mortality and Long-Term Morbidity in Children with Specific Isolated Congenital Anomalies: A European Register-Based Cohort Study.

OBJECTIVES: To compare 5-year survival rate and morbidity in children with spina bifida, transposition of great arteries (TGA), congenital diaphragmatic hernia (CDH) or gastroschisis diagnosed prenatally with those diagnosed postnatally. METHODS: Population-based registers' data were linked to hospital and mortality databases. RESULTS: Children whose anomaly was diagnosed prenatally (n = 1088) had a lower mean gestational age than those diagnosed postnatally (n = 1698) ranging from 8 days for CDH to 4 days for TGA. Children with CDH had the highest infant mortality rate with a significant difference (p < 0.001) between those prenatally (359/1,000 births) and postnatally (116/1,000) diagnosed. For all four anomalies, the median length of hospital stay was significantly greater in children with a prenatal diagnosis than those postnatally diagnosed. Children with prenatally diagnosed spina bifida (79% vs 60%; p = 0.002) were more likely to have surgery in the first week of life, with an indication that this also occurred in children with CDH (79% vs 69%; p = 0.06). CONCLUSIONS: Our findings do not show improved outcomes for prenatally diagnosed infants. For conditions where prenatal diagnoses were associated with greater mortality and morbidity, the findings might be attributed to increased detection of more severe anomalies. The increased mortality and morbidity in those diagnosed prenatally may be related to the lower mean gestational age (GA) at birth, leading to insufficient surfactant for respiratory effort. This is especially important for these four groups of children as they have to undergo anaesthesia and surgery shortly after birth. Appropriate prenatal counselling about the time and mode of delivery is needed.

Factors of poor prognosis in newborns with a prenatal diagnosis of gastroschisis in Bogota, Colombia.

OBJECTIVES: To identify factors associated with poor prognoses in newborns with a prenatal diagnosis of gastroschisis in eight hospitals in Bogota, Colombia, from 2011 to 2022. METHODS: A multi-center retrospective case-control study was conducted on newborns with gastroschisis in eight hospitals in Bogota, Colombia. Poor prognosis was defined as the presence of sepsis, intestinal complications, or death. RESULTS: The study included 101 patients. Preterm newborns under 32 weeks had a poor neonatal prognosis (OR 6.78 95 % CI 0.75-319). Oligohydramnios (OR 4.95 95 % CI 1.15-21.32) and staged closure with silo (OR 3.48; 95 % CI 1.10-10.96) were risk factors for neonatal death, and intra-abdominal bowel dilation of 20-25 mm was a factor for the development of intestinal complications (OR 3.22 95 % CI 1.26-8.23). CONCLUSIONS: Intra-abdominal bowel dilation between 20 and 25 mm was associated with intestinal complications, while oligohydramnios was associated with the risk of perinatal death, requiring increased antenatal surveillance of fetal wellbeing. Management with primary reduction when technically feasible is recommended in these infants, considering that the use of silos was associated with higher mortality.

Chest radiographic thoracic areas and respiratory outcomes in infants with anterior abdominal wall defects.

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 mm2/kg] than the gastroschisis infants [813, IQE 695-915 mm2/kg] No gastroschisis infant developed bronchopulmonary dysplasia (BPD). A CRTA of 1759 mm2 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.

Development of a skeletal muscle sheet with direct reprogramming-induced myoblasts on a nanogel-cross-linked porous freeze-dried gel scaffold in a mouse gastroschisis model.

PURPOSE: In this study, we attempted to create skeletal muscle sheets made of directly converted myoblasts (dMBs) with a nanogel scaffold on a biosheet using a mouse gastroschisis model. METHODS: dMBs were prepared by the co-transfection of MYOD1 and MYCL into human fibroblasts. Silicon tubes were implanted under the skin of NOG/SCID mice, and biosheets were formed. The nanogel was a nanoscale hydrogel based on cholesterol-modified pullulan, and a NanoClip-FD gel was prepared by freeze-drying the nanogel. 7 mm in length was created in the abdominal wall of NOG/SCID mice as a mouse gastroschisis model. Matrigel or NanoCliP-FD gel seeded with dMBs was placed on the biosheet and implanted on the model mice. RESULTS: Fourteen days after surgery, dMBs with Matrigel showed a small amount of coarse aggregations of muscle-like cells. In contrast, dMBs with NanoCliP-FD gel showed multinucleated muscle-like cells, which were expressed as desmin and myogenin by fluorescent immunostaining. CONCLUSION: Nanogels have a porous structure and are useful as scaffolds for tissue regeneration by supplying oxygen and nutrients supply to the cells. Combining dMBs and nanogels on the biosheets resulted in the differentiation and engraftment of skeletal muscle, suggesting the possibility of developing skeletal muscle sheets derived from autologous cells and tissues.

Clinical characteristics and outcome of omphalocele and gastroschisis: a 20-year multicenter regional experience.

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.

Improving outcomes for uncomplicated gastroschisis: clinical practice guidelines from the American Pediatric Surgical Association Outcomes and Evidence-based Practice Committee.

BACKGROUND: The authors sought better outcomes for uncomplicated gastroschisis through development of clinical practice guidelines. METHODS: The authors and the American Pediatric Surgical Association Outcomes and Evidenced-based Practice Committee used an iterative process and chose two questions to develop clinical practice guidelines regarding (1) standardized nutrition protocols and (2) postnatal management strategies. An English language search of PubMed, MEDLINE, OVID, SCOPUS, and the Cochrane Library Database identified literature published between January 1, 1970, and December 31, 2019, with snowballing to 2022. The Appraisal of Guideline, Research and Evaluation reporting checklist was followed. RESULTS: Thirty-three studies were included with a Level of Evidence that ranged from 2 to 5 and recommendation Grades B-D. Nine evaluated standardized nutrition protocols and 24 examined postnatal management strategies. The adherence to gastroschisis-specific nutrition protocols promotes intestinal feeding and reduces TPN administration. The implementation of a standardized postnatal clinical management protocol is often significantly associated with shorter hospital stays, less mechanical ventilation use, and fewer infections. CONCLUSIONS: There is a lack of comparative studies to guide practice changes that improve uncomplicated gastroschisis outcomes. The implementation of gastroschisis-specific feeding and clinical care protocols is recommended. Feeding protocols often significantly reduce TPN administration, although the length of hospital stay may not consistently decrease.

Improving care and survival of newborns with surgical conditions in Tanzania (TINY Tanzania): a focus on gastroschisis.

PURPOSE: Gastroschisis is associated with over 90% mortality in many sub-Saharan African countries. The introduction of the Gastroschisis Care Bundle at Muhimbili National Hospital (MNH) increased survival up to 60%. We aim to explain the impact of using implementation science methods to decentralize the care of babies with gastroschisis to other parts of Tanzania. METHODS: We used a Step-Wedge Implementation Science design to scale up gastroschisis care through training of providers, dissemination and current revision of evidence-based care protocols, advocacy, and engagement with stakeholders. We used mixed methods for data collection. Anonymous patient and provider evaluation data were collected using a nationwide Gastroschisis Database via REDCap. We evaluated the implementation and effectiveness of the care bundle in different hospitals in Tanzania. RESULTS: Decentralizing care nationally was feasible, acceptable, and adaptable. A total of nine trainings have been conducted training 420 providers (14 Master Trainers) reaching seven regions of Tanzania. The three advocacy national campaigns have ensured community reach and patient engagement. A countrywide gastroschisis database was developed to collect data on patients with gastroschisis, hosted locally at MNH with 332 patients' data entered in 1 year. The majority (90.2%) were treated using preformed silo bags with an overall survival of 28.5% in all centers. Late presentation and infection remain to be the main challenge. CONCLUSION: To achieve quality and sustainable surgical care, there is a need to design, implement, evaluate, and continuously improve context-relevant strategies to achieve and sustain the survival of neonates with congenital anomalies. Decentralization enables clear connectedness of hospitals, bringing care closer to patients.

Use of a new vertical traction device for early traction-assisted staged closure of congenital abdominal wall defects: a prospective series of 16 patients.

PURPOSE: Abdominal wall closure in patients with giant omphalocele (GOC) and complicated gastroschisis (GS) remains to be a surgical challenge. To facilitate an early complete abdominal wall closure, we investigated the combination of a staged closure technique with continuous traction to the abdominal wall using a newly designed vertical traction device for newborns. METHODS: Four tertiary pediatric surgery departments participated in the study between 04/2022 and 11/2023. In case primary organ reduction and abdominal wall closure were not amenable, patients underwent a traction-assisted abdominal wall closure applying fasciotens®Pediatric. Outcome parameters were time to closure, surgical complications, infections, and hernia formation. RESULTS: Ten patients with GOC and 6 patients with GS were included. Complete fascial closure was achieved after a median time of 7 days (range 4-22) in GOC and 5 days (range 4-11) in GS. There were two cases of tear-outs of traction sutures and one skin suture line dehiscence after fascial closure. No surgical site infection or signs of abdominal compartment syndrome were seen. No ventral or umbilical hernia occurred after a median follow-up of 12 months (range 4-22). CONCLUSION: Traction-assisted staged closure using fasciotens®Pediatric enabled an early tension-less fascial closure in GOC and GS in the newborn period.

[Nursing Experience Applying Transition Theory to Assist the Family of an Infant With Complex Congenital Gastroschisis].

This article describes the application of transition theory to assist a family with an infant with congenital complex gastroschisis. The nursing period, from March 3, 2023 to May 9, 2023, encompassed care from hospitalization to discharge. The author employed transition theory as a guide and used physical assessments, observations, and interviews for data collection as well as behavioral processes records. The primary nursing problem was identified as "preparation for family operation process enhancement/child's congenital disease and complex care needs, and the family's response to the challenges of the disease and care adaptation." The three phases of nursing care were summarized as: (1) the family adjustment to uncertainty, (2) undertaking caregiving roles and responsibilities, and (3) role development and family reconnection. The author established specific goals for each phase and provided corresponding interventions for the family. In the first phase, the author guided the family in expressing their concerns, and offered personalized health education information as well as psychological support to help them understand the progression of their child's disease and alleviate related anxiety and confusion. In the second phase, the author offered sleep guidance and customized home care schedules to support coping skill development and role functioning. In the third phase, the family was encouraged to explore the meaning of life while accompanying their child's growth in order to achieve spiritual growth and deepen the reconnection within the family. Ultimately, the family strengthened their confidence and capabilities in caregiving and embraced optimism and expectations for the future, enabling them to adapt smoothly to life after their child's return home. When families are confronted with their child's diagnosis with a congenital disease, they often find themselves in a state of self-doubt and faced with continuous challenges. Nurses may employ transition theory throughout the nursing process to better understand and address the evolving needs of both children and their families during the transition phase. Furthermore, transition theory may be applied to help nurses better assess, plan, and care for their patients, which can enhance the capabilities of families and facilitate their successful navigation through the challenging transition journey.

[Gastroschisis and omphalocele: Incidence and outcome].

INTRODUCTION: Gastroschisis and omphalocele are the most common congenital abdominal wall defects. The main purpose of this study was to investigate the incidence, other associated anomalies and the course of these diseases in Iceland. MATERIAL AND METHODS: The study was retrospective. The population was all newborns who were admitted to the NICU of Children's Hospital Iceland due to gastroschisis or omphalocele in 1991-2020. Furthermore, all fetuses diagnosed prenatally or post mortem where the pregnancy ended in spontaneous or induced abortion, were included. RESULTS: During the study period, 54 infants were born with gastroschisis and five with omphalocele. The incidence of gastroschisis was 4.11 and omphalocele 0,38/10,000 births. There was no significant change in the incidence of the diseases during the study period. In addition, five fetuses were diagnosed with gastroschisis and 31 with omphalocele where the pregnancy was terminated. In addition to gastroschisis in the live born infants and fetuses the most common associated anomalies were in the gastrointestinal or urinary tract but in infants and fetuses with omphalocele anomalies of the cardiac, central nervous or skeletal systems were the most common. Sixteen fetuses diagnosed with omphalocele had trisomy 18. Mothers aged 16-20 were more likely to give birth to an infant with gastroschisis than older mothers (p< 0.001). Primary closure was successful in 86% of the infants. Those reached full feedings significantly earlier and were discharged earlier. Overall survival rate was 95%. Three children were still receiving parenteral nutrition at discharge due to short bowel syndrome. CONCLUSIONS: The incidence of gastroschisis in Iceland is in accordance with studies in other countries but but the incidence of omphalocele is lower, which can be partly explained by spontaneous or induced abortions. Other anomalies associated with omphalocele are more severe than those associated with gastroschisis. Primary closure was associated with more benign course. Children with gastroschisis may need prolonged parenteral nutrition due to shortening of their intestines.

Acellular Bovine Pericardial Patch for Difficult Abdominal Closure in the Pediatric Population: Our Experience with Review of Literature.

Aims: Closure of congenital body wall defects in children can be a challenging task for the pediatric Surgeon. Biological prosthesis has been increasingly used for high-risk wound closure in adult patients with excellent outcomes and use in the pediatric population has also been reported. Here, we aim to study the outcome of abdominal wound repair with a tissue-engineered acellular bovine pericardial patch. Methods: Over a period of 21 months, a total of 15 children had undergone abdominal wound repair with bioprostheses, i.e., bovine pericardial patch at our institute. Patient demographics, cause of defect, an indication of patch use, rate of infection, postoperative recovery, recurrence, and outcome were studied. Results: A total of 15 patients underwent abdominal wall closure with acellular bovine pericardial patch. Nine out of 15 patients were neonates, of whom five had gastroschisis, two had a congenital diaphragmatic hernia, and two had ruptured omphalocele major. Of the rest 6 patients, 2 were patients of bladder exstrophy, 2 were older children of congenital diaphragmatic hernia with incisional hernias, and 2 were older children with omphalocele major. Out of the five patients with gastroschisis, two died during the early postoperative period due to sepsis. The wound healed in the rest 13 patients with mild skin dehiscence in two patients. Only one child had a recurrence. Conclusion: Reconstruction with acellular bovine pericardial patch is a viable option in children with high-risk abdominal wounds as it allows tensionless repair with excellent healing and minimal complications. Recurrence, if any, may disappear with time as remodeling of the prosthesis occurs along with the growth of the body wall of the child.

Mexican-American Women's Lifelong Residence in the United States Is Associated with an Increased Risk of Gastroschisis: A Population-Based Study.

OBJECTIVE: To determine whether nativity is associated with abdominal wall defects among births to Mexican-American women. STUDY DESIGN: Using a cross-sectional, population-based design, stratified and multivariable logistic regression analyses were performed on the 2014-2017 National Center for Health Statistics live-birth cohort dataset of infants of US-born (n = 1 398 719) and foreign-born (n = 1 221 411) Mexican-American women. RESULTS: The incidence of gastroschisis was greater among births to US-born compared with Mexico-born Mexican-American women: 36.7/100 000 vs 15.5/100 000, RR = 2.4 (2.0, 2.9). US-born (compared with Mexico-born) Mexican-American mothers had a greater percentage of teens and cigarette smokers, P < .0001. In both subgroups, gastroschisis rates were greatest among teens and decreased with advancing maternal age. Adjusting for maternal age, parity, education, cigarette smoking, pre-pregnancy body mass index, prenatal care usage, and infant sex), OR of gastroschisis for US-born (compared with Mexico-born) Mexican-American women was 1.7 (95% CI 1.4-2.0). The population attributable risk of maternal birth in the US for gastroschisis equaled 43%. The incidence of omphalocele did not vary by maternal nativity. CONCLUSIONS: Mexican-American women's birth in the US vs Mexico is an independent risk factor for gastroschisis but not omphalocele. Moreover, a substantial proportion of gastroschisis lesions among Mexican-American infants is attributable to factors closely related to their mother's nativity.

Longitudinal growth of children born with gastroschisis or omphalocele.

Normal childhood growth is an indicator of good health, but data addressing the growth of children born with abdominal wall defects (AWDs) are limited. The detailed growth phenotypes of children born with gastroschisis or omphalocele are described and compared to peers without AWDs from birth to adolescence. Data from 183 gastroschisis and 144 omphalocele patients born between 1993 and 2017 were gathered from Finnish nationwide registers and electronic health records. Weight (n = 3033), length/height (n = 2034), weight-for-length (0-24 months, n = 909), and body mass index measures (2-15 years, n = 423) were converted into sex- and age-specific Z-scores. Linear mixed models were used for comparisons. Intrauterine growth failure was common in infants with gastroschisis. Birth weight Z-scores in girls and boys were - 1.2 (0.2) and - 1.3 (0.2) and length Z-scores - 0.7 (0.2) and - 1.0 (0.2), respectively (p < 0.001 for all comparisons to infants without AWDs). During early infancy, growth failure increased in infants with gastroschisis, and thereafter, catch-up growth was prominent and faster in girls than in boys. Gastroschisis children gained weight and reached their peers' weights permanently at 5 to 10 years. By 15 years or older, 30% of gastroschisis patients were overweight. Infants with omphalocele were born with a normal birth size but grew shorter and weighing less than the reference population until the teen-age years. CONCLUSION: Children with gastroschisis and omphalocele have distinct growth patterns from fetal life onwards. These growth trajectories may also provide some opportunities to modulate adult health. WHAT IS KNOWN: • Intrauterine and postnatal growth failure can be seen frequently in gastroschisis and they often show significant catch-up growth later in infancy. It is assumed that part of the children with gastroschisis will become overweight during later childhood. WHAT IS NEW:  â€¢ The longitudinal growth of girls and boys with gastroschisis or omphalocele is described separately until the teenage years. The risk of gaining excessive weight in puberty was confirmed in girls with gastroschisis.

Retrospective review of antimicrobial use for gastroschisis patients in Kigali, Rwanda: can improved stewardship reduce late inpatient deaths?

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.

Prediction of Neonatal Growth Restriction in Fetuses With Gastroschisis by Early Third Trimester Ultrasonography Utilizing Contemporary Birth Weight Percentiles.

OBJECTIVE: To identify the estimated fetal weight (EFW) formula and threshold for the optimal prediction of fetal growth restriction (FGR) at 26-34 weeks' in fetuses with gastroschisis. METHODS: Late second and third trimester ultrasound data were used to calculate the EFW utilizing eight different formulas: Hadlock I-IV, Honarvar, Shepard, Siemer, and Warsof. EFW and birth weight percentiles were assigned from US population growth curves. FGR and small for gestational age (SGA) were defined as EFW and birth weight less than the tenth percentile for gestational age; Receiver operating characteristic (ROC) curves were used to compare formula performance for FGR diagnosis at 26-34 weeks' to identify an SGA birth weight. RESULTS: There were 170 newborns with gastroschisis; 46 (27%) were SGA. The mean gestational age at the time of ultrasound was 30.8 ± 1.7 weeks. The mean gestational age at birth was 36.3 ± 1.7 weeks. ROC curve analysis found the Hadlock III formula had the largest area under the curve (AUC) of 0.813 closely followed by Hadlock IV (AUC = 0.811) and Hadlock II (AUC = 0.808) for diagnosis of FGR correlating to neonatal SGA diagnosis. Hadlock II, Hadlock III, and Hadlock IV had the highest diagnostic accuracies when compared to the other EFW formulas. CONCLUSIONS: The Hadlock II, Hadlock III, and Hadlock IV formulas have comparable predictive performance in the optimal identification of FGR in fetuses with gastroschisis at 26-34 weeks'. A threshold of an EFW less than the 25.2th percentile is suggested.

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.