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.

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.

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.

[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.

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.

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.

Gastroschisis in Mozambique: current status and priorities for improving care from Hospital Central de Maputo, the largest and referral hospital of the country.

PURPOSE: Historically, gastroschisis was considered a death sentence in Mozambique. The purpose of this study was to evaluate the current state of gastroschisis management and outcomes in our facility and to identify potential areas of improvement in neonatal and surgical care. METHODS: A retrospective study was performed of all gastroschisis patients admitted to Hospital Central Maputo located in Maputo City/ Mozambique from 2019 to 2020. Demographic, perinatal, operative, and mortality data were obtained from neonatal and surgical logbooks. Descriptive analysis was performed. RESULTS: A total of 62 gastroschisis patients were admitted to the Hospital Central Maputo. No patients had a prenatal diagnosis. Many of the infants were born preterm (48%), and 68% had low birth weight. Only 15 (24%) patients underwent operative intervention (73% primary fascial closure and 27% sutured silo). There were only three survivors (5%) all of whom underwent primary closure. However, the overall survival rate for patients undergoing an attempt at surgical closure was 20%. CONCLUSION: While the mortality rate remains high for gastroschisis patients in Mozambique, there have been a few survivors when surgery is performed. Improvements in neonatal care are needed. Given the high mortality rates and limited resources, we plan to focus our surgical efforts on bedside closure techniques.

Factors Associated with Hospital Readmission One Year Post-Discharge in Infants with Gastroschisis.

INTRODUCTION: Gastroschisis is the most common congenital abdominal wall defect with a rising prevalence. Infants with gastroschisis are at risk for multiple complications, leading to a potential increased risk for hospital readmission after discharge. We aimed to find the frequency and factors associated with an increased risk of readmission. METHODS: A retrospective analysis of infants born with gastroschisis between 2013 and 2019 who received initial surgical intervention and follow-up care in the Children's Wisconsin health system was performed. The primary outcome was the frequency of hospital readmission within 1 year of discharge. We also compared maternal and infant clinical and demographic variables between those readmitted for reasons related to gastroschisis, and those readmitted for other reasons or not readmitted. RESULTS: Forty of 90 (44%) infants born with gastroschisis were readmitted within 1-year of the initial discharge date, with 33 (37%) of the 90 infants being readmitted due to reasons directly related to gastroschisis. The presence of a feeding tube (p < 0.0001), a central line at discharge (p = 0.007), complex gastroschisis (p = 0.045), conjugated hyperbilirubinemia (p = 0.035), and the number of operations during the initial hospitalization (p = 0.044) were associated with readmission. Maternal race/ethnicity was the only maternal variable associated with readmission, with Black race being less likely to be readmitted (p = 0.003). Those who were readmitted were also more likely to be seen in outpatient clinics and utilize emergency healthcare resources. There was no statistically significant difference in readmission based on socioeconomic factors (all p > 0.084). CONCLUSION: Infants with gastroschisis have a high hospital readmission rate, which is associated with a variety of risk factors including complex gastroschisis, multiple operations, and the presence of a feeding tube or central line at discharge. Improved awareness of these risk factors may help stratify patients in need of increased parental counseling and additional follow-up.

Three-Dimensional Ultrasound Evaluation of Lung Volume in Fetuses with Abdominal Wall Defect.

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 <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 < 0.001) and gastroschisis (p < 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 < 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 < 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.

Prenatal diagnosis of closed gastroschisis: What to expect in the most severe form of gastroschisis? Case report and literature review.

Closed gastroschisis (CG) and vanishing gastroschisis (VG) are the most severe forms of evolution of the malformation. In this case, a fetus presented with gastroschisis at 13 weeks, and the gastroschisis was not visualized at 22 weeks of gestation. Distal ileum, cecum, and ascending colon atresia were diagnosed at surgery, and the child is fully enteral-fed at 5 months of age. In a literature review of 43 cases of prenatal diagnosis of CS-VG, intrauterine death occurred in 4.5% of cases. 79.6% of the cases underwent surgical treatment. Of these cases, 20% of cases died due to complications of treatment, 26% were still under treatment and 54% are alive with enteral feeding. In 77% of the cases, the closure of the umbilical ring occurred after 26 weeks of gestation and the absence of prenatal diagnosis of CG-VG during pregnancy has a risk of 71% of death.

Hernia of Umbilical Cord: An Enigma.

Introduction: Hernia of the umbilical cord (HUC) is an uncommon problem which is often misdiagnosed, leading to inadequate treatment and various complications which cause increased morbidity and mortality in neonates. To address this issue, we took up this study. Materials and Methods: Patients diagnosed with HUC from January 1, 2017, to December 31, 2021, were retrospectively analyzed. The following data of all these patients were collected and retrospectively analyzed: demography, radiological investigations, echocardiography, contents of hernia, type of surgery performed, and outcome. Results: Eighteen out of 19 patients included in the study were taken up for surgery after baseline investigations and echocardiography. Out of 19 patients, 15 were discharged successfully and are on regular follow-up. Of the remaining four patients, an anastomotic leak occurred in two and they went into sepsis and succumbed. One of the patients died before any intervention and one succumbed on 3rd postoperative day due to sepsis. Conclusion: Timely referral and intervention can save precious lives. We need to educate doctors and health-care providers so that proper diagnosis and timely management can be done for this anomaly which is associated with less morbidity and a lower rate of associated anomalies.