A multicenter matched-cohort analysis of gastroschisis outcomes in infants born before 32 weeks gestation.

OBJECTIVE: To examine neonatal outcomes of infants with gastroschisis born <32 weeks' gestation compared to matched infants without gastroschisis. STUDY DESIGN: Retrospective matched-cohort analysis of infants with gastroschisis born <32 weeks' gestation at Children's Hospitals Neonatal Consortium (CHNC) NICUs from 2010 to 2022 compared to gestational age-matched controls. RESULTS: The study included 119 infants with gastroschisis and 357 matched infants; 60% of infants born 29-32 weeks, 23% born 26-28 weeks, and 16% born < 25 weeks. Mortality was not significantly different between groups (11% vs. 9%, p = 0.59). Preterm co-morbidities such as IVH, BPD, ROP, and PVL were similar, as were rates of surgical NEC. Infants with gastroschisis had longer hospital stays (92 vs. 67 days), higher CLABSI and UTIs, and were more likely to need feeding support at discharge. CONCLUSION: Compared to infants without gastroschisis, infants <32 weeks' gestation with gastroschisis had similar risks for inpatient mortality, NEC, and other preterm co-morbidities.

Special Populations-Surgical Infants.

Congenital gastrointestinal disorders and other surgical diagnoses share many common problems: increased nutritional requirements to prevent catabolism, enhance wound healing, and provide optimal growth; impaired motility and altered intestinal flora leading to feeding intolerance requiring long-term parenteral nutrition; gastroesophageal reflux and poor feeding mechanics requiring tube feedings and support; growth failure; poor barrier function and risk of infection; and other long-term sequelae. Consequently, the surgical "at-risk" infant requires specialized nutritional support to meet their increased requirements to ensure adequate growth and meet the increased demands from critical illness.

Single-Center Experience on Growth in Infants Born With End-Stage Kidney Disease.

OBJECTIVES: Children with chronic kidney disease display poor growth that impacts health outcomes; data on infants with severe congenital anomalies of the kidney and urinary tract (CAKUT) are limited. We examined growth patterns in infants with CAKUT requiring dialysis in the first 30 days. METHODS: This study evaluated infants with severe CAKUT from 2014 to 2018 surviving past 30 days. Somatic growth parameters as per standard infant curves and nutritional information were recorded. RESULTS: Twenty four infants met inclusion criteria. Seventeen infants received dialysis, demonstrating somatic growth disruption most profound at a 1-2 months postnatal age. Growth trends were improved compared to infants with CAKUT who did not require dialysis. Linear growth failed to normalize by 1 year of age. CONCLUSIONS: Infants with severe CAKUT are at high risk for early growth failure. Understanding of this deficit and impacts of early dialysis on growth and long-term outcomes are needed to identify targeted nutritional strategies.

Gastroschisis and low incidence of early-onset infection: a case for antimicrobial stewardship.

OBJECTIVE: Early onset infection (EOI) in gastroschisis is rare. Excess antibiotic exposure in neonates increases necrotizing enterocolitis and mortality. We evaluated antibiotic exposure and EOI in gastroschisis. STUDY DESIGN: Retrospective cohort analysis between 2010-2016 in the Children's Hospital Neonatal Database. Included: Infants ≥32 weeks with gastroschisis admitted <48 h. Excluded: major anomalies or surgical intervention prior to admission. PRIMARY OUTCOME: EOI diagnosis (<72 h). RESULTS: In 2021 patients with gastroschisis, median gestational age was 36 weeks (IQR 35, 37). 93.9% patients received empiric antibiotics after delivery, with median 7 days duration (IQR 3, 9). Only 13 patients (0.64%) had early positive blood culture. The rate of late onset blood stream infection (7.08%) was higher, and higher in complex (18%) than simple gastroschisis (4.8%, p < 0.001). CONCLUSION: Despite low incidence of EOI and risks of excess antibiotic exposure, neonates with gastroschisis are exposed to long courses of empiric antibiotics. These data should stimulate interinstitution work to improve antibiotic prescribing.

Gestational Age at Delivery and Neonatal Outcomes among Infants with Gastroschisis in the Children's Hospitals Neonatal Consortium (CHNC).

OBJECTIVE: The effect of gestational age (GA) on gastroschisis outcomes is unclear and delivery timing varies in practice. We aimed to correlate clinical outcomes of infants with gastroschisis and GA at delivery in the Children's Hospitals Neonatal Consortium (CHNC). STUDY DESIGN: This was a retrospective multicenter cohort study of infants with gastroschisis admitted to CHNC neonatal intensive care units (NICUs) from 2010 to 2016. Patients were categorized by GA: 32 to 346/7, 35 to 366/7, and ≥37 weeks. Respiratory and feeding interventions, mortality, length of stay, and common complications were compared. RESULTS: In 2021 for patients with gastroschisis, median GA at delivery was 36.3 weeks (interquartile range [IQR] 35.1, 37.3) and mean birth weight 2,425 g (IQR 2,100, 2,766). Overall mortality was low and there was no difference across GA groups. Infants <35 weeks' gestation had the greatest need for respiratory and feeding interventions. Complications such as medical necrotizing enterocolitis (NEC), cholestasis, and central line-associated blood stream infection were less common in infants ≥37 weeks. Feeding initiation and full feeds were earliest in term infants, compared with infants between 35 and 366/7 weeks, and longest in infants <35 weeks. Prematurity had a significant negative association with breast milk exposure. Enteral feeding tube support at discharge increased with prematurity. Compared with term, infants born between 35 and 366/7 weeks' gestation had a higher incidence of medical NEC and lower exposure to mother's milk at discharge but the need for respiratory interventions or tube feeding at discharge was similar. CONCLUSION: Premature infants with gastroschisis had more neonatal complications including respiratory interventions, longer NICU stay, longer time to full enteral feeds, and higher need for tube feeds at discharge as compared with those delivered at term. Differences were greatest for those <35 weeks GA. While overall mortality remains low, these results provide additional information about GA at birth in gastroschisis, with no evidence of benefit from preterm delivery. KEY POINTS: · Respiratory support was greatest for those with <35 weeks gestation.. · NEC and cholestasis increase with prematurity.. · Term infants have better feeding outcomes..

Gastroschisis with intestinal atresia leads to longer hospitalization and poor feeding outcomes.

OBJECTIVE: Compare in-hospital outcomes in gastroschisis with intestinal atresia versus simple gastroschisis (GS) using a national database. STUDY DESIGN: The Children's Hospitals Neonatal Database identified infants with gastroschisis from 2010 to 2016. RESULTS: 2078 patients with gastroschisis were included: 183 (8.8%) with co-existing intestinal atresia, 1713 (82.4%) with simple gastroschisis, the remainder with complex gastroschisis without atresia. Length of hospitalization was longer for those with atresia, and yielded higher rates of mortality, medical NEC, and intestinal perforation. They began enteral feedings later, were less likely to initiate feeds orally, and reached full feedings later. They were less likely to be receiving any maternal breast milk or breastfeeding at discharge and more likely than simple gastroschisis to be discharged with a feeding tube. CONCLUSION: A large multicenter cohort showed gastroschisis with atresia results in worse outcomes and complications, including necrotizing enterocolitis, feeding delays, and enteral feeding tube dependence.

Body Composition of Infants With Congenital Gastroschisis.

ABSTRACT: Infants born with congenital gastroschisis are at risk for intrauterine growth restriction, small for gestational size at birth, and growth failure during the newborn period despite advanced care. Body composition provides a more complete picture of proportional growth than weight and length alone. Fat-free mass (FFM) represents organ growth, and in preterm infants without gastroschisis, improved FFM deposition is associated with improved neurodevelopmental outcomes. There is limited literature regarding the body composition of infants with gastroschisis. This case series describes the body composition of 10 infants with gastroschisis.

EXIT-to-airway: Fundamentals, prenatal work-up, and technical aspects.

Ex-utero intrapartum treatment (EXIT) is a delivery strategy developed to manage a variety of prenatally diagnosed conditions in the transition to newborn life. This procedure allows control and provides time for intervention in otherwise life-threatening malformations, such as congenital upper airway obstructions. EXIT-to-airway has changed the outcome of fetuses with these anomalies. The main purpose of this intervention is to improve the safety of establishing a reliable airway at birth. Maximal but controlled uterine relaxation to maintain feto-maternal perfusion and thus gas exchange, while keeping the fetal and maternal well-being are the paradigms of any type of EXIT. The most important aspect of fetal airway management is to consolidate a highly trained, well-coordinated, multidisciplinary team that is prepared for every contingency. A comprehensive prenatal assessment, including ultrasound, fetal echocardiogram, fetal MRI, and genetic testing is imperative for patient selection. Extensive preoperative planning, ad-hoc team meetings, and surgical simulations for challenging cases are critical strategies to achieve the best outcomes. This article outlines the prenatal work-up, decision making, technical aspects, and principles for a successful EXIT-to-airway procedure.

Morbidity and cost burden of prenatal myelomeningocele repair.

OBJECTIVE: This study compared the morbidities and financial burden associated with prenatal open myelomeningocele repair versus postnatal repair. MATERIALS AND METHODS: The retrospective study cohort included 23 mother-infant dyads undergoing prenatal repair and 30 with postnatal repair. Financial, demographic, and medical information were obtained for mother-infant dyads from each infant's birth through the first year of life. RESULTS: Infants in the prenatal repair group were significantly affected by sequelae of prematurity, including apnea, bronchopulmonary dysplasia, and retinopathy of prematurity. Importantly, fewer of these infants required ventriculoperitoneal shunt procedures and the overall improved composite outcome of shunting or death. Infants in the postnatal repair group required more hospital readmissions in the first year of life. Financial costs for mother-infant dyads in the prenatal repair group were higher, driven by the length of stay in the neonatal intensive care unit and maternal hospital admissions. Kaplan-Meier curves analyzing the relative contributions of the length of stay and total charges in the population were constructed. The maternal contribution was markedly different between groups due to postoperative hospitalization, readmission, and close fetal surveillance required. Though these differences did not reach statistical significance, it highlights an important burden on families and the medical system. CONCLUSIONS: This is the first study to report the contributions of prematurity in relation to maternal and infant morbidity and financial costs. The benefits of prenatal myelomeningocele repair include lower risk for ventriculoperitoneal shunting and fewer hospital readmissions. The risk of preterm birth and its neonatal sequelae continue to be a significant burden.

Prenatal diagnosis of Proteus syndrome: Diagnosis of an AKT1 mutation from amniocytes.

Proteus syndrome is a mosaic genetic overgrowth disorder caused by a postzygotic, mosaic activating mutation in AKT1. Rare prenatal presentations include segmental tissue overgrowth, and skeletal and CNS anomalies. We present the first report of prenatally diagnosed and molecularly confirmed Proteus syndrome. Prenatal imaging identified megalencephaly, brain and eye malformations, focal soft tissue enlargement, and ambiguous genitalia. Exome sequencing performed on cultured amniocytes demonstrated an AKT1 pathogenic variant consistent with Proteus syndrome, and postnatal examination confirmed the diagnosis. Postnatal Sanger sequencing could not identify the AKT1 pathogenic variant. This case underscores the importance of prenatal exome sequencing on cultured amniocytes for mosaic overgrowth disorders, as well as provides additional information on the prenatal phenotype of Proteus syndrome, and highlights the impact of prenatal diagnosis on postnatal management.

Contemporary Outcomes of Patients with Isolated Bilateral Renal Agenesis with and without Fetal Intervention.

INTRODUCTION: Bilateral renal agenesis (BRA) is a lethal diagnosis, specifically meaning that natural survival beyond birth is not expected secondary to pulmonary hypoplasia. Limited contemporary data are available about intervention and the impact of restoring amniotic fluid volume in relation to the risk for lethal pulmonary hypoplasia and other factors that might influence survival in cases of fetal BRA. OBJECTIVE: We report the largest series of patients undergoing fetal intervention and postnatal care for BRA at a single comprehensive fetal center. METHODS: All patients with fetal BRA were reviewed from January 2004 to November 2017. Maternal and neonatal data were collected in an institutional review board-approved retrospective review. RESULTS: From 2014 to 2017, 20 singleton pregnancies with isolated fetal BRA were evaluated and 14 had amnioinfusion. Eight had serial infusions. Of those, there were 6 neonatal deaths. There were 2 neonatal survivors beyond 30 days; however, both died of sepsis on dialysis. One of these survivors received amnioinfusions by percutaneous approach and one via amnioport. There were no survivors to transplantation. CONCLUSION: Fetal intervention via amnioinfusion may promote pulmonary survivorship after birth, but postnatal survival remains poor. Future studies must place an emphasis on standardizing the postnatal approach to this patient population.

Emergency department utilization among pediatric spina bifida patients.

PURPOSE: There is a gap in knowledge regarding the use of emergency services by pediatric spina bifida patients. The goal of this study was to describe Emergency Department utilization patterns in this population. METHODS: Through a retrospective observational study, patients with spina bifida who visited the emergency department during a four-year period were identified; medical and demographic information was obtained though the Centers for Disease Control National Spina Bifida Patient Registry. Chief complaints and final diagnoses of visits were classified and related to medical needs of spina bifida to determine the appropriate care level. RESULTS: Among 303 children within the registry, 161 patients (53%) accounted for 579 visits. 70% of visits were for spina bifida-related complaints. Approximately half (51.7%) had a shunt-related chief complaint, although final diagnosis was largely unrelated to the shunt. Admission rate was 39%, higher than institutional baseline, and largely represented by genitourinary (GU) complaints. CONCLUSION: Pediatric patients with spina bifida presenting to a single center emergency department were most likely to present with shunt and urinary concerns; these patients were most likely to be admitted. This potentially suggests that improving outpatient care for bladder management may decrease emergency department use among this population.