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.

Limb-body wall complex: Literature review and case report.

INTRODUCTION: Body wall anomalies comprise a wide range of malformations. Limb-Body wall complex (LBWC) represents the most severe presentation of this group, with life threatening malformations in practically all the cases, including craniofacial, body wall defects, and limb anomalies. There is no consensus about its etiology and folding and gastrulation defects have been involved. Also, impaired angiogenesis has been proposed as a causative process. CASE REPORT: We present the case of a masculine stillborn, product of the first pregnancy in a 15-year-old, apparently healthy mother. He was delivered at 31 weeks of gestation due to an early rupture of membranes. He presented with multiple malformations including a wide body wall defect with multiple organ herniation and meromelia of the lower right limb. DISCUSSION AND CONCLUSIONS: LBWC represents a severe and invariably fatal pathology. There are no described risk factors, nevertheless, this case presented in a teenage mother, a well-described risk factor for other body wall anomalies. Its diagnosis allows us to discriminate between other pathologies that require prenatal or postnatal specialized treatment.

Gastroschisis: embriology, pathogenesis, risk factors, prognosis, and ultrasonographic markers for adverse neonatal outcomes.

Gastroschisis is the most common congenital defect of the abdominal wall, typically located to the right of the umbilical cord, through which the intestinal loops and viscera exit without being covered by the amniotic membrane. Despite the known risk factors for gastroschisis, there is no consensus on the cause of this malformation. Prenatal ultrasound is useful for diagnosis, prognostic prediction (ultrasonographic markers) and appropriate monitoring of fetal vitality. Survival rate of children with gastroschisis is more than 95% in developed countries; however, complex gastroschisis requires multiple neonatal interventions and is associated with adverse perinatal outcomes. In this article, we conducted a narrative review including embryology, pathogenesis, risk factors, and ultrasonographic markers for adverse neonatal outcomes in fetuses with gastroschisis. Prenatal risk stratification of gastroschisis helps to better counsel parents, predict complications, and prepare the multidisciplinary team to intervene appropriately and improve postnatal outcomes.

Implementación de paquete de medidas de conductas perinatales en recién nacidos con gastrosquisis congénita / Implementation of a perinatal bundles measures in newborns with congenital gastroschisis / ¿Implementação de um pacote de medidas comportamentais perinatais em recém-nascidos com gastrosquise congénita

Introducción: la gastrosquisis congénita (GC) es una patología con creciente demanda asistencial. Atentos a esto, en el Centro Hospitalario Pereira Rossell (CHPR) se implementó un protocolo de cuidados llevado adelante por un equipo multidisciplinario. En este trabajo nos proponemos evaluar el impacto en la sobrevida y morbilidad a partir de su aplicación. Material y método: estudio comparativo tipo antes y después de pacientes portadores de GC (n = 47), 29 de una cohorte histórica (GH) y 18 de una cohorte posaplicación de protocolo (GP). Con edad gestacional ≥ 36 semanas y peso al nacer ≥ 2500 g, asistidos en el CHPR entre los años 2016 y 2021. Resultados: no se observaron diferencias perinatales entre ambos grupos, ni en la incidencia de GQ complicadas. En el GP se observó mayor incidencia de cierre abdominal precoz (p < 0,01), menor necesidad de realización de silo quirúrgico (p < 0,01), menor tiempo de administración de ventilación mecánica (p = 0,03), de uso total de opioides (p < 0,01), de administración de nutrición parenteral (p = 0,03), e inicio más temprano de la alimentación enteral (p = 0,03). Sin diferencias en la sobrevida al egreso hospitalario. Conclusiones: la aplicación de un protocolo estandarizado se asoció a una reducción significativa en el tiempo de cierre abdominal, de ventilación mecánica, nutrición parenteral y de opioides, con inicio más precoz de la nutrición enteral.

Introduction: congenital gastroschisis is an increasingly demanded pathology, therefore a care protocol was implemented at the Pereira Rossell Pediatric Hospital (CHPR), and it has been carried out by a multidisciplinary team. In this paper, we aim at assessing the morbidity and mortality impact of survival and morbidity of this application. Materials and Methods: comparative before and after study of a historical cohort (GH, n=29) versus a post-protocol application cohort (GP, n=18), in patients with a gestational age ≥36 weeks and birth weight ≥2500 grams assisted in CHPR between 2016-2021. Results: no perinatal differences were observed between both groups or in the incidence of complicated GQ. In the GP, a higher incidence of early abdominal closure was decreased (GH 3.4% vs GP 67%, p<0.01), less need to perform a surgical silo (GH 100% vs GP 33%, p<0.01 ), shorter mechanical ventilation administration time (GH 2 days vs GP 0.5 days, p=0.03), total use of opioids (GH 3.5 days vs GP 7 days, p<0.01) , of administration of parenteral nutrition (GH 24.5 days vs GP 20 days, p=0.03), and earlier start of enteral feeding (GH 11 days vs GP 7 days, p=0.03). No differences in survival after hospital discharge (93% vs 89%, p=0.63). Conclusions: the application of a standardized protocol was linked to a significant reduction in the time of abdominal closure, mechanical ventilation, parenteral nutrition, and opioids, with earlier initiation of enteral nutrition.

Introdução: a gastrosquise congênita (GC) é uma patologia com demanda crescente de atendimento e, porém, foi implementado um protocolo de atendimento por equipe multidisciplinar no Centro Hospitalar Pediátrico Pereira Rossell (CHPR). Neste trabalho propomos avaliar o impacto na mortalidade e morbidade depois de sua aplicação. Material e Métodos: estudo comparativo antes e depois de pacientes com CG (n=47), sendo 29 de uma coorte histórica (GH) e 18 de uma coorte pós-aplicação de protocolo (GP). Idade gestacional ≥ 36 semanas e peso ao nascer ≥ 2.500 gramas atendidos no CHPR entre os anos de 2016-2021. Resultados: não foram observadas diferenças perinatais entre os dois grupos ou na incidência de QG complicada. No GP observou-se maior incidência de fechamento abdominal precoce (p<0,01), menor necessidade de silos cirúrgicos (p<0,01), menor tempo de administração de ventilação mecânica (p=0,03) e uso total de opioides. (p<0,01), administração de nutrição parenteral (p=0,03) e início mais precoce da alimentação enteral (p=0,03). Não houve diferenças na sobrevivência até a alta hospitalar. Conclusões: a aplicação de protocolo padronizado foi associada à redução significativa do tempo de fechamento abdominal, ventilação mecânica, nutrição parenteral e opioides, com início mais precoce da nutrição enteral.

Factors associated with cholestasis in newborns with gastroschisis / Fatores associados à colestase em recém-nascidos com gastrosquise

ABSTRACT Objective: To describe the incidence and to analyze risk factors associated with cholestasis in neonates with gastroschisis. Methods: This is a retrospective cohort study in a tertiary single center analyzing 181 newborns with gastroschisis between 2009 and 2020. The following risk factors associated with cholestasis were analyzed: gestational age, birth weight, type of gastroschisis, silo closure or immediate closure, days of parenteral nutrition, type of lipid emulsion, days of fasting, days to reach a full diet, days with central venous catheter, presence of infections, and outcomes. Results: Among the 176 patients evaluated, 41 (23.3%) evolved with cholestasis. In the univariate analysis, low birth weight (p=0.023), prematurity (p<0.001), lipid emulsion with medium-chain triglycerides and long-chain triglycerides (p=0.001) and death (p<0.001) were associated with cholestasis. In the multivariate analysis, patients who received lipid emulsion with fish oil instead of medium chain triglycerides/long chain triglycerides (MCT/LCT) emulsion had a lower risk of cholestasis. Conclusions: Our study shows that lipid emulsion with fish oil is associated with a lower risk of cholestasis in neonates with gastroschisis. However, this is a retrospective study and a prospective study should be performed to confirm the results.

RESUMO Objetivo: Analisar a incidência e os fatores de risco associados à colestase em recém-nascidos com gastrosquise. Métodos: Estudo de coorte retrospectivo em um único centro terciário, que analisou 181 recém-nascidos com gastrosquise entre 2009 e 2020. Foram examinados os seguintes fatores de risco associados à colestase: idade gestacional, peso ao nascer, tipo de gastrosquise, fechamento com silo ou fechamento imediato, dias de uso nutrição parenteral, tipo de emulsão lipídica, dias de jejum, dias para atingir a dieta completa, dias com cateter venoso central, presença de infecções e desfechos. Resultados: Dos 176 pacientes avaliados, 41 (23,3%) evoluíram com colestase. Baixo peso ao nascer (p=0,023), prematuridade (p<0,001), emulsão lipídica com triglicerídeos de cadeia média e triglicerídeos de cadeia longa (p=0,001) e óbito (p<0,001) foram associados à colestase. Na análise multivariada, os pacientes que receberam emulsão lipídica com óleo de peixe em vez da emulsão diária de triglicérides de cadeia média/triglicérides de cadeia longa (MCT/LCT) apresentaram menor risco de colestase. Conclusões: Nosso estudo mostra que a emulsão lipídica com óleo de peixe está associada a menor risco de colestase em neonatos com gastrosquise, porém este é um estudo retrospectivo, e um estudo prospectivo deve ser realizado para confirmar os resultados.

miRNA-143 expression is associated with inflammation and time of exposure to amniotic fluid in experimental gastroschisis.

OBJECTIVE: Gastroschisis (GS) is a congenital anomaly in the abdominal wall with the intestinal loops exiting laterally to the umbilicus. The contact of the loops with Amniotic Fluid (AF) causes an inflammatory process in the exposed part, leading to an extended hospital stay and an increased risk of morbidity due to alterations related to intestinal motility. The authors aimed to evaluate the time of exposure to the AF in the experimental GS and to search for potential biomarkers of intestinal inflammation by measuring microRNAs. METHODS: Rat fetuses were divided into three groups: a) CONTROL, b) GS reared on day 18 (GS = 18), and c) GS reared on day 19.5 (GS = 19) (term = 22 days). On day 21.5, the fetuses were removed for biometric parameters and biochemical analyses: 1) Biometrics: Body and Intestinal Weight (BW, IW), and intestinal-body weight ratio (IW/BW); 2) Descriptive histopathology and 3) miR-143 quantification by real-time Polymerase Chain Reaction (PCR). RESULTS: BW was higher in CONTROL than GS 18 and G19 (p < 0.05). IW, IW/BW, intestinal water, and mRNA-143 were higher in GS 18 and GS 19 than in CONTROL, and GS 18 was higher than GS 19 (p < 0.05). The average of the inflammation score from the intestinal wall with mucosal inflammation and intra-epithelial lymphocytes shows worst in GS 18 and GS 19 vs. CONTROL (p < 0.05). CONCLUSIONS: The tissue expression of mRNA-143 and the morphological changes in the intestine of GS worsened according to the time of exposure to AF, which could be a possible marker of fetal intestinal damage.

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.

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.

Vivência de mães no cuidado alimentar do filho com gastrosquise à luz de Ramona Mercer / Experience of mothers in the feeding care for children with gastroschisis in the light of Ramona Mercer / Vivencia de madres en el cuidado de alimentación de su hijo con gastrosquisis a la luz de Ramona Mercer

Objetivo: compreender a vivência materna no cuidado alimentar do filho com gastrosquise e discutir a assistência do enfermeiro a essas mães segundo as concepções de Ramona Mercer sobre o papel maternal. Métodos: estudo qualitativo realizado em duas unidades de terapia intensiva (neonatal e cirúrgica) de uma instituição federal no Rio de Janeiro. Foram entrevistadas 11 mães de recém-nascidos com gastrosquise que se alimentavam por via oral e sonda. Os dados foram analisados e interpretados à luz de Bardin e Ramona Mercer. Resultados: as falas evidenciaram dificuldades na construção da identidade materna, visto que alimentar o filho com gastrosquise é desafiador devido à instabilidade clínica, porém, o apoio da enfermagem mostrou-se fundamental nesse processo. Conclusão: dar voz e incluir as mães no cuidado faz com que elas se sintam seguras na criação do vínculo mãe-filho e estabeleçam estratégias para o desenvolvimento do papel materno, mesmo diante dos obstáculos impostos pela malformação

Objective: to understand the maternal experience in the feeding care of the infant with gastroschisis and discuss the assistance of the nurse to these mothers according to the conceptions of Ramona Mercer about the maternal role. Methods: qualitative study carried out in two intensive care units (neonatal and surgical) of a federal institution in Rio de Janeiro. A total of 11 mothers of newborns with gastroschisis who ate via oral and via tube were interviewed. The data were analyzed and interpreted in the light of Bardin and Ramona Mercer. Results: the answers evidenced the difficulties in building the maternal identity, given that feeding a child with gastroschisis is challenging due to the clinical instability; however, the support of the nursing personnel was essential in this process. Conclusion: giving voice and including the mothers in the care make them feel more secure in building the mother-child link and establishing strategies to the development of the maternal role, even in the face of obstacles imposed by the malformation.

Objetivo: comprender la vivencia materna en el cuidado de la alimentación del hijo con gastrosquisis y discutir la asistencia de enfermería a estas madres bajo la perspectiva de Ramona Mercer sobre el papel materno. Métodos: estudio cualitativo, realizado en dos unidades de cuidados intensivos (neonatal y quirúrgica) de una institución federal en Río de Janeiro. Se entrevistaron a 11 madres de recién nacidos con gastrosquisis, que se alimentaban vía oral y por sonda. Para el análisis e interpretación de los datos se utilizó la perspectiva de Bardin y de Ramona Mercer. Resultados: los relatos apuntaron a dificultades en la construcción de la identidad materna, pues alimentar al hijo con gastrosquisis es un desafío debido a la inestabilidad clínica; sin embargo, el apoyo de la enfermería resultó ser fundamental en este proceso. Conclusión: dar voz e incluir a las madres en el cuidado las hace sentir seguras en la creación del vínculo madre-hijo y establece estrategias para el desarrollo del rol materno, incluso ante obstáculos impuestos por esta malformación

Trends in prevalence of gastroschisis in Brazil from 2007 to 2020: A national population-based cross-sectional study.

BACKGROUND: Gastroschisis is a congenital abnormality of the abdominal wall which worldwide prevalence is increasing with time. Up to now, young maternal age is the main factor associated with gastroschisis. Brazil has a great proportion of adolescent mothers. OBJECTIVES: To estimate prevalence of gastroschisis in Brazil, explore the effect of very young maternal age, and analyze regional distribution and time trends. METHODS: Cross-sectional population-based study using data from official national information systems from 2007 to 2020, covering 9,831 gastroschisis cases. Low maternal age was assessed as risk factor through odds ratio. Prevalence time trends by maternal age and geographic area were analyzed using joinpoint regression. RESULTS: Overall prevalence of gastroschisis in Brazil was 2.47 per 10,000 births. From first to last biennium, there was a 23% relative increase. Last biennium prevalence was higher in the Central-west region and lower in the Northeast. Compared to mothers of 20-34 years old (YO), the odds-ratio for prevalence and 95% confidence intervals for gastroschisis were, as follows: (a) 10-14 YO-4.9 (4.3,5.5); (b) 15-19 YO-3.6 (3.5,3.8); and (c) 35-49 YO-0.3 (95% CI 0.3, 0.4). CONCLUSIONS: Prevalence of gastroschisis in Brazil relatively increased 23% from 2007 to 2020. This happened in all maternal age groups. Differences between regions may be associated to data quality. Young mothers had a higher prevalence of gastroschisis and still higher for those under 15 YO. Mother age over 34 YO was a protective factor.

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

SUMMARY 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.

Characterization of feeding skills and clinical markers of preterm newborns with gastroschisis in a neonatal therapy unit / Caracterização das habilidades de alimentação e marcadores clínicos de recém-nascidos prematuros com gastrosquise em unidade de terapia neonatal

ABSTRACT Objective To describe the clinical and feeding findings of premature infants with gastroschisis (GTQ) in a neonatal intensive care unit and compare them to preterm infants (NBs) without congenital anomalies. Methods A retrospective case-control study was conducted with 50 premature NBs (25 with GTQ and 25 without comorbidities - control group). The NBs were compared regarding demographic and clinical data: risk of mortality and speech-language assessment (nonnutritive and nutritive sucking). Subsequently, a multiple logistic regression model was applied to determine the variables associated with the negative speech therapy outcome (speech therapy discharge after more than 7 days considering the first speech therapy evaluation). Results The results of the first analysis indicated that there was a difference between the GTQ and the CG for the following variables: total time in days of hospitalization; use of mechanical ventilation (invasive x noninvasive); days of life on the date of the first speech-language assessment; use of alternative feeding route; and the GTQ group had worse results. The results of the multiple logistic regression indicated that the diagnosis of GTQ, the use of invasive mechanical ventilation, and the absence of adequate intraoral pressure during the first speech-language evaluation were risk factors for a negative speech-language outcome. Conclusion The feeding skills of preterm infants with QTG are related to the severity of the condition (gastrointestinal tract complications), requiring longer hospitalization, use of invasive mechanical ventilation, prolonged use of alternative feeding route and requiring more speech therapy to start oral feeding.

RESUMO Objetivo Descrever os achados clínicos e de alimentação de prematuros com gastrosquise (GTQ) em uma unidade de terapia intensiva neonatal comparando-os a recém-nascidos (RNs) prematuros sem anomalias congênitas. Método Foi realizado estudo caso controle de caráter retrospectivo com 50 RNs prematuros (25 com GTQ e 25 sem comorbidades - grupo controle). Os RNs foram comparados quanto aos dados demográficos clínicos: risco de mortalidade e de avaliação fonoaudiológica (sucção não nutritiva e nutritiva). Posteriormente foi aplicado o modelo de regressão logística múltipla a fim de determinar as variáveis associadas ao desfecho fonoaudiológico negativo (alta fonoaudiológica após mais de 7 dias considerando a primeira avaliação fonoaudiológica). Resultados Os resultados da primeira análise indicaram que houve diferença entre o GTQ e o GC para as seguintes variáveis: tempo total em dias de internação; uso de ventilação mecânica (invasiva x não invasiva); dias de vida na data da primeira avaliação fonoaudiológica; uso de via alternativa de alimentação, sendo que o grupo GTQ apresentou piores resultados. Os resultados da regressão logística múltipla indicaram que o diagnóstico de GTQ, uso de ventilação mecânica invasiva e ausência de pressão intraoral adequada durante a primeira avaliação fonoaudiológica foram fatores de risco para o desfecho fonoaudiológico negativo. Conclusão As habilidades de alimentação dos prematuros com GTQ está relacionada à gravidade do quadro (complicações do trato gastro digestivo), exigindo maior tempo de internação, uso de ventilação mecânica invasiva, uso prolongado de via alternativa de alimentação e necessidade de mais atendimento fonoaudiológico para iniciar a alimentação por via oral.

Factors associated with cholestasis in newborns with gastroschisis / Fatores associados à colestase em recém-nascidos com gastrosquise

ABSTRACT Objective: To describe the incidence and to analyze risk factors associated with cholestasis in neonates with gastroschisis. Methods: This is a retrospective cohort study in a tertiary single center analyzing 181 newborns with gastroschisis between 2009 and 2020. The following risk factors associated with cholestasis were analyzed: gestational age, birth weight, type of gastroschisis, silo closure or immediate closure, days of parenteral nutrition, type of lipid emulsion, days of fasting, days to reach a full diet, days with central venous catheter, presence of infections, and outcomes. Results: Among the 176 patients evaluated, 41 (23.3%) evolved with cholestasis. In the univariate analysis, low birth weight (p=0.023), prematurity (p<0.001), lipid emulsion with medium-chain triglycerides and long-chain triglycerides (p=0.001) and death (p<0.001) were associated with cholestasis. In the multivariate analysis, patients who received lipid emulsion with fish oil instead of medium chain triglycerides/long chain triglycerides (MCT/LCT) emulsion had a lower risk of cholestasis. Conclusions: Our study shows that lipid emulsion with fish oil is associated with a lower risk of cholestasis in neonates with gastroschisis. However, this is a retrospective study and a prospective study should be performed to confirm the results.

RESUMO Objetivo: Analisar a incidência e os fatores de risco associados à colestase em recém-nascidos com gastrosquise. Métodos: Estudo de coorte retrospectivo em um único centro terciário, que analisou 181 recém-nascidos com gastrosquise entre 2009 e 2020. Foram examinados os seguintes fatores de risco associados à colestase: idade gestacional, peso ao nascer, tipo de gastrosquise, fechamento com silo ou fechamento imediato, dias de uso nutrição parenteral, tipo de emulsão lipídica, dias de jejum, dias para atingir a dieta completa, dias com cateter venoso central, presença de infecções e desfechos. Resultados: Dos 176 pacientes avaliados, 41 (23,3%) evoluíram com colestase. Baixo peso ao nascer (p=0,023), prematuridade (p<0,001), emulsão lipídica com triglicerídeos de cadeia média e triglicerídeos de cadeia longa (p=0,001) e óbito (p<0,001) foram associados à colestase. Na análise multivariada, os pacientes que receberam emulsão lipídica com óleo de peixe em vez da emulsão diária de triglicérides de cadeia média/triglicérides de cadeia longa (MCT/LCT) apresentaram menor risco de colestase. Conclusões: Nosso estudo mostra que a emulsão lipídica com óleo de peixe está associada a menor risco de colestase em neonatos com gastrosquise, porém este é um estudo retrospectivo, e um estudo prospectivo deve ser realizado para confirmar os resultados.

miRNA-143 expression is associated with inflammation and time of exposure to amniotic fluid in experimental gastroschisis

Abstract Objective Gastroschisis (GS) is a congenital anomaly in the abdominal wall with the intestinal loops exiting laterally to the umbilicus. The contact of the loops with Amniotic Fluid (AF) causes an inflammatory process in the exposed part, leading to an extended hospital stay and an increased risk of morbidity due to alterations related to intestinal motility. The authors aimed to evaluate the time of exposure to the AF in the experimental GS and to search for potential biomarkers of intestinal inflammation by measuring microRNAs. Methods Rat fetuses were divided into three groups: a) CONTROL, b) GS reared on day 18 (GS = 18), and c) GS reared on day 19.5 (GS = 19) (term = 22 days). On day 21.5, the fetuses were removed for biometric parameters and biochemical analyses: 1) Biometrics: Body and Intestinal Weight (BW, IW), and intestinal-body weight ratio (IW/BW); 2) Descriptive histopathology and 3) miR-143 quantification by real-time Polymerase Chain Reaction (PCR). Results BW was higher in CONTROL than GS 18 and G19 (p < 0.05). IW, IW/BW, intestinal water, and mRNA-143 were higher in GS 18 and GS 19 than in CONTROL, and GS 18 was higher than GS 19 (p < 0.05). The average of the inflammation score from the intestinal wall with mucosal inflammation and intra-epithelial lymphocytes shows worst in GS 18 and GS 19 vs. CONTROL (p < 0.05). Conclusions The tissue expression of mRNA-143 and the morphological changes in the intestine of GS worsened according to the time of exposure to AF, which could be a possible marker of fetal intestinal damage.

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

ABSTRACT 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.

Oropharyngeal colostrum administration in neonates with gastroschisis: a randomized clinical trial / Administração orofaríngea de colostro em recém-nascidos com gastrosquise: ensaio clínico randomizado

ABSTRACT Objective: To evaluate the effect of colostrum therapy on days to start a suckling diet in newborns diagnosed with simple gastroschisis. Methods: Randomized clinical trial with newborns diagnosed with simple gastroschisis at a federal hospital in Rio de Janeiro who were randomized to receive oropharyngeal administration of 0.2mL of colostrum or a "sham procedure" during the first 3 days of life. The analysis included clinical outcomes such as days without food, days with parenteral feeding, days until the start of enteral feeding, days to reach complete enteral feeding, sepsis and length of hospital stay. Results: The onset of oral feeding (suction) in patients with simple gastroschisis in both groups occurred at a median of 15 days. Conclusion: The present study showed that there were no significant differences in the use of colostrum therapy and the number of days to the start of enteral feeding and suction diet between groups of newborns with simple gastroschisis.

RESUMO Objetivo: Avaliar o efeito da colostroterapia em dias para iniciar a dieta por sucção em recém-nascidos com diagnóstico de gastrosquise simples. Métodos: Ensaio clínico randomizado com recém-nascidos diagnosticados com gastrosquise simples em um hospital federal no Rio de Janeiro que foram randomizados para receber administração orofaríngea de 0,2mL de colostro ou "procedimento simulado", nos primeiros 3 dias de vida. A análise incluiu desfechos clínicos, como dias sem alimentação, dias com alimentação parenteral, dias para iniciar a alimentação enteral, dias para atingir a alimentação completa, sepse e tempo de internação. Resultados: O início da alimentação por via oral (sucção) na gastrosquise simples, em ambos os grupos, ocorreu com mediana de 15 dias. Conclusão: O presente estudo mostrou que não há diferenças significativas no uso de colostroterapia em dias para início de alimentação enteral e dieta por sucção entre grupos de recém-nascidos com gastrosquise simples.

Resultados materno y perinatal de pacientes con diagnóstico prenatal de gastrosquisis en un centro de Medellín / Maternal and perinatal outcomes of patients with prenatal diagnosis of gastroschisis in a center in Medellin

Objetivo: Describir los resultados maternos y perinatales de pacientes con diagnóstico prenatal de gastrosquisis atendidos en un centro de referencia obstétrica de Medellín. Método: Estudio observacional, descriptivo y retrospectivo, llevado a cabo en la Clínica Universitaria Bolivariana en fetos con diagnóstico prenatal de gastrosquisis desde el 1 de enero de 2010 hasta el 31 de julio de 2021. Resultados: Se identificaron 54 gestantes con diagnóstico prenatal de gastrosquisis. En el 63% era su primer embarazo y el 27,8% eran adolescentes. La duración promedio de la gestación fue de 35 semanas y 6 días. La cesárea fue la vía más común (98,1%) y la indicación más frecuente fue sufrimiento de asa 66,7%. El 55,6% de los neonatos requirieron más de una intervención quirúrgica para el cierre de la pared abdominal. Las complicaciones más frecuentes fueron anemia (66,7%) e íleo posoperatorio (72,2%). La mortalidad fue del 13%. Conclusiones: Se evidencian algunas características similares a las reportadas en otras series. La mayor presentación fue en primer embarazo, la causa de finalización de la gestación fue sufrimiento de asas (demostrando la importancia del seguimiento ecográfico), y las complicaciones más frecuentes fueron anemia e íleo posoperatorio presentados por la prematuridad. La mortalidad comparada con la de otras instituciones locales fue menor.

Objective: To describe the outcomes of maternal and perinatal in patients diagnosed with prenatal gastroschisis that received medical care at an obstetric reference center in Medellin. Method: Observational, descriptive and retrospective study in fetuses with a prenatal diagnosis of gastroschisis performed in the Clínica Universitaria Bolivariana between January 1st 2010 and July 31st 2021. Results: Were included 54 pregnant women with prenatal diagnosis of gastroschisis. The 63% were their first pregnancy and 27,8% were adolescents. The average duration of gestation was 35 weeks and 6 days. Cesarean section was the most common way of delivery (98,1%) and the most frequent indication was suffering from loop (66,7%). The 55,6% of neonates required more than one surgical intervention for closure of the abdominal wall. The most frequent complications were anemia (66,7%) and postoperative ileus (72,2%). A mortality of 13% was presented. Conclusions: Some characteristics like reported in other series are evident. The greatest presentation was in the first pregnancy, the cause of termination of pregnancy was suffering from loops (demonstrating the importance of ultrasound monitoring) and the most frequent complications were anemia and postoperative ileus presented by prematurity. Mortality, compared to other local institutions, was lower.

Pentalogy of cantrell. a stillbirth case report

ABSTRACT Introduction: Pentalogy of Cantrell is a rare congenital disorder characterized by midline birth anomalies. Its embryological origins are related to anomalies of the abdominal wall that prevent the closure of the thorax. Its etiology is not yet clear, but it has been associated with a failure of migration of the lateral plate mesoderm to the midline. Case description: A stillbirth at 25.2 weeks of gestation diagnosed with pentalogy of Cantrell. The mother was a 14-year-old teenager with no relevant history of disease. An obstetric ultrasound was performed at 19 weeks of gestation and revealed findings suggestive of pentalogy of Cantrell. The mother was informed of the potential risks and future complications for the fetus, yet she chose to continue with the pregnancy. At 25.2 weeks of gestation, the mother went to the emergency department due to pain in the hypogastrium accompanied by moderate vaginal bleeding and absence of fetal activity. Once fetal death was confirmed by ultrasound, labor was induced, resulting in stillbirth with anencephaly, thoracic hypoplasia, gastroschisis, and eventration of the liver. Conclusions: Adequate antenatal care, including strict ultrasound follow-up, is essential to detect future complications in the fetus; to provide advice on possible malformations incompatible with life, such as pentalogy of Cantrell; and to determine the best therapeutic approach.

RESUMEN Introducción. La pentalogía de Cantrell es una anomalía congénita rara que se caracteriza por malformaciones en la línea media del cuerpo, y cuyos orígenes embriológicos están relacionados con anormalidades de la pared abdominal que impiden el cierre del tórax. Su etiología aun no es clara, pero se ha asociado con una falla de la migración de los pliegues del mesodermo lateral a la línea media. Presentación del caso. Mortinato de 25.2 semanas de gestación diagnosticado con pentalogía de Cantrell. La madre era una menor de 14 años sin antecedentes patológicos. A las 19 semanas de gestación se realizó ecografía obstétrica que mostró imágenes sugestivas de pentalogía de Cantrell, y se le explicó a la gestante los riesgos y complicaciones futuras que presentaría el feto, pero esta decidió continuar con el embarazo. A las 25.2 semanas de gestación, la joven acudió al servicio de urgencias por dolor en hipogastrio acompañado de sangrado vaginal moderado y ausencia de actividad fetal. Una vez confirmada la muerte fetal mediante ecografía, se indujo el trabajo de parto, obteniéndose mortinato con anencefalia, hipoplasia de caja torácica, gastrosquisis y eventración del hígado. Conclusiones. La realización de adecuados controles prenatales, en los cuales se realice un seguimiento ecográfico estricto, es fundamental para detectar futuras complicaciones en el feto; brindar asesoría sobre posibles malformaciones que sean incompatibles con la vida, como la pentalogía de Cantrell, y establecer las mejores alternativas de manejo.

The complex search for the cause of gastroschisis.

BACKGROUND: To reveal the complex etiology of gastroschisis through two independent cases. CASES: Case 1 involves gastroschisis recurrence in a consanguineous marriage, and Case 2 concerns a fetus with gastroschisis whose mother had undergone gastroplasty. Methylation array was carried out in both cases (two fetuses with gastroschisis, their two mothers, one father from the consanguineous marriage), and in 16 controls (fetuses and their respective mothers). CONCLUSION: The two cases presented different noninherited methylation profiles.