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.

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.

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.

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.

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.

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.

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.

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.

Gastroschisis and late-onset neonatal sepsis in a tertiary referral center in Southeastern Brazil

Abstract Objectives: To analyze late-onset sepsis and to describe the etiological agents in newborns with gastroschisis. Methods: A retrospective cohort, including newborns with gastroschisis whose admissions occurred in the period between January 2012 to December 2018 in a tertiary referral center. Maternal and newborn characteristics, surgical procedures and evolution in hospitalization were verified. A bivariate analysis was performed with patients with proven late-onset neonatal sepsis and according to the simple or complex gastroschisis category, the prevalent microorganisms in positive cultures were identified, statistical tests were carried out and the significance level adopted was p < 0,05. Results are presented in proportions, averages and standard deviation or medians. The level of significance adopted was p < 0.05. Results: 101 newborns were analyzed, 45 (44.5%) were confirmed late-onset sepsis. The median birth weight was 2285+498 grams, and the gestational age was 35.9 +1.74weeks. The incidence of complex gastroschisis was 17.8%, the hospitalization time was 48.2+29.67 days and mortality was 9.9%. The newborns were divided into 2 groups: Group 1: late-onset sepsis (44.6%), and Group 2: no late-onset sepsis. The presence of complex gastroschisis was a factor associated with infection (p < 0.009). Fasting time (p < 0.001), parenteral nutrition time (p < 0.001), time to achieve full diet (p < 0.001), and hospitalization stay (p < 0.001) were higher in group 2. Gram-positive were the most frequent (51.1%), followed by Gram-negative (20%), and fungi (4.4%). Conclusions: Newborns with gastroschisis have a higher risk of evolving with late-onset sepsis, despite this study did not calculate the risk of sepsis statistically, and the main germs detected by cultures were gram-positive bacteria, specifically Staphylococcus epidermidis.

Gastroschisis annual incidence, mortality, and trends in extreme Southern Brazil

Abstract Objective: To analyze gastroschisis annual incidence, mortality rates, and trends in the Brazilian state of Rio Grande do Sul from the year 2000 to the year 2017. Method: Population-based study with the analysis of the temporal trend of gastroschisis annual incidence and mortality rates. Data were obtained from the Live Birth Information System and the Mortality Information System, with the analysis performed by polynomial regression modeling. Results: There were 2,612,532 live births, 705 hospitalizations, and 233 deaths due to gastroschisis. The annual incidence of gastroschisis was 2.69 per 10,000 live births. The annual incidence rate increased by 85% in the total period (p = 0.003), and mortality was 33% in the 2000-2017 period. Maternal age < 25 years was a risk factor for gastroschisis (p < 0.001). Children were more likely to be born weighing < 2,500 g (p < 0.001) and with a gestational age < 37 weeks (p < 0.001). The annual incidence trend was to increase, and the mortality trend was to decrease. Conclusion: Similar to what has been described in several regions/countries, there was a trend showing an 85% increase in the annual incidence of gastroschisis (p = 0.003) and the mortality was 33% with a trend of decreasing (p = 0.002).

Short and Medium-term Outcomes of Omphalocele and Gastroschisis: A Survey from a Tertiary Center / Resultados a curto e médio prazo do onfalocelo e gastrosquisis: uma investigação de um centro terciário

Abstract Objective To characterize and compare the outcomes of omphalocele and gastroschisis from birth to 2 years of follow-up in a recent cohort at a tertiary center. Methods This is a retrospective clinical record review of all patients with gastroschisis and omphalocele admitted to the Neonatal Intensive Care Unit between January 2009 and December 2019. Results There were 38 patients, 13 of whom had omphalocele, and 25 of whom had gastroschisis. Associated anomalies were present in 6 patients (46.2%) with omphalocele and in 10 (41.7%) patients with gastroschisis. Compared with patients with omphalocele, those with gastroschisis had younger mothers (24.7 versus 29.6 years; p=0.033), were born earlier (36 versus 37 weeks, p=0.006), had lower birth weight (2365±430.4 versus 2944.2±571.9 g; p=0.001), and had a longer hospital stay (24 versus 9 days, p=0.001). The neonatal survival rate was 92.3% for omphalocele and 91.7% for gastroschisis. Thirty-four patients were followed-up over a median of 24 months; 13 patients with gastroschisis (59.1%) and 8 patients with omphalocele (66.7%) had at least one adverse event, mainly umbilical hernia (27.3% vs 41.7%), intestinal obstruction (31.8% vs 8.3%), or additional surgical interventions (27.3% vs 33.3%). Conclusion Despite the high proportion of prematurity, low birth weight, and protracted recovery, gastroschisis and omphalocele (without chromosomal abnormalities) may achieve very high survival rates; on the other hand, complications may develop in the first years of life. Thus, a very positive perspective in terms of survival should be transmitted to future parents, but they should also be informed that substantial morbidity may occur in the medium term.

Resumo Objetivo Caracterizar e comparar os desfechos do onfalocelo e gastrosquisis desde o nascimento até aos 2 anos de seguimento numa coorte recente de um centro terciário. Métodos Este é um estudo retrospectivo em que foi feita uma revisão dos registos clínicos de todos os pacientes com gastrosquisis e onfalocelo que foram internados na unidade de cuidados intensivos neonatais, entre janeiro de 2009 e dezembro de 2019. Resultados Identificamos 38 pacientes, 13 dos quais tinham onfalocelo e 25 dos quais tinham gastrosquisis. Anomalias associadas estavam presentes em 6 pacientes (46.2%) com onfalocelo e 10 (41.7%) com gastrosquisis. Comparativamente com os pacientes com onfalocelo, os pacientes com gastrosquisis tinham mães mais jovens (24.7 versus 29.6 anos; p=0.033), nasceram mais precocemente (36 versus 37 semanas, p=0.006), com menor peso ao nascimento (2,365±430.4 versus 2,944.2±571.9 g; p=0.001), e o internamento teve uma duração mais longa (24 versus 9 dias, p=0.001). A taxa de sobrevivência neonatal foi de 92.3% para o onfalocelo e 91.7% para a gastrosquisis. Trinta e quatro pacientes foram seguidos durante umtempo mediano de seguimento de 24meses: 13 com gastrosquisis (59.1%) e 8 com onfalocelo (66.7%) apresentaram pelo menos um evento adverso, sobretudo hérnia umbilical (27.3% vs 41.7%), obstrução intestinal (31.8% vs 8.3%) ou intervenções cirúrgicas adicionais (27.3% vs 33.3%). Conclusão Apesar da alta proporção de prematuridade, de baixo peso e de recuperação lenta, os gastrosquisis, assim como os onfalocelos (sem anomalias cromossómicas), podem ter uma taxa de sobrevivência muito alta; por outro lado, nos primeiros anos de vida, podem surgir complicações não desprezíveis. Assim, aos futuros pais pode ser transmitida uma perspectiva muito positiva em termos de sobrevivência, embora eles também devam ser informados de que pode ocorrermorbidade substancial no médio prazo.

Gastroschisis in Brazil within a Global Context / Gastrosquise no Brasil em um Contexto Global

Introduction: global disparity in outcomes of gastroschisis is visible. Survival rates in high-income countries have improved since 1960, and are currently around 100%, due to progress in pediatric surgery and neonatal intensive care. However, in low- and middle-income countries mortality rates can reach all cases. Objective: this study aims to map the existing literature on gastroschisis in Brazil within a global context.Methods: scoping Review. PubMed, Scielo Brazil, Biblioteca Virtual em Saúde (BVS) and Google Scholar, were searched from January 2000 to May 2020.Results: eight studies met the inclusion criteria. The consolidated data of 912 patients were: Mean maternal age of 20.7 years, antenatal diagnosis rate of 80.2%, cesarean section rate of 77.7%. Mean of hospital stay of 40.8 days. Mean mortality rate of 25.3 %. Risk factors associated with death were: prematurity, low birth weight, low APGAR score, reinterventions, sepsis, birth-to-surgery interval greater than 4 hours, fewer prenatal visits, delayed prenatal diagnosis.Conclusion: studies on gastroschisis in Brazil were scarce, most of carried out in southeast of country. This study suggests that there are regional contrasts on gastroschisis outcomes in Brazil. The worst results are in regions with low economic resources, a similar situation found in international literature. This review should be validated with future studies to investigate the situation of pregnant women with fetuses with gastroschisis, especially in low-resource regions, in Brazil.

Introdução: a disparidade global nos resultados da gastrosquise é visível. Taxas de sobrevivência em países de alta renda melhoraram desde 1960 e atualmente estão em torno de 100%, devido ao progresso da cirurgia pediátrica e da terapia intensiva neonatal. No entanto, em países de media e baixa renda, as taxas de mortalidade continual elevadas. Objetivo: este estudo tem como objetivo mapear a literatura existente sobre gastrosquise no Brasil e discutir as evidências disponíveis em um contexto global.Método: revisão de escopo. Foram pesquisados o PubMed, o Scielo Brasil, a Biblioteca Virtual em Saúde (BVS) e o Google Scholar no período de janeiro de 2000 a maio de 2020.Resultados: oito estudos preencheram os critérios de inclusão e os dados encontrados de 912 pacientes foram: idade materna média de 20.7 anos, média do diagnóstico antenatal de 80.2%, taxa média de cesarianas de 77.7%, tempo de permanência hospitalar médio de 40.8 dias e taxa média de mortalidade 25.3%. Os fatores de risco associados ao óbito encontrados foram a prematuridade, baixo peso ao nascer, APGAR baixo, reintervenções cirúrgicas, sepse, intervalo nascimento-cirurgia maior que 4 horas, poucas consultas do pré-natal e diagnóstico antenatal tardio.Conclusão: estudos sobre gastrosquise no Brasil são escassos, a maioria realizada no Sudeste do país. Os piores desfechos estão em regiões com poucos recursos econômicos, situação semelhante à encontrada na literatura internacional. Esta revisão deve ser validada com estudos futuros que investiguem a situação de gestantes portadoras de fetos com gastrosquise, especialmente em regiões de poucos recursos, no Brasil.

Surgical repair of gastroschisis complicated by tracheostenosis in a very premature infant / 中华围产医学杂志

We describe the diagnosis and treatment of a very premature female infant with gastroschisis complicated by tracheostenosis. The pregnant woman, whose fetus was diagnosed with gastroschisis by ultrasound at 22 weeks in a local hospital, was admitted to the Second Affiliated Hospital of Wenzhou Medical University at 28 +1 weeks with oligohydramnios. Ultrasound after admission confirmed the previous diagnosis. A live baby girl was born by vaginal breech delivery at 29 +1 weeks after spontaneous rupture of the membranes. Because of the unstable oxygen saturation, the neonate finally received Silo in the delivery room prior to the closure of abdominal fissure 7 d after birth, and during the placement difficult endotracheal intubation was evident. She was diagnosed with having congenital tracheal stenosis via chest CT scans with 3-dimensional reconstruction 3 weeks after birth and received transbronchoscopic balloon dilatation at 3 months after birth. During the 2-year follow-up, she grew well without any complications.

Influence of birthplace on gastroschisis outcomes in a state in the southeastern region of Brazil

Abstract Objective: To characterize the influence of birthplace on outcomes of patients with gastroschisis admitted to three hospitals in a state in Brazil's southeastern region, according to condition inborn (born in a reference center) or outborn (born outside the reference center). Methods: Retrospective multicenter cohort study of patients with gastroschisis. The sample size utilized was of patients admitted in three hospitals with a diagnosis of gastroschisis ICD 10 Q79.3 between January 2000 to December 2018. Patients were divided into two groups, inborn and outborn. Characteristics of prenatal, perinatal and postoperative were compared using statistical tests. The level of significance adopted was P-value < 0.05. Results: In total, 144 cases of gastroschisis were investigated. The outborn patients group had higher rates of absence of antenatal diagnosis (p = 0.001), vaginal delivery (p = 0.001), longer time between birth and abdominal wall closure surgery (p = 0.001), to silo removal (p = 0.001), to first enteral feeding (p = 0.008), for weaning from mechanical ventilation (p = 0.034), used less peripherally inserted central catheter (PICC) and required more venous dissections (p = 0.001), and lower mean of serum sodium (p = 0.015). There were no differences in mortality rates and length of hospital stay between the inborn and outborn groups. Conclusion: Although outborn patients with gastroschisis were less likely to have an antenatal diagnosis and were more prone to a longer time to undergo surgical and feeding procedures, and to spend more time in mechanical ventilation, these disadvantages seemed not to reflect on the death rate and the length of hospital stay of patients from this group.

Análise do crescimento de recém-nascidos pré-termo com gastrosquise durante a Internação em Unidade de Terapia Intensiva Neonatal / Growth analysis of preterm newborns with gastroschisis during hospitalization in a neonatal intensive care unit

ABSTRACT BACKGROUND: Gastroschisis, especially complex type, prematurity and low birth weight are associated with a worse clinical outcome with higher mortality, higher incidence of sepsis and catheter-related infection, cholestasis, short bowel syndrome, greater number of days to achieve full diet, longer time of parenteral nutrition and longer hospitalization time. OBJECTIVE: To evaluate the growth of preterm newborns with gastroschisis during their hospitalization in the neonatal intensive care unit. METHODS: Descriptive study, based on a retrospective cohort (January 2012 to December 2018), including preterm newborns (gestational age less than 37 weeks) with simple and complex gastroschisis admitted in a tertiary neonatal intensive care unit. The following parameters were analyzed: maternal age, parity, type of delivery, birth weight, gender, gestational age, nutritional adequacy, type of gastroschisis, fasting time, parenteral nutrition time, time until achieving full enteral nutrition, hospitalization time, weight gain and outcome. The results were expressed in percentage, average, and median. RESULTS: A total of 101 newborns with gastroschisis were admitted, of which 59.4% were premature (80.7% of late preterm infants). From the maternal data, the mean age was 21.2 years and 68.3% were primiparous. Regarding childbirth: 80% were cesarean sections. From newborns: the average birth weight was 2137 g, 56.6% were female, the average gestational age was 34.8 weeks, the average weight gain was 20.8 g/day during hospitalization and 83.3% were discharged from the hospital. CONCLUSION: The growth analysis by weight gain (grams/day) during hospitalization in the intensive care unit showed that more than 90% of the sample presented acceptable or adequate weight gain.

RESUMO CONTEXTO: Gastrosquise, principalmente o tipo complexo, prematuridade e baixo peso ao nascer estão associados a um pior desfecho clínico com maior mortalidade, maior incidência de sepse e infecção relacionada ao cateter, colestase, síndrome do intestino curto, maior número de dias para atingir dieta plena, maior tempo de nutrição parenteral e maior tempo de internação. OBJETIVO: Avaliar o crescimento de recém-nascidos pré-termo com gastrosquise durante sua internação na unidade de terapia intensiva neonatal. MÉTODOS: Estudo descritivo, baseado em uma coorte retrospectiva (janeiro de 2012 a dezembro de 2018), incluindo recém-nascidos pré-termo (idade gestacional inferior a 37 semanas) com gastrosquise simples e complexa, admitidos em uma unidade de terapia intensiva neonatal de nível terciário. Foram analisados: idade materna, paridade, tipo de parto, peso de nascimento, gênero, idade gestacional, adequação nutricional, tipo de gastrosquise, tempos de jejum, de nutrição parenteral, até atingir nutrição enteral plena e de internação, ganho de peso e desfecho. Os resultados estão expressos em porcentagem, médias e medianas. RESULTADOS: Foram admitidos 101 recém-nascidos com gastrosquise, dos quais 59,4% eram prematuros (80,7% prematuros tardios). Dos dados maternos, a idade média foi de 21,2 anos e 68,3% eram primigestas. Com relação ao parto: 80% foram cesarianas. Dos recém-nascidos: o peso médio de nascimento foi de 2137 g, 56,6% eram do sexo feminino, a idade gestacional média foi de 34,8 semanas, o ganho médio de peso de 20,8 g/dia durante a internação e 83,3% receberam alta hospitalar. CONCLUSÃO: A análise do crescimento por meio de ganho de peso (gramas/dia) durante a internação na unidade de terapia intensiva mostrou que mais de 90% da amostra apresentou ganho aceitável ou adequado de peso.

Gastrosquisis / Gastroschisis

Introducción: La gastrosquisis es un defecto de la pared abdominal anterior, paraumbilical, habitualmente a la derecha con la protrusión de vísceras intraabdominales, las que flotan libremente en el líquido amniótico durante la vida intrauterina, solo recubiertas por su peritoneo visceral. Objetivo: Analizar la entidad a propósito de un caso portador de gastrosquisis compleja. . Caso clínico: Se describe una neonato de 4 días remitido al servicio de urgencias del Hospital Provincial de Cabinda, República de Angola, por presentar deshidratación severa y "evisceración". La paciente fallece 36 horas posteriores al ingreso. Conclusiones: Los neonatos con esta enfermedad se presentan como una emergencia quirúrgica que plantean un reto difícil para el cirujano tratante. Tiene una alta mortalidad aun con el tratamiento apropiado. Técnicas novedosas de tratamiento se acercan a las tasas de sobrevida esperadas para esta entidad(AU)

Introduction: Gastroschisis is a defect of the paraumbilical anterior abdominal wall, usually on the right with protrusion of intraabdominal viscera, which float freely in the amniotic fluid during intrauterine life, only covered by its visceral peritoneum. Objective: To analyze the entity apropos a case of complex gastroschisis. Clinical case: The case is described od a four-day-old neonate referred to the emergency service at Provincial Hospital of Cabinda, Republic of Angola, for presenting severe dehydration and "evisceration." The patient died 36 hours after admission. Conclusions: Neonates with this disease are presented as a surgical emergency that poses a difficult challenge for the treating surgeon. It has a high mortality even with the appropriate treatment. Novel treatment techniques are close to the expected survival rates for this entity(AU)

New clues to understand gastroschisis. Embryology, pathogenesis and epidemiology / Nuevas pistas para comprender la gastrosquisis. Embriología, patogénesis y epidemiología

Abstract gastroschisis is a congenital structural defect of the abdominal wall, most often to the right of the umbilicus, through which the abdominal viscera protrude. Its developmental, etiological and epidemiological aspects have been a hot topic of controversy for a long time. However, recent findings suggest the involving of genetic and chromosomal alterations and the existence of a stress-inducing pathogenetic pathway, in which risk factors such as demographic and environmental ones can converge. . We have conducted a review of the medical literature that gathers information on the embryonic development of the ventral body wall, the primitive intestine, and the ring-umbilical cord complex, as well as on the theories about its origin, pathogenesis and recent epidemiological evidence, for which we consulted bibliographic databases and standard search engines

Resumen La gastrosquisis es un defecto estructural congénito de la pared abdominal, localizado con mayor frecuencia a la derecha del ombligo, a través del cual sobresalen las vísceras abdominales. Durante mucho tiempo, sus aspectos evolutivos, etiológicos y epidemiológicos han sido un tema candente de controversia, aunque hallazgos recientes sugieren la participación de alteraciones genéticas, cromosómicas, y la existencia de una vía patogénica inductora de estrés, en la que factores de riesgo como los demográficos y ambientales pueden converger. Con el objetivo de ampliar la frontera del conocimiento sobre una malformación que ha mostrado una creciente prevalencia global, hemos efectuado una revisión que incluye información, del desarrollo embrionario de la pared corporal ventral, el intestino primitivo, el complejo anillo-cordón umbilical, y de las teorías acerca de su origen, patogénesis e información epidemiológica reciente.

Motor performance evaluation of newborns with gastroschisis after surgical correction / Avaliação do desempenho motor de recém-nascidos com gastrosquise após correção cirúrgica

INTRODUCTION: Gastroschisis is a congenital malformation that has risk factors for delayed neuropsychomotor development. That is why it is important to recognize early developmental changes in these newborns during hospitalization in the Neonatal Intensive Care UnitOBJECTIVE: To evaluate the motor performance of newborns with gastroschisis after surgical correctionMETHODS: An observational and retrospective study was carried out with newborns with gastroschisis, assessed by the Test of Infant Motor Performance at the Neonatal Intensive Care Center 2 of the Instituto da Criança e do Adolescente. The test allows the classification of the risk for developmental delay in 4 categories: within the average for age, low average, below average, and well below average. Maternal, neonatal, surgical, and motor assessment data were collected through electronic medical recordsRESULTS: Motor assessment was performed on 17 newborns, where 88.23% were classified as "below average" for age. The mean maternal age was 20 years, and the average gestational age and birth weight were 36.38 weeks and 2343.9 grams, respectively, with the majority being female. Simple gastroschisis accounted for 64.71%, and primary closure was possible in 82.35%. The average hospital stay of 53.24 days, and sepsis was the most frequent complication (64.71%CONCLUSION: Newborns with gastroschisis remain hospitalized for a long time and are susceptible to complications. It was possible to identify the delay in the development of these newborns early during hospitalization, which allows intervention by physiotherapy before the delay worsens

INTRODUÇÃO: A gastrosquise é uma malformação congênita que possui fatores de risco para o atraso no desenvolvimento neuropsicomotor. Por isso é importante o reconhecimento precoce das alterações do desenvolvimento nesses recém-nascidos durante a internação na Unidade de Terapia Intensiva NeonatalOBJETIVO: Avaliar o desempenho motor de recém-nascidos com gastrosquise após correção cirúrgicaMÉTODO: Foi realizado um estudo observacional e retrospectivo com recém-nascidos com gastrosquise, avaliados pelo Test of Infant Motor Performance no Centro de Tratamento Intensivo Neonatal 2 do Instituto da Criança e do Adolescente. O teste permite a classificação do risco para o atraso do desenvolvimento em 4 categorias: dentro da média para idade, média baixa, abaixo da média e muito abaixo da média. Foram coletados dados maternos, neonatais, cirúrgicos e da avaliação motora, através de prontuários eletrônicosRESULTADOS: A avaliação motora foi realizada em 17 recém-nascidos, onde 88,23% foram classificados como "abaixo da média" para idade. A idade média materna foi de 20 anos e a média de idade gestacional e peso ao nascimento de 36,38 semanas e 2343,9 gramas, respectivamente, sendo a maioria do sexo feminino. As gastrosquises simples representaram 64,71% e o fechamento primário foi possível em 82,35%. A média de permanência hospitalar de 53,24 dias e a sepse foi a complicação mais frequente (64,71%CONCLUSÃO: Os recém-nascidos com gastrosquise permanecem internados por tempo prolongado e estão suscetíveis a complicações. Foi possível a identificação precoce do atraso no desenvolvimento desses recém-nascidos durante a internação

Experiencia inicial del cierre sin sutura de la gastrosquisis. Reporte de casos en un hospital de la ciudad de Puebla, México / Initial experience of the sutureless closure of gastroschisis. Report of cases in a hospital in the city of Puebla, México

Resumen Introducción: La gastrosquisis es una de las malformaciones congénitas más frecuentes. En países subdesarrollados su mortalidad es mayor al 15% siendo la sepsis su principal causa. Su manejo implica el cierre de la pared abdominal mediante diferentes métodos, dependiendo del nivel de complejidad y del tipo de intervención. Objetivo: Describir la experiencia inicial del cierre sin sutura de la gastrosquisis, en una unidad de neonatología de Puebla, México. Materiales y métodos: Se realizó un estudio observacional, descriptivo, transversal y retrospectivo; en el cual se estudiaron 9 expedientes de recién nacidos con cierre sin sutura de gastrosquisis, en el Hospital General del Sur de Puebla. Resultados: Se estudiaron a los pacientes de Diciembre 2018 a Agosto 2019, de los cuales el 56% correspondía al sexo masculino. Se dividieron en 3 grupos: Cierre inmediatamente posterior a la cesárea (67%), retardo inmediato (11%), y retardo con uso del separador Alexis (22%). De los pacientes, el promedio fue de 25.67 + 1.56 días de estancia intrahospitalaria. Únicamente un paciente del grupo 2 presentó como complicación sepsis, el resto no manifestó complicación alguna. Conclusiones: El cierre sin sutura en nuestros pacientes resultó ser un procedimiento confiable y con buen pronóstico para su evolución, debido a resultados favorables en los días de estancia intrahospitalaria y el tiempo de inicio de la vía oral. MÉD.UIS.2020;33(3): 43-7.

Abstract Introducción: La gastrosquisis es una de las malformaciones congénitas más frecuentes. En países subdesarrollados su mortalidad es mayor al 15% siendo la sepsis su principal causa. Su manejo implica el cierre de la pared abdominal mediante diferentes métodos, dependiendo del nivel de complejidad y del tipo de intervención. Objetivo: Describir la experiencia inicial del cierre sin sutura de la gastrosquisis, en una unidad de neonatología de Puebla, México. Materiales y métodos: Se realizó un estudio observacional, descriptivo, transversal y retrospectivo; en el cual se estudiaron 9 expedientes de recién nacidos con cierre sin sutura de gastrosquisis, en el Hospital General del Sur de Puebla. Resultados: Se estudiaron a los pacientes de Diciembre 2018 a Agosto 2019, de los cuales el 56% correspondía al sexo masculino. Se dividieron en 3 grupos: Cierre inmediatamente posterior a la cesárea (67%), retardo inmediato (11%), y retardo con uso del separador Alexis (22%). De los pacientes, el promedio fue de 25.67 + 1.56 días de estancia intrahospitalaria. Únicamente un paciente del grupo 2 presentó como complicación sepsis, el resto no manifestó complicación alguna. Conclusiones: El cierre sin sutura en nuestros pacientes resultó ser un procedimiento confiable y con buen pronóstico para su evolución, debido a resultados favorables en los días de estancia intrahospitalaria y el tiempo de inicio de la vía oral. MÉD.UIS.2020;33(3): 43-7.

Gastrosquisis en Hospital IESS "Manuel Ygnacio Monteros". Presentación de caso clínico y revisión bibliográfica / Gastroschisis in Hospital del IESS "Manuel Ygnacio Ygnacio". Case Report and Literature Reviview.

INTRODUCCIÓN: La gastrosquisis es una anomalía que expone los órganos abdominales hacia el exterior. La prevalencia media global es de 1.36 por cada 10 000 nacidos vivos. En el Ecuador, según el Instituto Nacional de Estadísticas y Censos (INEC), se evidencia un aumento en el número de casos de egreso hospitalario alcanzando los 52 pacientes en el año 2010, los 85 en el 2012 y los 112 pacientes en el 2015. El objetivo del presente caso es realizar una descripción de esta patología y dar a conocer la importancia que tiene su diagnóstico prenatal con el fin de planificar su resolución quirúrgica para la atención inmediata al recién nacido luego del nacimiento. CASO CLÍNICO: recién nacido de madre primigesta de 16 años, sin más antecedentes prenatales, se realizó cuatro ultrasonidos durante el embarazo en casa de salud particular, los cuales no reportaron alteraciones. A las 37.4 semanas nace por parto eutócico, evidenciándose gastrosquisis. Se realizó laparotomía más peritoneostomía a las 24 horas de vida, y se programó cierre diferido. EVOLUCIÓN: recibió manejo integral en cuidados intensivos neonatales. Se realizó cierre definitivo a los 8 días. Permaneció hospitalizado por 22 días, recibió nutrición parenteral y se inició alimentación trófica a los 15 días de vida; se inició antibioticoterapia de primera línea, sin embargo por evolución tórpida, requirió rotación a antibiótico de amplio espectro. CONCLUSIÓN: la gastrosquisis se puede ser detectada por ecografía desde la semana doce de gestación con el objetivo de minimizar las complicaciones y planificar su resolución quirúrgica inmediatamente luego de su nacimiento.(au)

BACKGROUND: Gastroschisis is an anomaly in which abdominal organs are exposed outside the abdominal wall. The global average prevalence is 1.36 for each 10 000 newborns. In Ecuador, according to the National Institute of Statistics and Censuses (INEC), there is evidence of an increase in the number of hospital discharges with this diagnosis, reaching 52 in 2010, 85 in 2012, and 112 patients in 2015. The aim of this publication is to describe this pathology and expose the importance of prenatal diagnosis to plan prompt surgical intervention for the newborn after birth. CASE REPORT: newborn from a 16-year-old primigravid mother, with no other prenatal history, four ultrasounds were performed during pregnancy in a private health facility, which reported no alterations. At 37.4 weeks of pregnancy, the newborn was delivered by eutocic birth, showing gastroschisis. The patient underwent laparotomy and peritoneostomy at 24 hours of age, delayed closure was scheduled. EVOLUTION: the newborn received comprehensive care in the neonatal intensive care unit. Delayed closure was performed eight days later. He remained hospitalized for 22 days, he received parenteral nutrition and started trophic feeding at 15 days of age; first-line antibiotic therapy was initiated, however due to torpid evolution, he required a change to a broad-spectrum antibiotic. CONCLUSION: gastroschisis can be detected in ultrasound from the twelve week of pregnancy to minimize complications and program the surgical procedure immediately after birth.(au)