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).

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.

Gastrosquisis: análisis de situación entre los años 2011 y 2016 en el Hospital Pediátrico Pereira Rosell / Gastroschisis: study of the situation in the period between 2011-2016 at the Pereira Rossell Pediatric Hospital / Gastrosquise: análise da situação entre 2011 e 2016 no Hospital Pediátrico Pereira Rosell

La gastrosquisis es el defecto congénito más frecuente de la pared abdominal anterior. Se clasifican en simple o complicadas según si presentan o no anomalías a nivel intestinal. El diagnóstico prenatal se realiza en un 75%-95% de los pacientes. El tratamiento es quirúrgico, lográndose un cierre primario en el 80% de los casos. La mortalidad global reportada a nivel internacional es de un 10%. Se realizó un estudio observacional descriptivo retrospectivo transversal en el que se analizaron los pacientes con gastrosquisis operados en el Hospital Pediátrico del Centro Hospitalario Pereira Rossell entre el primero de enero de 2011 y el 30 de mayo de 2016. Se estudiaron las siguientes variables: incidencia de la patología, edad materna, edad gestacional al diagnóstico y al nacimiento, vía de nacimiento, anomalías asociadas, técnica quirúrgica, incidencia de complicaciones y mortalidad. En el período estudiado se intervinieron 62 pacientes con diagnóstico de gastrosquisis, de los cuales 48 fetos (77,4%) tenían diagnóstico prenatal. El 53,2% nacieron por parto vaginal y 53 % nacieron pretérmino. En el 85,5% se logró realizar un cierre primario. El 69,4% de los neonatos presentaron complicaciones, siendo las infecciosas las más frecuentes. La mortalidad fue de 12,9%, siendo la sepsis la causa más frecuente. Si bien hemos mejorado en el índice de diagnóstico prenatal y en la vía de nacimiento, y las cifras de tratamiento y mortalidad se encuentran dentro de las cifras publicadas a nivel internacional, existe un elevado número de complicaciones infecciosas, que condicionan la evolución y pronóstico de estos pacientes.

Gastroschisis is the most frequent congenital defect of the anterior abdominal wall. They are classified as simple or complicated according to whether or not they present abnormalities at the intestinal level. Prenatal diagnosis is made in 75%-95% of the patients. The treatment is surgical, achieving a primary closure in 80% of cases. The global mortality reported at an international level is 10%. A cross-sectional retrospective observational study was performed in which gastroschisis patients operated at the Pediatric Hospital of the Pereira Rossell Hospital Center between January 1, 2011 and May 30, 2016 were analyzed. The following variables were studied: the pathology, maternal age, gestational age at diagnosis and at birth, birth route, associated anomalies, surgical technique, incidence of complications and mortality. In the period under study, 62 patients diagnosed with gastroschisis were operated on, of which 48 fetuses (77.4%) had a prenatal diagnosis. 53.2% were born by vaginal delivery and 53% were born preterm. In 85.5% a primary closure was achieved. 69.4% of the neonates presented complications, infectious being the most frequent. Mortality was 12.9%, with sepsis being the most frequent cause. Although we have improved in the prenatal diagnosis index and in the route of birth, and the figures of treatment and mortality are among the figures published internationally, there is a high number of infectious complications, which condition the evolution and prognosis of these patients.

A gastrosquise é o defeito congênito mais freqüente da parede abdominal anterior. São classificadas como simples ou complicadas de acordo com a presença ou não de anormalidades no nível intestinal. O diagnóstico pré-natal é feito em 75% a 95% dos pacientes. O tratamento é cirúrgico, atingindo o fechamento primário em 80% dos casos. A mortalidade global reportada a nível internacional é de 10%. Um estudo observacional transversal foi realizado em gastrosquise retrospectiva que os pacientes operados no Hospital Pediátrico Pereira Rossell Hospital do Centro entre 1 de Janeiro de 2011 e 30 maio de 2016 foram analisados. As variáveis estudadas foram: a patologia, idade materna, idade gestacional no diagnóstico e no nascimento, rota nascimento, anomalias associadas, técnica cirúrgica, a incidência de complicações e mortalidade. No período em estudo, 62 pacientes diagnosticados com gastrosquise foram operados, dos quais 48 fetos (77,4%) tiveram o diagnóstico pré-natal. 53,2% nasceram por parto vaginal e 53% nasceram prematuros. Em 85,5%, um fechamento primário foi alcançado. 69,4% dos neonatos com o ser infeccioso mais freqüente. A mortalidade foi de 12,9%, sendo a sepse a causa mais freqüente. Temos melhorado, embora diagnóstico pré-natal no índice e na rota de nascimento, e os números de tratamento e mortalidade estão entre os números publicados Internacionalmente, há um elevado número de complicações infecciosas, que condicionam a evolução eo prognóstico desses pacientes.

Análise dos casos de gastrosquise no estado do Rio de Janeiro / Analysis of gastroschisis cases in the state of Rio de Janeiro

Essa tese teve como objetivo principal analisar os processos de cuidado e desfechos de recém-nascidos com gastrosquise no estado do Rio de Janeiro. Método: É apresentada em formato de coletânea de artigo, o primeiro artigo foi uma coorte de nascidos vivos construída a partir da linkagem probabilística do SINASC e do SIM em 10 anos (2005 a 2014). Após construção da coorte foram incluídas variáveis de caracterização do local de nascimento a partir do número do CNES do nascimento, com o objetivo de observar, além das características dos RN com gastrosquise, as características do local de nascimento. O segundo artigo trata-se de um estudo observacional tipo série de casos de RN internados com gastrosquise em um centro terciário no Rio de Janeiro no período de 5 anos (2013 a 2017). Resultados: No primeiro artigo destaca-se que os prematuros e os RN com baixo peso ao nascer tiveram maior chance de óbito, com significância estatística, valor de p=0,03 e 0,006, respectivamente. Em relação ao local de nascimento, o nascimento em uma unidade hospitalar em específico foi fator de proteção quando comparado ao nascimento ocorrido nas demais unidades. Além disso, nascer em unidades de UTIN tipo II aumentou o risco de óbito em 3,9 vezes. No segundo artigo observou-se que o tempo de internação não foi diferente entre os grupos dos expostos (nascidos fora do centro terciário) e não expostos (nascidos no centro terciário), no entanto a chance de morrer foi maior no grupo dos expostos. Conclusão: Esse estudo dá subsídios para a discussão de duas possíveis estratégias no tratamento à RN com gastrosquise. A primeira seria a centralização do nascimento e cuidado aos RN com gastrosquise em unidades terciárias. E a segunda seria a elaboração de diretrizes clínicas que padronizem o cuidado imediato e transporte dos RN com gastrosquise nascidos fora de centros terciários.

This thesis had as main objective to analyze the processes of care and outcomes of newborns with gastroschisis in the state of Rio de Janeiro. Method: The first article was a live birth cohort constructed from the probabilistic linkage of SINASC and SIM in 10 years (2005 to 2014). After the cohort was constructed, variables characterizing the place of birth were included based on the CNES number of the birth, in order to observe, besides the characteristics of the newborns with gastroschisis, the characteristics of the place of birth. The second article is a series-type observational study of infants hospitalized with gastroschisis at a tertiary center in Rio de Janeiro over a five-year period (2013 to 2017). Results: The first article shows that premature infants with low birth weight had a higher chance of death, with a statistical significance of p = 0.03 and 0.006, respectively. Regarding the place of birth, the birth in a specific hospital unit was a protection factor when compared to the birth occurred in the other units. In addition, being born in NICU type II units increased the risk of death by 3.9 times. In the second article, it was observed that the length of hospitalization was not different between the exposed and non-exposed (born in the tertiary center) groups; however, the chance of dying was higher in the exposed group. Conclusion: This study gives subsidies for the discussion of two possible strategies in the treatment of newborns with gastroschisis. The first would be centralization of birth and care for newborns with gastroschisis in tertiary units. And the second would be the elaboration of clinical guidelines that standardize the immediate care and transportation of newborns with gastroschisis born outside of tertiary centers.

Profile of newborns with gastroschisis diagnosis in a public hospital at Porto Alegre/RS

Objective: To know the profile of newborn diagnosed with gastroschisis and treated at a public hospital at Porto Alegre, southern Brazil. Method: A cross-sectional, descriptive, quantitative study was conducted to retrospectively analyze 54 medical records of neonates diagnosed with gastroschisis treated at the study hospital between January 2006 and January 2016. The analysis included all medical records of infants diagnosed with gastroschisis and born in the institution studied, as well as those who were transferred from other health institutions in the period examined. Medical records were searched through electronic consultation to the institution's Epidemiology Service, using the International Code of Diseases (ICD) 10 Q 793). Later, the files were accessed through the Medical Records and Statistics Service. Information was collected using a form containing the study variables. The data analysis was performed using SPSS software, version 21.0. The study complied with the ethical aspects of human research legislation. Results: The prevalence of gastroschisis was 0.11%, totaling 54 cases identified. Mean maternal age was 20.2 years; sex distribution was equal among newborns with the malformation; and 72.5% of cases had primary abdominal closure. Conclusion: The results for related causes and treatment, such as maternal age, drug use, and type of abdominal closure, were similar to findings from other studies on gastroschisis(AU)

Gastrosquisis: ¿una pandemia con tasas en aumento? Experiencia del estudio colaborativo latino americano de malformaciones congénitas (ECLAMC) en Chile. Período 1982-2014 / Gastroschisis: A pandemic with increasing rates? ECLAMC experience in Chile 1982-2015

Introducción: Numerosas publicaciones han alertado sobre el aumento sostenido de las tasas de prevalencia al nacimiento de gastrosquisis en todo el mundo, catalogado este hecho como una «pandemia¼ fuertemente asociada a edad materna baja. Objetivos: Comprobar si en Chile se ha producido también un aumento significativo de la tasa de prevalencia al nacimiento de gastrosquisis y si se relaciona con edad materna baja. Pacientes y método: Se usó la base de datos ECLAMC de 26 hospitales chilenos participantes. Las tasas de prevalencia entre hospitales y las categorías de edad materna se compararon con prueba de Chi cuadrado o prueba exacta de Fisher. Se estudió la serie temporal mediante comparación de tendencias lineales, usando comparaciones de medias vía t-test y Wilcoxon-test. Resultados: En el período 1982-2014 nacieron 721.901 niños, entre los que se encontró 107 casos de gastrosquisis (1,49/10.000), el 75,7% de ellos eran hijos de mujeres menores de 25 años. El promedio de edad materna de los casos fue de 21,6 años, y el promedio nacional es cercano a 28 años, según el Ministerio de Salud. A partir de 1995 esta tasa mostró una tendencia ascendente significativamente distinta de cero (p = 0,00714), con un aumento de 0,1591 cada año. Este incremento fue alrededor del 300% entre 1995 y 2014, respecto del período anterior (p < 0,00001). Conclusión: En Chile se ha producido un aumento significativo de las tasas de gastrosquisis después del año 1995, y esta anomalía se asocia fuertemente con baja edad materna.

Introduction: Several communications have reported a significant increase in the prevalence of gastroschisis at birth in the last three decades. In many Countries it is referred to as "a pandemic strongly associated to low maternal age". Objective: To verify if there has been a significant increase in the prevalence of gastroschisis at birth in Chile, and if this rate is associated with a low maternal age. Patients and methods: The study was performed using the ECLAMC data base from 26 Chilean Hospitals. Comparison between prevalence rates and maternal age categories were made using the X² test or Fisher exact Test. The time series was studied comparing average tendencies using the Student t test and Wilcoxon-test. Results: There were 721,901 newborns in the period 1982-2014, among which 107 were diagnosed with gastroschisis (1.48/10,000). Around three-quarters (75.7%) of them were born from mothers younger than 25. The mean maternal age was 21.6 years in mothers of children with gastroschisis, while the mean maternal age of all mothers from Chile was almost 28 years old. The gastroschisis prevalence at birth rate was constant at around 1/10,000 until 1994. From 1995 this rate has a significant increasing tendency of 0.1591 by year (P = .00714). This increase is about a 300% on average for the 1995-2014 period, compared with the previous one (P < .00001). Conclusion: There has been a significant increase in the gastroschisis prevalence at birth in Chile from 1995, and this anomaly is associated with a low maternal age.

Defectos de la pared abdominal. Estudio comparativo entre onfalocele y gastrosquisis / Abdominal wall defects. A Comparative investigation between omphalocele and gastroschisis

Introducción: Onfalocele y gastrosquisis son los defectos de la pared abdominal (DPA) más frecuentes, que por su importancia y las diferencias entre ellos merecen ser estudiados en forma especial. Objetivos: Establecer las tasas de prevalencia de onfalocele y gastroaquisis al nacimiento. Verificar variaciones de ellas en distintos períodos. Estudiar la morbimortalidad asociada y su sobrevida. Pacientes y Método: Se revisó la base de datos ECLAMC (Estudio Colaborativo Latino Americano de Malformaciones Congênitas) para el período 1996-2010. Resultados: Se pesquizarón 33 recién nacidos (RN) con DPA. (11,6 por 10.000 nacimientos). De ellos 19 fueron onfalocele (6,7 por 10.000) y 14 (4,9 por 10.000) gastrosquisis. Los niños con onfalocele tenían significativamente mayor asociación con otras malformaciones, su frecuencia en el sexo masculino fue mayor y mostró mayor letalidad que la gastrosquisis. Todos los niños con gastrosquisis nacieron vivos, con predominio del sexo femenino, sus madres eran significativamente más jóvenes. Conclusión: Onfalocele y gastros-quisis presentaron una prevalencia al nacimiento mayor que lo publicado, probablemente por ser el HCUCH un Centro de Referencia. Ambas anomalías mostraron un aumento significativo en sus tasas de prevalencia al nacimiento al compararlas con las obtenidas anteriormente por nosotros en el mismo hospital. Se demostró diferencias significativas entre estas dos entidades: Onfalocele tiene mayor mortinatalidad, mortalidad y letalidad, mayor frecuencia de malformaciones asociadas y mayor promedio de edad materna.

Introduction: Omphalocele and Gastroschisis are the two most common congenital abdominal wall (AWD) defects. Objectives: To determine birth prevalence of Omphalocele and Gastroaquisis; to verify variations in different periods and to study the associated morbidity, mortality and survival. Patients and Method: The database of the Latin American Collaborative Study of Congenital Malformations (ECLAMC) was searched between the years 1996 and 2010. Results: 33 newborns (NB) with AWD were investigated (11.6 per 10,000 births). 19 of the cases corresponded to omphalocele (6.7 per 10,000 births) and 14 to gastroschisis (4.9 per 10,000 births). Children with omphalocele had significantly greater association with other malformations and the frequency in males was higher and showed higher mortality rates than gastroschisis. All children with gas-troschisis were born alive; they were predominantly female infants whose mothers were significantly younger. Conclusion: Birth omphalocele and gastroschisis prevalence were higher than published information, perhaps due to the fact that the HCUCH (Clinical Hospital of Universidad de Chile) is a reference center. Both anomalies showed a significant increase in their birth prevalence rates when compared with those previously obtained by us in the same hospital. Significant differences between these two entities were described. Omphalocele presented higher stillbirth, mortality and fatality rates; higher frequency of associated malformations and higher average maternal age.

Evolution of critically ill patients with gastroschisis from three tertiary centers

OBJECTIVES AND INTRODUCTION: Gastroschisis is a congenital abdominal wall defect with increasing occurrence worldwide over the past 20-30 years. Our aim was to analyze the morbidity of newborns after gastroschisis closure, with emphasis on metabolic and hydroelectrolyte disturbances in patients at three tertiary university centers. METHODS: From January 2003 to June 2009, the following patient data were collected retrospectively: (A) Background maternal and neonatal data: maternal age, prenatal diagnosis, type of delivery, Apgar scores, birth weight, gestational age and sex; (B) Surgical modalities: primary or staged closure; and (C) Hospital course: levels of serum sodium and levels of serum albumin in the two first postoperative days, number of ventilation days, other postoperative variables and survival. Statistical analyses were used to examine the associations between some variables. RESULTS: 163 newborns were included in the study. Primary closure of the abdominal defect was performed in 111 cases (68.1 percent). The mean serum sodium level was 127.4¡6.7 mEq/L, and the mean serum albumin level was 2.35¡0.5 g/dL. Among the correlations between variables, it was verified that hyponatremia and hypoalbuminemia correlated with the number of days on the ventilator but not with the number of days on total parenteral nutrition (TPN); mortality rate correlated with infection. The final survival rate was 85.9 percent. CONCLUSION: In newborns with gastroschisis, more aggressive attention to hyponatremia and hypoalbuminemia would improve the outcome.

Prevalencia de defectos de la pared abdominal al nacer: estudio ECLAMC / Prevalence of abdominal wall defects in newborns: ECLAMC Study, 1982-2005

Introducción: Onfalocele y gastrosquisis son los defectos de la pared abdominal (DPA) más frecuentes. Ambos necesitan un diagnóstico precoz y tratamiento quirúrgico oportuno para sobrevivir. Objetivos: Determinar la prevalencia al nacimiento de los DPA en la maternidad del Hospital Clínico de la Universidad de Chile (HCUCH) y compararla con la del total de maternidades chilenas (MCh) y los resultados del Estudio Colaborativo Latino Americano de Malformaciones Congénitas (ECLAMC). Resultados: La prevalencia global de onfalocele fue de 3,4/ 10 000 nacimientos y 3,8/10 000 para gastrosquisis. La prevalencia de onfalocele fue 9/10.000 en HCUCH y 2,77/10 000 en MCh (p = 0,006) y la de gastrosquisis fue 1,9/10 000 en HCUCH y 1,1/10 000 en MCh (p = 0,036). El promedio de edad materna fue 24,2 años para gastrosquisis y 33,6 para onfalocele (p < 0,004). La sobrevida de gastrosquisis fue 100 por ciento versus 31,7 por ciento en onfalocele (p < 0,0425). Todos los RN con onfalocele, salvo uno, presentaban asociación con otras MC graves. El peso de nacimiento y edad gestacional fueron significativamente menores en onfalocele. Conclusión: La prevalencia de DPA fue significativamente mayor en el HCUCH que en el resto de las maternidades chilenas. Gastrosquisis se presentó en hijos de mujeres más jóvenes y onfalocele en las de mayor edad.

Is the incidence of gastroschisis rising in South Africa in accordance with international trends? : paediatric surgery

Objectives. Analysis of the incidence of gastroschisis compared with the incidence of exomphalos as a percentage of total paediatric surgical admissions. Design. Retrospective observational analysis using data from the ward admissions registers of the paediatric surgical wards of Pretoria Academic and Kalafong hospitals and from the weekly statistics sheets and audits thereof of the Paediatric Surgery Department at Pretoria Academic Hospital. Patient files from Pretoria Academic were used where available to confirm data. Setting. Pretoria Academic Hospital (PAH) and Kalafong Hospital (KH); Pretoria; from March 1981 to December 2001; excluding Kalafong Hospital data from February 1984 to 12 June 1984 and for 2001. Results. Forty-eight cases of gastroschisis and 139 cases of exomphalos were seen at PAH and KH out of 21 495 total paediatric surgery ward admissions. The average incidence of gastroschisis increased 35-fold from the 7-year period 1981 - 1987 to the 7-year period 1995 - 2001; while the average incidence of exomphalos compared across the same periods only showed a 1.82-fold increase. The incidence of gastroschisis rose above that of exomphalos in 1999 and remained so through to 2001. The incidence of exomphalos showed a general rising trend from 1981 to 2001 except for a sudden unexplained increase in 1995 and 1996. Gastroschisis presenting at PAH was associated with a mortality rate of at least 38.7. Reasons for this high mortality rate cannot be adequately evaluated owing to poor record keeping. Conclusions. The incidence of gastroschisis presenting at PAH and KH has risen dramatically relative to the incidence of the macroscopically similar exomphalos over the period 1981 - 2001; with the incidence of gastroschisis rising above the incidence of exomphalos. A larger scale study looking at birth prevalence of gastroschisis and exomphalos in South Africa is necessary to determine whether this represents a true differential change in the incidence of gastroschisis; reflecting internationally observed increases in the birth prevalence of this defect; or whether it is merely due to logistical changes within the administration of the Department of Health serving the area

Gastroschisis: a ten year review

From 1983 to 1993, 30 cases of gastroschisis were managed at the Mayaguez Medical Center. Ninety percent of these patients underwent primary closure of their abdominal wall defect. Three of 30 patients (10%) required silastic or goretex silos with final closure in an average of 8 days. There was no sex predilection, the average birth weight was 2.4 kg and the mean gestational age was 36 weeks. Thirty percent had associated anomalies, the majority were intestinal atresia, and/or undescended testicles. Twenty one (70%) of infants were delivered vaginally. Nine children (30%) were delivered via cesarean section. Four cesarean sections were done solely after prenatal ultrasonic identification of gastroschisis. There was no improvement in hospital stay, complications, or days until enteral feeds were tolerated when vaginally delivered patients were compared to those born by c-sections. In seven patients mesh sheeting (Marlex) was used for closure of late hernia defects. The mean hospital stay was 50 days and the mean time to enteral feedings 20 days. All patients required postoperative mechanical ventilation for an average of 4 days. There was no mortality. Our data and review of the literature do not support gastroschisis prenatal diagnosis as a sole indication for cesarean section. Our data showed favorable prognosis for most babies. Primary fascial closure can be accomplished safely in the majority of patients. No single operative strategy is ideal for all patients, and treatment of individual defects should be tailored to the degree of visceroabdominal disproportion