The Association of Prenatal Diagnoses with Mortality and Long-Term Morbidity in Children with Specific Isolated Congenital Anomalies: A European Register-Based Cohort Study.

OBJECTIVES: To compare 5-year survival rate and morbidity in children with spina bifida, transposition of great arteries (TGA), congenital diaphragmatic hernia (CDH) or gastroschisis diagnosed prenatally with those diagnosed postnatally. METHODS: Population-based registers' data were linked to hospital and mortality databases. RESULTS: Children whose anomaly was diagnosed prenatally (n = 1088) had a lower mean gestational age than those diagnosed postnatally (n = 1698) ranging from 8 days for CDH to 4 days for TGA. Children with CDH had the highest infant mortality rate with a significant difference (p < 0.001) between those prenatally (359/1,000 births) and postnatally (116/1,000) diagnosed. For all four anomalies, the median length of hospital stay was significantly greater in children with a prenatal diagnosis than those postnatally diagnosed. Children with prenatally diagnosed spina bifida (79% vs 60%; p = 0.002) were more likely to have surgery in the first week of life, with an indication that this also occurred in children with CDH (79% vs 69%; p = 0.06). CONCLUSIONS: Our findings do not show improved outcomes for prenatally diagnosed infants. For conditions where prenatal diagnoses were associated with greater mortality and morbidity, the findings might be attributed to increased detection of more severe anomalies. The increased mortality and morbidity in those diagnosed prenatally may be related to the lower mean gestational age (GA) at birth, leading to insufficient surfactant for respiratory effort. This is especially important for these four groups of children as they have to undergo anaesthesia and surgery shortly after birth. Appropriate prenatal counselling about the time and mode of delivery is needed.

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)

A retrospective review of gastroschisis epidemiology and referral patterns in northern Ghana.

PURPOSE: To describe the epidemiology and referral patterns of gastroschisis patients in northern Ghana. METHODS: A hospital-based retrospective review was undertaken at Tamale Teaching Hospital (TTH) Neonatal Intensive Care Unit (NICU) between 2014 and 2019. Data from gastroschisis patients were compared to patients with other surgical diagnoses. Descriptive and inferential statistics were performed with SAS. Referral flow maps were made with ArcGIS. RESULTS: From a total of 360 neonates admitted with surgical conditions, 12 (3%) were diagnosed with gastroschisis. Around 91% (n = 10) of gastroschisis patients were referred from other hospitals, traveling 4 h, on average. Referral patterns showed gastroschisis patients were admitted from three regions, whereas patients with other surgical diagnoses were admitted from eight regions. Only 6% (12/201) of expected gastroschisis cases were reported during the 6-year period in all regions. All gastroschisis deaths occurred within the first week of life. CONCLUSIONS: Improving access to surgical care and reducing neonatal mortality related to gastroschisis in northern Ghana is critical. This study provides a baseline to inform future gastroschisis interventions at TTH. Priority areas may include special management of low birth weight newborns, better referral systems, empowerment of community health workers, and increasing access to timely, affordable, and safe neonatal transport.

Neonatal surgical outcomes: a prospective observational study at a Tertiary Academic Hospital in Johannesburg, South Africa.

PURPOSE: The neonatal period is the most vulnerable period for a child. There is a paucity of data on the burden of neonatal surgical disease in our setting. The aim of this study was to describe the frequency with which index neonatal surgical conditions are seen within our setting and to document the 30-day outcome of these patients. METHODS: This was a single-centre prospective observational study in which all neonates with paediatric surgical pathology referred to the paediatric surgical unit with a corrected gestational age of 28 days were included. RESULTS: Necrotising enterocolitis was the most frequent reason for referral to the paediatric surgical unit (n = 68, 34.34%). Gastroschisis was the most frequent congenital anomaly referred (n = 20, 10.10%). The overall morbidity was 57.58%. Surgical complications contributed to 18.51% of morbidities. The development of gram negative nosocomial sepsis was the most frequent cause of morbidity (n = 98, 50.78%). Mortality at 30 days was 21.74% (n = 40). Sepsis contributed to mortality in 35 patients (87.5%), 16 of which had gram negative sepsis. CONCLUSION: Gram-negative sepsis was a major contributing factor in the development of morbidity and mortality in our cohort. Prevention and improvement in infection control are imperative if we are to improve outcomes in our surgical neonates.

Multidisciplinary Development of a Low-Cost Gastroschisis Silo for Use in Sub-Saharan Africa.

BACKGROUND: Gastroschisis silos are often unavailable in sub-Saharan Africa (SSA), contributing to high mortality. We describe a collaboration between engineers and surgeons in the United States and Uganda to develop a silo from locally available materials. METHODS: Design criteria included the following: < $5 cost, 5 ± 0.25 cm opening diameter, deformability of the opening construct, ≥ 500 mL volume, ≥ 30 N tensile strength, no statistical difference in the leakage rate between the low-cost silo and preformed silo, ease of manufacturing, and reusability. Pugh scoring matrices were used to assess designs. Materials considered included the following: urine collection bags, intravenous bags, or zipper storage bags for the silo and female condom rings or O-rings for the silo opening construct. Silos were assembled with clothing irons and sewn with thread. Colleagues in Uganda, Malawi, Tanzania, and Kenya investigated material cost and availability. RESULTS: Urine collection bags and female condom rings were chosen as the most accessible materials. Silos were estimated to cost < $1 in SSA. Silos yielded a diameter of 5.01 ± 0.11 cm and a volume of 675 ± 7 mL. The iron + sewn seal, sewn seal, and ironed seal on the silos yielded tensile strengths of 31.1 ± 5.3 N, 30.1 ± 2.9 N, and 14.7 ± 2.4 N, respectively, compared with the seal of the current standard-of-care silo of 41.8 ± 6.1 N. The low-cost silos had comparable leakage rates along the opening and along the seal with the spring-loaded preformed silo. The silos were easily constructed by biomedical engineering students within 15 min. All silos were able to be sterilized by submersion. CONCLUSIONS: A low-cost gastroschisis silo was constructed from materials locally available in SSA. Further in vivo and clinical studies are needed to determine if mortality can be improved with this design.

Gastroschisis in the neonatal period: A prospective case-series in a Brazilian referral center.

BACKGROUND/PURPOSE: Gastroschisis is increasing in incidence and has low mortality and high morbidity. We describe the clinical and surgical characteristics of gastroschisis patients in a Brazilian referral center. METHODS: Single-center prospective case series of gastroschisis patients. The following two groups were formed depending on the intestinal characteristics: simple and complex patients. RESULTS: In total, 79 patients were enrolled, 89% of whom were classified as simple and 11% as complex. The baseline characteristics were similar between the groups, with the exception of the illness severity score. The complex group had a significantly smaller defect size, more reoperations and worse clinical outcomes than the simple group, with the initiation of feeding taking 1.5 times longer, the duration of total parenteral nutrition taking twice as long, and the length of hospitalization being 2.5 times longer; the complex group also included all the deaths that occurred. Overall, the survival rate was 96%. Patients who underwent the sutureless technique had significantly fewer wound infections and a decreased duration of mechanical ventilation than sutured patients. CONCLUSIONS: This study provides a comprehensive picture of gastroschisis during the neonatal period in a Brazilian referral center, emphasizing the significantly higher risk for morbidity and mortality among complex patients than among simple patients and the few advantages of the sutureless technique over the sutured technique in terms of closing the defect. TYPE OF STUDY: Prognostic. LEVEL OF EVIDENCE: IV.

Intraoperative Findings Associated to Inpatient Mortality From Patients With Gastroschisis in Western Mexico.

BACKGROUND: Intraoperative findings during gastroschisis surgery are the main predictor associated with increased mortality. The aim of our study was to determine the type of surgical findings associated with inpatient mortality in a cohort of patients with gastroschisis from a university hospital in Western Mexico. MATERIALS AND METHODS: Infants with surgically repaired gastroschisis during the period 2011-2017 at the Dr. Juan I. Menchaca Civil Hospital of Guadalajara (Guadalajara, Mexico) were studied. Data regarding demographics, perinatal history, and intraoperative findings were collected and compared according to whether they were nonsurvivors (cases) or survivors (controls) at hospital discharge. Data were analyzed using logistic regression, determining its adjusted odds ratio (aOR) and its respective 95% confidence intervals (95% CIs). The proper adjustment of the model was verified using the Hosmer and Lemeshow test. RESULTS: Ninety-four patients with gastroschisis were studied, of which 13 were nonsurvivors (13.8%), and 81 (86.2%) were survivors at hospital discharge. In the group of survivors, primary surgical closure was performed more frequently (P = 0.018), whereas staged reduction with a silo predominated in the group of nonsurvivors (P = 0.018), and an increased frequency of complex gastroschisis (0.0001). After logistic regression analysis, intraoperative findings associated with nonsurvival were severe bowel matting (aOR: 7.3; 95% CI: 1.2-44), and prolapse of the small intestine and large intestine, plus any other organ (aOR: 15.9; 95% CI: 1.1-219.6). CONCLUSIONS: Mortality in our cohort was high (13.8%) and was significantly associated with severe bowel matting, and the prolapse of the small and large intestines, plus any other organ.

Reducing Gastroschisis Mortality: A Quality Improvement Initiative at a Ugandan Pediatric Surgery Unit.

INTRODUCTION: With modern treatment, survival of gastroschisis exceeds 90% in high-income countries. Survival in these countries has been largely attributed to prenatal diagnosis, delivery at tertiary facilities with timely resuscitation, timely intervention, parenteral nutrition and intensive care facilities. In sub-Saharan Africa, due to lack of these facilities, mortality rates are still alarmingly high ranging from 75 to 100%. In Uganda the mortality is 98%. AIM: The aim of this study was to reduce gastroschisis mortality in a feasible, sustainable way using a locally derived gastroschisis care protocol at a referring hospital in Western Uganda. METHODS: Data collection was performed from January to October 2018. Nursing staff were interviewed regarding the survival and management of gastroschisis babies. A locally derived protocol was created with staff input and commitment from all the team members. RESULTS: Four mothers absconded and 17 babies were cared for using the newly designed protocol. Seven survived and were well at one month post discharge follow-up, reducing the mortality for this condition from 98 to 59%. CONCLUSION: A dedicated team with minimal resources can significantly reduce the mortality in gastroschisis by almost 40% using a locally derived protocol.

Gastroschisis in Finland 1993 to 2014-Increasing Prevalence, High Rates of Abortion, and Survival: A Population-Based Study.

INTRODUCTION: The study aims to assess the changes in prevalence and mortality of gastroschisis, and to identify associated anomalies. MATERIALS AND METHODS: It is a population-based nationwide study. All gastroschisis cases were identified in the Finnish Register of Congenital Malformations and the Care Register for Health Care from 1993 to 2014 including live births, stillbirths, and terminations of pregnancy due to fetal anomalies. Associated anomalies were recorded, and analyzed, and prevalence and infant mortality were calculated. RESULTS: There were 320 cases of gastroschisis; 235 (73%) live births, 16 (5%) stillbirths, and 69 (22%) terminations of pregnancy. Live birth prevalence of gastroschisis in Finland was lower than generally reported (1.73 in 10,000). However, due to relatively high rates of abortion, our total prevalence of 2.57/10,000 was similar with other reports. The most common risk factor was young maternal age. Babies with gastroschisis were born prematurely, on average on the 36th week and most are delivered by caesarean section. There was a significant increasing trend in live birth prevalence (p = 0.0018). Overall infant mortality was 7.7% (18/235), 7.2% (16/222) in simple gastroschisis and 15% (2/13) in complex gastroschisis. Associated anomalies were rare both in aborted fetuses and neonates, and there was only one case with a chromosomal abnormality. CONCLUSION: Gastroschisis is usually an isolated anomaly with increasing birth prevalence and excellent survival rates. Regardless of the good prognosis, the abortion rates in Finland are higher than previously reported, and we hypothesize this to be due to lack of appropriate antenatal counselling.

Immediate versus silo closure for gastroschisis: Results of a large multicenter study.

BACKGROUND/PURPOSE: The optimal method to repair gastroschisis defects continues to be debated. The two primary methods are immediate closure (IC) or silo placement (SP). The purpose of this study was to compare outcomes between each approach using a multicenter retrospective analysis. We hypothesized that patients undergoing SP for ≤5 days would have largely equivalent outcomes compared to IC patients. METHODS: Gastroschisis patient data were collected over a 7-year period. The cohort was separated into IC and SP groups. The SP group was further stratified based on time to closure (≤5 days, 6-10 days, >10 days). Characteristics and outcomes were compared between groups. Multivariate logistic regression was also performed. RESULTS: 566 neonates with gastroschisis were identified including 224 patients in the IC group and 337 patients in the SP group. Among SP patients, 130 were closed within 5 days, 140 in 6-10 days, and 57 in >10 days. There were no significant differences in mortality, sepsis, readmission, or days to full enteral feeds between IC patients and SP patients who had a silo ≤5 days. IC patients had a significantly higher incidence of ventral hernias. Multivariate analysis revealed time to closure as a significant independent predictor of length of stay, ventilator duration, time to full enteral feeds, and TPN duration. CONCLUSIONS: Our data show largely equivalent outcomes between patients who undergo immediate closure and those who have silos ≤5 days. We propose that closure within 5 days avoids many of the risks commonly attributed to delay in closure. LEVEL OF EVIDENCE: Level II retrospective study.

Assessment of gastroschisis risk factors in Egypt.

AIM: Mortality in infants born with gastroschisis (GS) in low-to-middle-income countries (LMICs) is high. This study aimed to assess factors which might affect outcome in Egypt in order to improve survival. METHODS: A prospective study over a 15-month duration was completed. Variables assessed covered patient, maternal, antenatal, treatment, and complications. The Gastroschisis Prognostic Score (GPS) was used to predict outcome. A validated questionnaire was used to assess socioeconomic status. The main outcome was mortality. RESULTS: Twenty-four cases were studied. Median gestational age was 37 (26-40) weeks, and 9 (38%) were preterm. Mortality occurred in 15 (62%) infants. Median transfer time was 8 (1.5-35) hours, and 64% survived if transferred before 8 h. Median maternal age was 20 (16-27) years. All families were of a low or very-low socioeconomic level. Only 25% had antenatal scans. Most cases were simple GS, and only 3 (12.5%) were complex GS. Median length of stay was 14 (1-52) days, TPN duration was 12 (0-49) days, and days to full feeds was 5 (3-11) days. The GPS score ranged from 0 to 6 in the studied cases and negatively correlated with outcome (rS = -0.98; p = 0.03). CONCLUSION: The mortality of GS in Egypt is very high, mainly due to sepsis and prematurity. Young maternal age and poor socioeconomic status are linked to GS. The GPS is a good indicator of morbidity and mortality in a LMIC setting. Survival improved with better resuscitation and strict management protocols. More effort is needed to improve antenatal detection, and transfer time should be ideally below 8 h. LEVEL OF EVIDENCE: Level IV.

Mortality from gastroschisis in the state of Rio de Janeiro: a 10-year series / Mortalidade por gastrosquise no estado do Rio de Janeiro: uma série de 10 anos

ABSTRACT OBJECTIVE To analyze mortality and associated factors in a series of gastroschisis at birth in the state of Rio de Janeiro in a 10-year period (2005 to 2014). METHOD A retrospective cohort study, which related the databases of the Live Births Information System and the Mortality Information System by probabilistic linkage. Final database was constructed in two stages: preparation of the two initial databases and establishment of relationships between them. RESULTS Preterm newborns and those with low birthweight had higher risk of death, with statistical significance (p = 0.03 and p = 0.006, respectively). Regarding place of birth, although death frequency was higher in maternity units than in general hospitals (p = 0.04; OR = 0.5; 95%CI 0.3-1.0), it was observed that a unit characterized as a general hospital had a high birth frequency (61.2%). Furthermore, the comparative analysis of the risk of death between this unit and others showed a 7.5 higher risk of death in general hospitals and 3.2 higher in maternity units, with statistical significance (p < 0.001). Moreover, births in level II intensive care units had 3.9 times more risk of death compared with level III (p < 0.001). CONCLUSION This study foments the discussion of two possible strategies in the treatment of gastroschisis in newborns. First, the centralization of care in tertiary units, enabling malformation care to be analyzed in a more detailed and standardized manner. Second, and perhaps more feasible, the elaboration of clinical guidelines to standardize immediate care for gastroschisis in babies born outside tertiary centers, as well as the standardization of their transportation until arrival at the tertiary center.

RESUMO OBJETIVO Analisar a mortalidade e fatores associados em uma série de nascimentos com gastrosquise no estado do Rio de Janeiro em 10 anos (2005 a 2014). MÉTODO Estudo de coorte retrospectiva, no qual foram relacionadas as bases de dados do Sistema de Informação sobre Nascidos Vivos e do Sistema de Informação sobre Mortalidade por linkage probabilístico. A base de dados final foi construída em duas etapas, que consistiram em: preparo das duas bases de dados iniciais e estabelecimento de relações entre elas. RESULTADOS Os recém-nascidos pré-termo e os com baixo peso ao nascer tiveram maior chance de óbito, com significância estatística (p = 0,03 e p = 0,006, respectivamente). Em relação ao local de nascimento, embora a frequência de óbito tenha sido maior nas maternidades do que em hospitais gerais (p = 0,04; OR = 0,5; IC95% 0,3-1,0), foi observado que uma unidade caracterizada como hospital geral apresentou uma frequência alta de nascimentos (61,2%) e, na análise comparativa da chance de óbito dessa unidade com as demais, encontrou-se uma chance de morrer 7,5 maior em hospitais gerais e 3,2 maior em maternidades, com significância estatística (p < 0,001). Além disso, nascer em unidades de terapia intensiva tipo II aumentou a chance de óbito em 3,9 vezes em comparação com as do tipo III (p < 0,001). CONCLUSÃO Este estudo dá subsídios para a discussão de duas possíveis estratégias no tratamento de recém-nascidos com gastrosquise. A primeira seria a centralização do cuidado em unidades terciárias, possibilitando que o cuidado à malformação seja analisado de forma mais minuciosa e padronizada. A segunda, e talvez mais factível, seria a elaboração de diretrizes clínicas que padronizem o cuidado imediato aos bebês com gastrosquise nascidos fora de centros terciários, bem como a padronização do transporte deles até a chegada ao centro terciário.

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.

Gastroschisis during gestation: prognostic factors of neonatal mortality from prenatal care to postsurgery.

OBJECTIVE: To evaluate the prognostic factors related to mortality in neonates with gastroschisis, ranging from prenatal care to corrective surgery. STUDY DESIGN: A retrospective cohort study was conducted and included neonates with gastroschisis who underwent surgical correction from 2010 to 2015. The patients were divided into two groups based on postoperative outcomes: those who were discharged and those who died postoperatively. Data regarding demographics, prenatal history, delivery route, surgery and postoperative outcomes were collected in both groups. RESULTS: In total, 168 patients were eligible; 82 (48.8%) were discharged, and 86 (51.2%) died postoperatively. Fewer prenatal visits (4.85 versus 6.05 visits, P = 0.004), delayed prenatal diagnosis (27.6 versus 22.2 weeks of gestational age, P = 0.005), low birth weight (2159 versus 2444 g, P < 0.001), intestinal injury (OR 5.5, P = 0.001) and sepsis (OR 112.1, P < 0.001) were associated with infant mortality. The type of delivery and the mean time between birth and the first corrective surgery (16.2 versus 21.1 h, P = 0.071) were not associated with increased neonatal mortality; however, the newborns who underwent their first corrective surgery after less than 4 h of life had lower mortality, and surgery performed after more than 4 h of life increased the risk of mortality (OR 2.7, CI 1.2-6.3, P 0.014). CONCLUSION: The mortality rate was high (51.2%) and was associated with inadequate prenatal care, low birth weight, gestational age, the severity of intestinal injury, infection and septicemia.

Anterior abdominal wall defects managed at a tertiary maternal-fetal medicine service in New Zealand: What counselling advice can we offer parents?

BACKGROUND: Anterior abdominal wall defects, including gastroschisis and omphalocoele, are common fetal anomalies. The management remains complicated, and their diagnosis may lead to significant parental distress. Effective parental counselling may impact on parental perceptions of the defect and help guide pregnancy management. AIMS: Using contemporary data, we aimed to describe clinical outcomes of patients with gastroschisis or omphalocoele in order to provide information for clinicians to assist in parental counselling. MATERIALS AND METHODS: We followed a case-series of patients with anterior abdominal wall defects referred to our regional Maternal Fetal Medicine services from 2011 to 2016. Outcomes of interest antenatally included details of diagnosis, associated anomalies and outcomes of pregnancy and postnatally included the nature of surgical repair, hospital stay and secondary complications until initial discharge. RESULTS: Eighty babies with gastroschisis were referred antenatally, and 72 were liveborn. Forty-nine babies with omphalocoele were referred antenatally, and 24 were liveborn. One further neonate with omphalocoele was postnatally diagnosed. Seventy-one neonates with gastroschisis progressed to operation, 30 developed complications post-surgery, and 68 survived until initial discharge. Twenty-two neonates with omphalocoele progressed to surgery, only two developed complications, and 21 survived until initial discharge. Eight of the surviving neonates with omphalocoele had associated structural abnormalities. The median hospital stay was 27 and eight days for gastroschisis and omphalocoele, respectively. CONCLUSION: Neonates with gastroschisis can have complex postnatal periods. Omphalocoele is associated with high antenatal mortality, especially in the presence of associated abnormalities; however, surviving neonates may have uneventful postnatal periods.

Impact of disease-specific volume and hospital transfer on outcomes in gastroschisis.

BACKGROUND: Gastroschisis, a surgical condition requiring complex interdisciplinary care, may benefit from treatment at higher volume centers. Recent studies on surgical volume and outcomes have conflicting findings. METHODS: Data were collected prospectively on newborns ≥1500 g with gastroschisis born 2009-2015, admitted to 159 US centers, and separated into terciles based on number of annual gastroschisis repairs. Infants transferred after gastroschisis repair were excluded. RESULTS: There were 4663 infants included: 307 from 53 low, 1201 from 55 medium, and 3155 from 51 high volume centers. Infants at high volume centers had higher rates of intestinal atresia (P = 0.04) and outborn status (P < 0.0001). Outborn infants (N = 1134) had higher rates of gastrostomy/jejunostomy placement (P < 0.001). Mortality was universally low (2.0% low, 2.4% medium, and 1.7% high; 2.0% outborn and 1.9% inborn). On multivariate analysis, mortality, sepsis rates, and length of stay did not differ by center volume. Outborn status was associated with longer length of stay (P = 0.001), not mortality or sepsis. CONCLUSION: Infant characteristics and management vary based on gastroschisis surgical volume and transfer status. Center volume and early transfers were not associated with mortality. Further investigation to identify subsets of gastroschisis infants who would benefit from care at higher volume centers is warranted. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level II.

Closing gastroschisis: The good, the bad, and the not-so ugly.

PURPOSE: The diagnosis of "closing" or "closed gastroschisis" is made when bowel is incarcerated within a closed or nearly closed ring of fascia, usually with associated bowel atresia. It has been described as having a high morbidity and mortality. METHODS: A retrospective review of closing gastroschisis cases (n = 53) at six children's hospitals between 2000 and 2016 was completed after IRB approval. RESULTS: A new classification system for this disease was developed to represent the spectrum of the disease: Type A (15%): ischemic bowel that is constricted at the ring but without atresia; Type B (51%): intestinal atresia with a mass of ischemic, but viable, external bowel (owing to constriction at the ring); Type C (26%): closing ring with nonviable external bowel +/- atresia; and Type D (8%): completely closed defect with either a nubbin of exposed tissue or no external bowel. Overall, 87% of infants survived, and long-term data are provided for each type. CONCLUSIONS: This new classification system better captures the spectrum of disease and describes the expected long-term results for counseling. Unless the external bowel in a closing gastroschisis is clearly necrotic, it should be reduced and evaluated later. Survival was found to be much better than previously reported. TYPE OF STUDY: Retrospective case series with no comparison group. LEVEL OF EVIDENCE: Level IV.

Risk stratification and outcome determinants in gastroschisis.

Selection of outcome determinants and risk stratification are necessary to identify patients at higher risk for morbidity and mortality. This facilitates human and material resource allocation and allows for improved family counseling. While several different factors, including prenatal ultrasonographic bowel features, the timing and mode of delivery, and the features of bowel injury have been investigated in gastroschisis, there is still significant debate as to which of these best predicts outcome. This article reviews the different outcome predictors and risk prognostication schemata currently available in the literature to help guide clinicians caring for infants with gastroschisis.

A Gastroschisis bundle: effects of a quality improvement protocol on morbidity and mortality.

OBJECTIVE: Gastroschisis incidence is rising. Survival in developed countries is over 95%. However, in underdeveloped countries, mortality is higher than 15% often due to sepsis. The aim of this study was to evaluate the effect on morbidity and mortality of a Quality Improvement Protocol for out-born gastroschisis patients. METHODS: The protocol consisted in facilitating transport, primary or staged reduction at the bedside and sutureless closure, without anesthesia, PICC lines and early feeding. Data was prospectively collected for the Protocol Group (PG) treated between June 2014 through March 2016 and compared to the last consecutive patients Historical Group (HG). Primary outcome was mortality. SECONDARY OUTCOMES: need for and duration of mechanical ventilation (MV), time to first feed (TFF) after closure, parenteral nutrition (TPN), length of stay (LOS) and sepsis. Data were analyzed using χ2 and Mann-Whitney U tests. RESULTS: 92 patients were included (46 HG and 46 PG). Demographic data were homogeneous. Mortality decreased from 22% to 2% (p = 0.007). Mechanical ventilation use decreased from 100% to 57% (p = <0.001), ventilator days from 14 to 3 median days (p = <0.0001), TPN days: 27 to 21 median days (p = 0.026), sepsis decreased from 70% to 37% (p = 0.003) and anesthesia from a 100% to 15% (p = <0.001), respectively. No difference was found in NPO or LOS. CONCLUSION: A major improvement in the morbidity and mortality rates was achieved, with outcomes comparable to those reported in developed countries. It was suitable for all patients with gastroschisis. We believe this protocol can be implemented in other centers to reduce morbidity and mortality. LEVEL OF EVIDENCE: III.

A novel approach in the treatment of neonatal gastroschisis: a review of the literature and a single-center experience.

Gastroschisis is a congenital abdominal wall defect and its management remains an issue. We performed a review of the literature to summarize its evaluation, management and outcome and we describe a new type of surgical reduction performed in our center without anesthesia (GA), immediately after birth, in the delivery room. Between January 2002 and March 2013, we enrolled all live born infants with gastroschisis referred to the third-level Division of Obstetrics and Gynecology "San Camillo" of Rome. Two groups of infants were identified: group 1 in which gastroschis reduction was performed by the traditional technique and group 2 in which reduction was immediately performed after birth in the delivery room without GA. Twelve infants were enrolled in group 1, and seven infants in group 2. Statistical significance was observed between the groups regarding the hospital stay, for the duration of parenteral nutrition and full oral feeds (p = .004). Survival was similar between two groups. The reduction without GA performed immediately after birth in a delivery room encourages the relationship between the mother and her child and appears to be a safe and feasible technique in a selected group of patients with simple gastroschisis defect; for this reason, it could represent a valid alternative to traditional approach.