Chest radiographic thoracic areas and respiratory outcomes in infants with anterior abdominal wall defects.

OBJECTIVES: Infants with anterior abdominal wall defects (AWD) can suffer from pulmonary complications. Our aims were to determine if the chest radiographic thoracic areas (CRTAs) on day one differed between infants with exomphalos or gastroschisis, whether this related to differing severity of outcomes and if they were lower than those of controls indicating abnormal antenatal lung growth. METHODS: A review of infants with exomphalos or gastroschisis born between January 2004 and January 2023 was conducted. The control group was term, newborn infants ventilated for poor respiratory drive at birth. Chest radiographs on day one were analysed and the highest CRTA in the first 24 h after birth for each infant included in the analysis. RESULTS: The 127 infants with gastroschisis had a lower gestational age and birthweight than the 62 exomphalos infants and 130 controls (all p<0.001) The CRTAs of the controls were greater than the CRTAs of the exomphalos and the gastroschisis infants (p = 0.001). The median CRTA corrected for birthweight was lower in the exomphalos infants [688, IQR 568-875 mm2/kg] than the gastroschisis infants [813, IQE 695-915 mm2/kg] No gastroschisis infant developed bronchopulmonary dysplasia (BPD). A CRTA of 1759 mm2 had a sensitivity of 81 % and specificity of 71 % in predicting BPD in infants with exomphalos. CONCLUSIONS: Infants with gastroschisis or exomphalos had lower CRTAs than controls suggesting both groups had abnormal antenatal lung development. The CRTA was lower in the exomphalos infants who also had worse respiratory outcomes, hence CRTA assessment may a useful prognostic aid.

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.

Prenatal cannabis use disorder and gastroschisis in California, 2007-19.

BACKGROUND: Gastroschisis is a congenital anomaly of the abdominal wall with an unknown aetiology. Recent trends in the prevalence of gastroschisis suggest that changing environmental or behavioural factors may contribute. We examined whether prenatal cannabis use disorder was associated with gastroschisis. METHODS: The Study of Outcomes of Mothers and Infants is a population-based cohort compiled of California birth records that have been linked to Department of Health Care Access and Information hospitalization, emergency department and ambulatory surgery records. We included 2007-19 singleton live births (n = 5 774 656). Cannabis use disorder was measured by diagnosis codes at any visit during pregnancy or at birth. Gastroschisis was measured by diagnosis or surgical repair procedure codes at birth or during the first year of life. RESULTS: The prevalence of cannabis use disorder was about 1%. The prevalence of gastroschisis was 0.14% and 0.06% among those with and without cannabis use disorder, respectively. There were positive associations between cannabis use disorder and gastroschisis when using a multivariable model [adjusted risk ratio (aRR) = 1.3, 95% confidence interval (CI) 1.0, 1.7) and a matched sample approach (aRR = 1.5, 95% CI 1.1, 2.1). The association varied by maternal age and was largest among people aged >34 years (aRR = 2.5, 95% CI 1.0, 5.8). CONCLUSIONS: We confirm findings of a positive association between cannabis exposure and gastroschisis and add that it is strongest when maternal age is greater than 34 years. More investigation into whether the association is causal, and why the association varies by maternal age, is encouraged.

Assessment of the neonatal referral and transport system for patients with gastroschisis in Kenya.

PURPOSE: To assess the neonatal referral and transport system for gastroschisis patients referred to a tertiary level hospital in Kenya. METHODS: This was a prospective cross-sectional study carried out at Kenyatta National Hospital (KNH) which recruited patients with gastroschisis using consecutive sampling approach. Data were collected on pre-, intra-transit factors, time and distance covered. Assessment was done using pre and intra transit factors as per the standard transport protocols in literature. RESULTS: Twenty-nine patients presented with gastroschisis during the eight month study period. Mean age was 7.07 h. There were 16 (55.2%) males and 13 (44.8%) females. Mean birthweight was 2020 g, and a mean gestational age of 36.5 weeks. Mean duration of transit was 5 h. Mean distance from referring facility was 153.1 km. Most affected factors in the pre-transit protocol were lack of monitoring chart (0%), comment on blood investigations (0%), gastric decompression (3.4%), and prenatal obstetric scan (44.8%). For intra-transit scores, most affected were incubator use (0%), bowel monitoring (0%), functioning nasogastric tube (13.8%), and adequate bowel cover (34.5%). CONCLUSION: This study demonstrates that pre-transit and transit care of neonates with gastroschisis is inadequate in Kenya. Interventions needed, as identified by this study, to promote care of neonates with gastroschisis are advised.

Early growth and body composition assessed by air displacement plethysmography in infants born with simple gastroschisis.

BACKGROUND: Gastroschisis is an abdominal wall malformation usually associated with impaired growth. OBJECTIVE: To evaluate the growth and body composition of infants born with simple gastroschisis in a referral center. METHODS: This was a single-center, prospective case series of infants with simple gastroschisis who were measured at birth, at discharge, and at 3 months. Body composition was assessed via air-displacement plethysmography at discharge and at 3 months. The results were compared with those reported for healthy infants at an equivalent gestational age. RESULTS: Simple gastroschisis infants were lighter and smaller at birth and remained similar at 3 months. All anthropometric z scores decreased from birth to discharge, followed by an increase but not a full recovery toward 3 months. Overall, gastroschisis infants had a similar FM percentage, FM% (11.1 ± 4.7), but a lower FFM, FFM (2481 ± 478 g), at discharge. FM% (18.5 ± 5.3) decreased at 3 months, and FFM remained lower (3788 ± 722 g) but improved between the two exams. Boys had significantly more FFM than girls at both evaluations. The multiple regression analysis showed that male sex, prematurity, total parenteral nutrition duration, and exclusive breast milk diets were associated with differences in body composition. CONCLUSIONS: Infants with simple gastroschisis cared for in a referral center experienced growth failure at discharge and showed a similar FM% but lower FFM than healthy infants. At 3 months, they exhibited smaller FM% and FFM, but FFM improved after the first exam, representing a better protein accretion. TYPE OF STUDY: Prognostic. LEVEL OF EVIDENCE: IV.

Gastroschisis in Mozambique: current status and priorities for improving care from Hospital Central de Maputo, the largest and referral hospital of the country.

PURPOSE: Historically, gastroschisis was considered a death sentence in Mozambique. The purpose of this study was to evaluate the current state of gastroschisis management and outcomes in our facility and to identify potential areas of improvement in neonatal and surgical care. METHODS: A retrospective study was performed of all gastroschisis patients admitted to Hospital Central Maputo located in Maputo City/ Mozambique from 2019 to 2020. Demographic, perinatal, operative, and mortality data were obtained from neonatal and surgical logbooks. Descriptive analysis was performed. RESULTS: A total of 62 gastroschisis patients were admitted to the Hospital Central Maputo. No patients had a prenatal diagnosis. Many of the infants were born preterm (48%), and 68% had low birth weight. Only 15 (24%) patients underwent operative intervention (73% primary fascial closure and 27% sutured silo). There were only three survivors (5%) all of whom underwent primary closure. However, the overall survival rate for patients undergoing an attempt at surgical closure was 20%. CONCLUSION: While the mortality rate remains high for gastroschisis patients in Mozambique, there have been a few survivors when surgery is performed. Improvements in neonatal care are needed. Given the high mortality rates and limited resources, we plan to focus our surgical efforts on bedside closure techniques.

Gastroschisis Complicated by Colonic Atresia.

INTRODUCTION: Gastroschisis with colonic atresia is a rare association. Due to its rarity and variation in presentation, no standardized surgical treatment option exists. Complicated gastroschisis is associated with a higher morbidity and mortality than intestinal atresia or gastroschisis alone. METHODS: This is a case report of a neonate with congenital gastroschisis. On day of life 1, a silo was placed. On day of life 4, the upper portion of silo contents appeared more congested with dusky discoloration and the patient was oliguric. She was taken to the operating room for exploration. RESULTS: The patient required resection of the terminal ileum and an atretic portion of the cecum due to necrosis. Four days later, primary closure of the gastroschisis defect was performed with creation of an end ileostomy. Elective ileostomy takedown was performed 5 months later. She returned to the operating room for anastomotic revision and gastrostomy tube placement for intestinal dysmotility and prolonged ileus. Tube feedings were discontinued 3 months post-operatively. CONCLUSIONS: Gastroschisis with intestinal atresia is associated with increased hospital length of stay, longer duration of parenteral nutrition, more severe intestinal dysfunction, increased surgical complications, and higher mortality than gastroschisis or intestinal atresia alone. With associated colonic atresia, loss of a significant length of bowel due to necrosis, including the ileocecal region, is almost unavoidable. An already compromised blood supply to the atretic bowel is further impaired by a tight abdominal wall defect and postnatal increases in gravitational force. Complicated gastroschisis remains a complex surgical challenge requiring further discussion.

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.

Anatomy and embryology of abdominal wall defects.

Abdominal wall defects are one of the most frequently encountered human congenital anomalies. They are seen in as many as 1 in 2,000 live births with evidence to suggest that their incidence is increasing. While often discussed together abdominal wall defects consist mainly of two entities namely gastroschisis and omphalocele. There are marked differences in their theories of embryo-pathogenesis, clinical presentation/anatomy and overall outcomes. There is no clear consensus explaining the precise embryological mechanisms leading to the development of abdominal wall defects. Many clinicians and embryologists have attempted to explain the genesis of congenital abdominal wall defects because of failure of progression of various phases of normal embryonic development. This review summarizes the mechanisms involved in normal and abnormal development of the ventral abdominal wall leading to the development of gastroschisis and omphalocele.

Management of Abdominal Wall Defects.

Congenital abdominal wall defects vary from abdominal wall hernias to severe congenital structural anomalies that include gastroschisis, omphalocele, and prune belly syndrome. The conditions often carry various associated anomalies and require multidisciplinary treatment approaches. Complex surgical reconstructive techniques are frequently required and prenatal, perioperative, and long-term follow-up is critical to ensuring the best possible outcomes.

Comparison of three risk stratification scores in gastroschisis neonates: gastroschisis prognostic score, gastroschisis risk stratification index and complex gastroschisis.

PURPOSE: The aim of the study was to compare and evaluate the utility of three different risk stratification scores for gastroschisis neonates; simple/complex gastroschisis, gastroschisis prognostic score and risk stratification index. METHODS: Data of neonates born with gastroschisis between the years 1993 and 2015 were collected. The national registers and patient records of four Finnish University Hospitals were retrospectively reviewed. Logistic and linear regression analysis were performed to identify independent predictors for adverse outcomes. The efficacy of these prognostic methods was further assessed using ROC-curves and DeLong (1988) test. RESULTS: Gastroschisis risk stratification index was an acceptable predictor of in-hospital mortality, AUC 0.70, 95% CI 0.48-0.91, p = 0.049. Complex gastroschisis and gastroschisis prognostic score were able to predict short bowel syndrome, AUC 0.80, 95% CI 0.58-1.00, p = 0.012 and AUC 0.80, 95% CI 0.59-1.00, p = 0.012, respectively. CONCLUSION: There are three easily obtainable risk stratification scores for outcome prediction in gastroschisis patients, however, their predictive ability did not have a statistical difference in the present study. The Gastroschisis risk stratification index seemed to perform moderately well in mortality prediction.

Gastroschisis for the Gastroenterologist: Updates on Epidemiology, Management, and Outcomes.

Gastroschisis is a common congenital abdominal wall defect, likely influenced by environmental factors in utero, with increasing prevalence in the United States. Early detection of gastroschisis in utero has become the standard with improved prenatal care and screening. There are multiple surgical management techniques, though sutureless closure is being used more frequently. Postoperative feeding difficulty is common and requires vigilance for complications, such as necrotizing enterocolitis. Infants with simple gastroschisis are expected to have eventual catch-up growth and normal development, while those with complex gastroschisis have higher morbidity and mortality. Management requires collaboration amongst several perinatal disciplines, including obstetrics, maternal fetal medicine, neonatology, pediatric surgery, and pediatric gastroenterology for optimal care and long-term outcomes.

Gastroschisis prognostic score successfully identifies Brazilian newborns with high-risk gastroschisis.

BACKGROUND: The Gastroschisis Prognostic Score (GPS) stratifies patients as high or low risk based on the visual assessment of intestinal matting, atresia, perforation, or necrosis. Despite being a simple score, its applicability to low and middle-income countries (LMICs) remains unknown. We tested the hypothesis that GPS can predict outcomes in LMICs, by assessing the prognostic value of the GPS in a middle-income country. METHODS: This prospective study followed all newborns with gastroschisis in a Brazilian neonatal unit based in a public hospital from 2015-2019. Infants were stratified into low and high-risk cohorts based on the GPS. In addition to basic demographics, data collected included duration of parenteral nutrition (TPN), mechanical ventilation (MV), length of stay (LOS), suspicion of infection that led to the use of antibiotics, and mortality. Univariate and multivariate analyses were conducted to identify which outcomes the GPS independently predicted. RESULTS: Sixty-one newborns with gastroschisis were treated during the study period. The mean birth weight, gestational age, and 5' Apgar score were 2258 g, 36 weeks, and 9. Twenty-four infants (39.3%) were identified as low-risk (GPS < 2) and 37 (60.7%) as high-risk (GPS > 2). The high-risk group presented with prolonged TPN use (p<0.001), MV (p<0.001), and LOS (p:0.002). GPS did not predict antibiotic therapy or mortality. CONCLUSION: In the first study in a middle-income country, the GPS predicted several important clinical outcomes. The GPS is a reliable tool for parental counseling and resource allocation in diverse settings. LEVEL OF EVIDENCE: II.c (cohort prospective).

Outcome and management in neonates with gastroschisis in the third millennium-a single-centre observational study.

Gastroschisis is one of the most common congenital malformations in paediatric surgery. However, there is no consensus regarding the optimal management. The aims of this study were to investigate the management and outcome and to identify predictors of outcome in gastroschisis. A retrospective observational study of neonates with gastroschisis born between 1999 and 2020 was undertaken. Data was extracted from the medical records and Cox regression analysis was used to identify predictors of outcome measured by length of hospital stay (LOS) and duration of parenteral nutrition (PN). In total, 114 patients were included. Caesarean section was performed in 105 (92.1%) at a median gestational age (GA) of 36 weeks (range 29-38) whereof (46) 43.8% were urgent. Primary closure was achieved in 82% of the neonates. Overall survival was 98.2%. One of the deaths was caused by abdominal compartment syndrome and one patient with intestinal failure-associated liver disease died from sepsis. None of the deceased patients was born after 2005. Median time on mechanical ventilation was 22 h. Low GA, staged closure, intestinal atresia, and sepsis were independent predictors of longer LOS and duration on PN. In addition, male sex was an independent predictor of longer LOS. CONCLUSION: Management of gastroschisis according to our protocol was successful with a high survival rate, no deaths in neonates born after 2005, and favourable results in LOS, duration on PN, and time on mechanical ventilation compared to other reports. Multicentre registry with long-term follow-up is required to establish the best management of gastroschisis. WHAT IS KNOWN: • Gastroschisis is one of the most common congenital malformations in paediatric surgery with increasing incidence. • There is no consensus among clinicians regarding the optimal management of gastroschisis. WHAT IS NEW: • Although primary closure was achieved in 82% of the patients, mortality rate was very low (1.8%) with no deaths in neonates born after 2005 following the introduction of measurement of intraabdominal pressure at closure. • Low gestational age, staged closure, intestinal atresia, sepsis, and male sex were independent predictors of longer length of hospital stay.

Gastroschisis: A Successful, Prospectively Evaluated Treatment Model in a Middle-Income Country.

BACKGROUND: This research adopted a care protocol from high-income countries in a level II/III hospital in a middle-income country to decrease morbidity and mortality associated with gastroschisis. METHODS: We established a multidisciplinary protocol to treat patients with gastroschisis prospectively from November 2012 to November 2018. This included prenatal diagnosis, presence of a neonatologist and pediatric surgeon at birth, and either performing primary closure on the patients with an Apgar score of 8/9, mild serositis, and no breathing difficulty or placing a preformed silo, when unable to fulfill these criteria, under sedation and analgesia (no intubation) in the operating room or at the patients' bedside. The subsequent management took place in the neonatal intensive care unit. The data were analyzed through the Mann-Whitney and Student's t-distribution for the two independent samples; the categorical variables were analyzed through a chi-square distribution or Fisher's exact test. RESULTS: In total, 55 patients were included in the study: 33 patients (60%) were managed with a preformed silo, whereas 22 patients (40%) underwent primary closure. Prenatal diagnosis (P = 0.02), birth at the main hospital (P = 0.02), and the presence of a pediatric surgeon at birth (P = 0.04) were associated with successful primary closure. The primary closure group had fewer fasting days (P < 0.001) and a shorter neonatal intensive care unit length of stay (P = 0.025). The survival rate was 92.7% (51 patients). CONCLUSION: The treatment model modified to fit the means of our hospital proved successful.

Predicting Factors of Protracted Intestinal Failure in Children with Gastroschisis.

OBJECTIVE: To identify prenatal and neonatal predictors of short bowel syndrome-related intestinal failure (SBS-IF) in gastroschisis. STUDY DESIGN: This retrospective study included all patients with gastroschisis born between 2000 and 2017 who were enrolled in our home parenteral nutrition program, and all patients with gastroschisis born in our institution who survived 2 weeks, during the same time period. Prenatal ultrasound features, neonatal status, anatomic features, oral feeding, and parenteral nutrition dependency were analyzed. RESULTS: Among 180 patients, 35 required long-term parenteral nutrition (SBS-IF group) and 145 acquired full oral feeding within 6 months (oral feeding group). The mean follow-up was 7.9 years (IQR, 1.6-17.5 years) and 5.0 years (IQR, 0.1-18.2 years), respectively. Both bowel matting (OR, 14.23; 1.07-16.7; P = .039) and secondarily diagnosed atresia or stenosis (OR, 17.78; 3.13-100.98; P = .001) were independent postnatal predictors of SBS-IF. Eighteen children (51% of the SBS-IF group) were still dependent on artificial nutrition at the last follow-up. patients with SBS-IF who achieved full oral feeding had a median residual small-bowel length of 74 cm (IQR, 51-160 cm) vs 44 cm (IQR, 10-105 cm) for those still dependent on artificial nutrition (P = .02). An initial residual small bowel length of more than 50 cm was the best predictive cut-off for nutritional autonomy, with a sensitivity of 67% and a specificity of 100%. CONCLUSIONS: Bowel matting, complex gastroschisis, and secondary intestinal obstruction were associated with SBS-IF in gastroschisis. For patients with SBS-IF, a small bowel length of more than 50 cm was predictive of secondary nutritional autonomy.

The medical and surgical management of gastroschisis.

Gastroschisis (GS) is a full-thickness abdominal wall defect in which fetal intestine herniates alongside the umbilical cord into the intrauterine cavity, resulting in an intestinal injury of variable severity. An increased prevalence of gastroschisis has been observed across several continents and is a focus of epidemiologic study. Prenatal diagnosis of GS is common and allows for delivery planning and treatment in neonatal intensive care units (NICUs) by collaborative interdisciplinary teams (neonatology, neonatal nursing and pediatric surgery). Postnatal treatment focuses on closure of the defect, optimized nutrition, complication avoidance and a timely transition to enteral feeding. Babies born with complex GS are more vulnerable to complications, have longer and more resource intensive hospital stays and benefit from standardized care pathways provided by teams with expertise in managing infants with intestinal failure. This article will review the current state of knowledge related to the medical and surgical management and outcomes of gastroschisis with a special focus on the role of the neonatologist in supporting integrated team-based care.

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)