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Associated abnormalities in patients with omphalocele and gastroschisis
Source publication
Unlabelled:
Gastroschisis and omphalocele are the most common malformation of the anterior abdominal wall.The aim of the study was to determine the abdominal wall defect frequencies, survival, and mortalities in Ahvaz, Khuzestan province of Iran.Materiał and methods. All cases born with omphalocele or gastroschisis whom born in Imam Khomeini hospi...
Similar publications
An omphalocele, a fetal abdominal defect, is a very important congenital anomaly. Prenatal diagnosis of fetal omphalocele is crucial to clinical management.
To investigate the accuracy of prenatal diagnosis for fetal omphalocele, we undertook a retrospective and consecutive analysis of our ultrasound database between January 1994 and December 2011....
Background:
Although primary closure of the gastroschisis is possible in many cases, there have been various strategies published and materials used to cover the eviscerated bowel when the abdominal wall defect cannot be closed in one step, providing bowel protection and reduction of heat and fluid loss. There have been suggestions of coverage mat...
Gastroschisis is a congenital malformation of the abdominal wall and may be associated with significant neonatal mortality and morbidity. The primary objective of this study was to describe the neurodevelopmental outcomes of neonates with this condition.
Medical records of all neonates admitted with a diagnosis of gastroschisis to a tertiary surgic...
Patient: Female, 33
Final Diagnosis: Intramamniotic hemorrhage
Symptoms: Abdominal pain • uterine contractions • vaginal bleeding
Medication: —
Clinical Procedure: Cesarean delivery
Specialty: Obstetrics and Gynecology
Objective
Unusual clinical course
Background
A spontaneous intra-amniotic hemorrhage is rarely encountered during pregnancy. The...
Citations
... As a result of insufficient and delayed care, LMIC pediatric patients fare substantially worse after surgery than those in HIC. For example, surgical congenital anomalies have mortality rates as high as 80% in LMICs and many times higher than that of HIC, which is consistently under 10% [6,33,34]. In Nigeria, neonatal surgical mortality reaches 26�2%, with mortality from gastroschisis at 58�3%, esophageal atresia at 56�5%, and intestinal atresia at 37�2%, based on a recent prospective cohort study of 17 tertiary hospitals [35]. ...
The unmet need for pediatric surgery imposes enormous health and economic consequences globally, predominantly shouldered by Sub-Saharan Africa (SSA) where children comprise almost half of the population. Lack of knowledge about the economic impact of improving pediatric surgical infrastructure in SSA inhibits the informed allocation of limited resources towards the most cost-effective interventions to bolster global surgery for children. We assessed the cost-effectiveness of installing and running two dedicated pediatric operating rooms (ORs) in a hospital in Nigeria with a pre-existing pediatric surgical service by constructing a decision tree model of pediatric surgical delivery at this facility over a year, comparing scenarios before and after the installation of the ORs, which were funded philanthropically. Health outcomes measured in disability-adjusted life years (DALYs) averted were informed by the hospital’s operative registry and prior literature. We adopted an all healthcare payor’s perspective including costs incurred by the local healthcare system, the installation (funded by the charity), and patients’ families. Costs were annualized and reported in 2021 United States dollars ($). The incremental cost-effectiveness ratios (ICERs) of the annualized OR installation and operation were presented. One-way and probabilistic sensitivity analyses were performed. We found that installing and operating two dedicated pediatric ORs averted 538 DALYs and cost $177,527 annually. The ICER of the ORs’ installation and operation was $330 per DALY averted (95% uncertainty interval [UI] 315–336) from the all healthcare payor’s perspective. This ICER was well under the cost-effectiveness threshold of the country’s half-GDP per capita in 2020 ($1043) and remained cost-effective in one-way and probabilistic sensitivity analyses. Installation of additional dedicated pediatric operating rooms in Nigeria with pre-existing pediatric surgical capacity is therefore very cost-effective, supporting investment in children’s global surgical infrastructure as an economically sound intervention.
... The outcomes were better as compared to a recent study from Iran with only 25% survival rate. [17] In our study, the outcome of preterm patients, associated intestinal atresia, presence of edematous bowel with leathery peel, patients requiring silo due to VAD, NEC, and associated malformations was poor. This is also consistent with previously published series. ...
Background: Gastroschisis is one of the common congenital anterior abdominal wall defects with uncovered abdominal contents (usually intestines) protruding through it. Immediate reduction of the abdominal contents is crucial after birth because of the grave consequences of delayed management. The aim of our study was to evaluate the early outcomes of Gastroschisis at a tertiary care institute in India. Methods: This prospective observational study was undertaken over a one-year duration extending from January to December 2021 at our pediatric tertiary care teaching institute. Results: There were 30 male and 28 female patients, out of which 37 were preterm neonates. The mean birth weight was 2019±357g. Seven patients (12.07%) had major associated malformations. Fifty (86.21%) patients underwent primary skin flap closure. Staged reduction with silo was accomplished in five (8.62%) patients; three patients died during resuscitation before any therapeutic procedure could be undertaken. Among 55 (94.83%) patients with surgical procedures, only 26 (47.27%) could be salvaged with overall favorable (survival) outcomes in 44.83% of patients. Mortality was high (92.59%, 25/27) in the patients presenting with markedly edematous bowel with leathery peel. Seventeen (29.31%, 17/58) neonates died in the first 72 hours (postoperatively) due to complications of abdominal compartment syndrome, eight (13.79%) patients died due to postoperative sepsis with thrombocytopenia, and two (3.44%) had intestinal perforation. The duration of hospital stay in neonates who survived ranged from one to four weeks. Conclusion: Overall survival rates in our study were 44.83% markedly in contrast to the series published in the recent literature. The outcome of preterm (premature) patients, associated intestinal atresia, presence of edematous bowel with leathery peel, patients requiring silo due to viscero-abdominal disproportion, necrotizing enterocolitis, and associated malformations, was dismal.
... For example, surgical congenital anomalies have mortality rates as high as 80% in LMICs and many times higher than that of HIC, which is consistently under 10%. (6,28,29) In Nigeria, neonatal surgical mortality reaches 26·2%, with mortality from gastroschisis at 58·3%, esophageal atresia at 56·5%, and intestinal atresia at 37·2%, based on a recent prospective cohort study of 17 tertiary hospitals. (30) Nigeria is one of few LMICs that have published a national surgical, obstetric, anesthesia, and nursing plan after the World Health Assembly mandated that countries provide essential surgical care and anesthesia as part of their universal health coverage package.(31,32) ...
The unmet need for pediatric surgery incurs enormous health and economic consequences globally, predominantly shouldered by Sub-Saharan Africa (SSA) where children comprise almost half of the population. Lack of economic impact data on improving pediatric surgical infrastructure in SSA precludes informed allocation of limited resources towards the most cost-effective interventions to bolster global surgery for children. We assessed the cost-effectiveness of installing and maintaining a pediatric operating room in a hospital in Nigeria with a pre-existing pediatric surgical service by constructing a decision tree model of pediatric surgical delivery at this facility over a year, comparing scenarios before and after the installation of two dedicated pediatric operating rooms (ORs), which were funded philanthropically. Health outcomes measured in disability-adjusted life years (DALYs) averted were informed by the hospital’s operative registry and prior literature. A societal perspective included costs incurred by the hospital system, charity, and patients’ families. Costs were annualized and reported in 2021 United States dollars ($). The incremental cost-effectiveness ratios (ICERs) of the annualized OR installation were presented from charity and societal perspectives. One-way and probabilistic sensitivity analyses were performed. We found that the installation and maintenance of two pediatric operating rooms averted 1145 DALYs and cost $155,509 annually. Annualized OR installation cost was $87,728 (56% of the overall cost). The ICER of the OR installation was $152 per DALY averted (95% uncertainty interval [UI] 147-156) from the societal perspective, and $77 per DALY averted (95% UI 75-81) from the charity perspective. These ICERs were well under the cost-effectiveness threshold of the country’s half-GDP per capita in 2020 ($1043) and remained cost-effective in one-way and probabilistic sensitivity analyses. Installation of additional pediatric operating rooms in SSA with pre-existing pediatric surgical capacity is therefore very cost-effective, supporting investment in children’s global surgical infrastructure as an economically sound intervention.
... Studies have consistently shown that young women -with a mean age of 20 years -are more likely of delivering a fetus with gastroschisis when compared to the general obstetric population [24,25]. This can be dependent on lifestyle-related factors in this population including smoking, illicit drug use, alcohol consumption, in addition to low socioeconomic status, gestational age, prematurity and low weight [20,25,37]. Although many risk factors have been proposed, studies indicate that low maternal age is the strongest, clearest and most reproductive risk factor for gastroschisis [20,25,26]. ...
This is an integrative review aimed to analyze scientific evidence about the profile of newborns with gastroschisis and the implications to nursing care. Data was collected in virtual health databases such as BVS, MEDLINE/PUBMED and CINAHL between 2004 and 2016. We analyzed 19 publications that fit the established criteria. The characteristics of newborns with gastroschisis were grouped into four categories: (i) risk factors, prenatal diagnosis, maternal variables and neonatal profile for gastroschisis; (ii) clinical and surgical treatment and newborn care for gastroschisis; (iii) intestinal complications and postoperative of the newborn with gastroschisis; (iv) prognosis of the newborn with gastroschisis and the implications to the nursing care. The presence of this malformation compromises neonatal prognosis, and it is associated with obstetric and neonatal complications, like prematurity and low weight at birth, intestinal complications, sepsis and prolonged hospitalization. Data in the literature has shown that nursing care in gastroschisis is poorly documented. This highlights the necessity of developing more researches to sustain the systematization of nursing care to meet these newborn needs.
... Abdominal wall hernia is one of the most common diseases. Many studies have been conducted to find out its prevalence [1][2][3][4][5]. It varies from 3.9% to 18.3%, but this data mainly concerns groin hernias while, to our knowledge, there have been no epidemiological studies to reveal the overall prevalence of abdominal wall hernias, including umbilical and other types, in the general population. ...
... What makes our study different is that it was population-based, had a cross-sectional design and we examined the majority of residents that lived in the same settlement. The previously published studies were based not on examination of an entire population, but on registers of patients operated on for hernias, on data obtained from people who had visited primary care physicians, on databases of insurance companies and even on a simple extrapolation of available data [1,[3][4][5]9]. ...
Purpose
To find out the prevalence and risk factors for abdominal wall hernias in the general Russian population.
Methods
783 residents of Kryukovo rural community (Central Russia) of 10 > years were enrolled. Medical history was taken and clinical examination was performed documenting hernia presence. Ultrasound examination was performed in inconclusive situations. We considered residents with hernias at the time of examination, as well as those who had previously undergone hernia repair, to be hernia positive.
Results
There were 298 (38%) men and 485 (62%) women (mean age 49.3). Abdominal wall hernias have been found in 20.9% of population. 31.2% of men and 14.6% of women were affected (p < 0.0001). Umbilical hernias was found in 10.2%, groin hernias in 8.3%, incisional in 2.4% of residents. 72% of individuals with hernia reported local pain. 70% had never asked for medical advice before, 30% had undergone hernia repair before. Male gender, age, family history were confirmed as risk factors for any hernia with OR 3.9, 1.03 and 2.7, respectively (p < 0.01). For groin hernias male gender, age, family history and rough labour were confirmed with ORs 9.2, 1.03, 2.9 (p < 0.01) and 2.1 (p < 0.05). Risk factors for umbilical hernias were male gender with OR 2.2 (p < 0.01), age, family history and BMI with ORs 1.02, 1.8 and 1.08 (p < 0.05).
Conclusions
This study is the first to demonstrate prevalence of abdominal wall hernias and its risk factors in Russia. The results confirm that hernias are common in a general population pointing the need for improvement of surgical activity.
... Gastroschisis cases presented less frequently, but the condition is more difficult to manage in our setting and hence has a lower survival of just 12%. These findings are similar to those of Askarpour et al. [35] who also reported a higher prevalence of omphalocele than gastroschisis at their hospital, but a higher mortality rate from neonates undergoing surgery for gastroschisis than omphalocele. ...
Background
Congenital anomalies have risen to become the fifth leading cause of under-five mortality globally. The majority of deaths and disability occur in low- and middle-income countries including Ghana. This 3-year retrospective review aimed to define, for the first time, the characteristics and outcomes of neonatal surgical conditions in northern Ghana.
Methods
A retrospective study was conducted to include all admissions to the Tamale Teaching Hospital (TTH) neonatal intensive care unit (NICU) with surgical conditions between January 2014 and January 2017. Data were collected on demographics, diagnosis and outcomes. Descriptive analysis was performed on all data, and logistic regression was used to predict determinants of neonatal mortality. p < 0.05 was deemed significant.
Results
Three hundred and forty-seven neonates were included. Two hundred and sixty-one (75.2%) were aged 7 days or less at presentation, with males (n = 177, 52%) slightly higher than females (n = 165, 48%). The majority were delivered by spontaneous vaginal delivery (n = 247, 88%); 191 (58%) were born in hospital. Congenital anomalies accounted for 302 (87%) of the neonatal surgical cases and 45 (96%) deaths. The most common anomalies were omphalocele (n = 48, 13.8%), imperforate anus (n = 34, 9.8%), intestinal obstruction (n = 29, 8.4%), spina bifida (n = 26, 7.5%) and hydrocephalus (n = 19, 5.5%). The overall mortality rate was 13.5%. Two-thirds of the deaths (n = 30) from congenital anomalies were conditions involving the digestive system with gastroschisis having the highest mortality of 88%. Omphalocele (n = 11, 23.4%), gastroschisis (n = 7, 14.9%) and imperforate anus (n = 6, 12.8%) contributed to the most deaths. On multivariate analysis, low birthweight was significantly associated with mortality (OR 3.59, CI 1.4–9.5, p = 0.009).
Conclusion
Congenital anomalies are a major global health problem associated with high neonatal mortality in Ghana. The highest burden in terms of both caseload and mortality is attributed to congenital anomalies involving the digestive system, which should be targeted to improve outcomes.
... Introduction ccording to a report by the World Health Organization (WHO) in 2015, globally, congenital anomalies among Down's syndrome and neural tube defects (NTDs) are major causes of stillbirths, neonatal death, long-term illness, disability and childhood health problems (1). Despite powerful strides forward in the prenatal diagnosis of congenital anomalies in western countries, mortality rates from hospital-based data in low-and middle-income countries, particularly Iran, for common anomalies have risen to 20-85% (2,3). It is also well demonstrated that the risk of theses congenital anomalies increases significantly with the mother's age; the risk is even higher after the age of 35 (4). ...
Background:
The purpose of the study was to evaluate the use of contingent prenatal screening for the detection of Down's syndrome and neural tube defects (NTDs) in west of Iran.
Methods:
A prospective study was conducted on 653 pregnant women referred to a medical diagnostic laboratory (Imam Reza Clinic, Kermanshah, Iran) for contingent prenatal screening tests between October 2016 to September 2017.
Results:
Among 651 women screened in the first trimester, 8 (1.22%) pregnancies were screen-positive for Down's syndrome. In the second trimester, among 605 women, 25 (4.13%) had a positive result and all of these women voluntarily underwent amniocentesis. Overall, five pregnancies were complicated with chromosomal abnormalities, including five cases of Down's syndrome.
Conclusion:
In a nutshell, the contingent prenatal screening tests were found to be useful for estimation of Down's syndrome as well as NTDs in both young and older mothers in west of Iran. These tests should be performed for pregnant women before an invasive test for Down's syndrome.
... Mortality from GS in low-to-middle-income countries (LMICs) can reach up to 80-100% [45][46][47][48] which is in sharp contrast to the <10% in HICs [49]. Sepsis is a major culprit in most cases of neonatal mortality in LMICs [48]. ...
... Mortality from GS in low-to-middle-income countries (LMICs) can reach up to 80-100% [45][46][47][48] which is in sharp contrast to the <10% in HICs [49]. Sepsis is a major culprit in most cases of neonatal mortality in LMICs [48]. The Gastroschisis International (GiT) network has suggested that poor resuscitation combined with sepsis and abdominal compartment syndrome is directly linked to the poor outcome [50]. ...
Gastroschisis (GS) is one of the congenital abdominal wall defects, in which the bowel has prolapsed without a covering through a defect adjacent to (and nearly always to the right of) an otherwise normal umbilicus. Proper management of such cases gives them the opportunity to survive and thrive. In this chapter, simplified flowcharts for the initial management of GS, surgical intra-operative decisions and post-operative active follow-up of such cases will be presented and discussed. The first flowchart will discuss how to deal with a GS case from birth till the operative theatre, while the second flowchart will take the lead to guide the surgeon with the available surgical options and how to choose the suitable one for the case. Finally, the post-operative active follow-up fluid management and possible complications are discussed.
... Recent literature reports mortality rates of 80%, Iran; 36%, Turkey; and 6%-8%, Thailand. [8][9][10][11] In low-income countries, mortality remains high, with many SSA countries reporting mortality rates of 75%-100%. [12][13][14][15] Management of gastroschisis varies widely. ...
Introduction
There is a significant disparity in outcomes for neonates with gastroschisis in high-income countries (HICs) compared with low-income and middle-income countries (LMICs). Many LMICs report mortality rates between 75% and 100% compared with <4% in HICs.
Aim
To undertake a systematic review identifying postnatal interventions associated with improved outcomes for gastroschisis in LMICs.
Methods and analysis
Three search strings will be combined: (1) neonates; (2) gastroschisis and other gastrointestinal congenital anomalies requiring similar surgical care; (3) LMICs. Databases to be searched include MEDLINE, EMBASE, Scopus, Web of Science, ProQuest Dissertations and Thesis Global, and the Cochrane Library. Grey literature will be identified through Open-Grey, ClinicalTrials.gov, WHO International Clinical Trials Registry and ISRCTN registry (Springer Nature). Additional studies will be sought from reference lists of included studies. Study screening, selection, data extraction and assessment of methodological quality will be undertaken by two reviewers independently and team consensus sought on discrepancies. The primary outcome of interest is mortality. Secondary outcomes include complications, requirement for ventilation, parenteral nutrition duration and length of hospital stay. Tertiary outcomes include service delivery and implementation outcomes. The methodology of the studies will be appraised. Descriptive statistics and outcomes will be summarised and discussed.
Ethics and dissemination
Ethical approval is not required since no new data are being collected. Dissemination will be via open access publication in a peer-reviewed medical journal and distribution among global health, global surgery and children’s surgical collaborations and international conferences.
Conclusion
This study will systematically review literature focused on postnatal interventions to improve outcomes from gastroschisis in LMICs. Findings can be used to help inform quality improvement projects in low-resource settings for patients with gastroschisis. In the first instance, results will be used to inform a Wellcome Trust-funded multicentre clinical interventional study aimed at improving outcomes for gastroschisis across sub-Saharan Africa.
PROSPERO registration number
CRD42018095349.
... Recent literature reports mortality rates of 80%, Iran; 36%, Turkey; and 6%-8%, Thailand. [8][9][10][11] In low-income countries, mortality remains high, with many SSA countries reporting mortality rates of 75%-100%. [12][13][14][15] Management of gastroschisis varies widely. ...
Background: Gastroschisis has a great disparity in outcomes globally with less than 4% mortality in high-income countries (HICs) and over 90% mortality in many tertiary paediatric surgery centres across sub-Saharan Africa (SSA), including the study centres. Low-technology, cost-effective, evidence-based interventions are available with the potential for improved outcomes in low-resource settings.
Aim: To develop, implement and prospectively evaluate an interventional care bundle to reduce mortality from gastroschisis in seven tertiary paediatric surgery centres across SSA.
Study Design: A hybrid type 2 effectiveness-implementation study design will be utilised, which focusses equally on the effectiveness of the intervention through analysing clinical outcomes and effectiveness of the implementation through analysis of service delivery and implementation outcomes.
Methods: Using current literature an evidence-based, low-technology interventional care bundle has been developed with both pre-hospital and in-hospital components. During 2018 a systematic review, qualitative study and Delphi process will provide further evidence in order to optimise the interventional bundle and implementation strategy for use in low-resource environments. The interventional care bundle has core components, which will remain consistent across the seven sites, and adaptable components, which will be determined through in-country co-development meetings with all relevant members of the multi-disciplinary team. Across the seven study sites it is estimated that 180-289 patients will be included in the study, which has the potential to determine a difference in pre- and post-intervention mortality between 10-15%.
Implementation will take place over a 4-week period at each of the study sites between May - December 2019. Pre- and post-interventional data will be collected on clinical, service delivery and implementation outcomes between October 2018 to November 2020 (2-years of patient recruitment at each site with a 30-day post primary intervention follow-up period). The primary clinical outcome will be all-cause, in-hospital mortality. Secondary outcomes include the occurrence of a major complication, length of hospital stay and time to full enteral feeds. Service delivery outcomes include time to hospital and primary intervention and adherence to the pre-hospital and in-hospital management protocols. Implementation outcomes will include acceptability, adoption, appropriateness, feasibility, fidelity, coverage, cost and sustainability.
Expected Results: Pre- and post-intervention clinical outcomes will be compared using Chi-squared analysis, unpaired t-test and/ or Mann-Whitney test. Time-series analysis will be undertaken using Statistical Process Control to identify significant trends and shifts in outcome overtime. Multivariate logistic regression analysis will be used to identify both clinical and implementation factors affecting outcome with adjustment for confounders. P<0.05 will be deemed significant.
Outcome: This will be the first multi-centre interventional study to the investigator's knowledge aimed at reducing mortality from gastroschisis in low-resource settings. If successful, detailed evaluation of both the clinical and implementation components of the study will allow sustainability in the study sites and further scale-up.
ClinicalTrials.gov Registration: https://clinicaltrials.gov/ct2/show/NCT03724214
