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Between February 1994 and April 2004, we treated 40 children with gastroschisis and 26 children with omphalocele. We recorded the course of pregnancy, pre- and post-natal complications, delivery, operation, post-operative therapy, and long-term outcomes. Additionally, we conducted follow-up examinations of 37 of these 66 children (56%). We analysed...
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Context 1
... prolapsed organs included the stomach (5/40, 13%), urinary bladder or genital organs (2/ 40, 5%), and liver (1/40, 2.5%). There were associated anomalies in 11/40 (28%), all confined to the gastrointes- tinal tract (Table 1). ...
Similar publications
The outcome data of 132 patients treated at the Department of Pediatric Surgery in Mainz during the last 25 years were reviewed. Prenatal diagnosis of abdominal wall defects (AWDs) and associated malformations led to increasing selection of the patient population. The aim of primary closure of the abdominal wall can be achieved more frequently in g...
Citations
... Осложнённые формы ГШ не являются противопоказанием к выбору данной методики [41]. Её эффективность составляет 50-83% [42,43]. Преимуществами её являются предотвращение дальнейшего воспаления стенки кишки и профилактика патологических потерь жидкости с поверхности органов [44]. ...
Gastroschisis is a congenital malformation of the anterior abdominal wall with a defect to the right of the normally formed umbilical cord and eventration of the abdominal organs. Evented internal organs not covered with amniotic membrane are under the direct influence of amniotic fluid. Despite the increase in the incidence of the defect from 3.6 to 4.9 per 10,000 live births, over the past ten years mortality has sharply decreased to 5%. Antenatal diagnosis makes it possible to reliably identify cases of gastroschisis, starting from the 12th week of gestation, which is important especially when diagnosing complicated forms. Surgical correction involves primary radical plastic surgery of the anterior abdominal wall with simultaneous immersion of the eventrated organs into the abdominal cavity. However, in approximately every fifth patient, immediate correction is impossible due to the occurrence of viscero-abdominal disproportion and concomitant diseases. In these cases, temporary abdominal cavity occurs with delayed plasty of the anterior abdominal wall, enterostomy for intestinal decompression, resection of non-viable areas and interintestinal anastomoses. Among patients with complicated forms, there was an 8-fold increase in mortality. Despite significant advances in reducing mortality, the development of various treatment methods continues to provide an alternative method of correcting this lesion.
... Although omphalocele can occur as isolated anomalies, 35-80% of these defects are associated with other structural abnormalities [4][5][6][7][8]. Associated abnormalities include cardiac defects (e.g., ventricular septal defects, tetralogy of Fallot, or dextrocardia), genitourinary abnormalities (e.g., renal or bladder hypoplasia, multicystic nephropathies, hydronephrosis, or ureteral strictures/ duplication/ectopic placement), musculoskeletal disorders, gastrointestinal abnormalities (such as malrotation, intestinal or anal atresia), orofacial clefts, neural tube defects, and diaphragmatic defects [3,[8][9][10]. ...
... Although omphalocele can occur as isolated anomalies, 35-80% of these defects are associated with other structural abnormalities [4][5][6][7][8]. Associated abnormalities include cardiac defects (e.g., ventricular septal defects, tetralogy of Fallot, or dextrocardia), genitourinary abnormalities (e.g., renal or bladder hypoplasia, multicystic nephropathies, hydronephrosis, or ureteral strictures/ duplication/ectopic placement), musculoskeletal disorders, gastrointestinal abnormalities (such as malrotation, intestinal or anal atresia), orofacial clefts, neural tube defects, and diaphragmatic defects [3,[8][9][10]. Routine fetal echocardiography is recommended in omphalocele cases because of the substantially increased risk of structural cardiac abnormalities [11]. ...
... However, in a Dutch collaborative survey., 77% of children born with omphalocele eventually received post-secondary education, and the proportion is considered similar to the national population [43]. Over 93% of children with a history of omphalocele attended kindergarten at the expected age [8]. These studies showed that despite initial difficulties, most children with omphalocele eventually achieve age-appropriate neurodevelopment. ...
Background
Patients with omphalocele, a midline abdominal wall defect at the umbilical cord base, have a low survival rate. However, the long-term outcomes of fetuses with prenatally diagnosed omphalocele have scarcely been studied. Therefore, we investigated the ultrasonographic features, genetic characteristics, and maternal and fetal outcomes of fetuses with omphalocele and provided a reference for the perinatal management of such cases.
Methods
A total of 120 pregnant females with fetal omphalocele were diagnosed using prenatal ultrasonography at the Fujian Provincial Maternity and Child Health Hospital from January 2015 to March 2022. Amniotic fluid or cord blood samples were drawn at different gestational weeks for routine karyotype analysis, chromosomal microarray analysis (CMA) detection, and whole exome sequencing (WES). The maternal and fetal outcomes were followed up.
Results
Among the 120 fetuses, 27 were diagnosed with isolated omphalocele and 93 with nonisolated omphalocele using prenatal ultrasonography. Cardiac anomalies were the most observed cause in 17 fetuses. Routine karyotyping and CMA were performed on 35 patients, and chromosomal abnormalities were observed in five patients, trisomy 18 in three, trisomy 13 in one, and chromosome 8–11 translocation in one patient; all were non-isolated omphalocele cases. Six nonisolated cases had normal CMA results and conventional karyotype tests, and further WES examination revealed one pathogenic variant and two suspected pathogenic variants. Of the 120 fetuses, 112 were successfully followed up. Eighty of the 112 patients requested pregnancy termination. Seven of the cases died in utero. A 72% 1-year survival rate was observed from the successful 25 live births.
Conclusion
The prognosis of fetuses with nonisolated omphalocele varies greatly, and individualized analysis should be performed to determine fetal retention carefully. Routine karyotyping with CMA testing should be provided for fetuses with omphalocele. WES is an option if karyotype and CMA tests are normal. If the fetal karyotype is normal and no associated abnormalities are observed, fetuses with omphalocele could have a high survival rate, and most will have a good prognosis.
... Thirty-four patients were followed-up, with a median follow-up time of 24 months (range [11][12][13][14][15][16][17][18][19][20][21][22][23][24], and the outcomes are summarized in ►Table 4. Thirteen patients with gastroschisis (59.1%) and 8 patients with omphalocele (66.7%) had at least one adverse event during the followup. Ten patients (29.4%) needed further surgical interventions due to diverse comorbidities, and the details are given in ►Supplementary Table S1 of the supplementary material. ...
... Gastroschisis patients were born earlier and with lower birth weight than those with omphalocele, which is consistent with other studies. 11,23 All patients with gastroschisis and 84.6% of the patients with omphalocele were delivered by caesarean section. The observed rate for cesarean delivery was high, and this is mainly explained by the fact that a planned delivery in a tertiary care center, with access to appropriate neonatal and pediatric surgical services, is more readily achieved by caesarean section than by vaginal delivery. ...
... Our primary abdominal closure rate of 86.8% is similar to other published rates. 4,23,25 Staged repair with silo placement may be preferable for the unstable neonate, large defects and when gastroschisis is associated with significant intestinal damage. 4,7 In our study, one patient with a giant omphalocele and one patient with a large and complex gastroschisis developed compartment syndrome after primary closure and were converted to a staged reduction. ...
Objective To characterize and compare the outcomes of omphalocele and gastroschisis from birth to 2 years of follow-up in a recent cohort at a tertiary center.
Methods This is a retrospective clinical record review of all patients with gastroschisis and omphalocele admitted to the Neonatal Intensive Care Unit between January 2009 and December 2019.
Results There were 38 patients, 13 of whom had omphalocele, and 25 of whom had gastroschisis. Associated anomalies were present in 6 patients (46.2%) with omphalocele and in 10 (41.7%) patients with gastroschisis. Compared with patients with omphalocele, those with gastroschisis had younger mothers (24.7 versus 29.6 years; p = 0.033), were born earlier (36 versus 37 weeks, p = 0.006), had lower birth weight (2365 ± 430.4 versus 2944.2 ± 571.9 g; p = 0.001), and had a longer hospital stay (24 versus 9 days, p = 0.001). The neonatal survival rate was 92.3% for omphalocele and 91.7% for gastroschisis. Thirty-four patients were followed-up over a median of 24 months; 13 patients with gastroschisis (59.1%) and 8 patients with omphalocele (66.7%) had at least one adverse event, mainly umbilical hernia (27.3% vs 41.7%), intestinal obstruction (31.8% vs 8.3%), or additional surgical interventions (27.3% vs 33.3%).
Conclusion Despite the high proportion of prematurity, low birth weight, and protracted recovery, gastroschisis and omphalocele (without chromosomal abnormalities) may achieve very high survival rates; on the other hand, complications may develop in the first years of life. Thus, a very positive perspective in terms of survival should be transmitted to future parents, but they should also be informed that substantial morbidity may occur in the medium term.
... Time of exposure to meconium-filled amniotic fluid and potential bacterial translocation may explain this discrepancy of outcome. In the present study, the abdomen could be closed primarily in 66.6% of GS and 77.7% of OC new-borns, rates were comparable to literature, [24,25] we found no significantly advantages in secondary closures between silo-bag or patch. Our results support the statement of Maksoud-Filho et al., who found no distinction between primary closure, silo-bag or patch in terms of mortality, there was an extended parenteral nutrition and hospital stay in GS and OC children who were not primary closed. ...
Background:
Gastroschisis (GS) and omphalocele (OC) are congenital abdominal wall defects, the main difference between is the direct exposure of intestinal loops in amniotic fluid in children with a GS. This leads to a reduced primary closure rate and a higher number of intraoperative abnormalities and post-operative complications.
Aims and objectives:
We analysed abdominal wall defect patients over an 11-year period, aiming to assess the influence of meconium-contaminated amniotic fluid. This study has different objectives to show the consequence of functional outcome of abdominal wall defects (AWD) children in reliance to colour of amniotic fluid, to assess the effect of reduced bowel exposure time to meconium contaminated amniotic fluid on edematous inflammatory thickening of the bowel loops, to show an positively influence in the number of primary AWD closures, to demonstrate a reduced incidence of post-natal complications and to verify a better outcome of OC children because of failing exposure to amniotic fluid.
Methods:
A retrospective, observational case-control design was used to compare GS (n = 36) and OC (n = 18) children. Physical data, colour of amniotic fluid, pre- and perinatal problems, operative complications and surgical technique, post-operative complications, duration of intensive care unit (ICU) stay, mechanical ventilation, parenteral nutrition, commencement of oral feeding and total hospital stay were collected. Data were analysed with descriptive methods, t-test and non-parametric tests such as Wilcoxon and Kruskal-Wallis were performed in addition to the analysis of variance, including post hoc testing accepting a confidence interval of 95% (P < 0.05) by using IBM SPSS software, version 23 (IBM, Illinois, USA).
Results:
Rate of meconium-contaminated amniotic fluid is significantly higher in GS compared to OC (P < 0.001), delivery problems such as congenital infections are also significantly higher (P < 0.001), this yields in significantly more bowel loops anomalies and problems during surgery (P < 0.036) but had no significant influence on primary abdominal wall closures rate (P = 0.523). The post-surgical outcome of OC was significantly better as compared to GS. Within the GS, those with swollen intestines had significantly longer ICU stays (P = 0.045) due to extended mechanical ventilation (P = 0.007), parenteral nutrition (P = 0.011) and delayed initiation of oral feeding (P < 0.001. Same results were found for the duration of ICU stay (P = 0.008), mechanical ventilation (P = 0.006), parenteral nutrition (P = 0.011) and delayed initiation of oral feeding (P < 0.001) in secondary closures as compared to primary abdominal wall closures in the GS group.
Conclusions:
Worsen functional short-term outcome of GS children was directly addicted to meconium contamination of amniotic fluid due to swollen intestines and because of this more post-surgical problem including significantly extended hospital stays were observed.
... Omphaloceles are in a proportion of 35% to 70% associated with structural anomalies such as tetralogy of Fallot, dextrocardia, malrotation, intestinal atresia, ventricular septal defect, bladder or renal agenesis, ureteral stenosis orofacial clefts, diaphragmatic and neural tube defects [14][15][16]. 60% of non-liver-containing omphaloceles are linked to fetal aneuploidies such as trisomy 13, 18, or 21, triploids, Turner syndrome, and rare chromosomal deletions [17,18]. One-third of pregnancies with omphaloceles present polyhydramnios after the 20 th gestational week; fetal growth restriction is also associated with omphalocele [17,19]. ...
Omphalocele (exomphalos) represents one of the most frequent congenital abdominal wall defects. It presents as a defect of inconstant size and is located on the midline, at the base of the umbilical cord, the skin, fascia, and abdominal muscles being absent at this level. Omphaloceles are classified as liver-containing or non-liver-containing, the latter containing primarily bowel loops. We present the case of a 37-year-old pregnant woman with an early diagnosis of liver-containing omphalocele associating ductus venosus reversed flow, with the aim to highlight the importance of the first-trimester morphology scan and to develop a pilot study regarding the neurological development of infants after surgical repair of giant omphaloceles. The particularity of this case consists of a fetus with a positive diagnosis of a giant liver-containing omphalocele but with a small abdominal wall defect during the first-trimester morphology scan at 13 weeks and 3 days of gestation which associated ductus venosus reversed flow, presenting a normal karyotype postabortum. With a small defect, we can speculate the risk of strangling besides the mechanical traction exercised on the ductus venosus generating fetal distress, specifically fetal hypoxia at an early gestational age. In conclusion, the main issue, in this case, was if the fetal omphalocele and ductus venosus reversed flow indicated fetal hypoxia, what was the obstruction effect on the oxygenated blood pathway caused by the abdominal defect, and which were the long-term effects on infants with this complex pathology with an unknown outcome.
... 8 They require surgery soon after birth 9 and are exposed to several anesthetic and analgesic agents. 10,11 Studies assessing the childhood outcomes of these infants varied widely in their outcome measures and follow-up periods with some describing their overall health status or quality of life [12][13][14][15] and others evaluating their motor function [16][17][18][19] or behavior. 16,[20][21][22] Studies that specifically assessed their neurodevelopmental outcomes invariably relied on questionnaires 19 or different batteries of aptitude tests such as the Wechsler Intelligence Scale, [16][17][18][21][22][23] which are primarily designed to predict a child's ability to learn new skills. ...
... 10,11 Studies assessing the childhood outcomes of these infants varied widely in their outcome measures and follow-up periods with some describing their overall health status or quality of life [12][13][14][15] and others evaluating their motor function [16][17][18][19] or behavior. 16,[20][21][22] Studies that specifically assessed their neurodevelopmental outcomes invariably relied on questionnaires 19 or different batteries of aptitude tests such as the Wechsler Intelligence Scale, [16][17][18][21][22][23] which are primarily designed to predict a child's ability to learn new skills. On the other hand, we aimed to use performance on achievement tests, a measure intended to reflect what children are actually learning, 24 to compare the childhood academic performance of infants born with gastroschisis in to fetal death records using mother's name and child's date of death. ...
Objective
To compare third grade literacy and mathematics test proficiency among children born with gastroschisis vs. unaffected controls and identify predictors of academic proficiency among these children.
Study design
Infants born with gastroschisis (2000-2005) were identified from the Arkansas Reproductive Health Monitoring System. For each case, two controls were randomly selected from birth certificates and matched for hospital and month of birth, sex, and race/ethnicity. Data on re-hospitalization within the first 6 years and payer data were abstracted from the Arkansas Hospital Inpatient Discharge Database. Surviving cases and controls were linked to the Arkansas Department of Education database containing achievement test scores. The primary outcome was proficiency, defined as performance at or above grade level, on third grade achievement tests. Cases and controls who did not attend public schools could not be linked to education records. Multivariable logistic regression models evaluated the association between study characteristics and academic proficiency.
Results
The final study cohort included 47 cases and 63 controls. There was no statistically significant difference in rate of literacy (68% vs. 81%, P=0.65) or mathematics proficiency (89% vs 87%, P=0.15) between cases and controls. On multivariable analysis, a complex gastroschisis (defined as atresia, volvulus, necrosis, or perforation of the bowel) was associated with lower proficiency in literacy (AOR=0.1, 95% CI: 0.02, 0.58; P=0.01). No neonatal or maternal characteristics predictive of lower proficiency in mathematics were identified.
Conclusion
Among children born with gastroschisis, a complex gastroschisis was associated with lower proficiency on third grade literacy achievement tests.
... In total, 22 studies reported on paralytic ileus and entailed 1,332 patients and 120 events of paralytic ileus [35,42,46,48,49,51,61,65,81,82,96,98,100,103,109,112,114,115,132,151,153,155]. The pooled proportion of total paralytic ileus was 0.07 (95%-CI: 0.05-0.11; ...
Objectives
Ileus following surgery can arise in different forms namely as paralytic ileus, adhesive small bowel obstruction or as anastomotic stenosis. The incidences of these different forms of ileus are not well known after abdominal birth defect surgery in infants. Therefore, this review aims to estimate the incidence in general between abdominal birth defects.
Content
Studies reporting on paralytic ileus, adhesive small bowel obstruction or anastomotic stenosis were considered eligible. PubMed and Embase were searched and risk of bias was assessed. Primary outcome was the incidence of complications. A meta-analysis was performed to pool the reported incidences in total and per birth defect separately.
Summary
This study represents a total of 11,617 patients described in 152 studies of which 86 (56%) had a follow-up of at least half a year. Pooled proportions were calculated as follows; paralytic ileus: 0.07 (95%-CI, 0.05–0.11; I ² =71%, p≤0.01) ranging from 0.14 (95% CI: 0.08–0.23) in gastroschisis to 0.05 (95%-CI: 0.02–0.13) in omphalocele. Adhesive small bowel obstruction: 0.06 (95%-CI: 0.05–0.07; I ² =74%, p≤0.01) ranging from 0.11 (95% CI: 0.06–0.19) in malrotation to 0.03 (95% CI: 0.02–0.06) in anorectal malformations. Anastomotic stenosis after a month 0.04 (95%-CI: 0.03–0.06; I ² =59%, p=0.30) ranging from 0.08 (95% CI: 0.04–0.14) in gastroschisis to 0.02 (95% CI: 0.01–0.04) in duodenal obstruction. Anastomotic stenosis within a month 0.03 (95%-CI 0.01–0.10; I ² =81%, p=0.02) was reviewed without separate analysis per birth defect.
Outlook
This review is the first to aggregate the known literature in order approximate the incidence of different forms of ileus for different abdominal birth defects. We showed these complications are common and the distribution varies between birth defects. Knowing which birth defects are most at risk can aid clinicians in taking prompt action, such as nasogastric tube placement, when an ileus is suspected. Future research should focus on the identification of risk factors and preventative measures. The incidences provided by this review can be used in those studies as a starting point for sample size calculations.
... Ultrasound was used in 100% of the studies to diagnose gastroschisis in the prenatal period, with one study using morphological ultrasound [2] and another using three-dimensional ultrasound [33]. In seven studies, there were cases of fetuses that did not receive the diagnosis in the prenatal period (n ¼ 127) but at delivery [2,20,22,31,44,45,48]. ...
Objectives: The present systematic review aims to investigate the diagnosis, prognosis, delivery assistance, pregnancy results and postnatal management in gastroschisis.
Study design: The following data sources were evaluated: The CINAHL, Embase and MEDLINE/ PubMed databases were searched, observational and intervention studies published over the past 20 years. The quality of the studies was assessed using the Grading of Recommendations, Assessment, Development, and Evaluations (GRADE).
Results: A total of 3770 infants diagnosed with gastroschisis were included (44 studies); 1534 fetuses were classified as simple gastroschisis and 288 as complex gastroschisis. Intrauterine fetal demise occurred in 0.47% and elective termination occurred in 0.13%. Preterm delivery occurred in 23.23% and intrauterine growth restriction in 4.43%. Cesarean section delivery was performed in 54.6%. Neonatal survival was 91.29%. The main neonatal complications were: sepsis (11.78%), necrotizing enterocolitis (2.33%), short bowel syndrome (1.37%), bowel obstruction (0.79%), and volvulus (0.23%). Immediate surgical repair was performed in 80.1% with primary closure in
69%. The average to oral feeding was 33 (range: 11–124.5) days. Average hospital duration was 38 days and 89 days in neonates with simple and complex grastroschisis, respectively.
Conclusions: The present systematic review provides scientific data for counseling families with fetal gastroschisis.
... Primary reduction will often lead to higher intraabdominal pressure (IAP) compared to staged reduction. In 50-83% of cases a successful primary reduction without excessive increase of intraabdominal pressure can be achieved [36,37]. A staged reduction reduces duration of mechanical ventilation, risk of infection and time to enteral nutrition [38]. ...
Gastroschisis and omphalocele reflect the two most common abdominal wall defects in newborns. First postnatal care consists of defect coverage, avoidance of fluid and heat loss, fluid administration and gastric decompression. Definitive treatment is achieved by defect reduction and abdominal wall closure. Different techniques and timings are used depending on type and size of defect, the abdominal domain and comorbidities of the child. The present review aims to provide an overview of current treatments.
... However, in our series, there was a remarkable predominance of cesarean deliveries (98.8%). Our findings are discordant with the literature [16][17][18] because of our hospital protocols during the period of data collection. In the study by Henrich et al. [17], the average birth weight in the gastroschisis group was 2350 g, and that in the omphalocele group was 2980 g; the mean gestational ages at delivery were 36.2 and 38.8 weeks, respectively. ...
... Our findings are discordant with the literature [16][17][18] because of our hospital protocols during the period of data collection. In the study by Henrich et al. [17], the average birth weight in the gastroschisis group was 2350 g, and that in the omphalocele group was 2980 g; the mean gestational ages at delivery were 36.2 and 38.8 weeks, respectively. These data are very similar to ours, in which the mean birth weights were 2505 and 2982 g, respectively, which were significantly different (p < .001), ...
Background
An accurate estimated fetal weight (EFW) calculated with traditional formulae in cases of abdominal wall defects (AWDs) can be challenging. As a result of reduced abdominal circumference, fetal weight may be underestimated, which could affect prenatal management. Siemer et al. proposed a formula without the use of abdominal circumference, but it is not used in our protocols yet.
Objectives
Our aim was to evaluate the correlation of EFW and birth weight in fetuses with AWD by using Hadlock 1, Hadlock 2, and Siemer et al.’s formulae. Our secondary goal was to evaluate how often fetuses classified as small for gestational age (SGA) were in fact SGA at birth.
Study design
This was a retrospective cohort study of gestations complicated by gastroschisis and omphalocele at two tertiary-care centers in Brazil and Italy during an 8-year period. Of a total of 114 cases, 85 (44 cases of gastroschisis and 41 cases of omphalocele) met our criteria.
Results
The last prenatal scan was performed 5.2 (±4.1) days before birth. The mean gestational age at birth was 37.2 (±1.8) weeks. Correlation of EFW with birth weight was calculated with the three formulae with and without adjustment for weight gain between scan and birth, with the use of the Spearman coefficient. The correlation between EFW and weight at birth was positive according to all three formulae for the infants with gastroschisis. This finding was not confirmed in the infants with omphalocele. All formulae overestimated the number of SGA cases: although only 17.6% of fetuses were actually SGA at birth, the Hadlock formulae had classified nearly 35% of them as SGA, and Siemer et al.’s formula, 15.3%.
Conclusion
All three formulae yielded a good correlation between EFW in the last scan and birth weight in the infants with gastroschisis but not for those with omphalocele. Cases of SGA were overestimated.
