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Background: Although it is well-known that the presence of fetal anomalies is associated with maternal morbidity, granular information on these risks by type of anomaly is not available., Objective: To examine adverse maternal outcomes according to the type of fetal anomaly., Study Design: This was a repeated cross-sectional analysis of US vital statistics Live Birth/Infant Death linked data from 2011 to 2020. All pregnancies at 20 weeks or greater were included. Our primary outcome was severe maternal morbidity (SMM), defined as any maternal intensive care unit admission, transfusion, uterine rupture, or hysterectomy. Outcomes were compared between pregnancies with a specific type of fetal anomaly and pregnancies without any fetal anomaly. Fetal anomalies that were available in the dataset included anencephaly, meningomyelocele/spina bifida, cyanotic congenital heart disease, congenital diaphragmatic hernia, omphalocele, gastroschisis, cleft lip and/or palate, hypospadias, limb anomaly, and chromosomal disorders. If a fetus had more than one anomaly, it was classified as multiple anomalies. Adjusted relative risks (aRR) with 99% confidence intervals (99% CI) were calculated using modified Poisson regression. Adjusted risk differences (aRDs) were calculated using the marginal standardization form of predictive margins., Results: Of 35,760,626 pregnancies included in the analysis, 35,655,624 pregnancies had no fetal anomaly and 105,002 had isolated or multiple fetal anomalies. Compared to pregnancies without fetal anomaly, all fetal anomalies were associated with an increased risk of SMM except for gastroschisis and limb anomaly in order of aRRs (99% CI): 1.58 (1.29-1.92) with cleft lip and/or palate; 1.75 (1.35-2.27) with multiple anomalies; 1.76 (1.18-2.63) with a chromosomal disorder; 2.19 (1.82-2.63) with hypospadias; 2.20 (1.51-3.21) with spina bifida; 2.39 (1.62-3.53) with congenital diaphragmatic hernia; 2.66 (2.27-3.13) with congenital heart disease; 3.15 (2.08-4.76) with omphalocele; and 3.27 (2.22-4.80) with anencephaly. Compared to pregnancies without fetal anomaly, all fetal anomalies were associated with an increased absolute risk of SMM except for gastroschisis and limb anomaly in order of aRDs (99% CI): 0.26 (0.12-0.40) with cleft lip and/or palate, 0.34 (0.13-0.55) with multiple anomalies, 0.34 (0.02-0.66) with a chromosomal disorder, 0.54 (0.36-0.72) with hypospadias, 0.54 (0.17-0.92) with spina bifida, 0.63 (0.21-1.05) with congenital diaphragmatic hernia, 0.75 (0.56-0.95) with congenital heart disease, 0.97 (0.38-1.56) with omphalocele, and 1.03 (0.46-1.59) with anencephaly., Conclusion: The presence of fetal anomalies is associated with adverse maternal health outcomes. The risk of SMM varies according to the type of fetal anomaly. Counseling mothers about the maternal implications of fetal anomalies is paramount to help them make informed decisions regarding their pregnancy outcome., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Introduction: Umbilical hernias (UHs) are commonly underdiagnosed due to their asymptomatic presentation. The aim was to determine the prevalence of UHs by computed tomography (CT) in a trauma center, to assess the magnitude of their underdiagnosis., Methods: A cross-sectional study was designed, using CT studies to evaluate the integrity of the abdominal wall. The images were obtained from consecutive cases of adult patients (≥18 y) from the database of the radiology and imaging service during a 4-mo period. The sociodemographic features, type of CT scan, and description of the abdominal wall were obtained and compared with the radiology report. In the case of UH presence, the transversal, cephalocaudal, and anteroposterior lengths, as well as its content, were registered., Results: A total of 472 CT scans were included with a 67.6% (n = 319) prevalence of UH. These were most common in men (58.9%, n = 188/319), but women were more likely to have UH ≥ 10 mm (55%, n = 72/131). Of those with UH, 63.6% were unreported by radiology. The most common content was peritoneal fat (87.5%). UH had medians (interquartile range) of 9.1 mm (6.8, 12.5), 8.3 mm (6, 11.5), and 12.8 mm (8.6, 18.2) in its transversal, cephalocaudal, and anteroposterior lengths, respectively. Transversal length had a high positive correlation with cephalocaudal length (r = 0.877). This datum relationship can be explained by at least 76% due to this factor. Interobserver reliability analyses resulted in substantial reliability (ICC>0.85 and k > 0.85)., Conclusions: CT is an effective imaging tool for diagnosing UH. There is a high prevalence among the general adult population, with a high radiologic underreporting., (Copyright © 2024 Elsevier Inc. All rights reserved.)

There is an increase in the worldwide prevalence of congenital abdominal wall defects (CAWD), with gastroschisis (GS) and omphalocele (OC) being the most common. It is widely accepted that folic acid supplementation (FAS) in the maternal diet decreases the incidence of anomalies such as neural tube defects, but there is controversy regarding the possible beneficial role for other congenital malformations. Several epidemiological studies raise controversy regarding a possible relationship between vitamin supplementation with the occurrence of abdominal wall malformations. The aim of the present study is to obtain an updated review of the global frequency of CAWD in neonates and the relationship with FAS in the mothers. For this we have carried out a systematic search of epidemiological studies in different article databases between 2011 and 2022. The analysis of 25 studies conducted in different countries where cases of OC and/or GS are registered directly or together with other congenital defects shows that 60% inquire into the relationship of FAS with the incidence of CAWD. Half of them proposes a beneficial effect of FAS and the other half find no association, concluding that there is no unanimous evidence that FAS in the maternal diet decreases the incidence of CAWD. However, it seems that an influential factor to take into account is the nutritional habits of the mothers., (© 2024 The Author(s). Congenital Anomalies published by John Wiley & Sons Australia, Ltd on behalf of Japanese Teratology Society.)

Objective: Previous studies of alcohol consumption during pregnancy and omphalocele have produced mixed results. We updated an earlier analysis of National Birth Defects Prevention Study (NBDPS) data, adding six years of participants, to examine associations between maternal alcohol consumption and omphalocele., Methods: NBDPS was a multi-site, population-based case-control study in the United States. Cases were identified from birth defect surveillance programs in 10 states; controls were liveborn infants without a birth defect randomly selected from the same catchment areas. Mothers self-reported alcohol consumption during the periconceptional period (one month before through the third gestational month) via telephone interview. Our study included mothers of 410 omphalocele cases and 11,219 controls with estimated dates of delivery (EDDs) during 1997-2011. We used logistic regression to estimate adjusted odds ratios (AORs) and 95% confidence intervals (CIs) for periconceptional alcohol consumption and omphalocele. We performed a probabilistic bias analysis to evaluate the impact of alcohol exposure misclassification on our results., Results: Overall, 44% of case and 38% of control mothers reported periconceptional alcohol consumption; 22% and 17%, respectively, reported binge drinking. Any maternal periconceptional alcohol consumption was associated with modestly increased odds of omphalocele (AOR 1.35, 95% CI 1.09, 1.68), as was binge drinking (AOR 1.47, 95% CI 1.08, 2.01). Our bias analysis yielded estimates further from the null., Conclusions: We observed modest associations between maternal periconceptional alcohol consumption and omphalocele. Based on our bias analysis, studies of alcohol and birth defects not accounting for exposure misclassification may underestimate associations., Competing Interests: Declaration of competing interest The authors report no conflicts of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Gastroschisis is a serious birth defect with midgut prolapse into the amniotic cavity. The objectives of this study were to evaluate the prevalence and time trends of gastroschisis among programs in the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR), focusing on regional variations and maternal age changes in the population., Methods: We analyzed data on births from 1980 to 2017 from 27 ICBDSR member programs, representing 24 countries and three regions (Europe + (includes Iran) , Latin America, North America). Cases were identified using diagnostic codes (i.e., 756.7, 756.71, or Q79.3). We excluded cases of amniotic band syndrome, limb-body wall defect, and ruptured omphalocele. Programs provided annual counts for gastroschisis cases (live births, stillbirths, and legally permitted pregnancy terminations for fetal anomalies) and source population (live births, stillbirths), by maternal age., Results: Overall, gastroschisis occurred in 1 of every 3268 births (3.06 per 10,000 births; 95% confidence intervals [CI]: 3.01, 3.11), with marked regional variation. European + prevalence was 1.49 (95%CI: 1.44, 1.55), Latin American 3.80 (95%CI: 3.69, 3.92) and North American 4.32 (95%CI: 4.22, 4.42). A statistically significant increasing time trend was observed among six European + , four Latin American, and four North American programs. Women <20 years of age had the highest prevalence in all programs except the Slovak Republic., Conclusions: Gastroschisis prevalence increased over time in 61% of participating programs, and the highest increase in prevalence was observed among the youngest women. Additional inquiry will help to assess the impact of the changing maternal age proportions in the birth population on gastroschisis prevalence., (© 2024 The Authors. Birth Defects Research published by Wiley Periodicals LLC.)

Background: The Department of Defense Birth and Infant Health Research (BIHR) program leverages medical encounter data to conduct birth defect surveillance among infants born to military families. Omphalocele is a major abdominal wall defect with an annual prevalence of ~2 per 10,000 births in BIHR data, but an unexpected increase was observed during 2017-2019, reaching 6.4 per 10,000 births in 2018. To investigate this transient increase in prevalence, this study aimed to validate the omphalocele case algorithm among infants born 2016-2021., Methods: Omphalocele cases were identified by ICD-10 code Q79.2 (exomphalos) on one inpatient or two outpatient infant encounter records and validated using parental and infant electronic health records. Characteristics of true and false positive cases were assessed using bivariate analyses and compared over time., Results: Of 638,905 live births from 2016 to 2021, 230 met the ICD-10 case definition for omphalocele; 138 (60.0%) cases were eligible for validation, of which 68 (49.3%) were true positives. The geometric mean time from birth to first ICD-10 omphalocele diagnosis was 1.1 (standard error [SE] 0.1) days for true positives and 11.9 (SE 3.1) days for false positives. Among the 70 false positives, 36 (51.4%) were cases of confirmed umbilical hernia; rates of umbilical hernia and delayed omphalocele diagnoses (>30 days after birth) were elevated among false positives during 2017-2019., Conclusions: Higher misuse of ICD-10 code Q79.2 during 2017-2019 likely influenced the associated increase in omphalocele prevalence. Timing of diagnosis should be considered for omphalocele case definitions using medical encounter data., (© 2024 Wiley Periodicals LLC. This article has been contributed to by U.S. Government employees and their work is in the public domain in the USA.)

Introduction: Gastroschisis and omphalocele are the most common congenital abdominal wall defects. The main purpose of this study was to investigate the incidence, other associated anomalies and the course of these diseases in Iceland., Material and Methods: The study was retrospective. The population was all newborns who were admitted to the NICU of Children's Hospital Iceland due to gastroschisis or omphalocele in 1991-2020. Furthermore, all fetuses diagnosed prenatally or post mortem where the pregnancy ended in spontaneous or induced abortion, were included., Results: During the study period, 54 infants were born with gastroschisis and five with omphalocele. The incidence of gastroschisis was 4.11 and omphalocele 0,38/10,000 births. There was no significant change in the incidence of the diseases during the study period. In addition, five fetuses were diagnosed with gastroschisis and 31 with omphalocele where the pregnancy was terminated. In addition to gastroschisis in the live born infants and fetuses the most common associated anomalies were in the gastrointestinal or urinary tract but in infants and fetuses with omphalocele anomalies of the cardiac, central nervous or skeletal systems were the most common. Sixteen fetuses diagnosed with omphalocele had trisomy 18. Mothers aged 16-20 were more likely to give birth to an infant with gastroschisis than older mothers (p< 0.001). Primary closure was successful in 86% of the infants. Those reached full feedings significantly earlier and were discharged earlier. Overall survival rate was 95%. Three children were still receiving parenteral nutrition at discharge due to short bowel syndrome., Conclusions: The incidence of gastroschisis in Iceland is in accordance with studies in other countries but but the incidence of omphalocele is lower, which can be partly explained by spontaneous or induced abortions. Other anomalies associated with omphalocele are more severe than those associated with gastroschisis. Primary closure was associated with more benign course. Children with gastroschisis may need prolonged parenteral nutrition due to shortening of their intestines.

Normal childhood growth is an indicator of good health, but data addressing the growth of children born with abdominal wall defects (AWDs) are limited. The detailed growth phenotypes of children born with gastroschisis or omphalocele are described and compared to peers without AWDs from birth to adolescence. Data from 183 gastroschisis and 144 omphalocele patients born between 1993 and 2017 were gathered from Finnish nationwide registers and electronic health records. Weight (n = 3033), length/height (n = 2034), weight-for-length (0-24 months, n = 909), and body mass index measures (2-15 years, n = 423) were converted into sex- and age-specific Z-scores. Linear mixed models were used for comparisons. Intrauterine growth failure was common in infants with gastroschisis. Birth weight Z-scores in girls and boys were - 1.2 (0.2) and - 1.3 (0.2) and length Z-scores - 0.7 (0.2) and - 1.0 (0.2), respectively (p < 0.001 for all comparisons to infants without AWDs). During early infancy, growth failure increased in infants with gastroschisis, and thereafter, catch-up growth was prominent and faster in girls than in boys. Gastroschisis children gained weight and reached their peers' weights permanently at 5 to 10 years. By 15 years or older, 30% of gastroschisis patients were overweight. Infants with omphalocele were born with a normal birth size but grew shorter and weighing less than the reference population until the teen-age years., Conclusion: Children with gastroschisis and omphalocele have distinct growth patterns from fetal life onwards. These growth trajectories may also provide some opportunities to modulate adult health., What Is Known: • Intrauterine and postnatal growth failure can be seen frequently in gastroschisis and they often show significant catch-up growth later in infancy. It is assumed that part of the children with gastroschisis will become overweight during later childhood., What Is New:  • The longitudinal growth of girls and boys with gastroschisis or omphalocele is described separately until the teenage years. The risk of gaining excessive weight in puberty was confirmed in girls with gastroschisis., (© 2023. The Author(s).)

Introduction: We compared strategy outcomes and financial impact over the first two years of life (F2YOL) for patients with giant omphaloceles undergoing early repair (ER) (primary or staged) versus delayed repair (DR)., Methods: A retrospective review of giant omphaloceles (fascial defect > 5 cm/> 50% liver herniation) at a tertiary children's hospital between 1/1/2010 and 12/31/2019 was performed. Survival, length of stay, age at repair, ventilation days (VD), time to full enteral feeds, readmissions during the F2YOL, incidence of major associated anomalies, and total hospitalization charges during the F2YOL were compared. A subanalysis removing potential confounders and only including patients who underwent fascial closure within the F2YOL was also conducted., Results: Thirty four giant omphaloceles (23DR and 11ER) were identified. The median age (days) at repair was 289 [148, 399] DR versus 10 [5, 21] ER, P < 0.001. Total cohort two-year survival was significantly higher in the DR group (95.7% versus 63.6%, P = 0.03). Including patients with a tracheostomy there was no significant difference in VD during the index hospitalization. Excluding tracheostomy patients, the DR group had significantly fewer VD during the index hospitalization, 15 [0, 15] versus 18 [10, 54], P = 0.02 and over the F2YOL 6.5 [ 0, 21] versus 18 [14, 43], P = 0.03. There were no significant differences in the incidence/type of major associated anomalies, time to full enteral feeds, index length of stay, total hospital days, total admissions, or associated hospital charges. On subanalysis, there was no significant difference in VD or survival at any time., Conclusions: Delayed and early repair strategies for giant omphaloceles have equivalent outcomes in the index hospitalization and over the course of the first two years of life. These findings are useful for family counseling and expectation setting., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objectives: To estimate factors affecting survival in prenatally diagnosed omphalocele, factors predicting genetic abnormalities, and association of omphalocele and specific groups of anomalies., Methods: A retrospective observational study was performed, analyzing data of all omphalocele cases diagnosed prenatally in the perinatology clinic of a referral center. Demographic data, characteristics of the omphalocele (size, content, associated anomalies), results of genetic testing, pregnancy outcomes and postnatal outcomes were analyzed., Results: Sixty-nine fetuses with omphalocele were included. The prevalence of omphalocele in livebirth was 0.007 %. Overall survival during the study period was 73.9 %. Twenty-eight (71.7 %) out of 39 cases with associated anomalies who were born live, survived, whereas survival was 85.7 % in the isolated cases. The most common anomaly associated with omphalocele were cardiac defects with 42 %; followed by placental or umbilical cord anomalies (28.9 %), skeletal defects (27.5), genitourinary anomalies (20.2 %), central nervous system (18.8 %) and facial anomalies (7.2 %), respectively. Eighty-five percent of the fetuses had at least one additional anomaly or ultrasound finding. Skeletal abnormalities and staged surgical repair of omphalocele were associated with survival. Associated skeletal anomalies and staged repair significantly increase the risk of postnatal death (OR: 4.6 95 % CI (1.1-19.5) and (OR: 10.3 95 % CI (1.6-63.9), respectively)., Conclusions: Associated skeletal abnormalities and staged surgical repair are negatively associated with postnatal survival., (© 2023 Walter de Gruyter GmbH, Berlin/Boston.)

Purpose: To determine the prevalence of rectus diastasis (RD) in patients with inguinal hernia., Material and Methods: Multicenter, cross-sectional study. Patients with inguinal hernia were included in the study group (IH) and those with benign proctologic complaints created the control group (CG). Age, gender, BMI, family history for inguinal hernias, comorbid diseases, alcohol use, smoking, constipation, malignancy, chemotherapy, number of births, multiple pregnancies and prostate hypertrophy history of all patients in both groups were recorded. All patients were evaluated for RD and umbilical hernias by physical examination., Results: A total of 528 consecutive patients were included in the study (292 IH / 236 CG). Overall prevalence of RD was 35.6% and it was significantly higher in IH than in CG (46.9% vs 21.6%, p < 0.001). Also, umbilical hernia was more frequently detected in the patients with inguinal hernia. Other risk factors for RD were age, BMI, DM, BPH and smoking. The mean inter-rectus distance for 528 patients was 18.1 mm; it was 20.71 ± 10.68 mm in IH and 14.88 ± 8.82 in CG (p < 0.001). It was determined that the increase in age and BMI caused an increase in the inter-rectus distance, and that the presence of DM, inguinal hernia and umbilical hernia increased the inter-rectus distance quantitatively., Conclusions: The prevalence of RD seems to be higher in patients with inguinal hernia comparing to that in general population. Increased age, high BMI and DM were found to be independent risk factors for RD development., (© 2023. The Author(s), under exclusive licence to Springer-Verlag France SAS, part of Springer Nature.)

The aim of this study was to analyze umbilical hernia occurrences in patients who underwent laparoscopic or laparoendoscopic single-sight (LESS) cholecystectomy. Patients who underwent cholecystectomy by a single surgeon between 2015 and 2020 were surveyed. Data are presented as median (mean +/- standard deviation). Two hundred and fifty-three patients were sent the survey and 130 (51%) patients responded. The overall age was 57 (31 +/- 18) and the overall BMI was 30 (31 +/- 7). Twelve (9%) patients developed an umbilical hernia. Seventeen patients were active smokers and four (24%) developed an umbilical hernia. One hundred and thirteen patients were inactive smokers and eight (7%) developed an umbilical hernia. There was a statistical significance between umbilical hernia occurrence and smoking history ( P < .05). Active smokers have a higher risk of developing an umbilical hernia following a minimally invasive cholecystectomy, regardless of operative approach. Elective cholecystectomy should be reconsidered for current smokers.

Background: The objective of this paper is to describe the clinical and epidemiological profile and the early outcomes of patients with omphalocele born in a fetal medicine, pediatric surgery, and genetics reference hospital in Rio de Janeiro - Brazil. To determine its prevalence, describe the presence of genetic syndromes, and congenital malformations, emphasizing the characteristics of congenital heart diseases and their most common types., Methods: Using Latin-American Collaborative Study of Congenital Malformations (ECLAMC) database and records review, a retrospective cross-sectional study was performed, including all patients born with omphalocele between January 1st, 2016, and December 31st, 2019., Results: During the period of the study, our unity registered 4,260 births, 4,064 were live births and 196 stillbirths. There were 737 diagnoses of any congenital malformation, among them 38 cases of omphalocele, 27 were live born, but one was excluded for missing data. 62.2% were male, 62.2% of the women were multiparous and 51.3% of the babies were preterm. There was an associated malformation in 89.1% of the cases. Heart disease was the most common (45.9%) of which tetralogy of Fallot was the most frequent (23.5%). Mortality rate was 61.5%., Conclusions: Our data showed a good correspondence with the existing literature. Most patients with omphalocele had other malformations, especially congenital heart disease. No pregnancy was interrupted. The presence of concurrent defects showed a huge impact on prognosis, since, even if most survived birth, few remained alive and received hospital discharge. Based on these data, fetal medicine and neonatal teams must be able to adjust parents counseling about fetal and neonatal risks, especially when other congenital diseases are present., (© 2023. The Author(s).)

Objective: Omphalocele is known to be associated with genetic anomalies like trisomy 13, 18 and Beckwith-Wiedemann syndrome, but not with Turner syndrome (TS). Our aim was to assess the incidence of omphalocele in fetuses with TS, the phenotype of this association with other anomalies, their karyotype, and the fetal outcomes., Method: Retrospective multicenter study of fetuses with confirmed diagnosis of TS. Data were extracted from a detailed questionnaire sent to specialists in prenatal ultrasound., Results: 680 fetuses with TS were included in this analysis. Incidence of small omphalocele in fetuses diagnosed ≥12 weeks was 3.1%. Including fetuses diagnosed before 12 weeks, it was 5.1%. 97.1% (34/35) of the affected fetuses had one or more associated anomalies including increased nuchal translucency (≥3 mm) and/or cystic hygroma (94.3%), hydrops/skin edema (71.1%), and cardiac anomalies (40%). The karyotype was 45,X in all fetuses. Fetal outcomes were poor with only 1 fetus born alive., Conclusion: TS with 45,X karyotype but not with X chromosome variants is associated with small omphalocele. Most of these fetuses have associated anomalies and a poor prognosis. Our data suggest an association of TS with omphalocele, which is evident from the first trimester., (© 2023 The Authors. Prenatal Diagnosis published by John Wiley & Sons Ltd.)

Objective: To examine the prenatal diagnosis rates of bladder exstrophy (BE) and Omphalocele-Exstrophy-Imperforate anus-Spinal Defect Syndrome (OEIS) in a large cohort of patients over a 20-year period. We hypothesized that prenatal diagnosis rates improved over time due to evolving techniques in fetal imaging., Methods: A multi-institutional database was queried to identify BE or OEIS patients who underwent primary closure between 2000 and 2020. We retrospectively determined prenatal or postnatal diagnosis. Those with unknown prenatal history were excluded. Multivariable logistic regression was used to investigate temporal pattern in rate of prenatal diagnosis while adjusting for sex and treating institution., Results: Among 197 BE and 52 OEIS patients, 155 BE and 45 OEIS patients had known prenatal history. Overall prenatal diagnosis rates of BE and OEIS were 47.1% (73/155) and 82.2% (37/45), respectively. Prenatal diagnosis rate was significantly lower in BE compared to OEIS (P <.0001). The prenatal diagnosis rate for BE significantly increased over time (OR 1.10; [95%CI: 1.03-1.17]; P = .003). Between 2000 and 2005, the prenatal diagnosis rate of BE was 30.3% (10/33). Between 2015 and 2020, prenatal diagnosis rate of BE was 61.1% (33/54). Prenatal diagnosis rate for OEIS did not change over time. Rates of prenatal diagnosis did not differ by sex or treating institution., Conclusion: Rates of prenatal diagnosis of BE and OEIS are higher than previously reported. Prenatal diagnosis rate of BE doubled in the last 5 years compared to the first 5 years of the study period. Nonetheless, a significant proportion of both BE and OEIS patients remain undiagnosed prior to delivery., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Introduction: We evaluate the incidence, outcomes, and management of peri‑umbilical hernias after sutured or sutureless gastroschisis closure., Methods: A retrospective, longitudinal follow-up of neonates with gastroschisis who underwent closure at 11 children's hospitals from 2013 to 2016 was performed. Patient encounters were reviewed through 2019 to identify the presence of a peri‑umbilical hernia, time to spontaneous closure or repair, and associated complications., Results: Of 397 patients, 375 had follow-up data. Sutured closure was performed in 305 (81.3%). A total of 310 (82.7%) infants had uncomplicated gastroschisis. Peri-umbilical hernia incidence after gastroschisis closure was 22.7% overall within a median follow-up of 2.5 years [IQR 1.3,3.9], and higher in those with uncomplicated gastroschisis who underwent primary vs. silo assisted closure (53.0% vs. 17.2%, p< 0.001). At follow-up, 50.0% of sutureless closures had a persistent hernia, while 16.4% of sutured closures had a postoperative hernia of the fascial defect (50.0% vs. 16.4%, p< 0.001). Spontaneous closure was observed in 38.8% of patients within a median of 17 months [9,26] and most frequently observed in those who underwent a sutureless primary closure (52.2%). Twenty-seven patients (31.8%) underwent operative repair within a median of 13 months [7,23.5]. Rate and interval of spontaneous closure or repair were similar between the sutured and sutureless closure groups, with no difference between those who underwent primary vs. silo assisted closure., Conclusion: Peri-umbilical hernias after sutured or sutureless gastroschisis closure may be safely observed similar to congenital umbilical hernias as spontaneous closure occurs, with minimal complications and no additional risk with either closure approach., Levels of Evidence: Level II., Competing Interests: Declarations of Competing Interest None., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Omphalocele is an embryonic developmental error presented as a neonatal visceral umbilical herniation. Epidemiological and embryological studies are reviewed that revise our understanding of midgut development and defects in cattle. Google Scholar and PubMed were searched for omphalocele, exomphalos, calves, calf, cattle, epidemiology, genetics, disease presentation, and omphalocele treatments of bovine neonates. Omphalocele contains small intestine and sometimes liver and/or pyloric abomasum. This condition may arise from inadequately expanded abdominal cavity, herniated midgut entrapment, or inappropriate umbilical ring growth. Full-term neonates are otherwise healthy but comorbidities may be present. Limited epidemiological evidence suggests neonates, commonly female, of dairy dams have much higher omphalocele prevalence than humans but with fewer co-morbidities. Genetic or environmental influences may present at low incidences, especially with co-defects, while also epigenetics operate in metabolically challenged dairy cattle. Calf survival after surgical repair was commonly good if damage to eviscerated tissue was minimal and surgery prompt. Inadequate follow-ups prevented assessment of their commercial value. We also revised understanding of embryonic midgut development. There is little useful epidemiological information on bovine omphaloceles. To address this, we suggest: (i) Observations on omphaloceles should report herd and dam histories, abdominal disposition of contents, and comorbidities. (ii) Cases of surgical restitution must report full histories and thoroughly explore post-surgical follow-ups to assess animal commercial viability. There is a need for very large-scale multi-centre prospective observational studies on cattle health and productivity that should include omphaloceles., (© 2022. The Author(s), under exclusive licence to Springer Nature B.V.)

Background: Laparoscopic colorectal surgery (LCRS) requires a small laparotomy at the umbilicus. The wound is small and inconspicuous, but if the patient develops an umbilical incisional hernia (UIH), the wound is visible and the patient suffers from symptoms of discomfort. However, the incidence of UIH after LCRS and its risk factors are not well understood. The purpose of this study was to investigate the risk factors for UIH after LCRS for colorectal cancer., Methods: This was a single-centre retrospective study of 135 patients with colorectal cancer, conducted at our hospital from April 2013 to March 2019. The diagnosis of UIH was based on computed tomography and physical examination findings. Preoperative patient data such as enlargement of the umbilical orifice (EUO), subcutaneous fat thickness (SFT) and intraperitoneal thickness (IPT) were collected and analysed using univariate and multivariate analyses for the presence of risk factors for UIH., Results: A total of 135 patients who underwent LCRS were analysed. The incidence of UIH was 20.7%. Univariate analysis revealed significantly high body mass index (BMI) ≥ 25 (P = 0.032), EUO (P < 0.001), SFT ≥18 mm (P = 0.011), and IPT ≥61 mm (P < 0.01) in the UIH group. Multivariate analysis revealed significant differences in EUO (P < 0.001), SFT ≥18 mm (P = 0.046) and IPT ≥61 mm (P = 0.022)., Conclusion: EUO was the most important risk factor for UIH, followed by IPT and SFT. These findings are predictive indicators of the development of UIH after LCRS and can be assessed objectively and easily with preoperative computed tomography., (© 2022 Royal Australasian College of Surgeons.)

Background: Omphalocele and gastroschisis are the two most common congenital abdominal wall defects; however, no previous study has focused on gastrointestinal and hepatobiliary tract malformations in these two conditions. This study aimed to investigate the demographic characteristics, coexisting congenital gastrointestinal and hepatobiliary tract anomalies, hospital course, and outcomes of patients with gastroschisis and omphalocele., Methods: This is retrospective chart review of all patients admitted to one tertiary medical center in Taiwan between January 1, 2000 and June 30, 2020 with a diagnosis of gastroschisis or omphalocele. The medical records were reviewed to obtain demographic data regarding coexisting gastrointestinal and hepatobiliary tract anomalies and outcomes., Results: Of the 51 patients included, 21 had gastroschisis and 30 had omphalocele. Gastroschisis was associated with a significantly younger maternal age and a higher incidence of small for gestational age. Of the 30 patients with omphalocele, twelve had associated gastrointestinal and hepatobiliary anomalies. Seven of the 21 patients with gastroschisis had gastrointestinal anomalies, and none had hepatobiliary anomalies. Among the omphalocele patients, three (10%) had documented malrotation, and one developed midgut volvulus. Among gastroschisis patients, four patients (19%) had malrotation, and two developed midgut volvulus. There were no statistically significant differences in postoperative complications or mortality rates between those with and without gastrointestinal/hepatobiliary tract anomalies., Conclusion: The diversity of coexisting gastrointestinal and hepatobiliary tract anomalies is higher in the omphalocele than in gastroschisis. In addition, we demonstrate that patients with gastroschisis or omphalocele have a higher rate of intestinal malrotation and midgut volvulus., (Copyright © 2022 Taiwan Pediatric Association. Published by Elsevier B.V. All rights reserved.)

Purpose: We compared cases of anemia in gastroschisis versus omphalocele and investigated this clinical question., Methods: A multicenter study of five pediatric surgery departments in southern Japan was planned. Sixty patients were collected between 2011 and 2020, with 33 (gastroschisis: n = 19, omphalocele: n = 14) who met the selection criteria ultimately being enrolled. Anemia was evaluated before discharge and at the first outpatient visit., Results: Despite gastroschisis cases showed more frequent iron administration during hospitalization than omphalocele (p = 0.015), gastroschisis cases tended to show lower hemoglobin values at the first outpatient visit than omphalocele cases (gastroschisis: 9.9 g/dL, omphalocele: 11.2 g/dL). Gastroschisis and the gestational age at birth were significant independent predictors of anemia at the first outpatient visit, (gastroschisis: adjusted odds ratio [OR] 19.00, p = 0.036; gestational age at birth: adjusted OR 0.341, p = 0.028). A subgroup analysis for gastroschisis showed that the ratio of anemia in the 35-36 weeks group (8/10, 80.0%) and the > 37 weeks group (6/6, 100%) was more than in the < 34 weeks group (0/3, 0.0%)., Conclusions: Gastroschisis may carry an increased risk of developing anemia compared with omphalocele due to the difference of direct intestinal exposure of amnion fluid in utero., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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.

Background: Incisional hernia (HI), in open and laparoscopic surgery, is a very frequent complication. HI located in the umbilical region are called M3. The main aim of this study is to compare HI produced by the placement of an umbilical laparoscopic trocar (M3T) with those located in M3 in open surgery (M3O) in terms of basal characteristics, complications and recurrences; and secondarily the identification of risk factors., Method: Cross-sectional observational study based on the national prospective registry EVEREG during the period of July 2012-June 2018. The main variables were recurrences and postoperative complications. Both groups (M3T and M3O) were compared. Multiple logistic regression was performed to identify the risk factors of the entire cohort., Results: 882 had a follow-up time longer than 12 months. M3O group presented superior ASA-Class, more complex HI and previous repair. It also presented a higher recurrence rate at 12 and 24 months (8.6% vs. 2.5%; P < 0,0001 and 9.3% vs. 2.9%; P < 0.0001) and higher postoperative complications rate (21.9% vs. 14.6 %; P = 0.02). Previous repair, intervention length and associated procedures requirement were identified as risk factors for postoperative complications. Absence of a specialist present during surgery, previous repair, and the absence of complications were identified as risk factors for recurrence. In the PSM analysis no differences were detected in of complications and recurrences., Conclusions: HI M3O is more complex than M3T. The complexity is not related to the origin of the hernia but to its characteristics and those of the patient., (Copyright © 2021 AEC. Published by Elsevier España, S.L.U. All rights reserved.)

Evidence of the association between maternal sulfur dioxide (SO 2 ) exposure and the risk of omphalocele is limited and equivocal. We aimed to assess the aforementioned topic during the first trimester of pregnancy. A population-based case-control study was carried out in infants consisting of 292 cases of omphalocele and 7,950 healthy infant controls. Exposure to SO 2 , particulate matter with aerodynamic diameters ≤ 10 μm, and nitrogen dioxide was assessed by averaging the concentration from all stations in the mother's residential city. SO 2 exposure was categorized into three groups, with the lowest tertile defined as the reference category. Odds ratios (ORs) and 95% confidence intervals (CIs) were estimated using multivariable logistic regression models. Higher SO 2 exposure during the first trimester was significantly associated with omphalocele risk [per standard deviation (42 ug/m 3 ) increment: OR = 1.39, 95% CI = 1.22-1.65]. When focusing on shorter exposure windows, similar positive associations were observed for SO 2 exposure in the first and third months of pregnancy. In addition, compared with the lowest tertile, high SO 2 exposure in the second month of pregnancy increased the risk of omphalocele (OR = 2.80, 95% CI = 1.61-4.97). Maternal exposure to SO 2 during the first trimester may increase the risk of omphalocele in offspring., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Li, Huang, Li, Liu, Chen, Jiang, Chen and Zhuang.)

Purpose: Umbilical midline incisions for single incision- or reduced port laparoscopic surgery are still discussed controversially because of a higher rate of incisional hernia compared to conventional laparoscopic techniques. The aim of this study was to evaluate incidence and risk factors for incisional hernia after reduced port colorectal surgery., Methods: A total 241 patients underwent elective reduced port colorectal surgery between 2014 and 2020. Follow-up was achieved through telephone interview or clinical examination. The study collective was examined using univariate and multivariate analysis., Results: A total of 150 patients with complete follow-up were included into this study. Mean follow-up time was 36 (IQR 24-50) months. The study collective consists of 77 (51.3%) female and 73 (48.7%) male patients with an average BMI of 26 kg/m 2 (IQR 23-28) and an average age of 61 (± 14). Indication for surgery was diverticulitis in 55 (36.6%) cases, colorectal cancer in 65 (43.3%) patients, and other benign reasons in 30 (20.0%) cases. An incisional hernia was observed 9 times (6.0%). Obesity (OR 5.8, 95% CI 1.5-23.1, p = 0.02) and pre-existent umbilical hernia (OR 161.0, 95% CI 23.1-1124.5, p < 0.01) were significant risk factors for incisional hernia in the univariate analysis. Furthermore, pre-existent hernia is shown to be a risk factor also in multivariate analysis., Conclusion: We could demonstrate that reduced port colorectal surgery using an umbilical single port access is feasible and safe with a low rate of incisional hernia. Obesity and pre-existing umbilical hernia are significant risk factors for incisional hernia., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: To compare the frequency of seroma formation in laparoscopic para-umbilical hernia repair with and without primary closure of defect., Methods: The randomised parallel group study was conducted at the Department of Surgery, Bahawal Victoria Hospital, Bahawalpur, Pakistan, from October 1, 2019, to May 31, 2020, and comprised patients of both genders age 25-65 years diagnosed as a case of para-umbilical hernia. The patients were randomised into group A which subsequently had laparoscopic primary repair of defect with non-absorbable suture prior to mesh placement, and group B which had laparoscopic repair without primary closure of the defect. Case sheets of patients were prepared for age, gender, duration of disease, operating time, body mass index and the presence or absence of seroma formation. Data was analysed using SPSS 20., Results: Of the 100 patients, 35(35%) were males and 65(65%) were females with male-to-female ratio of 1:1.9, and an overall mean age of 43.92±10.77 years. Both groups had 50(50%) patients each. Post-operative seroma formation was noted in 1(2%) group A patient and 12(24%) group B patients (p=0.001)., Conclusion: Primary closure of the fascial defect in laparoscopic para-umbilical hernia repair resulted in decreased frequency of post-operative seroma formation.

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., Competing Interests: The authors have no conflict of interests to declare., (Federação Brasileira de Ginecologia e Obstetrícia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/).)

Folic acid (FA) can reduce the risk for selected birth defects other than neural tube defects. We examined whether FA has preventive effects against fetal abdominal wall defects (AWD) in a unique intervention cohort in China. Birth outcomes of 247 831 singleton births from a population-based cohort study with detailed pre-conceptional FA intake information were collected in China in 1993-1996. Information on births at 20 complete gestational weeks, including live births, stillbirths and pregnancy terminations, and all structural birth defects regardless of gestational week were recorded. The birth prevalence of omphalocele, gastroschisis and total fetal AWD was classified by maternal FA supplementation. The prevalence of total AWD was 4·30 per 10 000 births among women who took FA compared with 13·46 per 10 000 births among those who did not take FA in northern China and 6·28 and 5·18 per 10 000 births, respectively, in southern China. The prevalence of omphalocele was 0·54 per 10 000 births among women who took FA compared with 3·74 per 10 000 births among those who did not take FA in northern China and 1·79 and 1·44 per 10 000 births, respectively, in southern China. FA supplementation significantly prevented total AWD in multivariate analysis (relative risk 0·26, 95 % CI 0·11, 0·61) in northern China, although no preventive effect of FA on AWD was observed in southern China. FA supplementation successfully reduced the prevalence of AWD in northern China.

Purpose: This study aims to define the extent of additional surgical procedures after abdominal wall closure (AWC) in patients with gastroschisis (GS) and omphalocele (OC) with special focus on gastrointestinal related operations., Methods: A retrospective chart review was performed including all operations in GS and OC patients in the first year after AWC (2010-2019). The risk for surgery was calculated using the one-year cumulative incidence (CI)., Results: 33 GS patients (18 simple GS, 15 complex) and 24 OC patients (12 without (= OCL), 12 OC patients with liver protrusion (= OCL +)) were eligible for analysis. 43 secondary operations (23 in GS, 20 in OC patients) occurred after a median time of 84 days (16-824) in GS and 114.5 days (12-4368) in OC. Patients with complex versus simple GS had a significantly higher risk of undergoing a secondary operation (one-year CI 64.3% vs. 24.4%; p = 0.05). 86.5% of surgical procedures in complex GS and 36.3% in OCL + were related to gastrointestinal complications. Complex GS had a significantly higher risk for GI-related surgery than simple GS. Bowel obstruction was a risk factor for surgery in complex GS (one-year CI 35.7%)., Conclusion: Complex GS and OCL + patients had the highest risk of undergoing secondary operations, especially those with gastrointestinal complications., (© 2021. The Author(s).)

Purpose: The increase in prenatal diagnosis together with the high rates of associated anomalies in omphalocele has led to increased rates of termination of pregnancies. The aim of this study was to examine the national Swedish birth prevalence and survival rates among these patients., Methods: This study is based on a nationwide population-based cohort of all children born in Sweden between 1/1/1997 and 31/12/2016. All omphalocele cases were identified though the Swedish National Patient Register and the Swedish Medical Birth Register. Outcome of malformations and deaths were retrieved from the Swedish Birth Defects Register and the Swedish Causes of Death Register., Results: The study included 207 cases of omphalocele (42% females). The birth prevalence for omphalocele was 1/10,000 live births. About 62% of the cases had associated malformations and/or genetic disorders; most common was ventricular septal defect. The mortality within the first year was 13%. The rate of termination of pregnancy was 59%., Conclusion: The national birth prevalence for omphalocele in Sweden is 1/10,000 newborn, with high termination rates. Over half of the pregnancies with prenatally diagnosed omphalocele will be terminated. Among those who continue the pregnancy, 1-year survival rates are high., Type of Study: National register study LEVEL OF EVIDENCE: III., (© 2021. The Author(s).)

Objective: To examine the association between prenatal magnetic resonance imaging (MRI) based observed/expected total lung volume (O/E TLV) and outcome in neonates with giant omphalocele (GO)., Methods: Between 06/2004 and 12/2019, 67 cases with isolated GO underwent prenatal and postnatal care at our institution. MRI-based O/E TLVs were calculated based on normative data from Meyers and from Rypens and correlated with postnatal survival and morbidities. O/E TLV scores were grouped based on severity into <25% (severe), between 25% and 50% (moderate), and >50% (mild) for risk stratification., Results: O/E TLV was calculated for all patients according to Meyers nomograms and for 49 patients according to Rypens nomograms. Survival for GO neonates with severe, moderate, and mild pulmonary hypoplasia based on Meyers O/E TLV categories was 60%, 92%, and 96%, respectively (p = 0.04). There was a significant inverse association between Meyers O/E TLV and risk of neonatal morbidities (p < 0.05). A similar trend was observed with Rypens O/E TLV, but associations were less often significant likely related to the smaller sample size., Conclusion: Neonatal outcomes are related to fetal lung size in isolated GO. Assessment of Meyers O/E TLV allows identification of GO fetuses at greatest risk for complications secondary to pulmonary hypoplasia., (© 2021 John Wiley & Sons Ltd.)

Background: Trocar site hernia (TSH) is often underestimated after minimally invasive surgery. Scarce information is available about the incidence of TSH in patients undergoing laparoscopic hernioplasty. We aimed to evaluate the incidence and risk factors of umbilical TSH after laparoscopic TAPP hernioplasty in patients with and without an associated umbilical hernia., Methods: A retrospective analysis of a prospectively collected database of all patients who underwent laparoscopic inguinal TAPP repair during 2013-2018 was performed. After TAPP repair, the umbilical fascia was closed either by a figure-of-eight stitch with absorbable suture (G1) or by umbilical hernioplasty if it was present (G2). Multivariate logistic regression analysis was used to determine the TSH risk factors. Comparative evaluation regarding demographics, and operative and postoperative variables was performed., Results: A total of 535 laparoscopic TAPP repairs were included. There were 359 (67.1%) patients in G1 and 176 in G2 (32.9%). Surgical site infection was higher in G2 (G1: 0.6% vs G2: 5.7%, p = 0.001). Overall TSH rate was 3.9% after a mean follow-up of 20 (12-41) months. Performing a concomitant umbilical repair significantly increased the risk of umbilical TSH (G1: 2.2% vs G2: 7.4%, p = 0.004). TSH rates in G2 were similar in patients with simple suture or mesh repairs (p = 0.88). Rectus abdominis diastasis (OR 37.8, 95% CI:8.22-174.0, p < 0.001) and inguinal recurrence (OR 13.5, 95% CI:2.04-89.5, p = 0.007) were independent risk factors for TSH., Conclusion: Although trocar site hernia after laparoscopic TAPP repair has a low incidence, its risk is significantly increased in patients with a concomitant umbilical hernia repair, rectus abdominis diastasis, and/or inguinal recurrence., (© 2020. Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Omphalocele is a major congenital anomaly associated with significant morbidity and mortality. Regardless, the influence of maternal use of prescription drugs on the risk of omphalocele has only been addressed in a handful of studies. The aim of this study was to assess the influence of maternal risk factors and prescription drugs in early pregnancy on the risk of omphalocele., Methods: We performed a nationwide register-based case-control study in Finland. The analysis is based on the Finnish Register of Congenital Malformations and Drugs and Pregnancy databases, both upheld by the Finnish Institute for Health and Welfare. All omphalocele cases were identified between Jan 1, 2004, and Dec 31, 2014. Five age-matched controls from the same geographical region were randomly selected for each case. The main outcome measures were maternal risk factors for omphalocele. Our analysis compared the maternal characteristics and the use of prescription drugs during the first trimester of pregnancy between case and control mothers., Results: Mothers of 359 omphalocele cases were compared with 1738 randomly selected age and area-matched mothers of healthy infants between 1 January 2014 and 31 December 2014. Both maternal obesity (BMI ≥30) and diabetes increased the risk for omphalocele, and their co-occurrence accumulated this risk (aOR 5.06, 95% Cl 1.19-21.4). Similarly, history of multiple miscarriages was an independent risk factor (2.51, 1.16-5.43). The oral use of extended spectrum penicillins during the first trimester of pregnancy had a significant, protective influence (0.17, 0.04-0.71). These analyses were adjusted for sex, parity, and risk factors reported above. No significant changes in risk were observed with any other medication used during the first trimester., Conclusion: In conclusion, these findings may suggest that extended spectrum penicillins in the first trimester reduces the risk of omphalocle formation. Additionally, consistent with earlier studies, previous repeated miscarriages, maternal obesity, and diabetes were significant risk factors for omphalocele., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

The aim of this nationwide population-based case-control study was to assess the incidence of inguinal hernia (IH) among patients with congenital abdominal wall defects. All infants born with congenital abdominal wall defects between Jan 1, 1998, and Dec 31, 2014, were identified in the Finnish Register of Congenital Malformations. Six controls matched for gestational age, sex, and year of birth were selected for each case in the Medical Birth Register. The Finnish Hospital Discharge Register was searched for relevant diagnosis codes for IH, and hernia incidence was compared between cases and controls. We identified 178 infants with gastroschisis and 150 with omphalocele and selected randomly 1968 matched, healthy controls for comparison. Incidence of IH was significantly higher in gastroschisis girls than in matched controls, relative risk (RR) 7.20 (95% confidence interval [CI] 2.25-23.07). In boys with gastroschisis, no statistically significant difference was observed, RR 1.60 (95% CI 0.75-3.38). Omphalocele was associated with higher risk of IH compared to matched controls, RR 6.46 (95% CI 3.90-10.71), and the risk was equally elevated in male and female patients.Conclusion: Risk of IH is significantly higher among patients with congenital abdominal wall defects than in healthy controls supporting hypothesis that elevated intra-abdominal pressure could prevent natural closure of processus vaginalis. Parents should be informed of this elevated hernia risk to avoid delays in seeking care. We also recommend careful follow-up during the first months of life as most of these hernias are diagnosed early in life. What is Known: • Inguinal hernia is one of the most common disorders encountered by a pediatric surgeon. • Prematurity increases the risk of inguinal hernia. What is New: • Children with congenital abdominal wall defects have a significantly higher risk of inguinal hernia than general population. • Families should be informed of this elevated hernia risk to avoid delays in seeking care., (© 2021. The Author(s).)

Purpose: Several studies have reported high prevalence of undescended testis (UDT) among boys with congenital abdominal wall defects (AWD). Due to rarity of AWDs, however, true prevalence of testicular maldescent among these boys is not known. We conducted a national register study to determine the prevalence of UDT among Finnish males with an AWD., Methods: All male infants with either gastroschisis or omphalocele born between Jan 1, 1998 and Dec 31, 2015 were identified in the Register of Congenital Malformations. The data on all performed operations were acquired from the Care Register for Health Care. The register data were examined for relevant UDT diagnosis and operation codes., Results: We identified 99 males with gastroschisis and 89 with omphalocele. UDT was diagnosed in 10 (10.1%) infants with gastroschisis and 22 (24.7%) with omphalocele. Majority of these required an operation; 8/99 (8.1%) gastroschisis and 19/89 (21.3%) omphalocele patients. UDT is more common among AWD patients than general population with the highest prevalence in omphalocele., Conclusions: Cryptorchidism is more common among boys with an AWD than general population. Furthermore, omphalocele carries significantly higher risk of UDT and need for orchidopexy than gastroschisis. Due to high prevalence testicular maldescent, careful follow-up for UDT is recommended.

Congenital abdominal wall defects, namely, gastroschisis and omphalocele, are rare congenital malformations with significant morbidity. The long-term burden of these anomalies to families and health care providers has not previously been assessed. We aimed to determine the need for hospital admissions and the requirement for surgery after initial admission at birth. For our analyses, we identified all infants with either gastroschisis (n=178) or omphalocele (n=150) born between Jan 1, 1998, and Dec 31, 2014, in the Register of Congenital Malformations. The data on all hospital admissions and operations performed were acquired from the Finnish Hospital Discharge Register between Jan 1, 1998, and Dec 31, 2015, and compared to data on the whole Finnish pediatric population (0.9 million) live born 1993-2008. Patients with gastroschisis and particularly those with omphalocele required hospital admissions 1.8 to 5.7 times more than the general pediatric population (p<0.0001). Surgical interventions were more common among omphalocele than gastroschisis patients (p=0.013). At the mean follow-up of 8.9 (range 1.0-18.0) years, 29% (51/178) of gastroschisis and 30% (45/150) of omphalocele patients required further abdominal surgery after discharge from the neonatal admission.Conclusion: Patients with gastroschisis and especially those with omphalocele, are significantly more likely than the general pediatric population to require hospital care. Nevertheless, almost half of the patients can be treated without further surgery, and redo abdominal surgery is only required in a third of these children. What is Known: • Gastroschisis and omphalocele are congenital malformations with significant morbidity • There are no reports on the long-term need for hospital admissions and surgery in these children What is New: • Patients with abdominal wall defects are significantly more likely than the general pediatric population to require hospital care • Almost half of the patients can be treated without further surgery, and abdominal redo operations are only required in a third of these children.

Introduction: Rectus abdominis diastasis (RAD) is characterized by thinning and widening of the linea alba and laxity of the ventral abdominal muscle. RAD, when coexistent with umbilical hernia, is considered a risk factor for hernia recurrence. We investigated the impact of comorbid RAD in patients with umbilical hernia who had undergone hernia repair., Methods: We enrolled 30 patients who had undergone umbilical hernia repair using either a laparoscopic or anterior approach between April 2006 and May 2018. We diagnosed RAD according to preoperative CT. The outcomes of patients with umbilical hernia, the RAD group, and the non-RAD group were compared, especially in terms of recurrence., Results: Twenty-five patients (83%) presented with RAD, including three patients (12%) with postoperative recurrence who were allocated to the RAD group. The median BMI in the RAD group was 27.2 kg/m 2 . In the RAD group, a prosthesis mesh was used in 12 patients (48%), and nonabsorbable suture material was used in four patients (16%). There was no statistically significant difference between the two groups in terms of age, hernial orifice diameter, surgical technique, or operative time., Conclusion: The rate of comorbid umbilical hernia in Japanese patients with RAD was high, as was the recurrence rate of umbilical hernia. We strongly recommend preoperative detection of RAD. We also recommend mesh-based repair of the midline and nonabsorbable suture material to decrease the recurrence rate, irrespective of hernia size., (© 2020 Asia Endosurgery Task Force and Japan Society of Endoscopic Surgery and John Wiley & Sons Australia, Ltd.)

Background: The peri-operative management of omphalocoele in low- and middle-income countries is challenging owing to non-availability of neonatal intensive care units and equipment needed for the care of this anomaly., Aim: This study examined our experience in the management of omphalocoele and compared the pattern and outcome with a similar study from the same centre conducted four decades ago., Methods: A retrospective study of neonates managed for omphalocoele from 2003 to 2017 (Group A) was performed. Their demographic characteristics, clinical presentation, management modality and outcome were obtained and statistical analysis was performed to determine the predictors of mortality. The findings were also compared with the findings of a similar study (Group B) published from this centre four decades ago from 1973 to 1978., Results: A total of 95 patients were managed in Group A and 33 in Group B. Their ages ranged from 1 to 15 days with a median age of 1 day and a median gestational age at birth of 37 weeks (range - 36-43 weeks) in Group A and 5-72 h in Group B. There were 54 (56.8%) boys and 41 (43.2%) girls in Group A and 17 (51.5%) boys and 16 (48.5%) girls in Group B. Rupture of the sac was observed in 18 (18.9%) patients in Group A and 13 (39.4%) in Group B. Operative management was adopted for 55 (57.9%) patients in Group A compared to 14 (42.4%) in Group B. Mortality was recorded in 16 (16.8%) patients in Group A and 16 (48.5%) in Group B. Following further analysis in Group A, management outcome was noted to be significantly associated with the state of the sac (P = 0.011), presence of associated sepsis (P = 0.002) at presentation and management modality (P = 0.048) with only associated sepsis independently predicting mortality., Conclusion: Although epidemiological trend and clinical presentation are still similar, management outcome has improved over the years., Competing Interests: None

Objective: Describe the prevalence, perinatal and long-term outcomes of Beckwith-Wiedemann syndrome (BWS) among prenatally detected omphaloceles., Methods: All prenatally diagnosed omphaloceles from 2010 to 2015 within a single tertiary care centre were identified. An echocardiogram and detailed fetal ultrasound were performed, and amniocentesis was offered with karyotype/microarray analysis and BWS molecular testing. Perinatal, neonatal, and long-term outcomes were retrieved for BWS cases., Results: Among 92 omphaloceles, 62 had additional anomalies. Abnormal karyotypes were identified in 23/62 (37%) non-isolated and 2/30 (7%) isolated cases. One BWS case (5%) was identified among non-isolated omphaloceles and six BWS cases (37.5%) were identified among isolated omphaloceles after exclusion of aneuploidy. Among 19 BWS cases, 21% were conceived by ART. All omphaloceles underwent primary closure. Prenatally, macrosomia and polyhydramnios were seen in 42%. Macroglossia and nephromegaly were more commonly detected postnatally. Preterm birth occurred in 10/19 (53%) cases and cesarean deliveries were performed in 7/19 (40%) cases. Overall mortality was 20% (4/19). Embryonal tumors were diagnosed in 2/16 (12.5%) children, and neurodevelopmental outcomes were normal in 9/12 (75%) survivors., Conclusions: After excluding aneuploidy, BWS was identified in 37.5% and 5% of isolated and non-isolated omphaloceles, respectively. Omphaloceles were small-moderate size with good long-term surgical and neurodevelopmental outcomes when isolated., (© 2021 John Wiley & Sons Ltd.)

Introduction: Based on meta-analyses and registry data, the European Hernia Society and the Americas Hernia Society have published guidelines for the treatment of umbilical hernias. These recommend that umbilical hernia should generally be treated by placing a non-absorbable (permanent) flat mesh into the preperitoneal space with an overlap of the hernia defect of 3 cm. Suture repair should only be considered for small hernia defects of less than 1 cm. Hence, the use of a mesh in general is subject to controversial debate particularly for small (< 2 cm) umbilical hernias. This analysis of data from the Herniamed Registry now presents data on the treatment of small (< 2 cm) umbilical hernias over the past 10 years., Methods: Herniamed is an Internet-based hernia registry in which hospitals and surgical centers in Germany, Austria and Switzerland can voluntarily enter data on their routine hernia operations. Between 2010 and 2019, data were entered into the Herniamed Registry by 737 hospitals/surgery centers on a total of 111,765 patients with primary elective umbilical hernia repair. The prospective data were analyzed retrospectively for each year and statistically compared. Due to a higher number of cases, the years 2013 and 2019 were compared for the perioperative outcome and the years 2013 and 2018 for 1-year follow-up. Fisher's exact test was applied for unadjusted analyses between the years, using a significance level of alpha = 5%. For post hoc tests of single categories, a Bonferroni adjustment for multiple testing was implemented., Results: A mesh technique was used to treat 45.4% of all umbilical hernias. The proportion of small (< 2 cm) umbilical hernias in the total collective of umbilical hernias was 55.6%. Suture repair was used consistently over the 10-year period to treat around 75% of all small (< 2 cm) umbilical hernias. Preperitoneal mesh placement as recommended in the guidelines was used only in 1.8% of cases. Between 2013 and 2019, stable values of 2 and 0.7% were observed for the postoperative complications and complication-related reoperations, respectively, with no relevant effect identified for the surgical technique. At 1-year follow-up, significantly higher rates of pain at rest (2.6 vs. 3.3), pain on exertion (5.7 vs. 6.6), and recurrences (1.3 vs. 1.8) (all p < 0.05) were identified for 2018 compared with 2013., Conclusions: A suture technique is still used to treat 75% of patients with small (< 2 cm) umbilical hernias. The pain and recurrence rates are significantly less favorable for 2018 compared with 2013.

Purpose: The primary goal of this study was to determine the incidence of occult paraumbilical hernias during open primary umbilical hernia repair. The secondary objective was to further characterize the clinical features of these patients and hernias., Methods: This was a retrospective chart review of patients undergoing primary umbilical hernia repair at Shouldice Hospital, from 2007 to 2017. Inclusion criteria were utilized to elucidate patients, where a concomitant occult paraumbilical hernia was found. Descriptive statistics were used throughout., Results: 5850 patients underwent primary umbilical hernia repair, 459 (7.85%) patients had concomitant primary umbilical and paraumbilical hernias. There was a preoperative suspicion/diagnosis of a paraumbilical hernia in 166 (2.8%) of these patients. In 293 (5.01%) patients who had open primary umbilical hernia repair, at least one associated occult paraumbilical defect was found during surgery. Most of umbilical and concomitant occult paraumbilical hernias were small and medium size defects. The great majority of the reported occult paraumbilical hernias were found in the supraumbilical position at a distance of 3 cm or less from the top of the umbilical defect., Conclusion: The incidence of concomitant occult paraumbilical hernias in patients mildly overweight undergoing primary umbilical hernia repair is 5.01%, relevant to surgical decision-making. Since the great majority of these paraumbilical defects are superior to the umbilical defect, an adequate incision and dissection for at least 3 cm above the umbilical hernia may reduce the number of missed concomitant hernias and result in less presumed recurrences.

Purpose: The approach to repairing an initial umbilical hernia (IUH) varies substantially, and this likely depends on hernia size, patient age, sex, BMI, comorbidities including diabetes mellitus, and surgeon preference. Of these, only hernia size has been widely studied. This cross-sectional study aims to look at the practice pattern of umbilical hernia repair in the United States., Methods: A retrospective study was performed using data from the America Hernia Society Quality Collaborative. Patient characteristics included age, sex, hernia width, BMI, smoking status, and diabetes. Outcomes were use of mesh for repair, as well as surgical approach (open vs minimally invasive). Multivariate logistic regression was performed to assess the independent effect of age, sex, hernia width, BMI, smoking status, and diabetes on use of mesh and approach to repair., Results: 3475 patients were included. 74% were men. Mesh use was more common in men (67% vs 60%, P < 0.001). Mesh was used in 33% of repairs ≤ 1 cm, and 82% of repairs > 1 cm (P < 0.001). Younger patients were less likely to receive a mesh repair (54% if age ≤ 35 vs 67% for age > 35, P < 0.001). However, on multivariate analysis, mesh use was associated with increasing hernia width (OR 5.474, 95% CI 4.7-6.3) as well as BMI (OR 1.8, 95% CI 1.5-2.1) but not with age or sex., Conclusion: The majority of IUH are performed open. Patient BMI and hernia defect size contribute to choice of surgical technique including use of mesh. The use of mesh in 33% of hernias below 1 cm demonstrates a gap between evidence and practice. Patient factors including patient age and sex had no impact on operative approach or use of mesh.

Purpose: We sought to estimate the prevalence, incidence, and timing of surgery for elective and non-elective hernia repairs., Methods: We performed a retrospective cohort study, abstracting data on children < 18 years from the 2005-2014 DoD Military Health System Data Repository, which includes > 3 million dependents of U.S. Armed Services members. Our primary outcome was initial hernia repair (inguinal, umbilical, ventral, or femoral), stratified by elective versus non-elective repair and by age. We calculated prevalence, incidence rate, and time from diagnosis to repair., Results: 19,398 children underwent hernia repair (12,220 inguinal, 5761 umbilical, 1373 ventral, 44 femoral). Prevalence of non-elective repairs ranged from 6% (umbilical) to 22% (ventral). Incidence rates of elective repairs ranged from 0.03 [95% CI: 0.02-0.04] (femoral) to 8.92 [95% CI: 8.76-9.09] (inguinal) per 10,000 person-years, while incidence rates of non-elective repairs ranged from 0.005 [95% CI: 0.002-0.01] (femoral) to 0.68 [95% CI: 0.64-0.73] (inguinal) per 10,000 person-years. Inguinal (median = 20, interquartile range [IQR] = 0-46 days), ventral (median = 23, IQR = 5-62 days), and femoral hernias (median = 0, IQR = 0-12 days) were repaired more promptly and with less variation than umbilical hernias (median = 66, IQR = 23-422 days)., Conclusions: These data describe the burden of hernia repair in the U.S. The large variation in time between diagnosis and repair by hernia type identifies an important area of research to understand mechanisms underlying such heterogeneity and determine the ideal timing for repair., Level of Evidence: Prognosis study II.

Purpose: In this study, we aimed to comprehensively evaluate risk factors, ultrasound estimation of fetal weight, prenatal management, and pregnancy outcomes of gastroschisis and omphalocele at a metropolitan Australian hospital., Material and Methods: This was a retrospective single-center cohort study from 2006 to 2014 at a tertiary hospital with colocated neonatal surgical facilities. Demographic, pregnancy, ultrasound, birth and neonatal data were compared between gastroschisis and omphalocele. Correlation between routine (Hadlock 1 &2) and specific (Siemer) estimated fetal weight (EFW) estimation formulae with birth weight (BW) was made for those 50 gastroschisis cases with ≥2 third trimester scans and last scan ≤2 weeks prior to birth., Results: There were 126 abdominal wall defects: 83 gastroschisis and 43 omphalocele. Consistent with international literature, the average maternal age was lower for gastroschisis and rates of smoking higher, while there were more intrauterine deaths and pregnancy terminations in omphalocele. Gastroschisis mothers were more likely living outside Sydney, had more infections in pregnancy and were followed with a larger number of antenatal visits, with a shorter period from the last visit to birth. In omphalocele pregnancies, amniocentesis was more likely performed, with more abnormal results than in gastroschisis fetuses. All EFW formulae had a good correlation between Z score for the last US and actual BW (ICC 0.693-0.815), with Hadlock 2 being the best. Siemer formula had the best correlation from first to the last scan. Gastroschisis newborns were born earlier (36.8 versus 38.2 wks p  = .001), with smaller birthweight (2.52 versus 3.03 kg, p  < .001), a longer request of intensive care (central line, parenteral nutrition, intubation) and second surgery, along with more multisystem complications (average 1.5 versus 0.7, p  = .004) and a longer hospital stay (58.8 versus 36.8 d, p  < .001)., Conclusion: Demographic, antenatal, and pregnancy outcome data for abdominal wall defects correlated well with the international literature. Hadlock 1-2 gave the most consistent EFW estimate, with all formulae showing good correlation.

Background: Omphalocele is the second most common abdominal birth defect and often occurs with other structural and genetic defects. The objective of this study was to determine omphalocele prevalence, time trends, and mortality during early childhood, by geographical region, and the presence of associated anomalies., Methods: We conducted a retrospective study with 23 birth defect surveillance systems in 18 countries who are members of the International Clearinghouse for Birth Defects Surveillance and Research that submitted data on cases ascertained from 2000 through 2012, approximately 16 million pregnancies were surveyed that resulted in live births, stillbirths, or elective terminations of pregnancy for fetal anomalies (ETOPFA) and cases with omphalocele were included. Overall prevalence and mortality rates for specific ages were calculated (day of birth, neonatal, infant, and early childhood). We used Kaplan-Meier estimates with 95% confidence intervals (CI) to calculate cumulative mortality and joinpoint regression for time trend analyses., Results: The prevalence of omphalocele was 2.6 per 10,000 births (95% CI: 2.5, 2.7) and showed no temporal change from 2000-2012 (average annual percent change = -0.19%, p = .52). The overall mortality rate was 32.1% (95% CI: 30.2, 34.0). Most deaths occurred during the neonatal period and among children with multiple anomalies or syndromic omphalocele. Prevalence and mortality varied by registry type (e.g., hospital- vs. population-based) and inclusion or exclusion of ETOPFA., Conclusions: The prevalence of omphalocele showed no temporal change from 2000-2012. Approximately one-third of children with omphalocele did not survive early childhood with most deaths occurring in the neonatal period., (© 2020 Wiley Periodicals LLC.)

Gastroschisis and omphalocele are the two most common abdominal wall birth defects, and epidemiologic characteristics and frequency of occurrence as part of a syndromic condition suggest distinct etiologies between the two defects. We assessed complex patterns of defect co-occurrence with these defects separately using the Texas Birth Defects Registry. We used co-occurring defect analysis (CODA) to compute adjusted observed-to-expected (O/E) ratios for all observed birth defect patterns. There were 2,998 non-syndromic (i.e., no documented syndrome diagnosis identified) cases with gastroschisis and 789 (26%) of these had additional co-occurring defects. There were 720 non-syndromic cases with omphalocele, and 404 (56%) had additional co-occurring defects. Among the top 30 adjusted O/E ratios for gastroschisis, most of the co-occurring defects were related to the gastrointestinal system, though cardiovascular and kidney anomalies were also present. Several of the top 30 combinations co-occurring with omphalocele appeared suggestive of OEIS (omphalocele, exstrophy of cloaca, imperforate anus, spinal defects) complex. After the exclusion of additional cases with features suggestive of OEIS in a post-hoc sensitivity analysis, the top combinations involving defects associated with OEIS (e.g., spina bifida) were no longer present. The remaining top combinations involving omphalocele included cardiovascular, gastrointestinal, and urogenital defects. In summary, we identified complex patterns of defects that co-occurred more frequently than expected with gastroschisis and omphalocele using a novel software platform. Better understanding differences in the patterns between gastroschisis and omphalocele could lead to additional etiologic insights., (© 2020 Wiley Periodicals LLC.)

Background: Congenital Diaphragmatic Hernia (CDH) associated with Omphalocele is a rare condition, and only a few case reports are available in the literature. Both conditions are associated with some degree of pulmonary hypoplasia. We hypothesize that the combination of CDH with Omphalocele might be associated with poorer outcomes., Aim: The aim of this study was to describe the incidence of this association and postnatal outcomes from the largest database available for CDH., Methods: Data from the multicenter, multinational database on infants with CDH (CDHSG Registry) born from 2007 to 2018 was analyzed., Results: A total of 5730 entries were made into the registry during the study period. The incidence of Omphalocele associated with CDH was 0.63% (36 out of 5730). When comparing posterolateral Bochdalek hernias with Omphalocele (CDH + O) to CDH without Omphalocele (CDH-), CDH + O were born at significantly younger gestational ages. They were sicker directly after birth with significantly lower APGARs at all time points, but received ECMO significantly less often. The distribution of left vs right side or the defect size did not differ but CDH + O required patch in a significantly larger extent. CDH + O had surgical repair significantly later and had significantly higher rates of non-repairs and significantly lower survival rates. The morbidity was significantly higher with longer hospital stays and higher requirements for O2 at 30 DOL., Discussion: CDH associated with Omphalocele is a rare but more severe condition with higher mortality and morbidity rates. Newborns with these combined conditions can be difficult to stabilize or might pose complicated management problems due to pulmonary hypertension and/or pulmonary hypoplasia., Type of Study: Prognosis Study., Level of Evidence: Level I., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: This study was conducted to evaluate the incidence and early predictive factors of the development of protuberant umbilicus in pediatric umbilical hernia patients., Methods: In this retrospective visual and chart review, patients younger than 3 months with umbilical hernias who initially visited Ina Central Hospital from April 2011 to March 2017 and were followed until they started to walk (at the age of 1 year) were evaluated. The umbilici of the patients at the age of 1 year were classified into two types based on their appearance: concave and protuberant umbilici. Single-factor and logistic regression analyses of the association between the appearance of the umbilicus at the age of 1 year and various clinical data were performed., Results: Of the 103 patients, 72% had concave umbilici, and 28% had protuberant umbilici. Single-factor analysis showed significant differences in the umbilical shapes at the initial visit (P < 0.001) and straining habit (P < 0.001). The most ideal logistic regression model demonstrated that umbilici of the highly inflated balloon type (odds ratio, 27.00; 95% confidence interval odds ratio, 5.60-130.08) and crescent type (odds ratio, 14.34; 95% confidence interval odds ratio, 4.22-48.77) were more likely to develop into protuberant umbilici., Conclusions: Umbilical shapes at the initial visit can be used to predict the future development of protuberant umbilici in pediatric patients with umbilical hernias., (© 2020 Japan Pediatric Society.)