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We evaluate the incidence, outcomes, and management of peri‑umbilical hernias after sutured or sutureless gastroschisis closure.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.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%, p0.001). 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%, p0.001). 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.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.Level II.

Congenital omphalocele is among the most common antenatally diagnosed anomalies. As additional abnormalities are found in majority of cases, antenatal investigations target the search for additional structural anomalies and genetic disorders, including aneuploidy. Antenatal management focuses on the assessment of fetal well-being. Unfortunately, antenatal prediction of postnatal and long-term outcomes represents 2 less well-documented but crucial facets of this pathology. A large part of the prognosis relies on aspects that are difficult to predict such as quality of life, neurological development, and autonomy, which cause significant anxiety in expectant parents.This article offers a comprehensive review of antenatal management of omphalocele with a specific focus on predictive factors and long-term outcomes.We conducted an extensive literature review targeting management of fetal omphalocele. We had a specific interest in factors predictive of fetal and neonatal outcome as well as long-term consequences of omphalocele. Fetuses with large defects and those containing the liver are at higher risk of having a complicated postnatal course. Neonates may experience pulmonary hypoplasia, pulmonary hypertension, and gastroesophageal reflux. In selected cases, motor and cognitive delay may be present, but the overall life-long prognosis and quality of life is good.A multidisciplinary approach should be encouraged after the diagnosis of fetal omphalocele. In addition to clinical team experience, antenatal counseling should be based on objective and gestational age-dependent criteria and should include long-term outcomes.

Introduction : Umbilical access in laparoscopic surgery has been cited as a factor for increased complications in low-birth-weight infants and those less than three months old. In a previous series, 10.6% of pediatric surgeons reported complications in this population associated with umbilical access, citing carbon dioxide (CO2) embolism as the most common complication. To further examine the safety of this technique, we report our outcomes with blunt transumbilical laparoscopic access at our institution over four years. Methods : A retrospective review was performed of patients less than three months of age who underwent laparoscopic pyloromyotomy or inguinal hernia repair from 2016-2019. Operative reports, anesthesia records, and postoperative documentation were reviewed for complications related to umbilical access. Complications included bowel injury, vascular injury, umbilical vein cannulation, CO2 embolism, umbilical surgical site infection (SSI), umbilical hernia requiring repair, and death. Results : Of 365 patients, 246 underwent laparoscopic pyloromyotomy, and 119 underwent laparoscopic inguinal hernia repairs. Median age at operation was 5.9 weeks [4.3,8.8], and median weight was 3.9 kg [3.4,4.6]. Nine complications (2.5%) occurred: 5 umbilical SSIs (1.4%), 1 bowel injury upon entry requiring laparoscopic repair (0.2%), 1 incisional hernia repair 22 days postoperatively (0.2%), and 2 cases of hypotension and bradycardia upon insufflation that resolved with desufflation (0.5%). There were no intraoperative mortalities or signs/symptoms of CO2 embolism. Conclusion : In this series, umbilical access for laparoscopic surgery in neonates less than three months of age was safe, with minimal complications. Although concern for umbilical vessel injury, cannulation, and CO2 embolism exists, these complications are not exclusively associated with umbilical access technique.

We compared cases of anemia in gastroschisis versus omphalocele and investigated this clinical question.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.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%).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.

The umbilicus is the site of a number of well-recognized and unusual abnormalities. Well-known neonatal umbilical abnormalities include umbilical hernias, granulomas/polyps, and congenital remnants of development. In this article, we describe a rare case of an appendix draining through the umbilicus of a neonate. In the literature, there are only 15 cases with possible umbilical appendix. We describe this rare case along with a review of the literature and discuss the underlying pathophysiology.

Ectopia cordis is a rare congenital defect with the prevalence of 5 to 8 per million live births. Here we report a rare case of preterm female live birth with ectopia cordis associated with omphalocele, cleft lip, and palate. In this case, 14+ weeks ultrasound did not show any fetal abnormalities and parents were unaware of the condition until 35+ weeks when ultrasound detected the anomaly a few days before delivery. After delivery, they didn’t give consent for further intervention which led to neonatal mortality 3 hours after birth. If the condition was diagnosed in time, an earlier intervention could have been done.

Introduction Controversy exists over the best dressing for conservative management of exomphalos major. Here we describe our experience of using Manuka Honey. Methods Our regimen involved covering the sac with Manuka honey (Advancis Medical™) wrapped with gauze and crepe bandage. Initially, dressings were changed 3 times a week and then twice weekly until full epithelialisation. Babies went home after reaching full feeds, with our outreach nurses continuing dressings in clinic until the parents were trained to do them alone. Only patients needing management of co-morbidities were transferred to our unit. Patients would be reviewed by video consultation. Data was prospectively collected. Results From 2011–2019, 24 consecutive patients (11:13 M:F; median gestation 37 weeks, birth weight 3.1 kg) with exomphalos major were managed with honey dressings. Fourteen babies had significant associated anomalies of which 10 died of problems unrelated to the exomphalos. Time to full feeds 6 (2-58) days; time to discharge 21(7–66) days if no associated anomalies; time to epithelialisation 73 (27–199) days. Dressings were well tolerated. Definitive closure occurred at 17(11–38) months and was uneventful. No patient required fundoplication and all patients were orally fed. Only one patient developed a clinically significant infection. Conclusion This is the largest report of using Manuka honey for the management of exomphalos major. Benefits include early feeding, early discharge and a ‘normalisation’ of the neonatal period. Key to our success was the surgical outreach service supporting parents doing the dressings, first at the local hospital and then at home.

Aim The management of patients with congenital anterior abdominal wall defects remains challenging, particularly in cases of giant omphalocele. In 1948, San Martin described a surgical technique for the repair of large midline incisional hernias in adults without the need for a mesh. The purpose of this report is to describe our experience with this technique for the delayed closure of giant omphaloceles. Methods We retrospectively reviewed the outcomes of all patients with giant omphalocele managed with the San Martin technique between September 2013 and March 2019. Data collected included birth weight, gestational age, associated malformations, neonatal hospital stay, age at the time of the abdominal wall closure, days on mechanical ventilation (MV) after the closure, time to initiation of enteral feedings, intra- and postoperative complications, and postoperative hospital stay. Results A total of 8 patients were included in the study. The median birth weight was 3.190 (2.150 to 3.400) grams. The median gestational age was 35 (32 to 38) weeks. The median age at surgery was 6 (5 to 13) years. The median postoperative days on MV was 3 (3 to 11) days. Enteral feeding were initiated postoperatively at a median of 4 ( 2 to 4) days. There was one intraoperative complication (minor vascular injury). There were no short-term or long-term complications directly related to the surgical technique. The median postoperative hospital stay was 10 (6 to 16) days. The follow-up was 18 months to 8 years. Conclusion We believe that the San Martin technique is a valid alternative for the delayed closure of giant omphaloceles. Level of evidence According to the Journal of Pediatric Surgery this research corresponds to type of study level IV for case series with no comparison group.

Introduction: Giant omphaloceles can be a challenge for pediatric surgeons and neonatologists worldwide. It is a rare and low-frequency congenital anomaly with no standardized management schemes or treatment protocols. Over the past few decades, we have developed a simple and efficient staged management for giant omphaloceles that allows definitive closure in the neonatal period, the results of which we outline in this report. Material and methods: With IRB approval, a retrospective and multicentric cohort study was carried out between 1994 and 2019 with patients with giant omphalocele defined as an abdominal wall defect greater than 5 cm in diameter and/or that contains more than 50% of the liver within the sac. We included all patients managed with the nonsurgical silo technique. Data on demographics, gestational age, associated malformations, amnion reduction and inversion time, anatomic closure, requirement of a mesh, intra- and post-silo complications, mortality and follow-up were collected. The technique consists of the construction of a silo with an adhesive hydrocolloid dressing (DuodermⓇ) to achieve an omphalocele staged-reduction until complete abdominal reintegration of the liver and bowel preservation of the amnion sac. This also enables the simulation of abdominal closure before definitive surgical closure, being managed in the neonatal intensive care unit (NICU). Results: Forty patients, 21 of whom were female, were managed with this technique. The average weight was 2900 gs (890–3900), and the median gestational age was 38 weeks (28–40). In total, 37.5% of cases had an associated comorbidity. The average silo reduction time was 7.3 days (0–35), the average time of amnion inversion was 5 days (2–9), and the average time to closure was 14.6 days (6–38). Anatomical closure was achieved in 95% of cases. In 4 patients, an absorbable mesh was used to reinforce the anatomical closure, and in 2 patients (5%), a mesh (DualmeshⓇ) was required to achieve an abdominal closure. There was no mortality associated with this nonsurgical silo technique. The average follow-up time was 60 (6 - 288) months. Conclusion: The staged silo management of giant omphalocele in this series is safe and effective and reduces the time to closure and potential morbidity and mortality compared with traditional surgical or medical management.

The pathogenesis of omphalocele and gastroschisis is not obvious. Their etiology is disputed. The prevalence and the types of anomalies co-occurring with omphalocele and gastroschisis are variable in the different series published. The aim of this study was to estimate the frequency and the types of co-occurring anomalies in cases with gastroschisis and omphalocele. This study was performed in a well-described population of 387,067 consecutive births between 1979 and 2007. Hundred-one cases with omphalocele were registered (2.61 per 10,000), 75 (74.3%) had co-occurring anomalies comprising chromosomal anomalies (28 cases, 27.7%, including 18 trisomy 18), non-chromosomal syndromes (16 cases, 15.8%, including 3 cases with Beckwith-Wiedemann syndrome, 2 cases with the OEIS sequence, and one case with the Pentalogy of Cantrell complex), and 31 cases, 30.7% with MCA (multiple congenital anomalies). The most common MCA were musculoskeletal (23.5%), urogenital (20.4%), cardiovascular (15.1%), and central nervous (9.1%). Seventy-one cases of gastroschisis were ascertained (1.83 per 10,000). However, the prevalence increased during the study period. The frequency was highest in the mothers 15-19 years old. Sixteen out of the 71 cases with gastroschisis, (22.5%) had co-occurring anomalies including 11 cases of MCA and 5 cases with syndromes. To conclude, the frequency and the types of anomalies co-occurring with omphalocele and gastroschisis are peculiar. Therefore, cases with gastroschisis and omphalocele need to be screened for co-occurring anomalies.

Objective To describe the antenatal and pathological features of an immature umbilical cord teratoma associated with exomphalos, and to review the literature on this subject. Case presentation An abdominal wall defect, suspected to be an exomphalos, was identified during routine ultrasound examination performed at 13 weeks of gestation. The pregnancy was terminated. Fetopathological examination revealed an immature umbilical cord teratoma associated with exomphalos. Chromosomal microarray analysis was normal. Conclusions Umbilical cord teratomas, albeit very rare, should be emphasized as a possible differential diagnosis when abdominal wall defects are detected. Since cord teratomas may lead to adverse fetal or neonatal outcomes, close follow-up of the fetus is recommended.

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.

To characterize regional variation in the age of patients undergoing umbilical hernia repair to determine costs and subsequent care.We performed a cross-sectional descriptive study using a large convenience sample of US employer-based insurance claims from July 2012 to December 2015. We identified children younger than 18 years of age undergoing uncomplicated (not strangulated, incarcerated, or gangrenous) umbilical hernia repair as an isolated procedure (International Classification of Diseases, Ninth Revision procedure codes 53.41, 53.42, 53.43, or 53.49, International Classification of Diseases, Tenth Revision procedure code 0WQF0ZZ, or Current Procedural Terminology procedure codes 49580 or 49585).In all, 5212 children met criteria for inclusion. Children younger than age 2 years accounted for 9.7% of repairs, with significant variation by census region (6% to 14%, P .001). Total payments for surgery varied by age; children younger than 2 years averaged $8219 and payments for older children were $6137. Postoperative admissions occurred at a rate of 73.1 per 1000 for children younger than age 2 years and 7.43 for older children; emergency department visits were 41.5 per 1000 for children younger than age 2 years vs 15.9 for older children (P .001).Umbilical hernias continue to be repaired at early ages with large regional variation. Umbilical hernia repair younger than age 2 years is associated with greater costs and greater frequency of postoperative hospitalization and emergency department visits.

Several recurrent malformation associations affecting the development of the embryo have been described in which a genetic etiology has not been found, including LBWC, MURCS, OAVS, OEIS, POC, VACTERL, referred to here as "recurrent constellations of embryonic malformations" (RCEM). All are characterized by an excess of reported monozygotic discordant twins and lack of familial recurrence. We performed a comprehensive review of published twin data across all six phenotypes to allow a more robust assessment of the association with twinning and potential embryologic timing of a disruptive event. We recorded the type of twinning, any overlapping features of another RCEM, maternal characteristics, and the use of ART. Statistically significant associations included an excess of monozygotic twins and 80% discordance rate for the phenotype across all twins. There was an 18.5% rate of ART and no consistently reported maternal adverse events during pregnancy. We found 24 instances of co-occurrence of two RCEM, suggesting a shared pathogenesis across all RCEM phenotypes. We hypothesize the following timing for RCEM phenotypes from the earliest perturbation in development to the latest: LBWC, POC, OEIS, VACTERL, OAVS, then MURCS. The RCEM group of conditions should be considered a spectrum that could be studied as a group.

A study of the prevalence rates for selected isolated non-Mendelian congenital anomalies in the Hutterite Brethren of Alberta, Canada was undertaken to further examine longitudinal data in this isolated community that was last reported in 1985 (Lowry et al., 1985), although there are numerous publications on recessive disorders (Boycott et al., 2008; Triggs-Raine et al., 2016). Cases were ascertained from the Alberta Congenital Anomaly Surveillance System for the years 1997-2016. Since our initial results showed some surprising findings in the Hutterite Brethren, such as zero cases of spina bifida, cleft lip and palate, gastroschisis, and omphalocele, and a significant excess of cases with hypospadias, we extended the study to prior years (1980-1996) for selected anomalies. For the extended study period (1980-2016), there was a significant increased prevalence of hypospadias, tetralogy of Fallot and tricuspid atresia in the Hutterite population, and although not statistically significant, zero cases of cleft lip with cleft palate, gastroschisis and omphalocele were confirmed. Further research is needed to determine the precise effects of rural environmental exposures, lifestyle factors, and genetic associations for selected multifactorial congenital anomalies.

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.

BACKGROUND Neonates with abdominal wall defects are at an increased infection risk because of the defect itself and prolonged neonatal intensive care unit (NICU) stays. Antibiotic prophylaxis until closure of the defect is common. However, infection risk and antibiotic use have not been well quantified in these infants. METHODS A retrospective cohort study of infants with abdominal wall defects (gastroschisis and omphalocele) admitted to a single-center NICU from 2007 to 2018. Demographic and clinical information, including microbiologic studies, antibiotic dosing and surgical care, were collected. Antibiotic use was quantified using days of therapy (DOT) per 1000 patient-days. Sepsis was defined as culture of a pathogen from a normally sterile site. RESULTS Seventy-four infants were included; 64 (86%) with gastroschisis and 10 (14%) with omphalocele. Median day of closure was 8 days [interquartile range (IQR) 6-10, range 0-31]. All infants received ≥1 course of antibiotics; median antibiotic DOT/infant was 24.5 (IQR 18-36) for an average of 416.5 DOT per 1000 patient-days. Most antibiotic use was preclosure prophylaxis (44%) and treatment of small intestinal bowel overgrowth (24%). Suspected and proven infection accounted for 26% of all antibiotic use. Skin and soft tissue infection (13/74, 18%) and late-onset sepsis (11/74, 15%) were the most common infections; 2 infants had sepsis while on antibiotic prophylaxis. All infants survived to discharge. CONCLUSIONS Most antibiotic use among infants with abdominal wall defects was prophylactic. Infection on prophylaxis was rare, but 35% of infants had infection after prophylaxis. Improved stewardship strategies are needed for these high-risk infants.

Purpose To examine hospital-level variation in the timing of asymptomatic umbilical hernia repair in children. Methods Retrospective analysis of children undergoing umbilical hernia repair at 38 children's hospitals using the Pediatric Health Information System database (01/2013–12/2017). Early repair was defined as surgery performed at 3 years of age or younger. Repairs were categorized as emergent/urgent if associated with diagnostic or procedural codes indicating obstruction or strangulation, or if they occurred within 2 weeks of an emergency department encounter. Multivariable regression was used to calculate hospital-level observed-to-expected (O/E) ratios for early repair adjusting for emergent/urgent repair and patient characteristics. Results 23,144 children were included, of which 30% underwent early repair (hospital range: 6.9%–54.3%, p ≪ 0.001). Overall, 3.8% of all repairs were emergent/urgent, and higher rates of early repair did not correlate with higher rates of emergent/urgent repair across hospitals (r = − 0.10). Following adjustment, hospital-level O/E ratios for early repair varied 8.9-fold (0.19–1.70, p ≪ 0.001). Conclusion Timing of asymptomatic umbilical hernia repair varies widely across children's hospitals, and the magnitude of this variation cannot be explained by differences in patient characteristics or the acuity of repair. Many children may be undergoing repair of hernias that may spontaneously close with further observation. Level of Evidence. Level III (retrospective comparative study).

The 2 most common congenital abdominal wall defects are gastroschisis and omphalocele. Both are usually diagnosed prenatally with fetal ultrasonography, and affected patients are treated at a center with access to high-risk obstetric services, neonatology, and pediatric surgery. The main distinguishing features between the 2 are that gastroschisis has no sac and the defect is to the right of the umbilicus, whereas an omphalocele typically has a sac and the defect is at the umbilicus. In addition, patients with an omphalocele have a high prevalence of associated anomalies, whereas those with gastroschisis have a higher likelihood of abnormalities related to the gastrointestinal tract, with the most common being intestinal atresia. As such, the prognosis in patients with omphalocele is primarily affected by the severity and number of other anomalies and the prognosis for gastroschisis is correlated with the amount and function of the bowel. Because of these distinctions, these defects have different management strategies and outcomes. The goal of surgical treatment for both conditions consists of reduction of the abdominal viscera and closure of the abdominal wall defect; primary closure or a variety of staged approaches can be used without injury to the intra-abdominal contents through direct injury or increased intra-abdominal pressure, or abdominal compartment syndrome. Overall, the long-term outcome is generally good. The ability to stratify patients, particularly those with gastroschisis, based on risk factors for higher morbidity would potentially improve counseling and outcomes.

Objective Pulmonary hypertension (PH) has been described in the neonatal omphalocele population. This study was aimed to describe cardiac function and PH severity using echocardiography in newborns with giant omphalocele (GO) and with non-GO and determine if right ventricular (RV) dysfunction is associated with mortality. Study Design Retrospective, single-center analysis of first echocardiography among neonatal omphalocele patients born between 2004 and 2017 was conducted. Multivariate logistic and univariate Cox's regression was constructed to measure hazard ratio (HR) for death outcome. Results There were 32 newborns, of whom 18 were GO and 7 died. GO had increased systolic pulmonary arterial to systolic systemic blood pressure ratio (97% [isosystemic] vs. 73% [three-fourths systemic] p = 0.03). RV performance parameters (tricuspid annular plane excursion, HR = 0.40; fractional area change, HR = 0.90; and RV peak global longitudinal strain, HR = 1.39) were associated with mortality. These RV performance parameters remained associated in a multiple logistic regression accounting for gestational age and GO status. The overall population had abnormal eccentricity index and pulmonary artery acceleration time to RV ejection time ratio, two markers of PH. Conclusion Patients with omphalocele have increased pulmonary pressure, with GO being worse than non-GO. RV dysfunction at initial echocardiography was significantly associated with mortality.

PurposeOmphalocele is a congenital abdominal wall defect with an incidence of 1/4,200 births. Repair timing varies from the neonatal period to the first few years of life. Surgical technique has changed over the last two decades. We sought to establish improved surgical/ventilation protocols for patients with omphaloceles requiring abdominal reconstruction. MethodsAn IRB-approved retrospective review was performed on patients with omphalocele requiring abdominal wall reconstruction by Plastics and/or Pediatric Surgery at a pediatric tertiary-care referral center (January 2006-July 2021). Birth history, comorbidities, surgical details, ventilation data, complications/recurrence were extracted. Results Of 129 patients screened, seven required Plastic Surgery involvement. Defect size was 102.9 cm2 (range: 24 - 178.5); four patients required component separation; zero patients received mesh; zero complications/recurrences were recorded. Two patients required postoperative ventilation for 2.5 days, based on increased peak inspiratory pressures at surgery stop versus start time.ConclusionPatients with large defects secondary to omphalocele benefit from collaboration between Pediatric and Plastic Surgery for component separation and primary fascial closure without mesh. Future research should follow patients who mature out of pediatric clinics to evaluate the incidence of hernias in adults with Plastic Surgery-repaired omphaloceles.

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. Resumo Objetivo Caracterizar e comparar os desfechos do onfalocelo e gastrosquisis desde o nascimento até aos 2 anos de seguimento numa coorte recente de um centro terciário. Métodos Este é um estudo retrospectivo em que foi feita uma revisão dos registos clínicos de todos os pacientes com gastrosquisis e onfalocelo que foram internados na unidade de cuidados intensivos neonatais, entre janeiro de 2009 e dezembro de 2019. Resultados Identificamos 38 pacientes, 13 dos quais tinham onfalocelo e 25 dos quais tinham gastrosquisis. Anomalias associadas estavam presentes em 6 pacientes (46.2%) com onfalocelo e 10 (41.7%) com gastrosquisis. Comparativamente com os pacientes com onfalocelo, os pacientes com gastrosquisis tinham mães mais jovens (24.7 versus 29.6 anos; p=0.033), nasceram mais precocemente (36 versus 37 semanas, p=0.006), com menor peso ao nascimento (2,365±430.4 versus 2,944.2±571.9 g; p=0.001), e o internamento teve uma duração mais longa (24 versus 9 dias, p=0.001). A taxa de sobrevivência neonatal foi de 92.3% para o onfalocelo e 91.7% para a gastrosquisis. Trinta e quatro pacientes foram seguidos durante umtempo mediano de seguimento de 24meses: 13 com gastrosquisis (59.1%) e 8 com onfalocelo (66.7%) apresentaram pelo menos um evento adverso, sobretudo hérnia umbilical (27.3% vs 41.7%), obstrução intestinal (31.8% vs 8.3%) ou intervenções cirúrgicas adicionais (27.3% vs 33.3%). Conclusão Apesar da alta proporção de prematuridade, de baixo peso e de recuperação lenta, os gastrosquisis, assim como os onfalocelos (sem anomalias cromossómicas), podem ter uma taxa de sobrevivência muito alta; por outro lado, nos primeiros anos de vida, podem surgir complicações não desprezíveis. Assim, aos futuros pais pode ser transmitida uma perspectiva muito positiva em termos de sobrevivência, embora eles também devam ser informados de que pode ocorrermorbidade substancial no médio prazo.

To relate omphalocele and biliary atresia and investigate possible embryological correlations that justify the simultaneous occurrence. A female preterm newborn diagnosed as omphalocele; cesarean delivery, weight 2,500g, 46 XX karyotype. Initially, the newborn remained fasting and on parenteral nutrition, and enteral diet was introduced later, with good acceptance. On the 12th day of life, the newborn presented direct hyperbilirubinemia, increased levels of liver enzymes and fecal acholia, with a presumptive diagnosis of biliary atresia. However, the ultrasound was inconclusive, due to anatomical changes resulting from omphalocele. A surgical approach was chosen on the 37th day of life aiming to confirm diagnosis of biliary atresia and to repair omphalocele. During the surgical procedure, structural alterations compatible with biliary atresia were observed, later confirmed by pathological examination; a hepatoportoenterostomy was performed and the omphalocele was corrected. She evolved well in the postoperative period, with a decrease in direct bilirubin and liver enzymes, as well as resolution of fecal acholia, and was discharged in good clinical condition. This is a bizarre and extremely rare association, but the prognosis may be good when an early diagnosis is made and surgery performed, besides support and clinical management to prevent complications in the perioperative period. Although the pathogenesis of the diseases has not been fully defined yet, there is, to date, no direct relation between them. The association between omphalocele and biliary atresia is extremely uncommon, with only two published cases.

Background Kagami–Ogata syndrome is also known as paternal uniparental disomy 14 and related disorders and is caused by abnormal genomic imprinting in the long arm of the chromosome 14q32.2 region. Its clinical manifestations include polyhydramnios in the fetal stage, respiratory insufficiency because of a small thorax, abdominal wall abnormalities, and peculiar facial features after birth. Case presentation A 38-year-old Japanese primigravida woman was referred to our hospital in the 19th week of pregnancy for suspected omphalocele. She had a history of hypothyroidism but was prescribed orally administered levothyroxine (50 μg/day) prior to conception and was euthyroid. Her ultrasound scan prior to visiting our hospital revealed fetal omphalocele, heavy for date, and polyhydramnios. The mother was advised to be admitted for observation from 28 weeks of gestation for threatened premature delivery. She required amniodrainage at 29 and 32 weeks of gestation. At 35 weeks of gestation, the fetal membrane prematurely ruptured and she gave birth after an emergency Cesarean section. The infant was a male child with a birth weight of 3188 g, and was suspected to have Kagami–Ogata syndrome after birth based on thoracic hypoplasia, swallowing function abnormalities, and peculiar facial features. A definitive diagnosis was established by performing genetic testing of the infant after obtaining informed written consent from both the parents; the results of the genetic testing revealed hypermethylated intergenic-differentially methylated region and maternally expressed gene 3-differentially methylated region in the corresponding chromosome 14 region. Both the parents were genetically tested after adequate genetic counseling, which revealed a de novo microdeletion in a differentially methylated region. Conclusion Kagami–Ogata syndrome should have been suspected because of the presence of polyhydramnios and omphalocele during pregnancy. Respiratory insufficiency soon after birth, because of a small thorax, is expected in this disease and a diagnosis during pregnancy may have enabled appropriate care after birth.

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.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 12) 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.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 wksDemographic, 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.

To characterize factors associated with adverse neonatal outcomes in prenatally diagnosed omphalocele cases. Prenatally diagnosed omphalocele cases at a single referral center from 1 January 2009 to 31 December 2017 were retrospectively reviewed. Clinical variables and antenatal imaging measurements were collected. Associations between prenatal and neonatal characteristics and the adverse outcome of death or prolonged length of stay (LOS) were analyzed. Out of 63 fetal cases, 33 were live-born, > 50% had other anomalies, and neonatal mortality was 12%. Adverse outcomes were associated with neonatal variables, including lower median 1-min Apgar score, initial mechanical ventilation, and late-onset sepsis, but not approach to omphalocele closure. With multivariate analysis, death or prolonged LOS was associated only with low lung volumes by fetal MRI (OR 34 (3–422), p = 0.006). Low lung volumes by fetal MRI were associated with death or prolonged LOS in neonates with prenatally diagnosed omphalocele and may guide clinicians with counseling families.

OEIS is the acronym of a malformations complex association including omphalocele, exstrophy of bladder or cloaca, imperforate anus, and spinal defects. It has a very low prevalence, ranging from 1/82,000 to 1/200,000 live births (LB). The etiology of OEIS is unknown. Virtually all cases are sporadic, and specific associated risk factors uncertain. Objectives This study aimed to determine the prevalence, clinical spectrum, possible early pregnancy exposures, and demographic characteristics as potentially associated risk factors in a sample of Mexican cases. Methods We conducted a multihospital based case-control study on 12 cases with the OEIS complex identified in 1,195,020 LB born from January 1978 to December 2015. All comparisons performed were matching 1:3 the relation of cases and controls, respectively, considering the p-value of ≤.05 as statistically significant. Results The prevalence of OEIS was 1.004/100,000 (1/99,585) LB. The frequency of bladder/cloacal exstrophy was 75 and 25%, respectively, omphalocele was 83.3%, and imperforate anus and spinal defects, 75.0% each. Two pairs of twins discordant for the defect exhibited the severest OEIS phenotype. Except for the higher frequency of maternal first pregnancy trimester influenza infection, early perinatal mortality and a twining trend association, none other variable differed significantly. Discussion The prevalence of OEIS in our sample is within the highest reported worldwide. First-trimester pregnancy maternal influenza infection and twining emerge as associated risk factors for OEIS. Although twin zygosity was not defined, the observed severest phenotypes in twins endorse the hypothesis that OEIS and monozygotic twinning are features of disturbances on early blastogenesis.

We developed treatment guidelines (TGs) for appropriate transitional care of the genitourinary system in patients with persistent cloaca (PC), cloacal exstrophy (CE), or Mayer-Rokitansky-Küster-Häuser syndrome (MRKH). These TGs are in accordance with the Medical Information Network Distribution Service (Minds), published in 2014 in Japan. Clinical questions (CQs) concerning treatment outcomes of the genitourinary system, pregnancy and delivery, and quality of life in adulthood were prepared as six themes for PC and CE and five themes for MRKH. We were able to publish statements on chronic renal dysfunction, hydrometrocolpos, and pregnancy, based on four CQs about PC, four about CE, and two about MRKH, respectively. However, due to the paucity of proper manuscripts, we were unable to make conclusions about the correct timing and method of vaginoplasty for patients with PC, CE, and MRKH or the usefulness of early bladder closure for patients with CE. These TGs may help clarify the current treatments for PC, CE, and MRKH in childhood, which have been carried out on an institutional basis. To improve clinical outcomes, systematic clinical trials revealing comprehensive clinical data of the urinary and reproductive systems, especially the length of the common channel in PC, are essential.

Pediatric umbilical hernia repair is a common procedure that requires minimal tissue disruption. We examined variation in opioid prescription fills after repair of uncomplicated umbilical hernias to characterize the types and doses of medication used and persistent postsurgical use.Using the Truven Health Analytics MarketScan© Research Database for June 2012-September 2015, we identified pediatric patients undergoing umbilical hernia repair. We excluded patients with obstruction, gangrene, an earlier repair or a concurrent surgical procedure, and those without available pharmacy claim data. Analyses describe filled outpatient prescriptions by age, geographic region, drug type, quantity, and second prescriptions/refills.Of 4,407 procedures performed, 2,292 patients (52%) filled a prescription for postoperative opioids (age 0-1 years: 21.6%, age 2-3 years: 51.5%, age 4-5 years: 54.3%, 6 years or older: 57.9% [P.0001]). In the northeast United States, 42% of patients filled narcotic prescriptions, compared with 59% of patients in the south (P.0001). Hydrocodone/acetaminophen was most commonly prescribed (51%), followed by codeine/acetaminophen (30%). Durations were ≤3 days (50%), 4-10 days (46%), and10 days (4%). A total of 6% of patients filled a second opioid prescription within 30 days.Although many patients do not require opioids for umbilical hernia repair, most pediatric patients fill opioid prescriptions, including for prolonged courses and refills. Guidelines for appropriate prescribing of opioids after common, simple procedures, such as umbilical hernia repair, could improve the quality of care for children and impact the US epidemic of opioid abuse.

Omphalocele (exomphalos) is one of the most common abdominal wall defects. The size of the defect and the severity of the associated anomalies determine the overall morbidity and mortality. Routine prenatal screening and diagnosis of the abdominal wall defect and concurrent anomalies is important as it allows for effective prenatal counseling and optimal perinatal management. The purpose of this article is to discuss the approach to prenatal diagnosis and management of omphalocele.

Objective: To evaluate and compare the outcomes of pregnancies with prenatally detected gastroschisis and omphalocele. Materials and Methods: We retrospectively evaluated prenatally detected gastroschisis and omphalocele cases. Cases were compared in terms of maternal demographic and clinical characteristics as well as pregnancy and neonatal outcomes. Results: This study consisted of 17 gastroschisis and 30 omphalocele cases. Only one case with gastroschisis was terminated due to additional severe limb deformities. Seventeen out of 30 cases of omphalocele were terminated for various reasons (56.7%). All patients with gastroschisis had surgical repair, while 8 out of 13 omphalocele cases had surgery. One patient with an omphalocele died after surgery due to sepsis. Six cases of gastroschisis also died in the neonatal period due to various reasons (6/16, 37.5%). Conclusion: Additional genetic disorders are more frequent in those with omphalocele cases, and they are more frequently terminated during gestation that the gastroschisis fetuses.

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

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).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 into25% (severe), between 25% and 50% (moderate), and50% (mild) for risk stratification.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.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.

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.

Giant omphalocele and large gastroschisis remain challenging issues faced by pediatric surgeons and neonatal intensivists. In this report, we presented 3 neonatal cases with complex congenital abdo-minal wall defects that were successfully treated with vacuum-assisted closure (VAC). Case 1 had a ruptured giant omphalocele and was treated with VAC for 24 days. She was successfully discharged at 78 days old. Case 2 had large gastroschisis that was unretractable using silo reduction. She was treated with VAC for 19 days and was succes-sfully discharged at 69 days old. Case 3 had large gastroschisis, and his defect had been closed using Gore-tex after silo reduction. VAC was applied for 14 days, and the baby was discharged at 67 days old. The VAC system can be effectively used to assist with visceral reduction, promote granulation tissue development, and skin epithelialization. This method represents a life-saving treatment for neonates with giant omphalocele and large gastroschisis.

We report a boy with congenital hemihyperplasia, umbilical hernia and temporary neonatal hypoglycemia, who was confirmed to have BWS caused by paternal uniparental disomy of chromosome 11p15.5. Additional phenotypic features comprising scoliosis, nephromegaly, focal partial epilepsy and delayed psychomotor development were coherent with the underlying genotype. This case emphasizes the importance of identifying the underlying genetic variant in order to acknowledge and manage the associated clinical complications and specific risk profile.

Background Umbilical hernias are a frequent and well-known pathology in children or adults. Congenital umbilical hernias are commonly diagnosed in childhood, and in adulthood such a hernia is usually acquired. Umbilical hernia in pregnancy may result in serious obstetric complications including antepartum hemorrhage, intrauterine fetal demise, and preterm labor, particularly if incarcerated. Case presentation We present a rare case of a congenital umbilical hernia in a term pregnancy. The patient was a 34-year-old African (Malawian) woman, living with human immunodeficiency virus (HIV) and on antiretroviral treatment, gravida 4, with three previous vaginal deliveries, and with two babies weighing 4 kg at birth. We performed herniorrhaphy at caesarean section, and at 3 months of follow-up she had no evidence of a recurrent hernia. Conclusion Congenital umbilical hernias are commonly diagnosed in childhood but might first be seen by medical practitioners in adulthood. A patient-centered approach addressing patient complaints, associated risk factors, and possible complications is recommended. Primary repair at caesarean section is a feasible option.

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 (pp=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 childrenWhat 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

No studies of the efficacy and safety of surgical techniques for the primary closure of giant omphalocele have been performed in Colombia. To determine the mortality rate and factors associated with mortality in neonates with giant omphalocele subjected to the surgical technique of early closure with a surgical silo described by Abello in Barranquilla, Colombia from 1994 to 2019. Retrospective cohort study of 30 neonates diagnosed with giant omphalocele and subjected to early closure of the defect. Medical history data were collected, information bias was controlled for, and descriptive statistical analysis was performed using Fisher’s exact test and the Mann–Whitney U test in SPSS 25.0. Of the patients in the cohort, 36.7% presented technique-related complications, 56.7% developed sepsis, 23.3% had low birth weight, 26.7% were preterm births, 43.3% had other malformations, 26.7% had congenital heart defects, and 13.3% presented pulmonary hypertension. The mean hospital stay was 26 days. The mortality rate was 16.7%; it was significantly higher among patients with other malformations, congenital heart defects, pentalogy of Cantrell and pulmonary hypertension. The Abello technique for the treatment of giant omphalocele showed a high neonatal survival rate and a low rate of procedure-related complications. The main factors associated with the death of neonatal patients were the presence of other malformations, congenital heart defect, pentalogy of Cantrell and pulmonary hypertension.

We sought to estimate the prevalence, incidence, and timing of surgery for elective and non-elective hernia repairs.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.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).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.Prognosis study II.

Management of infants with OEIS complex is challenging and not standardized. Expeditious surgery after birth has been recommended to limit soilage of the urinary tract and optimize intestinal function. However, clinical instability secondary to comorbidities is common in this population and early operation carries risk. We sought to define the risk/benefit profile of delaying repair.All newborn patients with OEIS managed by our institution between Sep 2017 and Oct 2019 were reviewed. Comorbidities were evaluated, including cardiopulmonary pathologies and associated malformations.Ten patients with OEIS were managed. Patients underwent early (2 patients, repair at 0-2 days) or delayed (6 patients, repair at 6-87 days) first-stage exstrophy repair. Two patients died prior to repair (progressive respiratory failure, severe genetic anomalies). Repairs were delayed secondary to cardiac conditions, neurosurgical interventions, medical disease, and/or delayed transfer. Delayed repair patients had longer lengths of stay and use of parenteral nutrition. No patients experienced urinary tract infections prior to repair.Delaying first-stage exstrophy repair to allow physiologic optimization is safe. All repaired patients were discharged home, without parenteral nutrition or supplemental oxygen.

Patient: Female, Newborn Final Diagnosis: Gallbladder agenesis Symptoms: Asymptomatic Medication:— Clinical Procedure: — Specialty: Pediatrics and Neonatology Objective: Rare co-existance of disease or pathology Background: Methimazole embryopathy is caused by maternal methimazole intake during early pregnancy. It causes fetal malformations such as choanal atresia, esophageal atresia, aplasia cutis, omphalomesenteric duct remnants, urachal remnants, and omphalocele. Gallbladder agenesis is sometimes complicated with other malformations, but there have been no reports of gallbladder agenesis due to methimazole or concomitant methimazole embryopathy with gallbladder agenesis. Case Report: The mother of a male neonate had taken methimazole for hyperthyroidism until pregnancy was recognized at 7 weeks of gestation. Ultrasonography at 12 weeks and 4 days of gestation showed the fetus had a cystic lesion in the umbilical region. The child was born at the gestational age of 38 weeks and 5 days. At birth there was omphalocele, omphalomesenteric fistula, and a scalp defect, and the child was diagnosed with methimazole embryopathy. Ultrasonography could not identify the gallbladder. Emergency surgery was performed for omphalocele with omphalomesenteric fistula on day 0. The intestine, including the omphalomesenteric fistula, was resected. Postoperative blood testing revealed hypothyroidism, so the patient was administered levothyroxine. Although MRI did not detect the gallbladder, it showed dilatation of the biliary duct. Hypothyroidism was well controlled by levothyroxine, so the patient was discharged with outpatient follow-up for the gallbladder agenesis. Six months later, the patient is asymptomatic. Conclusions: Concomitant gallbladder agenesis with methimazole embryopathy has not been previously reported. In the case of a dilated common bile duct, even when asymptomatic in the neonatal period, gallbladder agenesis demands long-term follow-up because of possible manifestation of choledocholithiasis or biliary malignant tumors.

Terminal myelocystocele is a rare type of neural tube malformation, consisting of a skin-covered lumbosacral mass, highly associated with other complex abdominal malformations within the OIES complex (omphalocele, imperforate anus, exstrophy of the cloaca and spinal defects). We present a case of a premature female with an extensive lumbosacral mass at birth, as well as an omphalocele, cloacal exstrophy, renal abnormalities, and sacral agenesis. Lumbar magnetic resonance imaging revealed a meningocele sac herniating through the bone defects and containing a syringocele sac. Advanced imaging techniques showed turbulent cerebrospinal fluid flow. At control, 4 weeks later, the defect doubled in size. The myelocystocele sac was evacuated and closed, and the patient persisted with paraparesis. The role of cerebrospinal fluid flow analysis is well established in Chiari-type malformations, in which turbulent flow within the syrinx is related to a better outcome after surgery. It is possible that the same principle could be applied to other spinal malformations, as shown in this case of terminal myelocystocele.

A vaginally delivered, term infant presented on the fourth day of life with lethargy, abdominal distension and bilious vomiting. He accepted breastfeeds and passed meconium on the first day. However, on the second day, he developed rapidly increasing abdominal distension, non-bilious vomiting and

Purpose This study aimed to describe sac ligation and sequential closure for the management of giant omphalocele (GO) and analyze its outcomes. Methods The medical records of 13 neonates with GO treated at a tertiary general hospital between July 2012 and April 2020 were reviewed. Sac ligation and progressive external compression were performed on most cases immediately after birth. Staged closure with or without a prosthetic patch was conducted after a period of sac suspension. Results Sac ligation-traction-compression was performed on 12 cases, of which 10 underwent staged closure, one with delayed closure. One patient with coexistent esophageal atresia was deemed ineligible for surgery. Among those who had undergone staged closure, 9 survived; however, one neonate who complicated with bilateral diaphragmatic eventration and severe ventilator-associated pneumonia died from multiple-organ failure. Pentalogy of Cantrell was excluded. One patient in whom primary closure was performed after birth died aged 29 h. Pneumonia was the most common infection among patients (5/13), with three having ventilator-associated pneumonia. The median durations of mechanical ventilation and hospital stay were 22.2 days (range, 1–151) and 44.2 days (range, 2–152), respectively, and 25.6 days and 46.4 days, respectively, among patients with staged closure. Among five infants who required oxygen support for more than 28 days, four had pulmonary hypoplasia. Conclusions Aside from abdominal wall defects, other major comorbidities and pulmonary hypoplasia influence the prognosis of GO. Sac ligation and staged closure is a effective choice for GO. Type of Study: Retrospective Study Level of Evidence: Level IV

The purpose of this report was to evaluate the safety and efficacy of the simple laparoscopic percutaneous extraperitoneal closure (LPEC) method for treating ovarian hernia patients, including newborns and low-birth-weight infants.We retrospectively reviewed the cases of ovarian hernia that were treated in our institution from May 2012 to September 2017.Thirty-four infants were included in this study (right side, n = 8; left side, n = 22; bilateral, n = 4). The mean corrected age was 2.3 ± 2.8 months and the mean body weight was 4.4 ± 1.6 kg. The contralateral positive rate was 53%, and 34% of the cases showed sliding of the fallopian tube. Umbilical hernias were observed in 24 cases (71%). The mean operative time was 53 ± 20 minutes. The LPEC procedure was successful in 32 cases; two cases were converted to open surgery.Small infants with ovarian hernia have some specific features such as a shortened round ligament and the presence of peritoneum cavities that require skillful techniques when being surgically repaired. The simple LPEC procedure can be performed safely with a low risk of recurrence, even in infants with sliding of the fallopian tube.

Introduction Surgical management of giant omphalocele has evolved at a slow pace, but evidence on the survival of patients who underwent primary staged closure is scattered and atomized. Objective To analyze the studies about of mortality associated with neonatal primary staged closure of giant omphalocele. Methods Systematic review in three databases using ex-ante search protocol and selection of studies following the phases suggested by PRISMA and MOOSE criteria. Reproducibility and evaluation of methodological quality were guaranteed by using CARE and STROBE. Results Seven studies of clinical cases with nine patients, and six cross-sectional studies with 85 individuals were analyze. These were conducted in the USA mainly, between 1985 and 2018. In the case studies, the death was 11,1% due to hepatic necrosis and portal system angiomatosis. On the cross-sectional studies, mortality was registered in 18.8% of patients due to coarctation of the aorta, heart, kidney, intestinal, respiratory or multiple organ failure, an anomaly of venous return, prematurity, ruptured omphalocele, pulmonary hypoplasia, trisomy 13, ARDS, sepsis, and septic shock. The main complication was wound infection with subsequent confection of the silo, found in 5.4% of patients. Conclusion Only a few studies on staged closure of giant omphalocele were found on a low number of patients. The high survival rate and the low percentage of complications on the 94 analyzed patients suggests the effectiveness and safety of the procedure. Levels of Evidence. According to the Journal of Pediatric Surgery this research corresponds to type of study level II for retrospective studies, and level IV for case series with no comparison group.