Anatomic parameters of omphaloceles and their association with anatomic, genetic, or syndromic malformations: a retrospective study.

PURPOSE: This retrospective study aims to describe anatomical parameters of omphaloceles and to analyze their association with anatomical, genetic, or syndromic malformations. METHODS: Cases were selected from digital records of two university centers, a certified regional registry and personal records. Patients from 1998 to 2018 with omphalocele and live birth (LB), termination of pregnancy due to fetal anomaly (TOPFA) and fetal death (FD) were included. Cases born outside Western Switzerland and/or with upper or lower coelosomy were excluded. RESULTS: We analyzed 162 cases with the following distribution: 57 (35%) LB, 91 (56%) TOPFA and 14 (9%) FD. TOPFA was significantly more frequently performed in cases with non-isolated omphalocele, i.e., omphaloceles with associated major malformations (especially cardiovascular and genitourinary), genetic/chromosomal anomalies, or syndromes. For LB, associated anatomical malformations, genetic or chromosomal anomalies were not significantly associated with the size of the omphalocele or the liver involvement. CONCLUSIONS: The proportion of cases resulting in TOPFA was higher among fetuses with major malformations, genetic or chromosomal anomalies. Despite the large size of this cohort, and in contrary to previous publications, the size of the omphalocele and/or liver involvement does not allow for conclusions regarding the presence or number of associated malformations, genetic or chromosomal anomalies.

Spontaneous bowel evisceration through umbilical hernia in an adult non-cirrhotic patient.

Few cases of spontaneous bowel evisceration (SBE) through umbilical hernias (UHs) in adult patients have been reported in the literature. Interestingly, the spontaneous rupture of the hernia sac is a rare complication usually seen in adult cirrhotic patients with persistent ascites or in patients with congenital wall defects. A man in his early 50s was admitted to our emergency department with SBE through a long-standing acquired UH. He was not clinically cirrhotic, although being HCV positive. Surgeons performed an urgent laparotomy with ileal resection, latero-lateral ileal anastomosis and direct hernioplasty without mesh. Given the rarity of this presentation, we reported it and reviewed the available literature on this subject. Elective hernioplasty is currently suggested to lower the risk of complications. Mesh placement should be preferred, but only if comorbidities and infectious risks do not contraindicate its use. In emergency situations, a direct hernia repair is preferred.

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

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

Septic Shock After Endoscopic Sleeve Gastroplasty: A Post-procedural Complication?

BACKGROUND: ESG is a safe and effective technique in the obesity management, usually indicated in class I and II obesity. It is also an acceptable treatment in patients with class III obesity who have high surgical risk or refuse surgery. This procedure results in a significant weight loss and important improvement in metabolic comorbidities. Nevertheless, there are several procedure-related complications. Few cases of gastric perforation following ESG have been reported. We present a case of septic shock after ESG with preoperative diagnostic uncertainties. METHODS: We present the case of a 54-year-old male with a BMI of 43.6 kg/m2 who underwent ESG 7 days before in an external center. The patient came to the emergency department presenting abdominal pain, nausea, and vomiting since the day after the procedure. Physical examination revealed hemodynamic instability, altered level of consciousness, diffuse abdominal pain, and a painful umbilical lump due to a complicated umbilical hernia. Emergent surgery was decided after preoperative assessment. RESULTS: Intraoperative gastroscopy was performed, viewing a gastric ischemic ulcer covered with fibrin and a mucosal defect and suspecting a covered gastric perforation. Firstly, we performed an open approach to the complicated umbilical hernia. Subsequently, an exploratory laparoscopy was performed through the hernial ring, where a fibrin-covered area was evidenced in the anterior face of the gastric body, adhered to the round ligament by a transmural suture of the ESG. Additionally, multiple transmural sutures were observed adhered to the greater omentum and lesser sac and an intramural hematoma in the greater gastric curvature. No intra-abdominal free fluid was evidenced. A laparoscopic barbed suture of the area covered with fibrin was performed, after its release from the round ligament. The adhesions of the sutures and metallic material from the ESG were released. Finally, two abdominal drains were placed in the anterior and posterior gastric face. The patient presented superficial incisional surgical site infection and was discharged 6 days after laparoscopic surgery. CONCLUSIONS: ESG is a novel procedure, which has proven to be an effective alternative in the treatment of obesity. However, this technique may have major complications that can require urgent surgery.

Age and Probability of Spontaneous Umbilical Hernia Closure.

This cohort study of children younger than 6 years uses electronic health records to investigate whether a child's age is associated with the probability of spontaneous umbilical hernia closure and to refine guidelines for surgical repair.

Provincial Review of Adherence to Age-specific Guidelines for Umbilical Hernia Repair and Trends in Management.

BACKGROUND: Umbilical hernia (UH) is a common pediatric condition, for which delaying surgical repair for asymptomatic UH until after age 3 is recommended due to a high incidence of spontaneous closure. We aimed to determine the adherence to guidelines, rate of urgent surgical repair, outcomes, cost, and interinstitutional referral patterns of UH repair in the province of Quebec (Canada). METHODS: This was a population-based retrospective cohort study of children 28 days to 17 years old who underwent UH repair between 2010 and 2020 using health administrative databases. Children who had multiple procedures, or prolonged peri-operative stays were excluded. Early repair was defined as elective surgery at or under age 3. RESULTS: Of the 3215 children, 1744 (54.2%) were female, and 1872 (58.2%) were treated in a tertiary children's hospital. Guidelines were respected for 2853 out of 3215 children (89.7%). Patients living over 75 km from their treating hospitals (OR 2.36, 95% CI 1.33-4.16, P < 0.01), with pre-existing comorbidities (OR, 2.82; 95% CI, 1.96-4.05; P < 0.001), or being treated in a tertiary center (OR 2.10, 95% CI 1.45-3.03, P < 0.001) had a higher risk of early repair. Repair at or under age 3 and urgent surgery were associated with significant cost increases of 411$ (P < 0.001) and 558$ (P < 0.001), respectively. CONCLUSION: Quebec has a high rate of adherence to age-specific guidelines for UH repair. Future research should explore factors that explain transfers into tertiary centers, and the extent to which these reflect efficient use of resources. LEVEL OF EVIDENCE: level III. TYPE OF STUDY: Retrospective comparative study.

A case series of successfully managing exomphalos major with awake graduated compression dressing and early enteral feeding.

INTRODUCTION: Exomphalos anomaly is defined as the herniation of abdominal viscera into the base of the umbilical cord, with only a membranous sac covering these contents. It has an incidence of approximately 1 in 4000-6000 births. Management of exomphalos major (EM) remains controversial and limited, with very few studies to guide decision-making. METHOD: This is a case series of four neonates with EM treated at a tertiary paediatric referral centre between 2018 and 2021 with a gradual compression dressing technique. RESULTS: Four neonates were diagnosed with EM. The average gestational age was 38 + 5 (range 38 + 2 - 39 + 2), and the average birth weight was 3.1 kg (range 2.56 - 3.49 kg). The defect size ranged between 5 and 7 cm. All patients were commenced on gradual compression dressing between days 1 and 3 of life. Dressings were applied at the bedside in the general neonatal ward. The average time taken to reach full feeds was 1 week; only one patient required parenteral nutrition. Three underwent surgical repair at two and 16 weeks of age; one had delayed repair at the age of 1 year because of the COVID-19 pandemic. None required patch repair. None required prolonged ventilation after repair. CONCLUSION: This case series describes a successful compression dressing technique that reduces sac content without the need for general anaesthetic or respiratory compromise, whereby simultaneous enteral feeding is tolerated.

Use of Bilateral Rectus Sheath Blocks in Open Umbilical Hernia Repair.

BACKGROUND: In recent years, the rectus sheath block (RSB) has become increasingly prevalent in laparoscopic surgery. However, there is currently no definitive research on its use in the open repair of umbilical hernias with cirrhotic ascites. OBJECTIVE: In this study, we assessed the safety and clinical efficacy of ultrasound-guided (US-guided) bilateral RSBs in open umbilical hernia repair for patients diagnosed with cirrhotic ascites. STUDY DESIGN: Seventy-two patients diagnosed with umbilical hernias that presented with cirrhotic ascites and who were admitted to our hospital were randomly divided into 2 groups. These categories were labeled the RSB group (Group R) and the local infiltration group (Group L); we used US-guided RSBs in Group R and local infiltration in Group L. SETTING: The clinical outcomes of the patients in each group were compared to one another. Heart rate (HR), systolic blood pressure (SBP), and diastolic blood pressure (DBP) were recorded at various time points in both groups. METHODS: Measurements of the patients' outcomes were taken before anesthesia (T0), at the beginning of surgery (T1), at the time of the separation of the hernia sac (T2), at the end of surgery (T3), 6 hours postoperatively (T4), and 24 hours postoperatively (T5). On the Visual Analog Scale (VAS), pain scores at rest (T1-T3) and during activity (T4-T5) were recorded, as were the incidence of perioperative remedial analgesia and adverse effects. RESULTS: Compared to T0, both groups' HR was significantly higher at T1-T3 (P < 0.05). The SBP and DBP were also significantly higher (P < 0.05). At T1-T3, the HR of Group R was significantly slower than that of Group L (P < 0.05), and at T4-T5, the VAS score for activity in Group R was significantly lower than that of Group L (P < 0.05). Group R had a significantly lower incidence of intraoperative remedial analgesia and postoperative nausea and vomiting than did Group L (P < 0.05). Neither group required postoperative remedial analgesia, and no patient experienced adverse reactions during the perioperative period. LIMITATIONS: This study has limitations in its sample size, lack of blood ammonia levels, and absence of data on patient satisfaction, necessitating future studies to address these issues. CONCLUSION: US-guided RSBs are an efficient method of anesthesia for open umbilical hernia repair in patients diagnosed with cirrhosis. This technique not only provides precise anesthesia and appropriate analgesia but also results in a low incidence of postoperative nausea and vomiting.

[Gastroschisis and omphalocele: Incidence and outcome].

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.

Incarcerated Littre's Umbilical Hernia: A Case Report.

Littre's hernia is an extremely rare type of hernia which has Meckel's diverticulum as its content. A 63-year-old male, presented to the emergency department with chief complaints of swelling and pain around the umbilicus. The patient was diagnosed with an incarcerated umbilical hernia. Following the emergency laparotomy, the intraoperative finding depicted an umbilical Littre's hernia. The patient underwent open Meckel's diverticulectomy with mesh repair. Preoperative diagnosis of Littre's hernia is unlikely due to its low incidence and lack of specific radiological and clinical findings, but the role of computed tomography scan and ultrasound are important in differentiating between strangulated or incarcerated bowel and omentum and in guiding the urgency of operative management. Keywords: case reports; hernia; Meckel diverticulum.

Associations between maternal periconceptional alcohol consumption and risk of omphalocele among offspring, National Birth Defects Prevention Study, 1997-2011.

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.

Gastroschisis prevalence patterns in 27 surveillance programs from 24 countries, International Clearinghouse for Birth Defects Surveillance and Research, 1980-2017.

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.

Investigation of a transient increase in omphalocele prevalence in a birth cohort of TRICARE beneficiaries.

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.

Switching from robotic-assisted extended transabdominal preperitoneal (eTAPP) to totally extraperitoneal (eTEP) hernia repair for umbilical and epigastric hernias.

Our study aimed to assess the safety and effectiveness of the robotic-assisted extended totally extraperitoneal (eTEP) repair compared to transabdominal preperitoneal (eTAPP) repair with a suprapubic trocar insertion to treat umbilical and epigastric hernias. On a prospectively maintained database, we identified patients who underwent either eTEP or eTAPP for treating umbilical and epigastric hernias. During the study period, 53 patients were included, 32 in the eTEP group and 21 in the eTAPP group. The mean age was 59.0 ± 13.9 years, 45 patients (84.9%) were male, and the mean BMI was 28.0 ± 5.9 kg/m2. Most hernias were umbilical (81.1%) and primary (83.0%). The operative time for eTEP was slightly shorter than for eTAPP (106 ± 43 min vs. 126 ± 74 min, p = 0.232). Postoperatively, only one case of bleeding and one seroma were recorded. No complication occurred during a mean follow-up of 11.3 ± 6.4 months in the eTEP group and 20.5 ± 9.7 months in the eTAPP group. In conclusion, our study showed that the eTEP with suprapubic approach was safe and feasible in the treatment of epigastric and umbilical hernias. According to our experience, shorter operative time, integrity of the posterior layers and increased overlap size are the main surgical reasons of switching from eTAPP to eTEP.