UNASSIGNED: To evaluate the clinical features and perinatal outcomes of antenatally diagnosed fetuses with omphalocele and gastroschisis.
UNASSIGNED: This was a retrospective, single-center, cohort study of prenatally diagnosed fetuses with omphalocele and gastroschisis followed-up and delivered at a university hospital. Demographic, pregnancy, birth and perinatal outcomes were compared between gastroschisis and omphalocele.
UNASSIGNED: A total of 75 fetuses with omphalocele and 21 cases with gastroschisis were evaluated. The mean maternal age of women carrying a fetus with omphalocele was significantly higher than the women with gastroschisis (p=0.001). Associated structural anomalies were found in 53.3% and 4.7% of fetuses with omphalocele and gastroschisis, respectively (p<0.001). The rate of chromosomal anomaly was 8.3% in pregnancies with omphalocele. In liveborn pregnancies, the mean gestational age at delivery and birth weight did not differ between the study groups. Time to postoperative oral intake, duration of parenteral nutrition and length of hospital stay were significantly longer in babies with gastroschisis than omphalocele (p<0.01). Rates of termination, intrauterine, neonatal and infant death of fetuses with omphalocele were 25.3%, 6.7%, 10.7% and 2.7% respectively. Time to postoperative oral intake, duration of parenteral nutrition and duration of hospitalization were significantly longer in babies with complex compared to simple gastroschisis (p<0.01). Survival rates were 95.2%, 82.9% and 20% in fetuses with gastroschisis, isolated and non-isolated omphalocele, respectively.
UNASSIGNED: Associated structural and chromosomal anomalies were significantly more common in fetuses with omphalocele compared to those with gastroschisis. Prognosis of fetuses with omphalocele depended on the associated structural and chromosomal anomalies, whereas bowel compromise was the main determining factor in gastroschisis.

Congenital umbilical cord hernia is often misdiagnosed and easily confused with a small omphalocele. It is different from postnatally diagnosed umbilical hernias and is believed to arise from persistent physiological mid-gut herniation. Its incidence is estimated to be 1 in 5,000, although some articles reported the incidence rate of approximately 0.2%. It is very important to clearly diagnose these cases in the antenatal period so that the patients receive appropriate management postnatally and to decrease the stress in the parents as well. Umbilical cord hernias have not been associated with other chromosomal anomalies. In this article, we describe a case of a patient who was diagnosed with an umbilical cord mass for which the course prenatally and postnatally was uncomplicated. It is very important to avoid misdiagnosing these cases, so that patients are appropriately treated in the postnatal course and to prevent complications such as bowel injury during cord clamping.

OBJECTIVE: Although serum screening for aneuploidies has become less prevalent, maternal-serum alpha-fetoprotein (MSAFP) screening for body-wall defects remains widespread. We explored whether MSAFP screening is associated with earlier omphalocele detection than ultrasound alone.
METHODS: This is a retrospective cohort study of prenatally detected omphalocele cases at our center from 2007 to 2023. We explored the association between MSAFP screening, gestational age at omphalocele detection, and clinical outcomes.
RESULTS: Among 101 pregnancies with prenatally diagnosed omphalocele, 27 (26.7%) had MSAFP screening. The median gestational age at MSAFP screening was 17 weeks 4 days. Of those who received MSAFP screening, 11 (41%) had values ≥2.5 multiples of the median (MoM) and 16 (59%) were not elevated. MSAFP results did not correlate with omphalocele size and were not associated with prenatal or postnatal outcomes. MSAFP screening did not result in earlier suspicion for or confirmation of omphalocele (P = .97 and P = .87, respectively). In contrast, first-trimester ultrasound screening was associated with earlier suspicion for and confirmation of omphalocele (P < .01 and P = .01, respectively). There were no clinical or demographic differences between those who received MSAFP screening and those who did not (including body mass index or commute distance to an urban center).
CONCLUSIONS: MSAFP screening is not associated with earlier omphalocele detection. Furthermore, in pregnancies with prenatally diagnosed omphalocele, the results of MSAFP screening are not predictive of clinical outcomes.

BACKGROUND: Omphalocele (OM) is a congenital defect of the abdominal wall. The main goal of the surgical management is the survival of the neonate. However, the residual scar following the surgery can be extremely burdensome and negatively impact the quality of life (QoL) of these patients. The aim of this study is to assess the cosmetic results of the surgical treatment, the level of satisfaction of patients and surgeons, and the influence of the scar on the QoL of the patient.
METHODS: We conducted an observational retrospective cross-sectional study collecting all data regarding patients born with OM, operated at our Centre between 1998 and 2021. The cosmetic results of the surgical repair were evaluated using the validated Patient and Observer Scar Assessment Scale (POSAS). The assessment of the quality of life determined by the presence of the scar was conducted using PedQL 4.0. At last, the patients were visited by two paediatric surgeons and a medical student, which then scored the cosmetic result of the scar. Statistical analysis was conducted with Spearman linear correlation and Mann-Whitney test. A P-value below 0.05 was considered statistically significant.
RESULTS: In our study, we included a total of 19 patients, with a mean of 12 years of age at the time of the evaluation. The parameters with the major influence on the patient\'s general opinion of the scar were stiffness, thickness, and irregularity. We discovered significant differences in median values of all scores between the giant OM group and the nongiant OM group, in favour of the latter. Finally, we found a low grade of concordance between PedsQL filled by parents and patients.
CONCLUSIONS: The POSAS scale is a valid, feasible, and reliable tool for the assessment of the aesthetic outcome of surgical procedures. The original size of the defect is the most important factor acting on the result. However, it is crucial that any decision on plastic surgery to improve the looks of the scar must be postponed to the adult age of the patient.
METHODS: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

OBJECTIVE: To identify predictors of morbidity in GO through an analysis of associated anomalies, defect characteristics and management strategies.
METHODS: PubMed and Ovid EMBASE databases were searched from 2013 to 2023. Primary end points assessed correlation of morbidity with gestational age (GA), birth weight (BW), eviscerated organs, defect size, associated anomalies and management strategy.
RESULTS: Twenty articles were included for analysis with a total of 1009 GO. Median GA was 37 weeks (27-41), with a median BW of 2700 g (900-6000). 143 cardiovascular anomalies, 238 pulmonary anomalies, 98 musculoskeletal anomalies, 53 urogenital anomalies, 94 gastrointestinal anomalies, 11 neurological anomalies and 43 chromosomal anomalies were identified. 82 had unspecified additional anomalies, of which 20 and 17 were defined as major and minor, respectively. 123 neonates were managed with primary closure, 206 neonates with staged closure and 312 neonates with conservative treatment. Complications included sepsis, respiratory compromise, feeding dysfunction and closure-related adverse events. Long-term morbidity included home ventilation (n = 72), long-term parental nutrition (n = 36), and delayed motor neurodevelopment (n = 21). Main predictors of morbidity were pulmonary hypertension/hypoplasia, major congenital anomalies, greater defect size and liver herniation.
CONCLUSIONS: Key predictors of poor outcomes include the presence of additional congenital anomalies, defect size, liver herniation and pulmonary hypertension/hypoplasia.

We herein present a case of a ruptured giant omphalocele with congenital short small intestine. Vacuum-sealing drainage and carboxymethylcellulose silver dressing promoted wound healing after repair, avoided abdominal compartment syndrome, and reduced the risks of multiple procedures. We review the perioperative management of omphaloceles in congenital short small intestines.

Omphalocele and gastroschisis are the most common types of abdominal wall defects. Comprehensive local experience helps parents to make decisions on the pregnancy and foresee the disease journey. A retrospective review of abdominal wall defect patients in all three pediatric surgical centers in Hong Kong between January 2003 and February 2023 was conducted. All patients consecutively diagnosed with omphalocele and gastroschisis were included, excluding other forms. Data of demographics and short- and long-term outcome parameters were collected. A total of 99 cases were reviewed and 85 patients met the inclusion criteria. Diagnoses include omphalocele major (n = 49, 57.6%), omphalocele minor (n = 22, 25.9%) and gastroschisis (n = 14, 16.5%), with mean gestational age 37 weeks (SD 2.2) and birth weight 2.7 kg (SD 0.6). Omphalocele is most commonly associated with cardiovascular (n = 28, 39.4%) and chromosomal defects (n = 11, 15.5%). Surgical procedures including primary repair (n = 38, 53.5%), staged closure (n = 30, 42.3%) with average 8.6 days (SD 4.7) of silo reduction, and conservative management (n = 3, 4.2%) were performed. The mortality rate was 14.1% (n = 10) and the complication rate was 36.6% (n = 26). The majority of patients had normal intellectual development (92.5%) and growth (79.2%) on the latest follow-up. For gastroschisis, one patient (7.1%) had intestinal atresia. Surgical procedures included primary repair (n = 9, 64.3%) and staged closure (n = 5, 35.7%) with average 8 days (SD 3.5) of silo reduction. Complication rate was 21.4% (n = 3), with one mortality (7.1%). All patients had normal intellectual development and growth. The mean follow-up time of this series is 76.9 months (SD 62.9). Most abdominal wall defects in our series were managed surgically with a good overall survival rate and long-term outcome. This information is essential during antenatal and postnatal counseling for parents.

Omphalocele is a malformation of the abdominal wall varying in size and located at the base of the umbilical cord. Meckel\'s diverticulum is the most common congenital malformation of the gastrointestinal tract with an increased prevalence in newborns with congenital malformations of the umbilicus and gastrointestinal tract. The association between Meckel\'s diverticulum and omphalocele has been described in rare cases. We present the case of a newborn diagnosed with both entities.

OBJECTIVE: We analysed closure techniques in the treatment of giant omphalocele. A challenging pathology where there lacks consensus.
METHODS: Cochrane, MEDLINE and EMBASE were searched between 1 January 1992 and 31 December 2022 using terms and variations: omphalocele, exomphalos, giant, closure and outcome. Papers were selected using Preferred Reporting Items for Systematic review and Meta-Analyses 2020 criteria. Data collected included demographics, timing and technique of surgical repair, morbidity and mortality.
RESULTS: We identified 342 papers; 34 met inclusion criteria with a total 356 neonates. Initial non-operative management was described in 26 papers (14 dressings, eight silo, four serial sac-ligation). Operative techniques by paper were as follows: Early closure: nine primary suture closure without patch, two primary closure with patch and four mixed methods. Delayed closure: five simple, four-component separation technique, four tissue expanders, one Botox/pneumoperitoneum and two with patch. Median number of procedures was two (1-6) in the early group versus three (1-4) in the delayed. The most favourable was early primary closure with biological patch. The most unfavourable was delayed closure with patch. Cumulative reported mortality remained high, mostly due to non-surgical causes.
CONCLUSIONS: Definitions of giant omphalocele in the literature were heterogeneous with a variety of management approaches described.

Omphalocele is an abnormality where fetal abdominal organs protrude through the abdominal wall. We report a case of a 13-year-old female with a history of omphalocele repair who presented with acute periumbilical pain, nausea, and vomiting. Computed Tomography scan showed a para-ovarian cyst and mild dilation of the small bowel. During laparoscopy, the right ovary and fallopian tube were detached from the uterus and located behind the cecum. Despite this displacement, the ovary appeared to have retained functionality with intact blood supply. Authors hypothesize that surgical repair led to pelvic adhesion that caused torsion and avulsion of the fallopian tube and utero-ovarian ligament that led to the displacement. This anatomical change should be taken into consideration in surgical patients with history of omphalocele repair.