Pediatrics omphalocele

BACKGROUND: Beckwith-Wiedemann syndrome (BWS) is an overgrowth disorder that exhibits etiologic genomic imprinting characterized by molecular heterogeneity and phenotypic variability. Associations with localized developmental dysplastic chondromatous lesions and cortical neuronal heterotopias have not previously been described. CASE PRESENTATION: A 33-week gestational age female had an omphalocele and intractable hypoglycemia at birth. The placenta demonstrated placental mesenchymal dysplasia...

BACKGROUND: Acquired neonatal intestinal diseases have an array of overlapping presentations and are often labeled under the dichotomous classification of necrotizing enterocolitis (which is poorly defined) or spontaneous intestinal perforation, hindering more precise diagnosis and research. The objective of this study was to take a fresh look at neonatal intestinal disease classification using unsupervised machine learning. METHODS: Patients admitted to the University of Florida Shands Neonatal Intensive Care Unit January 2013-September 2019 diagnosed with an intestinal injury, or had imaging findings of portal venous gas, pneumatosis, abdominal free air, or had an abdominal drain placed or exploratory laparotomy during admission were included...

PURPOSE: Mothers of infants born with congenital gastrointestinal surgical anomalies experience a unique caregiving role. Whether these challenges result in more mental health diagnoses than the general population is unknown. This study assessed mental health diagnoses in mothers of children born with congenital surgical anomalies (CSA) compared to mothers of children without CSA. METHODS: A total of 780 mothers of children with CSA (case-mothers) and 6994 control-mothers were included...

INTRODUCTION: Texas consistently accounts for approximately 10% of annual national births, the second highest of all US states. This temporal study aimed to evaluate incidences of neonatal surgical conditions across Texas and to delineate regional pediatric surgeon accessibility. METHODS: The Texas Birth Defects Registry was queried from 1999 to 2018, based on 11 well-established regions. Nine disorders (30,476 patients) were identified as being within the operative scope of pediatric surgeons: biliary atresia (BA), pyloric stenosis (PS), Hirschsprung's disease, stenosis/atresia of large intestine/rectum/anus, stenosis/atresia of small intestine, tracheoesophageal fistula/esophageal atresia, gastroschisis, omphalocele, and congenital diaphragmatic hernia...

No abstract text is available yet for this article.

PURPOSE: The purpose of this study was to explore echocardiographic parameters of the left ventricle (LV) in relation to the outcomes of omphalocele neonates with pulmonary hypertension (PH). METHODS: This retrospective study was conducted among omphalocele patients with PH born from 2019 to 2020. Patients in this study did not have additional severe malformations or chromosomal aberrations. Patients who died under palliative care were excluded. The echocardiographic parameters of LV were obtained within 24 h after birth...

Beckwith-Wiedemann syndrome (BWS) is a rare genetic disorder, distinguished by the following characteristics: macrosomia, macroglossia, abdominal wall deformities such as omphalocele, visceromegaly, hemihypertrophy and elevated risk of developing tumors such as nephroblastoma or hepatoblastoma. A 2.5-year-old female patient came to the Department of Pediatric and Preventive Dentistry with a complaint of abnormally large tongue along with difficulty in swallowing and slurred speech. On clinical examination, the built of the patient was greater than normal...

OBJECTIVE: The multidisciplinary antenatal diagnosis staff bring together practitioners who are involved in the management of the antenatal period at birth. This project was designed following the French experience to institute multidisciplinary consultation meetings for prenatal diagnosis (MCMPD) in Benin. METHODS: This is a descriptive cross-sectional study examining the knowledge, attitudes, and practices of Beninese practitioners in the field of MCMPD to develop the pilot phase of the project...

UNLABELLED: Normal childhood growth is an indicator of good health, but data addressing the growth of children born with abdominal wall defects (AWDs) are limited. The detailed growth phenotypes of children born with gastroschisis or omphalocele are described and compared to peers without AWDs from birth to adolescence. Data from 183 gastroschisis and 144 omphalocele patients born between 1993 and 2017 were gathered from Finnish nationwide registers and electronic health records. Weight (n = 3033), length/height (n = 2034), weight-for-length (0-24 months, n = 909), and body mass index measures (2-15 years, n = 423) were converted into sex- and age-specific Z-scores...

AIM: Exomphalos is a congenital anomaly found in 1/4500 newborns. Choice of non-operative management of exomphalos major unamenable to primary repair is controversial. This study aims at reviewing conservative management modalities and compare outcomes and complications. METHODS: A systematic review was performed according to PRISMA guidelines of all English publications in MEDLINE and EMBASE databases. Search words were exomphalos OR omphalocoele AND conservative OR non-operative AND management...

INTRODUCTION: Rural children have worse health outcomes compared to urban children. One mechanism for this finding may be decreased access to specialized care at children's hospitals. The objective of this study was to evaluate the hospital types where complex surgical care in infants is performed nationally. METHODS: This study examined infants (<1 y old) in the Kids' Inpatient Database from 2009 to 2019 who underwent surgery for one of the following conditions: esophageal atresia, gastroschisis, omphalocele, Hirschsprung disease, anorectal malformation, pyloric stenosis, small bowel atresia, congenital diaphragmatic hernia, and necrotizing enterocolitis...

We describe a 2-month-old female infant with macroglossia, macrosomia, omphalocele, neonatal hypoglycemia, earlobe creases, low nasal bridge, midface retrusion, syndromic facies and multiple cutaneous and hepatic hemangiomas (HH). Genetic evaluation confirmed the diagnosis of Beckwith-Wiedemann Syndrome (BWS) with mosaic uniparental disomy 11 as the underlying genetic mechanism suggested by partial hypermethylation of H19/IGF2:IG-DMR and partial hypomethylation of KCNQ1OT1:TSS-DMR on chromosome 11p15.5. Pediatric endocrinology and cardiology assessments were normal...

The main goal of surgical treatment for gastroschisis and omphalocele is the reduction of viscera in the abdominal cavity and closure of the abdomen, but the challenge is to succeed without the detrimental effects of increased intraabdominal pressure. In this regard, we performed a retrospective study for all patients admitted for gastroschisis and omphalocele to the Neonatal Intensive Care Unit of 'Marie Sklodowska Curie' Emergency Clinical Hospital for Children, from January 2011 until June 2021. Our aim was to highlight the presence of postoperative abdominal compartment syndrome...

Solid tumors or predisposition syndromes are increasingly suspected before birth. However optimal management and outcomes remain unclear. We have performed a ten-year retrospective study of oncologic indications of prenatal diagnosis in public hospitals in Marseille. Data were obtained from prenatal diagnosis center and hospital imaging databases and pediatric oncology department files. Fifty-one cases were identified, 40 with mass: adrenal 17, sacrococcygeal 9, cardiac 7, abdominal 4, ovarian 1, cervical 2; 8 with developmental abnormalities (omphalocele 4, macroglossia 4), 3 WITH familial predisposition syndromes (familial rhabdoid 2, Li-Fraumeni 1)...

No abstract text is available yet for this article.

OBJECTIVE: To determine whether nativity is associated with abdominal wall defects among births to Mexican-American women. STUDY DESIGN: Using a cross-sectional, population-based design, stratified and multivariable logistic regression analyses were performed on the 2014-2017 National Center for Health Statistics live-birth cohort dataset of infants of US-born (N=1,398,719) and foreign-born (N=1,221,411) Mexican-American women. RESULTS: The incidence of gastroschisis was greater among births to US-born compared with Mexico-born Mexican-American women: 36...

AIM OF THE STUDY: Children with omphalocele have an increased prevalence of Beckwith Wiedemann syndrome (BWS) and thus a suspected increased risk of developing embryonal tumors, e.g. Wilms tumor, hepatoblastoma, neuroblastoma and rhabdomyosarcoma. The aim of this study was to examine the prevalence of BWS and the risk of embryonal tumors amongst patients born with omphalocele. METHODS: A population-based cohort was used, including all children born in Sweden 1/1 1997-31/12 2016...

BACKGROUND: The study aimed to estimate the prevalence of major congenital anomalies of the alimentary system and the abdominal wall in Thailand using a nationwide hospital discharge database from the National Health Security Office (2017-2020). METHODS: The study extracted data from records with International Classification of Diseases-10 (ICD-10) codes related to esophageal malformation (ESO), congenital duodenal obstruction (CDO), jejunoileal atresia (INTES), Hirschsprung's disease (HSCR), anorectal malformation (ARM), abdominal wall defects (omphalocele (OMP) and gastroschisis (GAS)), and diaphragmatic hernia from the database with patient age selection set to less than 1 year...

BACKGROUND: The objective of this paper is to describe the clinical and epidemiological profile and the early outcomes of patients with omphalocele born in a fetal medicine, pediatric surgery, and genetics reference hospital in Rio de Janeiro - Brazil. To determine its prevalence, describe the presence of genetic syndromes, and congenital malformations, emphasizing the characteristics of congenital heart diseases and their most common types. METHODS: Using Latin-American Collaborative Study of Congenital Malformations (ECLAMC) database and records review, a retrospective cross-sectional study was performed, including all patients born with omphalocele between January 1st, 2016, and December 31st, 2019...

BACKGROUND: Higher risk of recurrence has been reported in pediatric inguinal hernia patients with specific comorbidities. The purpose of this systematic review was to investigate which comorbidities predispose to recurrent pediatric inguinal hernias (RPIHs). METHODS: A comprehensive search of six databases was performed, reviewing the literature to date on RPIHs and the co-occurrence of comorbidities. English-language publications were considered for inclusion...