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Gastroenterologie Clinique Et Biologique 2006
Review
Topics: Anti-Inflammatory Agents, Non-Steroidal; Ascites; Ascitic Fluid; Diuretics; Dopamine Antagonists; Hepatorenal Syndrome; Hernia, Umbilical; Humans; Liver Cirrhosis; Liver Transplantation; Metoclopramide; Mineralocorticoid Receptor Antagonists; Punctures; Randomized Controlled Trials as Topic; Recurrence; Spironolactone; Vasodilator Agents
PubMed: 16885871
DOI: 10.1016/s0399-8320(06)73334-6 -
Pediatrics and Neonatology Jul 2022
Topics: Gastrointestinal Diseases; Hernia, Umbilical; Humans; Rupture
PubMed: 35396198
DOI: 10.1016/j.pedneo.2022.03.002 -
Pediatric Surgery International May 2020Neonatal surgery for abdominal wall defects is not performed in a centralized manner in Germany. The aim of this study was to investigate whether treatment for abdominal...
PURPOSE
Neonatal surgery for abdominal wall defects is not performed in a centralized manner in Germany. The aim of this study was to investigate whether treatment for abdominal wall defects in Germany is equally effective compared to international results despite the decentralized care.
METHODS
All newborn patients who were clients of the major statutory health insurance company in Germany between 2009 and 2013 and who had a diagnosis of gastroschisis or omphalocele were included. Mortality during the first year of life was analysed.
RESULTS
The 316 patients with gastroschisis were classified as simple (82%) or complex (18%) cases. The main associated anomalies in the 197 patients with omphalocele were trisomy 18/21 (8%), cardiac anomalies (32%) and anomalies of the urinary tract (10%). Overall mortality was 4% for gastroschisis and 16% for omphalocele. Significant factors for non-survival were birth weight below 1500 g for both groups, complex gastroschisis, volvulus and anomalies of the blood supply to the intestine in gastroschisis, and female gender, trisomy 18/21 and lung hypoplasia in omphalocele.
CONCLUSIONS
Despite the fact that paediatric surgical care is organized in a decentralized manner in Germany, the mortality rates for gastroschisis and omphalocele are equal to those reported in international data.
Topics: Abdominal Wall; Birth Weight; Delivery of Health Care; Digestive System Surgical Procedures; Female; Gastroschisis; Germany; Hernia, Umbilical; Humans; Infant, Newborn; Male
PubMed: 32219563
DOI: 10.1007/s00383-020-04647-7 -
Romanian Journal of Morphology and... 2014Trisomy 18 or Edwards syndrome is a rare chromosomal anomaly, associated with mild to severe intellectual disabilities and multiple congenital anomalies. Trisomies 18... (Review)
Review
Trisomy 18 or Edwards syndrome is a rare chromosomal anomaly, associated with mild to severe intellectual disabilities and multiple congenital anomalies. Trisomies 18 and 13 are lethal, only 5-10% of patients surviving the first year of life. Although prenatal biological and ultrasound investigations are mandatory and free and the detection rate of chromosomal abnormalities is high, the birth of children with no real chance at a normal life being thus avoided by therapeutic abortion, the parents of the here presented child did not benefit from medical examination or prenatal tests, unfortunately the case of many families in Romania. The policy of limiting medical intervention in newborns with Edwards syndrome due to the broad spectrum of severe congenital malformations, severe mental retard and reduced life expectancy is unanimously accepted, but yet difficult to apply from an ethical point of view. That is why very important for both healthcare providers and families to have accurate and detailed knowledge of survival, disease course, and quality of life so that they can make fully informed decisions regarding care of these babies. The particularity of this case is the association of multiple congenital anomalies in a male newborn with trisomy 18, almost all apparata and systems being affected, with the presence of an omphalocele and complete right labiopalatine cleft, which are less frequent at children with trisomy 18.
Topics: Abnormalities, Multiple; Calcinosis; Chromosomes, Human, Pair 18; Ear, External; Heart Defects, Congenital; Hematopoiesis; Hernia, Umbilical; Humans; Hyperplasia; Jaw Abnormalities; Male; Microstomia; Trisomy; Trisomy 18 Syndrome
PubMed: 24715190
DOI: No ID Found -
Taiwanese Journal of Obstetrics &... Sep 2009To present the prenatal magnetic resonance imaging (MRI) findings of concomitant craniorachischisis and omphalocele, review the literature, and discuss the pathogenesis. (Review)
Review
OBJECTIVE
To present the prenatal magnetic resonance imaging (MRI) findings of concomitant craniorachischisis and omphalocele, review the literature, and discuss the pathogenesis.
CASE REPORT
A 20-year-old, gravida 2, para 0, woman was referred to genetic counseling at 17 weeks of gestation because of multiple congenital malformations in the fetus. Level II ultrasound revealed acrania, a ventricular septal defect, an upward-turned face, and omphalocele containing the intestines. MRI revealed normal extremities, exencephaly, hyperextension of the fetal head, significant shortening of the spinal column, marked lordosis and hyperextension of the malformed spine, an upward-turned face, and absence of a neck. A diagnosis of iniencephaly associated with anencephaly, rachischisis and omphalocele was made. Amniocentesis revealed a karyotype of 46,XY. Postnatal X-ray showed anencephaly with total spina bifida of the cervical and thoracic spine.
CONCLUSION
Prenatal MRI is able to provide a clear whole-body image of the fetus and its relationship with the placenta. Prenatal MRI is very useful in the differential diagnosis of concomitant craniorachischisis and omphalocele from amniotic band sequence, limb body-wall complex with craniofacial defect and Disorganization human homologue.
Topics: Female; Hernia, Umbilical; Humans; Magnetic Resonance Imaging; Male; Neural Tube Defects; Pregnancy; Prenatal Diagnosis; Young Adult
PubMed: 19797022
DOI: 10.1016/S1028-4559(09)60306-5 -
Scientific Reports Jan 2021Analyze the biometric parameters and the size (area) of abdominal wall defect (AWD) in fetuses with gastroschisis and omphaloceles and correlate them with the herniated...
Analyze the biometric parameters and the size (area) of abdominal wall defect (AWD) in fetuses with gastroschisis and omphaloceles and correlate them with the herniated internal organs. We studied 22 fetuses (11 with AWDs and 11 without anomalies). In all fetuses we evaluated the xiphopubic distance (XPD) and iliac crest distance (ICD). In fetuses with AWDs we dissected the abdominal wall and measured the width and length of the defect for calculating its area and studying the correlation between the size of the defect with the organs that were herniated. For statistical analysis, the Anova and Tukey post-test were used (p < 0.05). The XPD in the control group had mean of 4.2 mm (2.3-5.9; SD ± 1.11), while in the AWDs it was 4.2 mm (2.9-5.5; SD ± 0.98) (p = 0.4366). The ICD had mean values of 2.5 mm (1.6-3.4; SD ± 0.58) in the control group, and 2.3 mm (1.2-3.0; SD ± 0.56) in AWDs fetuses (p = 0.6963). The number of herniate organs do not have significant correlation with the area of the defect (r = 0.2504, p = 0.5068). There is no correlation between the size (area) of abdominal wall defects and the number of the internal organs that herniated. Therefore, the hole size is not a predictor of the severity of the gastroschisis or omphalocele.
Topics: Abdominal Wall; Case-Control Studies; Female; Fetus; Gastroschisis; Gestational Age; Hernia, Abdominal; Hernia, Umbilical; Humans; Male; Pregnancy
PubMed: 33420099
DOI: 10.1038/s41598-020-79599-y -
European Journal of Pediatrics Dec 2023Normal 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...
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. Linear mixed models were used for comparisons. Intrauterine growth failure was common in infants with gastroschisis. Birth weight Z-scores in girls and boys were - 1.2 (0.2) and - 1.3 (0.2) and length Z-scores - 0.7 (0.2) and - 1.0 (0.2), respectively (p < 0.001 for all comparisons to infants without AWDs). During early infancy, growth failure increased in infants with gastroschisis, and thereafter, catch-up growth was prominent and faster in girls than in boys. Gastroschisis children gained weight and reached their peers' weights permanently at 5 to 10 years. By 15 years or older, 30% of gastroschisis patients were overweight. Infants with omphalocele were born with a normal birth size but grew shorter and weighing less than the reference population until the teen-age years.
CONCLUSION
Children with gastroschisis and omphalocele have distinct growth patterns from fetal life onwards. These growth trajectories may also provide some opportunities to modulate adult health.
WHAT IS KNOWN
• Intrauterine and postnatal growth failure can be seen frequently in gastroschisis and they often show significant catch-up growth later in infancy. It is assumed that part of the children with gastroschisis will become overweight during later childhood.
WHAT IS NEW
• The longitudinal growth of girls and boys with gastroschisis or omphalocele is described separately until the teenage years. The risk of gaining excessive weight in puberty was confirmed in girls with gastroschisis.
Topics: Infant; Male; Pregnancy; Child; Adult; Female; Adolescent; Humans; Gastroschisis; Hernia, Umbilical; Overweight; Birth Weight; Fetal Growth Retardation
PubMed: 37819418
DOI: 10.1007/s00431-023-05217-4 -
European Journal of Pediatrics Jul 2021Congenital abdominal wall defects, namely, gastroschisis and omphalocele, are rare congenital malformations with significant morbidity. The long-term burden of these...
Congenital abdominal wall defects, namely, gastroschisis and omphalocele, are rare congenital malformations with significant morbidity. The long-term burden of these anomalies to families and health care providers has not previously been assessed. We aimed to determine the need for hospital admissions and the requirement for surgery after initial admission at birth. For our analyses, we identified all infants with either gastroschisis (n=178) or omphalocele (n=150) born between Jan 1, 1998, and Dec 31, 2014, in the Register of Congenital Malformations. The data on all hospital admissions and operations performed were acquired from the Finnish Hospital Discharge Register between Jan 1, 1998, and Dec 31, 2015, and compared to data on the whole Finnish pediatric population (0.9 million) live born 1993-2008. Patients with gastroschisis and particularly those with omphalocele required hospital admissions 1.8 to 5.7 times more than the general pediatric population (p<0.0001). Surgical interventions were more common among omphalocele than gastroschisis patients (p=0.013). At the mean follow-up of 8.9 (range 1.0-18.0) years, 29% (51/178) of gastroschisis and 30% (45/150) of omphalocele patients required further abdominal surgery after discharge from the neonatal admission.Conclusion: Patients with gastroschisis and especially those with omphalocele, are significantly more likely than the general pediatric population to require hospital care. Nevertheless, almost half of the patients can be treated without further surgery, and redo abdominal surgery is only required in a third of these children. What is Known: • Gastroschisis and omphalocele are congenital malformations with significant morbidity • There are no reports on the long-term need for hospital admissions and surgery in these children What is New: • Patients with abdominal wall defects are significantly more likely than the general pediatric population to require hospital care • Almost half of the patients can be treated without further surgery, and abdominal redo operations are only required in a third of these children.
Topics: Abdominal Wall; Child; Female; Gastroschisis; Hernia, Umbilical; Hospitals; Humans; Infant; Infant, Newborn; Morbidity
PubMed: 33666724
DOI: 10.1007/s00431-021-04005-2 -
Pediatric Surgery International May 2020Major congenital abdominal wall defects (gastroschisis and omphalocele) may account for up to 21% of emergency neonatal interventions in low- and middle-income... (Review)
Review
Major congenital abdominal wall defects (gastroschisis and omphalocele) may account for up to 21% of emergency neonatal interventions in low- and middle-income countries. In many low- and middle-income countries, the reported mortality of these malformations is 30-100%, while in high-income countries, mortality in infants with major abdominal wall reaches less than 5%. This review highlights the challenges faced in the management of newborns with major congenital abdominal wall defects in the resource-limited setting. Current high-income country best practice is assessed and opportunities for appropriate priority setting and collaborations to improve outcomes are discussed.
Topics: Abdominal Wall; Disease Management; Gastroschisis; Health Resources; Hernia, Umbilical; Humans; Incidence; Infant; Infant Mortality; Infant, Newborn
PubMed: 32200405
DOI: 10.1007/s00383-020-04638-8 -
Birth Defects Research Nov 2019In this report, the National Birth Defects Prevention Network (NBDPN) examines and compares gastroschisis and omphalocele for a recent 5-year birth cohort using data...
BACKGROUND/OBJECTIVES
In this report, the National Birth Defects Prevention Network (NBDPN) examines and compares gastroschisis and omphalocele for a recent 5-year birth cohort using data from 30 population-based birth defect surveillance programs in the United States.
METHODS
As a special call for data for the 2019 NBDPN Annual Report, state programs reported expanded data on gastroschisis and omphalocele for birth years 2012-2016. We estimated the overall prevalence (per 10,000 live births) and 95% confidence intervals (CI) for each defect as well as by maternal race/ethnicity, maternal age, infant sex, and case ascertainment methodology utilized by the program (active vs. passive). We also compared distribution of cases by maternal and infant factors and presence/absence of other birth defects.
RESULTS
The overall prevalence estimates (per 10,000 live births) were 4.3 (95% CI: 4.1-4.4) for gastroschisis and 2.1 (95% CI: 2.0-2.2) for omphalocele. Gastroschisis was more frequent among young mothers (<25 years) and omphalocele more common among older mothers (>40 years). Mothers of infants with gastroschisis were more likely to be underweight/normal weight prior to pregnancy and mothers of infants with omphalocele more likely to be overweight/obese. Omphalocele was twice as likely as gastroschisis to co-occur with other birth defects.
CONCLUSIONS
This report highlights important differences between gastroschisis and omphalocele. These differences indicate the importance of distinguishing between these defects in epidemiologic assessments. The report also provides additional data on co-occurrence of gastroschisis and omphalocele with other birth defects. This information can provide a basis for future research to better understand these defects.
Topics: Abdominal Wall; Abnormalities, Multiple; Adult; Congenital Abnormalities; Digestive System Abnormalities; Female; Gastroschisis; Hernia, Umbilical; Humans; Infant; Infant, Newborn; Live Birth; Male; Maternal Age; Middle Aged; Mothers; Population Surveillance; Pregnancy; Prevalence; Racial Groups; Registries; Risk Factors; United States
PubMed: 31642616
DOI: 10.1002/bdr2.1607