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Extended spectrum penicillins reduce the risk of omphalocele: A population-based case-control study.Journal of Pediatric Surgery Sep 2021Omphalocele is a major congenital anomaly associated with significant morbidity and mortality. Regardless, the influence of maternal use of prescription drugs on the...
BACKGROUND
Omphalocele is a major congenital anomaly associated with significant morbidity and mortality. Regardless, the influence of maternal use of prescription drugs on the risk of omphalocele has only been addressed in a handful of studies. The aim of this study was to assess the influence of maternal risk factors and prescription drugs in early pregnancy on the risk of omphalocele.
METHODS
We performed a nationwide register-based case-control study in Finland. The analysis is based on the Finnish Register of Congenital Malformations and Drugs and Pregnancy databases, both upheld by the Finnish Institute for Health and Welfare. All omphalocele cases were identified between Jan 1, 2004, and Dec 31, 2014. Five age-matched controls from the same geographical region were randomly selected for each case. The main outcome measures were maternal risk factors for omphalocele. Our analysis compared the maternal characteristics and the use of prescription drugs during the first trimester of pregnancy between case and control mothers.
RESULTS
Mothers of 359 omphalocele cases were compared with 1738 randomly selected age and area-matched mothers of healthy infants between 1 January 2014 and 31 December 2014. Both maternal obesity (BMI ≥30) and diabetes increased the risk for omphalocele, and their co-occurrence accumulated this risk (aOR 5.06, 95% Cl 1.19-21.4). Similarly, history of multiple miscarriages was an independent risk factor (2.51, 1.16-5.43). The oral use of extended spectrum penicillins during the first trimester of pregnancy had a significant, protective influence (0.17, 0.04-0.71). These analyses were adjusted for sex, parity, and risk factors reported above. No significant changes in risk were observed with any other medication used during the first trimester.
CONCLUSION
In conclusion, these findings may suggest that extended spectrum penicillins in the first trimester reduces the risk of omphalocle formation. Additionally, consistent with earlier studies, previous repeated miscarriages, maternal obesity, and diabetes were significant risk factors for omphalocele.
Topics: Case-Control Studies; Female; Hernia, Umbilical; Humans; Infant; Penicillins; Pregnancy; Pregnancy Trimester, First; Risk Factors
PubMed: 33250216
DOI: 10.1016/j.jpedsurg.2020.10.034 -
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 Pediatric Surgery :... Apr 2021The aim of this study is to assess the changes in prevalence, mortality and termination pregnancy of omphalocele, and to identify associated anomalies.
INRODUCTION
The aim of this study is to assess the changes in prevalence, mortality and termination pregnancy of omphalocele, and to identify associated anomalies.
MATERIALS AND METHODS
A population-based nationwide register study. All cases with omphalocele were identified in the Finnish Register of Congenital Malformations and the Care Register for Health Care from 1993 to 2014 including live births, stillbirths, and terminations of pregnancy due to fetal anomalies. Associated anomalies were recorded and analyzed, and perinatal and infant mortality and prevalence were calculated.
RESULTS
There were 600 cases with omphalocele including 229 live births, 39 stillbirths, and 332 (55%) abortions. Birth prevalence in Finland was 1.96 per 10,000 births with no consistent trend over time. However, total prevalence was much higher (4.71/10,000) because more than half of these families chose option for the termination of pregnancy. Omphalocele is often complicated with other anomalies; most commonly chromosomal abnormalities (9.3%), heart defects (6.3%), central nervous system anomalies (3.0%), gastrointestinal, and urogenital malformations (both 2.0%). Proportion of chromosomal and central nervous system abnormalities were even higher in terminated pregnancies. Overall infant mortality was 22%. Total 1-year survival rates for isolated omphalocele, cases with multiple anomalies and neonates with chromosomal defects were 80, 88, and 17%, respectively.
CONCLUSION
Omphalocele is a rare congenital anomaly, often associated with other malformations. Our data suggest that isolated cases may be more common than previously thought. In the absence of chromosomal defects, survival is reasonably good. Regardless, more than half of these pregnancies are often terminated.
Topics: Abnormalities, Multiple; Abortion, Eugenic; Adult; Female; Finland; Hernia, Umbilical; Humans; Infant; Infant, Newborn; Live Birth; Pregnancy; Prevalence; Registries; Retrospective Studies; Stillbirth
PubMed: 32131131
DOI: 10.1055/s-0040-1703012 -
Annals of Plastic Surgery May 2022Tissue expander-assisted component separation can be used to increase the amount of skin, muscle, and fascial components available for repair of congenital abdominal...
BACKGROUND
Tissue expander-assisted component separation can be used to increase the amount of skin, muscle, and fascial components available for repair of congenital abdominal wall defects via a staged approach without the need for flap reconstruction. We present the largest case series to date using a tissue expander-assisted component separation technique for treatment of congenital abdominal wall defects in a pediatric patient population.
METHODS
A retrospective chart review of 9 patients with large congenital abdominal wall defects not initially amenable to primary repair between 2009 and 2020 was performed. Patients first underwent placement of tissue expanders, followed by removal once they had reached a sufficient expander volume. Component separation, with and without mesh placement, was performed to achieve abdominal wall closure.
RESULTS
The average age of patients at primary repair was 3.2 years (SD ±1.7 years). Eight patients (88.8%) had congenital omphalocele, and 1 patient (11.1%) had gastroschisis; none were amenable to primary repair. The average size of the defects before closure was 87.6 cm2 (SD = 33.6 cm2). Eighteen tissue expanders were placed in 9 patients, 72.2% of which were placed in the plane between the external and internal oblique muscles. Patients were seen in clinic an average of 6.8 times (SD, ±3.3 visits) for volume expansion into the tissue expander, receiving an average of 32.0 mL in each per visit. An average of 4.3 months (SD, ±1.8 months) elapsed between placement and removal of the expanders. At the time of tissue expander removal and abdominal wall closure, the defects ranged from 30 to 132 cm2 (mean, 54 cm2). All defects were successfully repaired using a component separation and bilateral fasciocutaneous flap advancement. Two patients (18.2%) experienced infection of the surgical site and seroma, both of which required debridement. One patient (9.1%) experienced partial thickness skin necrosis that was managed nonsurgically. The overall complication rate was 36.4%.
CONCLUSIONS
Omphalocele and gastroschisis can produce abdominal wall defects that are not amenable to primary repair. Staged reconstruction using tissue expander-assisted component separation is a safe and effective method of obtaining adequate local soft tissue to achieve primary closure.
Topics: Humans; Child; Child, Preschool; Tissue Expansion Devices; Gastroschisis; Abdominal Wall; Hernia, Umbilical; Retrospective Studies
PubMed: 37740465
DOI: 10.1097/SAP.0000000000003138 -
American Journal of Medical Genetics.... Nov 2020Gastroschisis and omphalocele are the two most common abdominal wall birth defects, and epidemiologic characteristics and frequency of occurrence as part of a syndromic...
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.
Topics: Abnormalities, Multiple; Adult; Anus, Imperforate; Cloaca; Congenital Abnormalities; Female; Gastroschisis; Hernia, Umbilical; Humans; Infant, Newborn; Male; Maternal Age; Pregnancy; Registries; Software; Spine; Texas; Young Adult
PubMed: 32885608
DOI: 10.1002/ajmg.a.61830 -
Cureus Oct 2021Mesh is beneficial in the repair of umbilical hernias. But it may cause chronic pain due to inflammatory reactions, which may impair the patient's quality of life.
BACKGROUND
Mesh is beneficial in the repair of umbilical hernias. But it may cause chronic pain due to inflammatory reactions, which may impair the patient's quality of life.
OBJECTIVES
To assess and compare the quality of life of patients following umbilical hernia repair with and without mesh.
METHODS
During the study period, 45 patients underwent umbilical hernia repair. The study was conducted at King Abdul-Aziz University Hospital (KAUH), KSA. Data were collected using medical records, and each patient was contacted by telephone, to fill the "Carolina Comfort Scale (CCS)" survey. The survey assesses the grade of pain, sensation of mesh, and movement limitation in different situations.
RESULTS
A non-significant difference was found between mean quality of life (QOL) scores of mesh and non-mesh groups. The relationship between CCS and gender was not significant in both groups. However, males had significantly higher CCS scores in mesh-treated cases. There was no statistically significant relationship between CCS and comorbidity, nationality, or symptoms. The overall CCS score did not differ statistically between mesh-treated and non-mesh-treated cases. Conclusion: The CCS score did not differ between mesh-treated and non-mesh-treated cases. It is suggested that future multicentric studies with a larger sample size be conducted.
PubMed: 34824932
DOI: 10.7759/cureus.19016 -
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 -
Hernia : the Journal of Hernias and... Jun 2021
Topics: Hernia, Abdominal; Hernia, Umbilical; Herniorrhaphy; Humans
PubMed: 34076774
DOI: 10.1007/s10029-021-02436-2 -
BMC Pregnancy and Childbirth Jun 2023The 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,...
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.
RESULTS
During the period of the study, our unity registered 4,260 births, 4,064 were live births and 196 stillbirths. There were 737 diagnoses of any congenital malformation, among them 38 cases of omphalocele, 27 were live born, but one was excluded for missing data. 62.2% were male, 62.2% of the women were multiparous and 51.3% of the babies were preterm. There was an associated malformation in 89.1% of the cases. Heart disease was the most common (45.9%) of which tetralogy of Fallot was the most frequent (23.5%). Mortality rate was 61.5%.
CONCLUSIONS
Our data showed a good correspondence with the existing literature. Most patients with omphalocele had other malformations, especially congenital heart disease. No pregnancy was interrupted. The presence of concurrent defects showed a huge impact on prognosis, since, even if most survived birth, few remained alive and received hospital discharge. Based on these data, fetal medicine and neonatal teams must be able to adjust parents counseling about fetal and neonatal risks, especially when other congenital diseases are present.
Topics: Infant, Newborn; Child; Pregnancy; Humans; Male; Female; Hernia, Umbilical; Tertiary Care Centers; Retrospective Studies; Cross-Sectional Studies; Brazil; Heart Defects, Congenital; Congenital Abnormalities; Prevalence
PubMed: 37286958
DOI: 10.1186/s12884-023-05741-z -
Journal of Perinatal Medicine Nov 2023To estimate factors affecting survival in prenatally diagnosed omphalocele, factors predicting genetic abnormalities, and association of omphalocele and specific groups... (Observational Study)
Observational Study
OBJECTIVES
To estimate factors affecting survival in prenatally diagnosed omphalocele, factors predicting genetic abnormalities, and association of omphalocele and specific groups of anomalies.
METHODS
A retrospective observational study was performed, analyzing data of all omphalocele cases diagnosed prenatally in the perinatology clinic of a referral center. Demographic data, characteristics of the omphalocele (size, content, associated anomalies), results of genetic testing, pregnancy outcomes and postnatal outcomes were analyzed.
RESULTS
Sixty-nine fetuses with omphalocele were included. The prevalence of omphalocele in livebirth was 0.007 %. Overall survival during the study period was 73.9 %. Twenty-eight (71.7 %) out of 39 cases with associated anomalies who were born live, survived, whereas survival was 85.7 % in the isolated cases. The most common anomaly associated with omphalocele were cardiac defects with 42 %; followed by placental or umbilical cord anomalies (28.9 %), skeletal defects (27.5), genitourinary anomalies (20.2 %), central nervous system (18.8 %) and facial anomalies (7.2 %), respectively. Eighty-five percent of the fetuses had at least one additional anomaly or ultrasound finding. Skeletal abnormalities and staged surgical repair of omphalocele were associated with survival. Associated skeletal anomalies and staged repair significantly increase the risk of postnatal death (OR: 4.6 95 % CI (1.1-19.5) and (OR: 10.3 95 % CI (1.6-63.9), respectively).
CONCLUSIONS
Associated skeletal abnormalities and staged surgical repair are negatively associated with postnatal survival.
Topics: Pregnancy; Female; Humans; Hernia, Umbilical; Placenta; Prenatal Diagnosis; Pregnancy Outcome; Ultrasonography, Prenatal; Retrospective Studies
PubMed: 37531153
DOI: 10.1515/jpm-2023-0197