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Prenatal Diagnosis Mar 2021An omphalocele is a congenital defect in the abdominal wall characterized by absent abdominal muscles, fascia, and skin. The characteristic ultrasound appearance... (Review)
Review
An omphalocele is a congenital defect in the abdominal wall characterized by absent abdominal muscles, fascia, and skin. The characteristic ultrasound appearance includes a midline defect with herniation of abdominal contents into the base of the umbilical cord. Other anatomic abnormalities are seen in approximately 50% of cases, most notably cardiac defects (19%-32%). Approximately, 50% of cases are associated with genetic and multiple malformation syndromes including trisomy 13/18, pentalogy of Cantrell and Beckwith-Wiedemann syndrome. Therefore, a thorough evaluation is recommended, including detailed anatomic survey, fetal echocardiogram, genetic counseling, and prenatal diagnostic testing. Overall prognosis depends on the size of the omphalocele, genetic studies, and associated anomalies. Early prenatal diagnosis remains important in order to provide parental counseling and assist in pregnancy management. Delivery should occur at a tertiary care center. Timing and mode of delivery should be based on standard obstetric indications with cesarean delivery reserved for large omphalocele (>5 cm) or those that involve the fetal liver. Neonatal management involves either primary or staged reduction, both of which can be associated with a prolonged neonatal hospitalization.
Topics: Female; Hernia, Umbilical; Humans; Infant, Newborn; Magnetic Resonance Imaging; Parents; Pregnancy; Prenatal Diagnosis; Professional-Patient Relations; Truth Disclosure
PubMed: 33540475
DOI: 10.1002/pd.5886 -
The British Journal of Surgery Oct 2021The definition, classification and management of rectus diastasis (RD) are controversial in the literature and a variety of different surgical treatments have been...
BACKGROUND
The definition, classification and management of rectus diastasis (RD) are controversial in the literature and a variety of different surgical treatments have been described. This article reports on the European Hernia Society (EHS) Clinical Practice Guideline for RD.
METHOD
The Guideline group consisted of eight surgeons. The Grading of Recommendation, Assessment, Development and Evaluation (GRADE) approach and the Appraisal of Guidelines for Research and Evaluation (AGREE) instrument were used. A systematic literature search was done in November 2018 and updated in November 2019 and October 2020. Nine Key Questions (KQs) were formulated.
RESULTS
Literature reporting on the definition, classification, symptoms, outcomes and treatments was limited in quality, leading to weak recommendations for the majority of the KQs. The main recommendation is to define RD as a separation between rectus muscles wider than 2 cm. A new classification system is suggested based on the width of muscle separation, postpregnancy status and whether or not there is a concomitant hernia. Impaired body image and core instability appear to be the most relevant symptoms. Physiotherapy may be considered before surgical management. It is suggested to use linea alba plication in patients without concomitant hernia and a mesh-based repair of RD with concomitant midline hernias.
CONCLUSION
RD should be defined as a separation of rectus muscles wider than 2 cm and a new classification system is suggested.
Topics: Hernia, Umbilical; Hernia, Ventral; Herniorrhaphy; Humans; Physical Therapy Modalities; Postoperative Care; Rectus Abdominis; Surgical Mesh
PubMed: 34595502
DOI: 10.1093/bjs/znab128 -
The British Journal of Surgery Feb 2020Umbilical and epigastric hernia repairs are frequently performed surgical procedures with an expected low complication rate. Nevertheless, the optimal method of repair... (Review)
Review
BACKGROUND
Umbilical and epigastric hernia repairs are frequently performed surgical procedures with an expected low complication rate. Nevertheless, the optimal method of repair with best short- and long-term outcomes remains debatable. The aim was to develop guidelines for the treatment of umbilical and epigastric hernias.
METHODS
The guideline group consisted of surgeons from Europe and North America including members from the European Hernia Society and the Americas Hernia Society. The Grading of Recommendations, Assessment, Development and Evaluation (GRADE) approach, the Scottish Intercollegiate Guidelines Network (SIGN) critical appraisal checklists, and the Appraisal of Guidelines for Research and Evaluation (AGREE) instrument were used. A systematic literature search was done on 1 May 2018, and updated on 1 February 2019.
RESULTS
Literature reporting specifically on umbilical and epigastric hernias was limited in quantity and quality, resulting in a majority of the recommendations being graded as weak, based on low-quality evidence. The main recommendation was to use mesh for repair of umbilical and epigastric hernias to reduce the recurrence rate. Most umbilical and epigastric hernias may be repaired by an open approach with a preperitoneal flat mesh. A laparoscopic approach may be considered if the hernia defect is large, or if the patient has an increased risk of wound morbidity.
CONCLUSION
This is the first European and American guideline on the treatment of umbilical and epigastric hernias. It is recommended that symptomatic umbilical and epigastric hernias are repaired by an open approach with a preperitoneal flat mesh.
Topics: Europe; Hernia, Abdominal; Hernia, Umbilical; Herniorrhaphy; Humans; Laparoscopy; Practice Guidelines as Topic; Plastic Surgery Procedures; Societies, Medical; Surgical Mesh; United States
PubMed: 31916607
DOI: 10.1002/bjs.11489 -
NeoReviews Jun 2020The 2 most common congenital abdominal wall defects are gastroschisis and omphalocele. Both are usually diagnosed prenatally with fetal ultrasonography, and affected... (Review)
Review
The 2 most common congenital abdominal wall defects are gastroschisis and omphalocele. Both are usually diagnosed prenatally with fetal ultrasonography, and affected patients are treated at a center with access to high-risk obstetric services, neonatology, and pediatric surgery. The main distinguishing features between the 2 are that gastroschisis has no sac and the defect is to the right of the umbilicus, whereas an omphalocele typically has a sac and the defect is at the umbilicus. In addition, patients with an omphalocele have a high prevalence of associated anomalies, whereas those with gastroschisis have a higher likelihood of abnormalities related to the gastrointestinal tract, with the most common being intestinal atresia. As such, the prognosis in patients with omphalocele is primarily affected by the severity and number of other anomalies and the prognosis for gastroschisis is correlated with the amount and function of the bowel. Because of these distinctions, these defects have different management strategies and outcomes. The goal of surgical treatment for both conditions consists of reduction of the abdominal viscera and closure of the abdominal wall defect; primary closure or a variety of staged approaches can be used without injury to the intra-abdominal contents through direct injury or increased intra-abdominal pressure, or abdominal compartment syndrome. Overall, the long-term outcome is generally good. The ability to stratify patients, particularly those with gastroschisis, based on risk factors for higher morbidity would potentially improve counseling and outcomes.
Topics: Gastroschisis; Hernia, Umbilical; Humans; Infant, Newborn
PubMed: 32482700
DOI: 10.1542/neo.21-6-e383 -
The Surgical Clinics of North America Oct 2022Congenital abdominal wall defects vary from abdominal wall hernias to severe congenital structural anomalies that include gastroschisis, omphalocele, and prune belly... (Review)
Review
Congenital abdominal wall defects vary from abdominal wall hernias to severe congenital structural anomalies that include gastroschisis, omphalocele, and prune belly syndrome. The conditions often carry various associated anomalies and require multidisciplinary treatment approaches. Complex surgical reconstructive techniques are frequently required and prenatal, perioperative, and long-term follow-up is critical to ensuring the best possible outcomes.
Topics: Abdominal Wall; Digestive System Abnormalities; Female; Gastroschisis; Hernia, Umbilical; Humans; Pregnancy; Plastic Surgery Procedures
PubMed: 36209747
DOI: 10.1016/j.suc.2022.07.011 -
Seminars in Pediatric Surgery Dec 2022Abdominal wall defects are one of the most frequently encountered human congenital anomalies. They are seen in as many as 1 in 2,000 live births with evidence to suggest... (Review)
Review
Abdominal wall defects are one of the most frequently encountered human congenital anomalies. They are seen in as many as 1 in 2,000 live births with evidence to suggest that their incidence is increasing. While often discussed together abdominal wall defects consist mainly of two entities namely gastroschisis and omphalocele. There are marked differences in their theories of embryo-pathogenesis, clinical presentation/anatomy and overall outcomes. There is no clear consensus explaining the precise embryological mechanisms leading to the development of abdominal wall defects. Many clinicians and embryologists have attempted to explain the genesis of congenital abdominal wall defects because of failure of progression of various phases of normal embryonic development. This review summarizes the mechanisms involved in normal and abnormal development of the ventral abdominal wall leading to the development of gastroschisis and omphalocele.
Topics: Pregnancy; Female; Humans; Gastroschisis; Hernia, Umbilical; Abdominal Wall
PubMed: 36446303
DOI: 10.1016/j.sempedsurg.2022.151230 -
LaeknabladidGastroschisis and omphalocele are the most common congenital abdominal wall defects. The main purpose of this study was to investigate the incidence, other associated...
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.
Topics: Pregnancy; Infant; Female; Child; Infant, Newborn; Humans; Gastroschisis; Hernia, Umbilical; Retrospective Studies; Incidence; Prenatal Diagnosis
PubMed: 38420959
DOI: 10.17992/lbl.2024.03.785 -
Revista Brasileira de Ginecologia E... Jan 2022To characterize and compare the outcomes of omphalocele and gastroschisis from birth to 2 years of follow-up in a recent cohort at a tertiary center.
OBJECTIVE
To characterize and compare the outcomes of omphalocele and gastroschisis from birth to 2 years of follow-up in a recent cohort at a tertiary center.
METHODS
This is a retrospective clinical record review of all patients with gastroschisis and omphalocele admitted to the Neonatal Intensive Care Unit between January 2009 and December 2019.
RESULTS
There were 38 patients, 13 of whom had omphalocele, and 25 of whom had gastroschisis. Associated anomalies were present in 6 patients (46.2%) with omphalocele and in 10 (41.7%) patients with gastroschisis. Compared with patients with omphalocele, those with gastroschisis had younger mothers (24.7 versus 29.6 years; = 0.033), were born earlier (36 versus 37 weeks, = 0), had lower birth weight (2365 ± 430.4 versus 2944.2 ± 571.9 g; = 0.001), and had a longer hospital stay (24 versus 9 days, = 0.001). The neonatal survival rate was 92.3% for omphalocele and 91.7% for gastroschisis. Thirty-four patients were followed-up over a median of 24 months; 13 patients with gastroschisis (59.1%) and 8 patients with omphalocele (66.7%) had at least one adverse event, mainly umbilical hernia (27.3% vs 41.7%), intestinal obstruction (31.8% vs 8.3%), or additional surgical interventions (27.3% vs 33.3%).
CONCLUSION
Despite the high proportion of prematurity, low birth weight, and protracted recovery, gastroschisis and omphalocele (without chromosomal abnormalities) may achieve very high survival rates; on the other hand, complications may develop in the first years of life. Thus, a very positive perspective in terms of survival should be transmitted to future parents, but they should also be informed that substantial morbidity may occur in the medium term.
Topics: Birth Weight; Female; Gastroschisis; Hernia, Umbilical; Humans; Infant, Low Birth Weight; Infant, Newborn; Retrospective Studies
PubMed: 35092954
DOI: 10.1055/s-0041-1736299 -
Veterinary Research Communications Dec 2022Omphalocele is an embryonic developmental error presented as a neonatal visceral umbilical herniation. Epidemiological and embryological studies are reviewed that revise... (Review)
Review
Omphalocele is an embryonic developmental error presented as a neonatal visceral umbilical herniation. Epidemiological and embryological studies are reviewed that revise our understanding of midgut development and defects in cattle. Google Scholar and PubMed were searched for omphalocele, exomphalos, calves, calf, cattle, epidemiology, genetics, disease presentation, and omphalocele treatments of bovine neonates. Omphalocele contains small intestine and sometimes liver and/or pyloric abomasum. This condition may arise from inadequately expanded abdominal cavity, herniated midgut entrapment, or inappropriate umbilical ring growth. Full-term neonates are otherwise healthy but comorbidities may be present. Limited epidemiological evidence suggests neonates, commonly female, of dairy dams have much higher omphalocele prevalence than humans but with fewer co-morbidities. Genetic or environmental influences may present at low incidences, especially with co-defects, while also epigenetics operate in metabolically challenged dairy cattle. Calf survival after surgical repair was commonly good if damage to eviscerated tissue was minimal and surgery prompt. Inadequate follow-ups prevented assessment of their commercial value. We also revised understanding of embryonic midgut development. There is little useful epidemiological information on bovine omphaloceles. To address this, we suggest: (i) Observations on omphaloceles should report herd and dam histories, abdominal disposition of contents, and comorbidities. (ii) Cases of surgical restitution must report full histories and thoroughly explore post-surgical follow-ups to assess animal commercial viability. There is a need for very large-scale multi-centre prospective observational studies on cattle health and productivity that should include omphaloceles.
Topics: Pregnancy; Humans; Cattle; Animals; Female; Hernia, Umbilical; Veterinarians; Embryonic Development; Cattle Diseases; Observational Studies, Veterinary as Topic
PubMed: 36107281
DOI: 10.1007/s11259-022-09994-5