Omphalocele

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Shiori Tsuruhisa - One of the best experts on this subject based on the ideXlab platform.

Erik D. Skarsgard - One of the best experts on this subject based on the ideXlab platform.

  • Immediate versus staged repair of Omphaloceles.
    Seminars in pediatric surgery, 2019
    Co-Authors: Erik D. Skarsgard
    Abstract:

    Abstract Omphalocele is an abdominal wall defect involving the umbilical ring which results in visceral herniation of small and large intestine, liver, spleen and sometimes gonads. The covering of the herniated viscera by a fused membrane consisting of peritoneum, Wharton's jelly and amnion projects viscera from mechanical injury and exposure to chemical irritants in amniotic fluid. Omphalocele is usually diagnosed before birth, is variable in size, and is frequently associated with chromosomal and somatic anomalies, syndromes, and variable degrees of pulmonary hypoplasia which can be lethal. In this article we examine surgical closure options for Omphaloceles ranging from early primary fascial repair for small Omphaloceles to a staged repair, often facilitated by an amnion preserving silo, which may be necessary for giant Omphaloceles that cannot be closed primarily. We also review some of the adjuncts to abdominal wall reconstruction including tissue expansion and mesh. Conservative management (paint and wait) of giant Omphaloceles is described elsewhere.

Joanne Baerg - One of the best experts on this subject based on the ideXlab platform.

  • outcomes in Omphalocele correlate with size of defect
    Journal of Pediatric Surgery, 2019
    Co-Authors: Steven L Raymond, Cynthia D Downard, Shawn D St Peter, Joanne Baerg, Faisal G Qureshi, Steven W Bruch, Paul D Danielson, Elizabeth Renaud, Saleem Islam
    Abstract:

    Abstract Background Omphaloceles can be some of the more challenging cases managed by pediatric surgeons. Single center studies have not been meaningful in delineating outcomes due to the length of time required to accumulate a large enough series with historical changes in management negating the results. The purpose of this study was to evaluate factors impacting the morbidity and mortality of neonates with Omphaloceles. Methods A multicenter, retrospective observational study was performed for live born neonates with Omphalocele between 2005 and 2013 at nine centers in the United States. Maternal and neonatal data were collected for each case. In-hospital management and outcomes were also reported and compared between neonates with small and large Omphaloceles. Results Two hundred seventy-four neonates with Omphalocele were identified. The majority were delivered by cesarean section with a median gestational age of 37 weeks. Overall survival to hospital discharge was 81%. The presence of an associated anomaly was common, with cardiac abnormalities being the most frequent. Large Omphaloceles had a significantly longer hospital and ICU length of stay, time on ventilator, number of tracheostomies, time on total parenteral nutrition, and time to full feeds, compared to small Omphaloceles. Birth weight and defect size were independent predictors of survival. Conclusion This is the largest contemporary study of neonates with Omphalocele. Increased defect size is an independent predictor of neonatal morbidity and mortality. Level of Evidence Level II.

  • long term complications and outcomes in Omphalocele
    Seminars in Pediatric Surgery, 2019
    Co-Authors: Joanne Baerg, Amanda Munoz
    Abstract:

    After a diagnosis of Omphalocele during pregnancy, questions regarding long-term prognosis are of primary importance for parents. It is imperative that their questions are answered with substantiated data to promote confident decisions for their children. They frequently express concerns regarding long-term survival, quality of life, need for more operations, feeding issues, motor and cognitive development, cosmesis, and the unique difficulties of giant Omphaloceles. The available outcome studies that address these questions are discussed.

  • The use of ECMO for gastroschisis and Omphalocele: Two decades of experience.
    Journal of pediatric surgery, 2017
    Co-Authors: Joanne Baerg, Arul Thirumoorthi, Andrew O. Hopper, Edward Tagge
    Abstract:

    Abstract Purpose The aim was to review the respiratory failure causes and outcomes of infants with Omphalocele or gastroschisis receiving ECMO and reported to the Extracorporeal Life Support Organization (ELSO). Methods Gastroschisis and Omphalocele infants supported with ECMO and reported to the ELSO Registry between 1992 and 2015 were retrospectively reviewed. Clinical variables, diagnosis of respiratory failure (pulmonary hypertension (PHN), congenital heart defects (CHD), congenital diaphragmatic hernia (CDH), and sepsis), and outcomes were recorded. Univariate analysis was performed using Student's t -test for continuous or Fisher's exact test for categorical variables. Results Fifty-two infants with gastroschisis (41) (79%) or Omphalocele (11) (21%) were identified. The survival to discharge rate of 51% for gastroschisis remained stable and was significantly higher ( P =0.05). The overall mortality rate for Omphalocele was 82%. Omphalocele had significantly more PHN ( P P P =0.04) had significantly more sepsis ( P =0.02), and none had a CDH. Conclusion Infants with gastroschisis requiring ECMO support have significantly better survival than Omphaloceles, and respiratory failure is significantly associated with sepsis. The majority of Omphalocele infants die despite ECMO, and respiratory failure is associated PHN and CDH. The association of Omphalocele, PHN, and CDH merits further investigation. Study type and evidence level Retrospective comparative study of Registry Database, Level 3.

  • high prevalence of pulmonary hypertension complicates the care of infants with Omphalocele
    Neonatology, 2017
    Co-Authors: Shandee Hutson, Shawn D St Peter, Joanne Baerg, Andrew O. Hopper, Douglas D Deming, Donna A Goff
    Abstract:

    Background Omphalocele is one of the most common abdominal wall defects. Many newborn infants born with Omphalocele present with significant respiratory distress at birth, requiring mechanical ventilatory support, and have clinical evidence of pulmonary hypertension. Little information exists on the prevalence of and risk factors associated with pulmonary hypertension in this cohort of infants. Objectives To describe the prevalence of and risk factors associated with pulmonary hypertension among infants with Omphalocele. Methods This is a multicenter retrospective chart review of demographic data and clinical characteristics of infants with Omphalocele admitted to the neonatal intensive care units of Loma Linda University Children's Hospital and Children's Mercy Hospital between 1994 and 2011. Echocardiogram images were reviewed for pulmonary hypertension, and statistical analyses were performed to identify risk factors associated with the presence of pulmonary hypertension. Results Pulmonary hypertension was diagnosed in 32/56 (57%) infants with Omphalocele. Compared to infants without pulmonary hypertension, infants with pulmonary hypertension were more likely to have a liver-containing defect (16/32 [50%] vs. 5/24 [21%], p = 0.03), require intubation at birth (18/32 [56%] vs. 6/24 [17%], p = 0.03), and die during initial hospitalization (12/32 [38%] vs. 2/24 [8%], p = 0.01). Conclusion The majority of infants with Omphalocele have evidence of pulmonary hypertension which is associated with increased mortality. Echocardiograms to screen for pulmonary hypertension should be obtained at ≥2 days of life in infants with Omphalocele, especially in those with liver within the Omphalocele sac and/or in those infants who require intubation at birth to screen for pulmonary hypertension.

Rene M H Wijnen - One of the best experts on this subject based on the ideXlab platform.

  • Omphalocele at school age: What do parents report? A call for long-term follow-up of complex Omphalocele patients.
    Early human development, 2019
    Co-Authors: Annelieke Hijkoop, Rene M H Wijnen, Dick Tibboel, André B. Rietman, Titia E. Cohen-overbeek, Joost Van Rosmalen, Hanneke Ijsselstijn
    Abstract:

    Abstract Objective Many children with Omphalocele experience morbidity in early life, which could affect long-term outcomes. We determined parent-reported outcomes in school-aged children treated for minor or giant Omphalocele. Study design We sent paper questionnaires to the parents of all children treated for Omphalocele in 2000–2012. Giant Omphalocele was defined as defect diameter ≥ 5 cm with liver protruding. Motor function (MABC-2 Checklist) was compared with Dutch reference data; cognition (PedsPCF), health status (PedsQL), quality of life (DUX-25) and behavior (Strengths and Difficulties Questionnaire; SDQ) were compared with those of controls (two per child) matched for age, gender and maternal education level. Possible predictors of cognition and behavior were evaluated using linear regression analyses. Results Of 54 eligible participants, 31 (57%) returned the questionnaires. MABC-2 Checklist scores were normal for 21/26 (81%) children. Cognition, health status, quality of life and behavior were similar to scores of matched controls. One quarter (26%) of children with Omphalocele scored ≤ − 1 standard deviation on the PedsPCF, compared with 9% of matched controls (p = 0.07). Giant Omphalocele and presence of multiple congenital anomalies (MCA) were most prominently associated with lower PedsPCF scores (giant Omphalocele: β −22.11 (95% CI: −43.65 to −0.57); MCA -23.58 (−40.02 to −7.13)), although not significantly after correction for multiple testing. Conclusions Parent-reported outcomes of children with Omphalocele at school age are reassuring. Children with an isolated, minor Omphalocele do not need extensive long-term follow-up of daily functioning. Those with a giant Omphalocele or MCA might be at risk for delayed cognitive functioning at school age; we recommend long-term follow-up to offer timely intervention.

  • Omphalocele from diagnosis to growth and development at 2 years of age
    Archives of Disease in Childhood, 2019
    Co-Authors: Annelieke Hijkoop, Rene M H Wijnen, T E Cohenoverbeek, Dick Tibboel, Nina C J Peters, Rosan L Lechner, Yolande Van Bever, Annabel P J M Van Gilsfrijters, Hanneke Ijsselstijn
    Abstract:

    Objectives To compare the prenatal frame of reference of Omphalocele (ie, survival of fetuses) with that after birth (ie, survival of liveborn neonates), and to assess physical growth and neurodevelopment in children with minor or giant Omphalocele up to 2 years of age. Design We included fetuses and neonates diagnosed in 2000–2012. Physical growth (SD scores, SDS) and mental and motor development at 12 and 24 months were analysed using general linear models, and outcomes were compared with reference norms. Giant Omphalocele was defined as defect ≥5 cm, with liver protruding. Results We included 145 fetuses and neonates. Of 126 (87%) who were diagnosed prenatally, 50 (40%) were liveborn and 35 (28%) survived at least 2 years. Nineteen (13%) neonates were diagnosed after birth. Of the 69 liveborn neonates, 52 (75%) survived and 42 children (81% of survivors) were followed longitudinally. At 24 months, mean (95% CI) height and weight SDS were significantly below 0 in both minor (height: −0.57 (−1.05 to –0.09); weight: −0.86 (−1.35 to –0.37)) and giant Omphalocele (height: −1.32 (−2.10 to –0.54); weight: −1.58 (−2.37 to –0.79)). Mental development was comparable with reference norms in both groups. Motor function delay was found significantly more often in children with giant Omphalocele (82%) than in those with minor Omphalocele (21%, P=0.002). Conclusions The prenatal and postnatal frames of reference of Omphalocele differ considerably; a multidisciplinary approach in parental counselling is recommended. As many children with giant Omphalocele had delayed motor development, we recommend close monitoring of these children and early referral to physical therapy.

  • closure of giant Omphaloceles by the abdominal wall component separation technique in infants
    Journal of Pediatric Surgery, 2008
    Co-Authors: Floortje C Van Eijck, Robert P Bleichrodt, P N M A Rieu, Ivo De Blaauw, Marc H W A Wijnen, F H J M Van Der Staak, Rene M H Wijnen
    Abstract:

    BACKGROUND/PURPOSE: Several techniques have been described to repair giant Omphaloceles. There is no procedure considered to be the criterion standard worldwide. The aim of the present prospective study was to analyze the early and late results of secondary closure of giant Omphaloceles using the component separation technique (CST) in infants. METHODS: From January 2004 to January 2007, 10 consecutive pediatric patients with a giant Omphalocele were treated at our department. Initially, patients were treated conservatively. After epithelialization of the Omphalocele, the abdominal wall was reconstructed using CST. Patients were monitored for complications during admission, and all patients were seen for follow-up. RESULTS: Component separation technique was performed at median age of 6.5 months (range, 5-69 months). The median diameter of the hernia was 8 cm (range, 6-9 cm). There was no mortality. The postoperative course was uneventful in 7 patients. Complications were seen in 3 patients (infection, skin necrosis, and hematoma). Median hospital stay was 7 days. After median follow-up of 23.5 months (range, 3-39 month), no reherniations were found. CONCLUSIONS: The CST is a safe 1-stage procedure for secondary closure in children with a giant Omphalocele without the need for prosthetic material and with good clinical outcome.

Michael K. Bohlmann - One of the best experts on this subject based on the ideXlab platform.

  • Seasonality of Omphalocele in northern Germany
    Ultraschall in Der Medizin, 2008
    Co-Authors: A. Hornemann, Jan Weichert, M. Thill, Altgassen C, D. W. Luedders, Michael K. Bohlmann
    Abstract:

    PURPOSE: Omphalocele is a rare congenital abdominal wall defect that has been attributed to environmental factors such as increased levels of herbicides in surface water. We wanted to verify a seasonal variation of the incidence of Omphalocele and thus to identify possible environmental factors in an area in Northern Germany that is characterized by a predominantly agricultural setting. MATERIALS AND METHODS: A retrospective analysis of all pregnancies (n = 28 935) screened by ultrasound at our University Hospital between January 1, 1993 and December 31, 2007 was carried out. After identification of fetuses with persisting Omphalocele, analysis for additional structural defects and for chromosomal anomalies, such as trisomy 18, was carried out. We further categorized all patients with regard to the date of conception during summer (April-September) and winter months (October-March). Categorical variables were compared by the Mann-Whitney-U test and were considered statistically significant when two tailed analyses yielded p = 0.05. RESULTS: There was no significant difference in the seasonal distribution of conception neither for all fetuses (p = 0.70) nor for fetuses with Omphalocele (p = 0.94). The results were constant for fetuses with isolated Omphalocele and with additional malformation and for those with Omphalocele and associated chromosomal anomalies. The incidence of fetal Omphalocele has remained relatively stable within the last 15 years. No significant difference was found in the seasonal incidence of Omphalocele in patients from an urban or rural background. CONCLUSION: Since we were unable to demonstrate a relationship between the time of conception and the incidence of fetal Omphalocele in our cohort of pregnant women from a predominantly agricultural background, other factors should be investigated for possible associations with the onset of Omphalocele.

  • Seasonality of Omphalocele in northern Germany
    Ultraschall in der Medizin (Stuttgart Germany : 1980), 2008
    Co-Authors: A. Hornemann, Jan Weichert, M. Thill, D. W. Luedders, C Altgassen, Michael K. Bohlmann
    Abstract:

    Omphalocele is a rare congenital abdominal wall defect that has been attributed to environmental factors such as increased levels of herbicides in surface water. We wanted to verify a seasonal variation of the incidence of Omphalocele and thus to identify possible environmental factors in an area in Northern Germany that is characterized by a predominantly agricultural setting. A retrospective analysis of all pregnancies (n = 28 935) screened by ultrasound at our University Hospital between January 1, 1993 and December 31, 2007 was carried out. After identification of fetuses with persisting Omphalocele, analysis for additional structural defects and for chromosomal anomalies, such as trisomy 18, was carried out. We further categorized all patients with regard to the date of conception during summer (April-September) and winter months (October-March). Categorical variables were compared by the Mann-Whitney-U test and were considered statistically significant when two tailed analyses yielded p = 0.05. There was no significant difference in the seasonal distribution of conception neither for all fetuses (p = 0.70) nor for fetuses with Omphalocele (p = 0.94). The results were constant for fetuses with isolated Omphalocele and with additional malformation and for those with Omphalocele and associated chromosomal anomalies. The incidence of fetal Omphalocele has remained relatively stable within the last 15 years. No significant difference was found in the seasonal incidence of Omphalocele in patients from an urban or rural background. Since we were unable to demonstrate a relationship between the time of conception and the incidence of fetal Omphalocele in our cohort of pregnant women from a predominantly agricultural background, other factors should be investigated for possible associations with the onset of Omphalocele. Georg Thieme Verlag KG Stuttgart, New York.

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