Long term complications and outcomes in omphalocele

Baerg, Joanne; Munoz, Amanda

doi:10.1053/j.sempedsurg.2019.04.004

Cited by 27 publications

(35 citation statements)

References 25 publications

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“…Patients with isolated omphalocele have the best 1-year survival, which is greater than 90%, but this is a rather small group of omphalocele cases (around 30%). Giant omphalocele (defect containing more than 75% of liver in the sac and/or defect diameter larger than 5 cm) have an in-hospital mortality of up to 20% [55].…”

Section: Treatment Outcomementioning

confidence: 99%

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Major abdominal wall defects in the low- and middle-income setting: current status and priorities

2020

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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.Publisher's Note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

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Section: Treatment Outcomementioning

confidence: 99%

“…Long-term medical problems occur such as gastroesophageal reflux, pulmonary insufficiency, recurrent lung infections or asthma, feeding difficulties, and failure to thrive [55,56]. Special attention has been focused on the neurodevelopmental outcome of patients with omphalocele.…”

Section: Treatment Outcomementioning

confidence: 99%

Major abdominal wall defects in the low- and middle-income setting: current status and priorities

2020

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“…Severe respiratory insufficiency, in particular pulmonary hypoplasia and resultant pulmonary hypertension, result in significantly increased morbidity and mortality among fetuses with omphalocele. 31,32 This is observed more often with giant omphalocele which is thought to cause an abnormally narrow chest, poor chest expansion in utero, and inadequate lung growth. 30 The role of magnetic resonance imaging (MRI) in the evaluation of omphalocele is unclear but may be useful in the prediction of fetal lung volumes.…”

Section: F I G U R E 4 Omphalocele At 12 Weeks Gestation (A)mentioning

confidence: 99%

“…[30][31][32] A ratio of total lung volume to expected lung volume of less than 50% predicts a more complicated postnatal course, with longer hospitalization and prolonged ventilator support requirements, but is not directly correlated with mortality in the absence of pulmonary hypertension. [30][31][32] In cases of multiple anomalies, bowel dilation, or "giant" omphaloceles where lung volumes can be significantly reduced, MRI can be helpful to determine overall prognosis ( Figure 5). 32 Further studies are needed to clearly delineate the utility of MRI and as such ultrasound remains the gold standard for prenatal diagnosis.…”

Section: F I G U R E 4 Omphalocele At 12 Weeks Gestation (A)mentioning

confidence: 99%

Omphalocele—What should we tell the prospective parents?

2021

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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. Key Points What is already known about this topic? � Omphalocele can be accurately diagnosed on prenatal ultrasound � A significant proportion of omphalocele cases are associated with chromosomal abnormalities or genetic syndromes � Isolated cases of small omphalocele can be associated with a good prognosis � In the absence of other obstetric indications delivery should be at term What does this study add? � The role of magnetic resonance imaging for prognostic prediction remains unclear. � Further studies are needed to determine the role of stated exome sequencing. 1 | INTRODUCTION Omphalocele is one of the most common types of abdominal wall defects. With improved sensitivity of prenatal ultrasound and expanding prenatal diagnostic options, clinicians are tasked with not only providing the diagnosis, but counseling the patient regarding prenatal and postnatal management options. In this manuscript, we review the essential points for counseling when the diagnosis of fetal omphalocele is made in order to answer common questions that might arise in discussion with parents. Ideally, counseling should be provided by a multidisciplinary team consisting of obstetricians, genetic counselors, maternal-fetal medicine specialists, neonatologists, and pediatric surgeons.

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“…Larger abdominal wall defects may result in feeding difficulties and poor nutritional status, including poor weight gain and smaller stature, especially in infancy, and patients with omphaloceles may require reoperations with a higher likelihood to experience small-bowel obstruction. 2 Thus, it can be inferred that a combination of a complex abdominal wall from prior surgeries or incomplete repair of the omphalocele and poor nutritional status may indicate poorer wound healing and increased risk for small-bowel obstruction and infection for patients with acute cholecystitis being considered for cholecystectomy. Other options to achieve gallbladder drainage include percutaneous and endoscopic routes.…”

mentioning

confidence: 99%