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7 Cards in this Set

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What's an omphalocele
refers to a defect in the abdominal wall through which intraabdominal contents are extruded into the base of the umbilical cord; an amnioperitoneal membrane typically covers the defect (but may rupture before or at the time of delivery)
What can it be associated with?
-40-60% a/w chromosomal abnormalities.
-Beckwith Wiedeman Sx
-Pentalogy of Cantrell
-Midline abnormalities
What do you look for in Beckwith Wiedeman Sx?
Abnormality of chrom 11p15
-macroglossia,
-visceromegaly,
-gigantism
-either umbilical hernia or omphalocele
-GU abnormalities
-increased risk for Wilms, liver, adrenocortical cancers
What is involved in Pentalogy of Cantrell?
-Epigastric omphalocele
-Defective (cleft)sternum
-Ventral diaphragmatic defect
-Intrinsic cardiac abnormality (VSD)
-Anterior pericardial deficiency
What's the immediate care of an omphalocele baby?
The immediate care of the newborn with omphalocele involves sterile wrapping of the bowel to preserve heat and minimize insensible fluid loss, insertion of an orogastric tube to decompress the stomach, stabilizing the airway to ensure adequate ventilation, and establishing peripheral intravenous access.
-Antibiotics/ IVF
What is the definitive treatment for an omphalocele?
Small defects (<2 cm) can generally be managed by primary direct closure, while medium to large defects require a staged procedure ( with silos).
How can you diagnose omphalocele prenatally?
-Elevated maternal AFP
-Ultrasound