Which clinical history findings are characteristic of pediatric omphalocele?

Updated: Nov 05, 2019
  • Author: James G Glasser, MD, MA, FACS; Chief Editor: Dharmendra J Nimavat, MD, FAAP  more...
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Answer

In an omphalocele, the diameter of the abdominal wall defect varies between 4 and 12 cm; the defect is located centrally, or in the epigastrium or the hypogastrium.

With a large omphalocele, dystocia may occur and cause liver injury.

The omphalocele sac is ruptured in 10%-20% of cases; rupture may occur in utero or during delivery.

Infants with Beckwith-Wiedemann syndrome have the following characteristics:

  • Omphalocele (generally small)

  • Macroglossia and coarse, rounded facial features

  • Visceromegaly with hyperplasia of the pancreatic islet cells causing neonatal hypoglycemia, which may be severe

  • Genitourinary abnormalities

Increased incidence of Wilms tumors, liver tumors (hepatoblastoma), and adrenocortical neoplasms; surveillance by ultrasonography is indicated

Pediatric omphalocele and gastroschisis (abdominal Pediatric omphalocele and gastroschisis (abdominal wall defects). Note the enlarged tongue in this infant with Beckwith-Wiedemann syndrome.

Pentalogy of Cantrell includes the following components [31] :

  • Epigastric omphalocele

  • Cleft sternum

  • Anterior (retrosternal) diaphragmatic hernia of Morgagni

  • Absent pericardium

  • Cardiac defects (ectopia cordis and ventricular septal defects)

    Pediatric omphalocele and gastroschisis (abdominal Pediatric omphalocele and gastroschisis (abdominal wall defects). This infant has pentalogy of Cantrell: an epigastric omphalocele, cleft sternum, anterior (retrosternal) diaphragmatic hernia of Morgagni, absent pericardium, and cardiac defects (ectopia cordis, ventricular septal defects).

Giant omphalocele features are as follows:

  • Large, centrally located, abdominal wall defect

  • Ectopic liver, located outside of the abdominal cavity, within the omphalocele sac

  • Small, undeveloped abdominal and thoracic cavities

  • Restrictive lung disease and pulmonary hypoplasia associated with the hypoplastic thoracic cavity

  • Best to accomplish operative closure in stages (to avoid excessive intra-abdominal pressure) [32]


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