Omphalocoele

Posted by e-Medical PPT
Omphalocoele is an Anterior abdominal wall defect at the base of the umbilical cord through which intraabdominal contents are extruded

Gastroschisis is a linear defect of the abdominal wall that permits extrusion of the viscera without involving the umbilicus
It is just lateral to the umbilicus.

Umbilical hernias occur when intestines do return to the abdomen, but later herniate through the umbilicus
Defect in linea alba,subcutaneous tissue and skin covering the protruding bowel
Frequent in premature infants

Clinical Findings (Omphalocoele)
Defect may vary from 2-10 cm
Sac is composed of amnion, Wharton’s jelly and peritoneum
50% have
accompanying liver,spleen, testes/ovary
>50% have associated defects
Location:Epigastric,Central or Hypogastric
Cord attachment is on the sac
Umbilical cord inserts onto the membrane
Large defects can extend from umbilicus to costal margin – contain liver, small and large bowel

Etiology
Result of disturbances in organogenesis during embryonic period (Errors in Midgut Development)
Three theories of Omphalocoele formation
• Persistence of primitive body stalk beyond 12 weeks
• Failure of bowel to return to the abdomen at 10 weeks
• Failure of complete lateral-body fold migration & body wall closure

Associated anomalies
40% have chromosomal abnormalities (Trisomy 13, 18, 21, Turner’s and Klinefelter synd)
45-88% infants have associated malformations
• Cardiovascular, genitourinary, CNS
• Beckwith-Wiedman syndrome
• Pentalogy of Cantrell
Prognosis based on associated anomalies

Share Medical Presentations