Present since birth. Due to failure of the midgut to return to the coelum in fetal life due to failure of the 2nd stage of rotation
1.Exomphalos minor: Small defect persisting at the umbilicus through which a peritoneal sac protrudes through the umbilical cord
Contents: Loop of intestine or Meckel's diverticulum (Littre's)
Coverings: Thin layer of Wharton's jelly + amniotic membrane
:Complications
During ligation of the cord a loop of intestine may be entangled in the ligature Always TWIST the cord to reduce the contents and ligate it 3-4 cm from the abdomen
Desiccation of the coverings and rupture of the hernia
- :Exomphalos Major
A large defect at or above the umbilicus through which a large wide necked sac protrudes
Contents: Large portion of viscera may be left lobe of liver
Covering: A layer of Amniotic membrane only
Complication: Desiccation of the covering, rupture and peritonitis
Treatment: Immediate operation
- Small defects: Contents reduced, sac excised, and abdominal muscles approximated
- Large defects: Create skin flaps by undermining of the skin and release incisions carried in the loin to cover the sac with skin without tension. Later after a few years repair the hernia