Gastroschisis and exomphalos are both examples of congenital visceral malformations.

Gastroschisis

Gastroschisis describes a congenital defect in the anterior abdominal wall just lateral to the umbilical cord.

Management

• vaginal delivery may be attempted
• newborns should go to theatre as soon as possible after delivery, e.g. within 4 hours

Exomphalos (omphalocoele)

In exomphalos (also known as an omphalocoele) the abdominal contents protrude through the anterior abdominal wall but are covered in an amniotic sac formed by amniotic membrane and peritoneum.

Associations

• Beckwith-Wiedemann syndrome
• Down's syndrome
• cardiac and kidney malformations

Management

• caesarean section is indicated to reduce the risk of sac rupture
• a staged repair may be undertaken as primary closure may be difficult due to lack of space/high intra-abdominal pressure
  • if this occurs the sacs is allowed to granulate and epithelialise over the coming weeks/months
  • this forms a 'shell'
  • as the infant grows a point will be reached when the sac contents can fit within the abdominal cavity. At this point the shell will be removed and the abdomen closed