12/10/94 (RAHC)

Types of Abdominal Wall Defects

  1. Omphalocele/Exomphalos: Congenital herniation of abdominal contents at the umbilicus (i.e. into the umbilical cord). Occasionally divided into:
  1. Gastroschisis: Full thickness abdominal wall defect situated almost always to the right of the umbilicus without a covering membrane. A bridge of skin separates it from the umbilicus.

  2. Prune Belly Syndrome: Congenital deficiency of abdominal musculature, urinary tract dilatation and cryptorchidism. There are three grades:
  3. Others: e.g. Bladder extrophy.


Omphalocele: 1:6,000
Gastroschisis: 1:20,000 - 30,000
Prune Belly: 1:50,000


  1. Omphalocele: 30% have associated congenital defects (? up to 50%).
  2. Gastroschisis: Congenital associations are uncommon (?5 - 25%) but 22% are premature. The gut may be infarcted or perforated.

  3. Prune Belly: >90% have associated defects:


Abdominal wall and bowel develop conjointly over the 3rd to 12th weeks in utero, final bowel fixation occurring after birth.

Week 3: the embryo has cephalic, caudal and lateral folds:

Weeks 4 - 10: Gut growth is faster than abdominal wall growth forcing the gut into the cord.

Weeks 10 - 12: Gut returns to the abdomen with counterclockwise rotation.

Gastroschisis has been blamed on intrauterine disruption of the right omphalomesenteric artery but it may be just a different expression of the same defect that causes omphalocele.

The embryology of Prune Belly Syndrome is unknown.

Initial Management

The Problems:

  1. Fluid management:
  2. Heat management:
  3. Infection control:
  4. Gastric Distension:
  5. Associations:

Surgical Management

Anaesthetic Considerations

  1. Usual problems of neonatal anaesthesia:
  2. Compounded by:
    • problems of prematurity when present - R.D.S.
    • associated cardiac defects
    • associated pulmonary hypoplasia
    • increased heat and fluid loss
    • hypoglycaemia in Beckwith-Weidemann
  3. Additional problems:
  4. Analgesia: