Abdominal wall defects – Gastroschisis and Exomphalos

Abdominal wall defects refer to birth defects where the layers of the abdomen including muscles and skin have failed to form properly. These include 2 main types of defects, namely Gastroschisis and Exomphalos.

Gastroschisis refers to a defect in the abdominal wall where skin and muscles are absent and as a result the organs in the abdomen are protruding through to the outside. This commonly occurs to the right side of the umbilical cord and is often first noticed at birth. Some cases can be diagnosed by ultrasound before birth. Because the intestines have been exposed to the amniotic fluid before birth and to the environment after birth, they are often swollen and inflamed.

There are different options for management of Gastroschisis and each child is assessed for the most appropriate treatment for them. This may include surgery shortly after birth to place the organs and intestines back into the abdomen and this may be done in the neonatal ICU at the bedside or in theatre. If this is not possible then all the organs and intestines are placed in a special bag (silo) and slowly pushed in over the coming days. Following this the hole is closed.

Even after the intestines and organs have been placed back in the abdomen these children require long hospital admissions before they are able to feed properly and will require a central line to allow feeding directly into the blood vessels until they are able to feed normally again.

An omphalocoele or exomphalos is similar to a gastroschisis except the organs push out of the abdomen through a defect at the umbilicus (or belly button). The organs and intestines push out into the umbilical cord and are covered with a thin layer. Because of this they are often not as inflamed and swollen as in gastroschisis. Like with gastroschisis, this problem may only be noticed at birth but can be diagnosed with an ultrasound before birth.

Children with an omphalocoele often have problems with other organs including their hearts and kidneys. All cases of omphalocoele will require surgical repair. The type of surgery and timing of surgery will depend on each child's condition at birth as well as the size of the abdominal wall defect. Sometimes these are closed surgically shortly after birth, but larger defects are often treated to allow the skin to grow over followed by surgical repair of the muscles a few months later.