gastroschisis_exomphalos_flashcards
What are Gastroschisis and Exomphalos examples of?
Congenital visceral malformations
What is Gastroschisis?
A congenital defect in the anterior abdominal wall just lateral to the umbilical cord
What is the management approach for Gastroschisis?
Vaginal delivery may be attempted; newborns should go to theatre as soon as possible after delivery, e.g. within 4 hours
What is Exomphalos also known as?
Omphalocoele
Describe Exomphalos.
The abdominal contents protrude through the anterior abdominal wall but are covered in an amniotic sac formed by amniotic membrane and peritoneum
List some conditions associated with Exomphalos.
Beckwith-Wiedemann syndrome, Down’s syndrome, cardiac and kidney malformations
What is the management approach for Exomphalos?
Caesarean section is indicated to reduce the risk of sac rupture; a staged repair may be undertaken if primary closure is difficult
What is the purpose of allowing the sac to granulate and epithelialise in Exomphalos management?
It forms a ‘shell’ which, as the infant grows, allows the abdominal contents to eventually fit within the abdominal cavity
When can the abdomen be closed in Exomphalos management?
When the sac contents can fit within the abdominal cavity, the shell will be removed and the abdomen closed
summarise Gastroschisis and exomphalos
Gastroschisis and exomphalos
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
A 32-year-old mother, gravida 5 presents in labour having had no antenatal follow up. The neonate is born with the bowel protruding out of the abdomen but you note that it has a peritoneal covering protecting it.
What is the optimal management of the protruding bowel?
Cover in cling-film and surgically correct within first 5 days of life
Cover in cling-film and allow natural correction
Immediate surgical correction
Removal of the protruding bowel and formation of a permanent stoma
Staged closure starting immediately with completion at 6-12 months
Staged closure starting immediately with completion at 6-12 months
Exomphalos should have a gradual repair to prevent respiratory complications. Gastroschisis requires urgent correction
This is an example of exomphalos (omphalocele), these are usually detected antenatally but some are missed and other patients don’t engage with antenatal care. The key differential is gastroschisis in which a paraumbilical abdominal wall defect results in abdominal contents being outside the body, without a peritoneal covering. The prognosis is good if operated on as soon as possible and whilst waiting the bowel should be protected with cling-film. Intestinal function will take time to normalise and thus the child may require TPN for a few weeks.
Omphalocele may be repaired quickly or in stages and often a staged repair is preferred (especially with larger defects) as returning the abdominal contents can cause respiratory insufficiency or inability to close the abdomen, both of which can result in death. Therefore gradual closure allows the pulmonary system to adapt to the increased abdominal contents over 6-12 months. There is no need for a cling-film covering as the peritoneum will already be protecting the bowel in omphalocele.
