Gastroschisis_and_Exomphalos_flashcards
What are Gastroschisis and Exomphalos?
Gastroschisis and Exomphalos are both examples of congenital visceral malformations.
What is Gastroschisis?
Gastroschisis describes a congenital defect in the anterior abdominal wall just lateral to the umbilical cord.
What is the management 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 (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.
What syndromes and malformations are associated with Exomphalos?
Beckwith-Wiedemann syndrome, Down’s syndrome, cardiac and kidney malformations.
What is the management for Exomphalos?
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.
Why is a caesarean section indicated for Exomphalos?
A caesarean section is indicated to reduce the risk of sac rupture.
What is the process of staged repair for Exomphalos?
The sac is allowed to granulate and epithelialise over the coming weeks/months, forming 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.
What happens to the sac in Exomphalos if primary closure is difficult?
The sac is allowed to granulate and epithelialise over the coming weeks/months, forming a ‘shell’.
summarise
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 35-week neonate is admitted to the neonatal intensive care unit. They were born via emergency Caesarean section to a 16-year-old mother in preterm labour. The mother was a late booker to antenatal health services and her antenatal history including significant tobacco and alcohol use. On examination, the baby has intestinal loops protruding through a hole in the abdomen to the left of the umbilicus. What is the most likely diagnosis?
Gastroschisis
Omphalocele
Foetal alcohol syndrome
Anencephaly
Congenital diaphragmatic hernia
Gastroschisis is associated with socioeconomic deprivation (maternal age <20, maternal alcohol/tobacco use)
Important for meLess important
Gastroschisis and omphalocele present similarly, but gastroschisis refers to a defect lateral to the umbilicus whereas omphalocele refers to a defect in the umbilicus itself.
Foetal alcohol syndrome presents with small head, flattened philtrum and thin upper lip.
Anencephaly is a neural tube defect causing absence of brain, skull and scalp.
Congenital diaphragmatic hernia refers to herniation of abdominal viscera into the thoracic cavity.
