Gastroschisis.

Further information for clinicians and Care

Pathway for management of patients

Introduction
With increased first trimester ultrasound the diagnosis may well be made at
12 weeks rather than at the time of the anomaly scan. The diagnosis is made
by the appearance of free floating loops of bowel which are, in the majority of
case, to the right of the umbilicus.

Most mothers are young, average age around 20.
Incidence on the increase - currently 2-3 / 10,000.
Increased incidence linked to social drug usage but not proven.
Defect thought to arise as a result of a vascular problem, perhaps within the
right vitilline vessel. (Cocaine causes vascular constriction hence drug
hypothesis)

Gastoschisis is usually isolated.

Importantly there is no increased risk of chromosome anomaly. Risk is
therefore age related risk (Note: if using triple test then AFP will be raised
and will give a positive result for a NTD and therefore a lower than
expected Downs risk)


Antenatal complications

Common

IUGR- most babies will be small. Mean birth weight 2.35Kg (5lb 3oz).
Ultrasound will of course often suggest that the AC measurement is
even smaller as the bowel is not included in the AC measurement.
Velocity rather than absolute size is therefore more important.
Preterm delivery. Mean delivery gestation 36-37 weeks.
Increased bowel dilatation both intra and extra abdominal loops.
Difficult to know what the long term significance of this is as there is no
current data to suggest that babies with significant dilation benefit form
earlier delivery. However if we see significant dilatation or this is
increasing we may well consider delivery if the gestation is appropriate.

Rare
Antenatal stillbirth. Still occurs but has become rare (we believe as a
result of increased surveillance)
Vanishing bowel. Very rarely with an early diagnosis of gastroschisis
the defect appears to disappear. If the diagnosis was certain then this
can be indicative of bowel ischemia of such a degree that the bowel
becomes atretic and falls off. These children are born lacking, usually,
the majority of their small bowel. These children will fail to thrive. Small
bowel transplantation has been performed but is associated with many
problems.
Intrapartum complications
CTG anomalies. Both antenatal and intrapartum CTG recordings
demonstrate decreased variability. This is thought to be as a result of
stretching of the bowel mesentery
Meconium stained liquor At the time of ARM the liquor is usually
discoloured. This is usually with bile rather than meconium.
There is no indication for caesarean section unless there is an obstetric
indication. Points one and two will tend to indicate possible fetal
distress and as such the percentage of cases born by caesarean
section is higher than average.


Delivery
Paediatrician present for delivery and prepared for arrival of baby with
gastroschisis.
Post natal management aimed at keeping the bowel as healthy as
possible. The bowel is wrapped in cling film to prevent fluid loss and
bowel positioned to prevent compromise of blood supply
Surgery usually occurs within three hours of delivery. In recent Leeds
randomised trial mean 2.5 hours.
Present review of management. Standard care is to aim for primary
closure, however if this is not possible then the bowel is placed within a
silo and the defect is closed approximately 7 10 days later. Presently
assessing the use of primary silos inserted on the ward and then
closure (either with or without a general anaesthetic) on day 5.

Although there has never been a study which demonstrates a benefit of
delivery in a paediatric surgical centre this is one condition where we should
definitely strive to achieve that goal. Delivery outside of a surgical unit will
delay closure. Perhaps more important is the potential bowel damage as a
result of distortion of the blood supply.


Post natal management
Bowel exposed to amniotic fluid for weeks tends to be matted and is
slow to respond when replaced in abdomen. Feeding is slow and
gradual. TPN is usually required. Full feeding usually takes 4 5
weeks to establish.
Approximately 10% of babies will have an associated bowel atresia
which may be suspected on antenatal scanning. These will require
further surgery although this cannot be performed until the bowel has
had time to return to normal. Often 4- 6 weeks post delivery.
Babies will spend a minimum of 6-8 weeks in hospital post delivery.


Survival
Overall 90-95 % survival
Long term outcome good.


Care Pathway

Patients should be seen at least once antenatally. Ideally that visit should be
on a Monday morning at 19 -21 weeks when we would perform an ultrasound
scan looking at the defect as well as hunting for other anomalies.
The parents should then have the opportunity to meet with Mr Ian Sugarman,
consultant paediatric surgeon, who has a clinic on a Monday morning.


Follow up scans
These should be performed at:

26, 30 32, 34 and 36 weeks.
At each visit we would assess the following
BPD , HC, AC and Femur length.
Liquor volume / AFI
Umbilical artery Doppler
Evidence of intra or extra abdominal bowel dilation.


Indication for referral back to Leeds
Evidence of growth restriction with failure of growth to follow own
centile.
Evidence of significant bowel dilation. extra abdominal loop of >
15mm, intrabdominal loop > 20mm.
If pregnancy exceeds 39 weeks.
Or in any other concern.