|It is the profound wish
of all parents that their baby will be "normal" and healthy
and one of their biggest nightmares is that the child should have
a major anatomical problem requiring emergency surgery. The birth
of a baby should be a time of joy and celebration - congratulations
given to the proud parents and tender warnings of the tribulations
to come! Serious congenital conditions changes this moment to one
of anxiety, fear and guilt. I have not met a parent who would not
willingly change places with their child to take over that child's
Gastroschisis and Exomphalos are dramatic conditions
where the problem is obvious for all to see. It is the role of the
paediatric surgeon together with a vast team of technicians, paediatricians,
specialist nurses, general practitioners and parental support groups
to ensure the best possible outcome for the child and to reduce
the stress for the parents.
A number of advances have improved the service
we can now give: Antenatal ultrasound scanning is able to identify
these conditions early on in pregnancy in the majority of women.
This allows for counselling long before the child is due, such that
the parents have a realistic knowledge of what is and what is not
likely and possible, it also allows for the birth of the baby to
be planned. It also allows for the parents to contact support groups
such as GEEPS. At this time of anxiety it is vital that all forces
speak with one voice to avoid confusion.
Improvements in obstetric and neonatal care has
increased the early survival of babies.The administration of
TPN allows for babies to survive and grow even when the intestines
cannot be used for several weeks.
Surgical techniques now take into consideration
the long term aesthetic implications of surgery. We do try to maintain
the belly button! (I am assured by my older patients that without
a belly button either "you cannot be pulled up to heaven "or"
"your bottom will fall off!")
There are a number of techniques now available
to surgically increase the length of bowel where this has been damaged
by the condition, however there remain a number of outstanding problems
with such babies:
For some babies, with Exomphalos in particular,
there are associated problems with the genetic makeup of the baby
and also with the major systems such as the as the heart, kidneys
or brain. Such problems may be identified early in pregnancy. If
the problems are of such magnitude that survival is not possible,
or the quality of life will be dire, parents may choose, after appropriate
counselling, not to continue with this pregnancy. Similarly, although
doctors will not actively end life, if after birth there are problems
as above, then most doctors would be very sympathetic towards parents
who want to limit the medical intervention given.
In some cases of Gastroschisis, there is not sufficient
bowel to sustain normal life. Whereas TPN
can keep babies alive for many years, this is not without problems
such as infection, blockage of lines and liver failure. Bowel transplant
is approaching being a viable medical treatment, but is enormously
expensive and not without significant problems.
The number of cases of babies with Gastroschisis
has increased dramatically in the Western world over the past decade.
The cause of this increase is not known, although there are a number
There are fewer than 100 surgeons in the
U.K. operating on babies with Gastroschisis and Exomphalos. We all
meet frequently and the standard of surgical care is likely to be
very similar whatever the region of the country you are in. Paediatric
surgeons are a dedicated lot and care passionately for their charges.
They cannot work miracles, but will work with you to ensure the
best possible future for your baby.
Consultant Paediatric Surgeon
University Hospital of Wales, Cardiff