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Introduction

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 suffering.

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 of theories.

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.

Simon Huddart

Consultant Paediatric Surgeon
University Hospital of Wales, Cardiff
January 2002

Diagnosis of the condition Top of page
Diagnosis is, in most cases of gastroschisis and exomphalos, made when the pregnant mother attends hospital for a routine ultrasound scan at around 16-20 weeks.

The Mother may be offered further investigative tests to discover weather the baby has any other problems. These tests may include a more detailed ultrasound scan, an amniocentesis or cordocentisis. But the amniocenteses and cordocentisis are invesive tests and carry a risk of miscarriage, therefore the mother should receive guidance from her consultant as to weather these tests are deemed appropriate.

Termination of the pregnancy Top of page
As most cases are detected when the Mother attends hospital for an ultrasound scan,the option of having the pregnancy terminated will be available and probably offered to her.

Before making a decision you should be given an opportunity to find out as much as you can about your child’s condition and it’s severity. Seek advice about the condition from the obstetrician, your GP and if possible a neonatal surgeon who can explain in general terms what will be done once he baby is born.

GEEPS can give you information sheets on the condition and this website aims to provide a realistic insight into what might happen after the baby is born. We can also put you in touch with other parents who have been through a similar situation.

Although most parents who contact GEEPS do decide to carry on with the pregnancy, we can also support those parents who choose, or are strongly advised, to terminate the pregnancy. Some parents feel that they could not cope with the unborn child’s condition and other baby’s are so badly affected that termination of the pregnancy is advised. It is an agonising choice to make and we fully support and sympathise with those parents who take the decision to terminate.

Finally, remember that everyone will offer their advice and opinions, from professional health workers to family and friends, but the final decision is yours and you should take as much time as you need to make the choice that you are happy with and not feel pressurised by anyone.

Preparing for the birth Top of page
After diagnosis has been made the mother will likely be placed under consultant care.

Regular ultrasound scans to monitor the progress of the baby and to assess the amount and condition of the Gastroschisis/Exomphalos are also likely. This will help the obstretician and the surgeon to decide on the best time and place for the baby to be delivered.

Towards the end of the pregnancy ask if an appointment can be made to meet the paediatric surgeon who will be operating on the baby and ask any questions or talk about worries that you may have. Also ask if arrangements can be made for you to b shown around the Intensive care unit or Special care unit where the baby will be nursed after surgery.

Most hospitals have facilities for one or both parents to sleep at the hospital whilst their baby is receiving nursing care. The standard of accommodation on offer varies, and in some hospitals it may be a put-up-upbed in a corner of the ward, where other hospitals have purpose built facilities for parents. GEEPS recommends that you ask if these facilities for parents are available, very often the information is not voluntarily given. Let the hospital know if you would like to stay with your child as soon as possible so that the appropriate arrangements can be made in advance.

At the time of birth Top of page
Most parents assume that the mother will have a caesarian section because they cannot possibly see how a vaginal birth would not further damage the bowel and the organs. Often a vaginal delivery is possible and the options will be discussed with the parents and a decision will be based on what is best for the baby and mother. Obviously if the defect was not picked up during the mother's pregnancy then she will be delivered according to her progress at the time of labour.

Studies have shown that the majority of Gastroschisis and Exomphalos babies are born slightly earlier than the normal 40 weeks gestation. (36-38 weeks seems to be the average).

If an elective caesarian section is to take place then the opportunity to have an epidural or spinal block will probably be offered. GEEPS mothers who have undergone an elective section have, on the whole, chosen this option so that they are awake when their baby is born. If the baby is to be transferred to another hospital for its surgery particular consideration should be given to having an epidural/spinal block. A caesarian section is major abdominal surgery and it will be a few days before the mother will feel comfortable enough to make a journey to another hospital to visit the baby.

What happens after the baby is born Top of page

The paediatric team caring for the baby will ensure that the baby is breathing normally. A tube will be put up the baby's nose and into his/her stomach. (The tube is called a naso gastric tube or N.G.T. or N.G. tube). The N.G. tube will be left in place and is used to get rid of excess air which could make the baby's bowel more swollen. The baby's abdomen and exposed bowel will then be wrapped in a medical cling film which prevents further damage and infection and will also keep the baby warm. When the team is satisfied that the baby is in a stable condition they may bring him/her to you and you may be able to have a quick cuddle.

The baby will then be placed in an incubator and taken to the intensive care unit to enable preparation for surgery. Blood tests will be done and a drip inserted into the baby to enable fluids and antibiotics to be given. Sometimes the baby might need a little extra oxygen and this will be given via a head box or mask.

The surgeon and surgical team will then assess the baby's condition and will decide on one of two types of operation.

If there is only a small amount of bowel outside of the baby's abdomen, it will be put back inside and the wound area and closed. This is called a primary repair.

Sometimes if the Gastroschisis/Exomphalos is large or if the abdominal cavity is small the surgeons will construct a pouch in which the Gastroschisis/Exomphalos is placed. The pouch is then suspended from the ceiling of the incubator and gravity along with a reduction of the size of the pouch will help the Gastroschisis/Exomphalos to slip back inside the abdomen.

This may take a week or more in total and only then will the final closure occur. Occasionally there is not enough skin to cover the affected area, and a patch of silicone meshing or gortex patch is stitched to the skin. Over the next few months the skin will slowly grow over the patch. This type of surgery is called a staged repair.

After the operation Top of page

The baby will be taken back to the intensive Care Unit and nursed there. In most cases the baby will be on a ventilator for a few days. When the ventilator is removed the baby usually needs extra oxygen for a few days and this will be given a through a head box or blown by tube straight into the incubator.

Throughout the baby's time in hospital he/she will be closely monitored. When the baby first comes back from theatre after surgery monitoring may take place as often as half-hourly. The baby's heart rate, temperature, blood pressure and breathing will be watched and adjustments to equipment and pain relief made accordingly. Intravenous drips will also be topped up and the bags supplying the drips changed at the appropriate times.

Helping with the care of your baby Top of page

The nursing staff will encourage your participation in the care of your baby as soon as you feel ready. As baby progresses you will be able to do more. You might start by washing baby's face and body, changing the sheets etc. The nurses will help you until you feel confident of doing things on your own. Take one step at a time and don't feel pressured into tackling more than you feel you can manage until you are ready.

Feeding the baby Top of page

Because the bowel has been laying outside of the abdomen for a long time it does not work properly after the birth. Sometimes it has been so badly damaged that some of it may be removed.

The baby will not be fed by mouth (orally) until everything has healed and the bowel is working. This can sometimes take several weeks. During this time the baby will receive all necessary fluids and calories intravenously (by drip).

Although breast feeding is normally encouraged, some babies cannot absorb breast milk very easily and to start with they may be given a special formula feed. It is best to speak to the team caring for your baby if you wish to try breast feeding. Mothers are normally asked to express their milk and it is frozen for later use. To begin with baby will be given small amounts of breast milk to see if it is tolerated. If it is, the amount will slowly be increased and if all goes well the mother may be able to try breast feeding.

The eventual outcome Top of page

Once the abdomen has healed and the bowel is working properly the baby should make good progress.

Usually he/she will be in ICU for at least a further 4-6 weeks or so. Some babies require several months stay in hospital before they are able to go home. Once the baby is tolerating milk and feeding well and you are happy and confident about caring for your baby, it will be time to go home.

If a lot of damaged bowel has been removed then the baby may need extra feeds and calories to help normal growth. You will be shown how to prepare the high calorie formula feeds by the nurses before you take your baby home.

The baby will have a scar on its abdomen from the operation(s). Obviously the size of the scar depends on the initial amount of bowel and organs outside at birth. "Tidy up" operations may be performed when the baby is a few years old. The operation literally tidies up the scarring. Some children grow up conscious that they have no tummy button and the surgeon may "make" a tummy button from existing skin when performing tidy up operations.

Taking your baby home Top of page

Some parents can't wait to take their baby home and start a "normal" life, free from hospital routine. Other parents worry about leaving the safety of the hospital and the support received from the nursing staff.

If, when you get your baby home, you have any queries then you should immediately contact either your G.P. or the hospital where your baby stayed.

As with any new baby, try to get as much practical help as possible. Family, friends and neighbours can do all sorts of jobs for you. Take advantage of them.

Your baby's Health Visitor will become a regular visitor once you are at home. The Health Visitor is a valuable source of local information and will know all sorts of organisations that could be of use. Ask if there is any nursing support available. The community nurse may come and help with dressings if your baby still needs them.

Outpatients appointments Top of page

When your baby is discharged from hospital, arrangements will be made for him/her to attend an outpatient clinic. To begin with the appointments will take place on a regular basis and it may be some time before you are seeing the consultant every six months or once a year. Use these visits to discuss any serious worries you have.

Before your visit write down any questions you want to ask and jot down the answers if you think you may forget them later. If you don't understand a question the consultant is asking you then ask them to rephrase it and make sure you understand what you are being told. If you think of a question you meant to ask after you leave the hospital, then make a note of it for your next visit or write to the consultant about it.

You may have to wait a considerable time for your doctor/consultant once you arrive at the hospital. Appointment times are approximate. Make sure your car is parked where it won' get towed away or check the bus/train timetables for the next few hours. Take a couple of your baby's favourite toys and a change of nappies and cloths. If you are bottle-feeding then take along a feed just in case you need it. As the scouts say - be prepared!