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Pre-Operative Discussions


I. Embryology

Gastroschisis is a birth defect in which the abdominal organs are outside the abdomen, unprotected and exposed to the environment. Returning the organs to the abdomen or covering them with a protective silo is an urgent surgical procedure. The intestines normally develop outside the abdomen and enter the abdomen during the 10th–12th week of the pregnancy. The abdominal muscles are then supposed to close and seal them inside. For some reason in one out of 2500 babies the intestines either don’t enter the abdomen or the muscles fail to close and the intestines come back out. Occasionally, one or both testicles in males or one or both ovaries in females may also stick out through this hole.

Approximately 10% of babies with gastroschisis will have a second birth defect involving the intestine, in which the hole that is supposed to go through the intestines from the mouth to the anus is blocked at one or more places.

II.  Closure

Placing the intestines and other organs inside the abdomen after birth is an urgent surgical procedure. In approximately 85% of cases, the organs can be placed into the abdomen with a single operation performed within several hours of the baby’s birth. In the other babies, the intestines may be so swollen and inflamed that they will not fit into the abdomen. In this case the intestines are placed inside a plastic bag, or silo, which is elevated above the baby’s abdomen. Raising the intestine in this silo will encourage the swelling to resolve and over the next 2–10 days the intestine can be gradually squeezed into the abdomen. Once the intestines have been squeezed into the abdomen, the baby is taken to the operating room, the silo is removed and the hole in the muscles is sewn closed.

III. Ventilator

In all babies with gastroschisis, because the abdomen developed without the organs inside, the abdominal cavity will be too small to receive them; so when the intestines and other organs are placed inside, the abdomen will be very full, and even tight. This is similar to what you are experiencing as your uterus enlarges with the fetus and pushes up on your diaphragm, making it more difficult to take a deep breath and to breathe. Your baby will have trouble breathing after the organs have been squeezed inside and will need assistance breathing with a ventilator, a mechanical device that can do all or part of the baby’s effort to breathe. He/she may need the ventilator support for one or more days after birth, depending on his/her recovery and any other medical issues.

IV. Dysmotility and Nutrition

In babies with gastroschisis after the intestines are placed inside the abdomen they will be sluggish to work for 2–6 weeks after birth. The stomach and intestines will still make their juices, but the muscles in the intestine will be weak and cannot push even the intestinal juices further down the intestines. This is called dysmotility and it means that your baby will not be able to take any liquids, formula, breast milk or food by mouth for 2–6 weeks after birth. After this time, when feedings are started, the volume of each feeding will have to be gradually increased as the coordination in the intestinal muscles improves. During this time the baby will have a tube inserted through his/her mouth into the stomach to suck out the stomach juices, otherwise he/she would throw them up.

Because your baby cannot take a bottle, the doctors/nurses will need to insert a special IV called a central venous catheter, or a PICC line, that threads into the large veins outside the heart, where the blood flows the fastest. Into these veins, we can give high-calorie, high-protein intravenous feedings and actually make your baby grow as he/she recovers from the surgery. This is called parenteral nutrition or TPN.

V. Hospitalization

You should expect your baby to be in the hospital for at least four weeks, if his/her recovery goes smoothly. Many times the ability of the baby to tolerate bottle/breast feedings may be gradual and prolonged. Some of the babies with gastroschisis may have other unrelated birth defects or medical conditions which may prolong their hospitalization. Babies with the more complicated/complex conditions may be in the hospital for months after birth.

VI. Who will be taking care of your baby?

There will be a team of physicians, surgeons, nurses and nurse practitioners who will coordinate your baby’s care.  Other medical specialists may be consulted in your baby’s care as needed.

The pediatric surgeon who is on call the day that your baby is born is the surgeon who will perform your baby’s surgery. This may or may not be the same pediatric surgeon that you met during your prenatal interview.

Neonatologists are pediatricians with advanced training in the care of the medical conditions of premature babies and babies with birth defects. There is a group of neonatologists at the Children’s Hospital and at least one of them is in the hospital 24 hours per day. They will be readily available to supervise your baby’s care and to answer your questions throughout his/her stay in the Newborn Intensive Care Unit (NICU).

Working with the neonatologists are neonatal nurse practitioners and with your pediatric surgeon are pediatric surgical nurse practitioners. These nurses have advanced training in the care of premature babies and medical emergencies and coordinate the care of your baby among the physicians and surgeons.

At your baby’s bedside will be a pediatric staff nurse who has advanced training in care for babies in the NICU environment. These are the nurses that will have the most frequent contact with your baby and you.

VII.  Outcome

Among babies born with a gastroschisis, 95% survive and most of these children grow and develop normally, and as adults, lead productive lives.

In some cases of gastroschisis the muscles of the abdominal wall may start to close before the baby is born and the muscles may strangle the intestine outside the abdomen resulting in severe damage. This damage may leave an intestine that is shorter than normal and unable to digest food. This condition may require the need for intravenous feedings over many months or years and may lead to the consideration for a small bowel transplant. Other babies may have a long enough intestine but the dysmotility may linger for months or never improve; these babies may also be considered for small bowel transplantation.