The term omphalocele comes from the Greek (omphalos = the navel; and -zele = hernia) and means something like "umbilical cord hernia". During a normal pregnancy, the abdominal organs are displaced outwards into the umbilical cord outside the abdominal wall (physiological umbilical cord hernia) and move back into the abdominal cavity towards the end of the 3rd month of development. In the case of an omphalocele, the abdominal organs (intestine, liver or spleen) are therefore partially located inside the umbilical cord, which protects them from the amniotic fluid as a thin membrane (hernia sac). However, as the organs are already located outside the abdominal cavity during pregnancy, the abdominal wall cannot develop normally, resulting in a very small abdominal cavity.

The diagnosis is usually made before birth as part of the ultrasound examinations during pregnancy; an omphalocele is visible to the gynecologist from the 12th week of pregnancy at the earliest.

Omphalocele occurs in 1:5000 children, boys are more frequently affected than girls. It is not hereditary and there is therefore no higher risk for other children of the same mother. Ultimately, the cause of omphalocele is still unclear. Children with omphalocele often have other malformations, e.g. of the heart, kidneys or intestines. The prognosis usually depends on the accompanying malformations and the size of the abdominal wall defect. Children with prenatally known omphalocele should therefore be born in a center with well-founded neonatological and pediatric surgical expertise.

Treatment is usually surgical and consists of moving the contents of the hernia sac back into the abdominal cavity. For small omphaloceles, this can be done as a primary closure. In the case of large defects, the treatment strategy must be determined on a case-by-case basis.

Ideally, you will have already familiarized yourself with the upcoming operation and what to expect after the birth by talking to us before the birth. The Women's Clinic of the MHH offers a prenatal consultation in which pediatricians and pediatric surgeons inform the future parents in detail about the respective diseases and their treatment options.

After your child is born in the Women's Clinic, the entire lower half of your child's body is placed in a sterile plastic bag in the delivery room to prevent bacterial colonization and to mechanically protect the omphaloceles from drying out. We also start prophylactic antibiotic therapy. A gastric tube is inserted to prevent the leaked intestinal loops from filling excessively with air. As the children must remain fasting on the first day of life before the operation (in the case of a primary obstruction), a venous line is inserted and the children are supplied with sufficient fluids.

There are basically two different ways of treating an omphalocele: primary closure and delayed closure of the abdominal wall.

 

Primary closure of the abdominal wall

If immediate closure of the omphalocele is possible, this should be performed as soon as possible after birth. Once the operating room has been prepared, your child will be taken to the operating room. The omphalocele sac is removed and the intestines are pushed back into the abdominal cavity. The abdominal wall is then closed with a suture.

 

Delayed abdominal wall closure

If the abdominal wall defect is too large, there are several options for treatment, which we will discuss with you on a case-by-case basis:

• Repeated closure by tightening the abdominal wall.
• Temporary covering of the abdominal wall defect with a synthetic material ("patch") until the abdominal wall is permanently closed.
• Conservative procedure: An antiseptic ointment and sterile compresses are applied to the defect daily until the omphalocele is skinned over. The skinned omphalocele can then be closed at a later date.

In both primary closure and delayed (secondary) closure, children are usually not fed by mouth (enteral) in the first few days and receive the required fluids via a drip (venous access, intravenous). After the procedure, a tube is left in place, which is inserted via the nostril into your child's stomach and drains the digestive juices.

During this time, the child and mother are cared for by our pediatric nurses and medical team on the infant ward. When the digestive juices are transported from the intestine and your child has a bowel movement, we start to feed your child by mouth. However, it is not possible to predict how long it will take for the bowel to function normally. Every child takes its own time. However, full enteral feeding is typically delayed in children with omphalocele (several days to weeks from surgery to full feeding).
When your child is drinking the food completely and gaining weight steadily, the feeding tube will be withdrawn and you can be discharged home.

Due to the great variability of the disease spectrum and clinical course, it is not possible to predict when, how often and where the first post-operative check-ups will take place after discharge. They are usually carried out by your pediatrician, but we will also visit you at regular intervals in our pediatric surgery outpatient clinic.