Omphalocele

The omphalocele is a condition in which the baby is born with the contents (intestines and sometimes organs) of the abdomen lying outside of the abdomen. The contents of the abdomen are covered with the amniotic membrane and the peritoneum. The umbilical cord comes out of the top of this sac. The amniotic membrane is one of the coverings of the baby formed inside the placenta while the baby is in development. The peritoneum is the smooth shiny covering (membrane) which lines the abdomen. This is the same membrane lining our abdomen as adults. If the defect in the abdominal wall is large, other organs (liver and spleen) may also be located outside the abdomen.

CAUSES

The muscles of the abdominal wall do not develop and close properly during the pregnancy.

SYMPTOMS

There is a sac sticking out of the abdomen. It is filled with intestines and at times, organs are clearly seen.

DIAGNOSIS

A diagnosis can be made during pregnancy with ultrasound.

OTHER PROBLEMS BABIES WITH OMPHALOCELE MAY HAVE INCLUDE:

  • The sac may break (rupture) while inside the womb (uterus) or during delivery.

  • Babies may be born before their due date (premature).

  • Babies may have other bowel problems and heart defects.

  • Babies may have the Beckwith-Wiedemann Syndrome. This is a collection of problems that includes:

  • A large tongue (macroglossia).

  • A large baby (gigantism).

  • Increased insulin secretion (hyperinsulinemia).

  • Low blood sugar (hypoglycemia).

  • If the abdominal wall defect is large, babies may have poor lung development and problems with breathing.

  • There may be heart defects present along with other abnormal organs.

  • The survival rate of babies born with an omphalocele varies. It often depends on what is wrong with the other organs.

TREATMENT

  • The surgical repair of the abdominal wall involves placing the organs that are outside the abdomen back into the abdomen. If the contents of the abdomen do not fit into the abdomen through the hole in the abdominal wall, a germ free (sterile) pouch or "silo" is created. It can hold the abdominal contents until everything can be pushed back inside. The surgery is usually done hours after birth.

  • Sometimes the omphalocele is so big that it will not fit back into the baby's abdomen. The skin around the omphalocele can grow around it and in time cover the defect. Later, when the baby is older, the abdominal muscles and skin can be repaired.

  • When the omphalocele is diagnosed during the pregnancy, the mother should take special care of herself. The baby should be monitored very closely until born.

  • The baby should be delivered carefully and surgery performed as soon as possible. The baby should be delivered in hospital that has the surgical expertise and is set up to operate on an omphalocele.

POSSIBLE OPTIONS DURING SURGERY (SURGEON'S CHOICES)

  • Sometimes the skin can be stretched over the contents of the abdomen. After this, the walls (muscles and other layers) of the abdomen can be repaired at a later date.

  • A patch of special material can be sewn to the skin edges to cover the abdominal contents. This will be removed at a later surgery.

  • A sack can be built out of special material. This sack is sewn to the skin with the contents of the abdomen. Over a period of time, the abdomen grows and more of its contents fit inside. Once the abdomen is large enough, the sack can be removed with a second surgery. The wall of the abdomen can be repaired.

HOME CARE INSTRUCTIONS

  • Keep all your caregiver's appointments.

  • Follow your caregiver's advice and instructions.

  • Maintain a healthy pregnancy by getting enough rest, eating healthy and taking your prenatal supplements (vitamins, medications, etc.)

SEEK MEDICAL CARE DURING THE PREGNANCY IF:

  • You develop a fever.

  • You have contractions of the uterus.

  • You develop vaginal bleeding.

  • You do not feel the baby moving or it is moving less than usual.

WHEN WILL YOUR BABY BE BETTER?

  • It generally takes a few weeks until the baby can take food by mouth. The type of food taken will be guided by your caregiver.

  • In the first weeks after surgery the baby is at an increased risk for bowel inflammation called Necrotizing Enterocolitis. This is a dangerous condition which your caregiver will discuss with you if necessary.

  • Your caregiver will tell you in how to care for your baby's patch or pouch at home if one was created.

SEEK MEDICAL CARE FOR THE BABY AFTER THE SURGERY IF:

  • A fever develops, 100° F (37.8° C) or higher develops.

  • Feeding problems develop.

  • Vomiting green or yellow material develops.

  • Any kind of excessive, persistent or projectile vomiting develops.

  • There are less bowel movements.

  • The area of the surgery becomes red or swollen.

  • There is fluid drainage or pus from the surgery area.