Gastroschisis

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Gastroschisis
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Abdominal wall defect, intestines outside the body
Complications	Short bowel syndrome, intestinal atresia, sepsis
Onset	Prenatal
Duration	Chronic
Types	N/A
Causes	Unknown, possibly genetic and environmental factors
Risks	Young maternal age, smoking, alcohol use, recreational drug use
Diagnosis	Prenatal ultrasound, physical examination
Differential diagnosis	Omphalocele, bladder exstrophy
Prevention	None known
Treatment	Surgical repair
Medication	N/A
Prognosis	Generally good with treatment
Frequency	1 in 2,000 to 5,000 live births
Deaths	N/A

Other Names: Congenital fissure of the abdominal cavity; Laparoschisis Gastroschisis is a birth defect that occurs when a baby's intestines extend outside of the body through a hole next to the belly button. This type of defect is known as an abdominal wall defect. Sometimes other organs are also involved.

Cause

The exact cause of a gastroschisis is usually not known, but it is more common in babies born to young mothers or to mothers who may have used alcohol or tobacco during their pregnancies. Babies with gastroschisis are born with a hole in the abdominal wall. The child's intestines often stick out (protrude) through the hole. The condition looks similar to an omphalocele. An omphalocele, however, is a birth defect in which the infant's intestine or other abdominal organs protrude through a hole in the belly button area and are covered with a membrane. With gastroschisis, there is no covering membrane. Abdominal wall defects develop as a baby grows inside the mother's womb. During development, the intestine and other organs (liver, bladder, stomach, and ovaries, or testes) develop outside the body at first and then usually return inside. In babies with gastroschisis, the intestines (and sometimes the stomach) remain outside the abdominal wall, without a membrane covering them. The exact cause of abdominal wall defects is not known.

Riskfactors

Mothers with the following may be at higher risk of having babies with gastrochisis:

• Younger age
• Fewer resources
• Poor nutrition during pregnancy
• Use tobacco, cocaine, or methamphetamines
• Nitrosamine exposure (chemical found in some foods, cosmetics, cigarettes)
• Use of aspirin, ibuprofen, acetaminophen
• Use of decongestants that have the chemical pseudoephedrine or phenylpropanolamine
• Babies with gastroschisis usually do not have other related birth defects.

Symptoms

A gastroschisis is usually seen during a prenatal ultrasound. It can also be seen when the baby is born. There is a hole in the abdominal wall. The small intestine is often outside the abdomen near the umbilical cord. Other organs that may also be seen are the large intestine, stomach, or gallbladder. Usually the intestine is irritated by the exposure to amniotic fluid.The baby may have problems absorbing food. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 80%-99% of people have these symptoms

• Gastroschisis

30%-79% of people have these symptoms

• Intestinal atresia

5%-29% of people have these symptoms

• Abnormality of mesentery morphology

Diagnosis

Gastroschisis is typically diagnosed on routine ultrasound examinations before a baby is born. These babies may need to be born in hospitals with specialists who are experienced in treating gastroschisis.

Treatment

If gastroschisis is found before birth, the mother will need special monitoring to make sure her unborn baby remains healthy. Treatment for gastroschisis involves surgery. Usually the infant's abdominal cavity is too small for the intestine to fit back in at birth. So a mesh sack is stitched around the borders of the defect and the edges of the defect are pulled up. The sack is called a silo. Over the next week or two, the intestine returns into the abdominal cavity and the defect can then be closed. The baby's temperature must be carefully controlled, because the exposed intestine allows a lot of body heat to escape. Because of the pressure involved in returning the intestines to the abdomen, the baby may need support to breathe with a ventilator. Other treatments for the baby include nutrients by IV and antibiotics to prevent infection. Even after the defect is closed, IV nutrition will continue as milk feedings must be introduced slowly.

Prognosis

The baby has a good chance of recovering if there are no other problems and if the abdominal cavity is large enough. A very small abdominal cavity may result in complications that require more surgeries. Plans should be made for careful delivery and immediate management of the problem after birth. The baby should be delivered in a medical center that is skilled at repairing abdominal wall defects. Babies are likely to do better if they do not need to be taken to another center for further treatment. Possible Complications Because of the exposure to amniotic fluid, babies' intestines may not work normally even after the organs are put back inside the abdominal cavity. Babies with gastroschisis need time for their intestines to recover and become used to taking feedings. A small number of babies with gastroschisis (about 10-20%) may have intestinal atresia (parts of the intestines that did not develop in the womb). These babies require further surgery to relieve obstruction. The increased pressure from the misplaced abdominal contents can decrease blood flow to the intestines and kidneys. It can also make it difficult for the baby to expand the lungs, leading to breathing problems. Another possible complication is bowel death necrosis. This occurs when intestinal tissue dies due to low blood flow or infection. This risk may be reduced in babies who receive breast milk rather than formula. This condition is apparent at birth and will be detected in the hospital at delivery if it has not already been seen on routine fetal ultrasound exams during pregnancy. If you have given birth at home and your baby appears to have this defect, call the local emergency number (such as 911) right away.

NIH genetic and rare disease info

• NIH info on Gastroschisis

Gastroschisis is a rare disease.

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