Subject: Re: Gastroschisis
Forum: The Gastroenterology and Liver Diseases Forum
Topic Area:
Posted by HFHSM.D.-ym on March 17, 1998 at 12:55:42:
In Reply to: Gastroschisis posted by Penny Bessire on March 11, 1998 at 21:13:10:

Is anything known about the adult health of these infants born with this birth defect. My 24 year old daughter developed gastrointestinal problems about 5 years ago and was diagnosed with "Cron's Disease". She was a very healthy, bright and athletic child while growing up but since has had a resection, hernia, gallstones, etc. She is often ill and hospitalized due to bowel obstructions.
Thank you!
_Dear Penny,

You implied that your daughter may have had gastroschisis as an infant. Gastroschisis is a congenital abnormality due to the herniation of abdominal contents. Unlike another congenital defect called omphalocele these abdominal contents are not covered by a sac and are therefore exposed to amniotic fluid in utero. This exposure damages the intestine and causes thickening of its walls. Up to 21% of patients with gastroschisis have associated anomalies including intestinal atresia (incomplete formation of the intestine), volvulus (twisting of intestinal loops), undescended testicles (in males) and gallbladder and kidney agenesis (incomplete development of these organs).

The incidence of gastroschisis is approximately 1:10,0000 births. A recent population based study over a 14 year period showed a sharp rise in the occurrence of gastroschisis in the infants born to women 15-19 years of age. The reasons for this are poorly understood. Socially disadvantaged women with a history of smoking and substance abuse are also at higher risk.

Abdominal wall defects are usually diagnosed in the prenatal period with ultrasound. If an amniocentesis (sampling of amniotic fluid) is performed it is extremely rare to find chromosomal abnormalities. These infants are more often delivered by Caesarean section. Although initial survival of these infants is very good, early corrective surgery must be performed. Sometimes during corrective surgery intestine must be resected and this may lead to the development of intestinal malabsorption later in life. If intestinal atresia or intestinal narrowing are present these may also necessitate resection of large amounts of intestine. After surgery these infants often require total parenteral nutrition (intravenous nutrition) which may also be associated with infectious and non-infectious complications. Nevertheless, the surgery is often successful and associated with a low mortality rate.

I was able to find two recent reports which assess the long term morbidity and quality of life in survivors of gastroschisis. In the first study 83/94 patients with omphalocele or gastroschisis survived initial treatment and 61 had long term follow-up. If congenital abnormalities were not present survival was favorable. Nineteen patients required 31 reoperations, most for abdominal wall hernias and the sequelae of intestinal atresia. Current quality of life (10-20 years after birth) was described as good in 80% of patients. In the second study 23/35 patients with gastroschisis (age range 12-23 years old) were questioned about their general health, growth, abdominal symptoms, cosmetic concerns, education, employment and fertility. 22 patients were in good health and overall growth was normal. Eight patients underwent further surgery related to gastroschisis, including two for adhesive small bowel obstruction (scar tissue forming in the abdomen after surgery-this can cause intestinal blockage) and three for scar complications. In 13 patients the absence of an umbilicus (belly button) caused emotional distress during childhood. These reports suggest that once corrective surgery is performed most gastroschisis survivors can expect normal growth and good health, however, a significant percentage of patients will require reoperation.

Although your daughter could have Crohns disease it is also possible that she has complications related to having gastroschisis as an infant and undergoing corrective surgery (i.e. if in fact she had gastroschisis as an infant). Crohns disease is a diagnosis that is usually made after biopsies are taken from the intestine and reviewed by a pathologist. Although characteristic clinical and radiographic findings can be present to suggest the diagnosis, these findings can be mimicked by other diseases and should not be relied upon without confirmation. I hope you find this information helpful. Good luck to you and your daughter.

This response is being provided for general informational purposes only and should not be considered medical advice or consultation. Always check with your personal physician when you have a question pertaining to your health.

If you would like to be seen at our institution please call 1-800-653-6568, our Referring Physicians Office and make an appointment to see Dr. Muszkat one of our experts in Gastroenterology.

HFHSM.D.-ym
Keywords: gastroschisis, survivors

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