St. Louis Blues hockey player TJ Oshie is having an amazing year.
He became the darling of the ice rink at the 2014 Sochi Olympics when he helped the US team beat against rival Russia. Oshie scored the winning goal in a double over-time shootout.
And now, he and fiancée Lauren Cosgrove have become parents for the first time, to daughter Lila Grace, born on St. Patricks Day. But little Lila’s birth was less than routine. Before she was born, the couple learned that Lila suffered from a rare birth defect called gastroschisis, where some of her intestines lie outside the body through a hole in the abdominal wall. Surgery to replace the intestines and close the hole were necessary shortly after birth.
Lila underwent the surgery at St. Louis Children’s Hospital (where I did my pediatrics residency) and is doing very well post-operatively. Here’s a little more about it:
Gastroschisis is a birth defect of the abdominal (belly) wall. The baby’s intestines stick outside of the baby’s body, through a hole beside the belly button. The hole can be small or large and sometimes other organs, such as the stomach and liver, can also stick outside of the baby’s body.
Gastroschisis occurs early during pregnancy when the muscles that make up the baby’s abdominal wall do not form correctly. A hole occurs which allows the intestines and other organs to extend outside of the body, usually to the right side of belly button. Because the intestines are not covered in a protective sac and are exposed to the amniotic fluid, the bowel can become irritated, causing it to shorten, twist, or swell.
Gastroschisis is similar to a disorder called an omphalacoele. In an omphalacoele, the defect is in the tissues surrounding the umbilical cord and the intestines or other abdominal organs stick out of the belly button area, and are covered with a membrane. In gastroschisis the problem is in the abdominal wall muscles and the intestines are found to one side of the umbilical cord.
The Centers for Disease Control and Prevention (CDC) estimates that about 1,871 babies are born each year in the United States with gastroschisis.
Because the intestines are floating in the amniotic fluid, there is frequently more protein in the fluid. This could result in a high AFP (alpha fetoprotein) level- a test frequently done during pregnancy to look for birth defects. The disorder may also be discovered on a prenatal ultrasound– most commonly during the second trimester.
Soon after the baby is born, surgery will be needed to place the abdominal organs inside the baby’s body and repair the defect.
If the gastroschisis defect is small (only some of the intestine is outside of the belly), it is usually treated with surgery soon after birth to put the organs back into the belly and close the opening. If the gastroschisis defect is large (many organs outside of the belly), the repair might done slowly, in stages. The exposed organs might be covered with a special mesh and slowly moved back into the belly. After all of the organs have been put back in the belly, the opening is closed.
After the repair, most infants with gastroschisis will do perfectly well. However, those who had a large amount of abdominal contents outside the belly, or had to have sections of bowel removed may have problems with feeding, digestion of food, and absorption of nutrients.