GASTROSCHISIS By: Rizka Hanifa Gastroschisis is one of several congenital abdominal wall defects that evolves in the first

four post-conception-weeks. It is generally accepted that this congenital abdominal wall defect is embryologically different from omphalocele. The anomaly is thought to be the result of a defect at the site where the second umbilical vein involutes. Non rotation of the bowel always accompanies this anomaly and there is an increase in intestinal abnormalities including atresia (mostly involving the small instestine) perforation, and infarction resulting from in utero midgut volvulus or vascular thrombosis. Worldwide, the incidence of gastroschisis is approximately 1 in 4,000-6,000 live births. Infants with gastroschisis typically are slightly premature (35-37 weeks of gestation) and frequently have growth retardation with birth weights from approximately 2000-2500 g. A rising incidence of this anomaly is documented in the medical literature of every decade since 1940s. Incidence of associated anomalies remains relatively stable at 10%, including mostly gastrointestinal and cardiac anomalies. Therefore, gastroschisis represents a majority of the congenital gastrointestinal anomalies encountered in fetal and newborn healthcare. Most case of gastroschisis are suspected by abnormal maternal serology and reliably confirmed fetal by ultrasound. Ultrasound diagnosis in the late first trisemester is possible as physiologic return of the intestines to the peritoneal cavity is normally complete by 11 weeks. The identifying sonographic features of gastrochisis are the findings of multiple loops of bowel floating freely in amniotic fluid. The characteristics gross appearance of gastrochisis involves a defect to the right of the umbilicus that has smooth edges and is usually less than 4 cm in diameter. The defect is frequently separated from the base of the umbilical cord by a small bridge of skin. The intestines are usually thickened and matted with no overlying membrane. The remainder of the pregnancy will be closely monitored. On average, spontaneous labor occurs around 36 weeks with gastroschisis. Most women deliver vaginally, unless there is a medical condition that necessitates a cesarean delivery or if the fetus is having difficulty before birth. After birth, the baby is immediately moved from the delivery room to an adjacent stabilization room, where breathing status is assessed, an IV is placed and a nasogastric (NG)

tube (a tube that runs through the nose) is placed to decompress the intestine this prevents reflux of gastric contents into the lungs. Throughout, careful positioning of the baby is critical to maintain adequate blood flow to the intestine. Repair of gastroschisis involves returning the extra-abdominal contents back into the abdominal cavity, followed by abdominal wall closure. This can either be performed with an immediate primary gastroschisis repair or, more commonly, a repair done in a series of steps (staged), depending upon postnatal assessment of the condition of the exposed intestine. At the time of delivery, the exposed abdominal contents are placed in a moistened bag for the infants transfer to the N/IICU. The baby undergoes surgical repair immediately upon admission to the N/IICU. The intestine is returned to the abdominal cavity and the abdominal wall is closed during one procedure. A staged gastroschisis repair is performed if the amount of intestine outside the abdomen is especially large, dilated or if the babys condition is unstable. A silastic pouch is placed around the herniated intestine, and reduced over 5-10 days. The outcome for patients with gastroschisis has dramatically improved. Whereas the mortality was 80 to 90% three or four decades ago, the survival is now more than 90%. The improvement in outcome is related to the availability of intravenous nutrition and the use of staged closure when indicated. Late complications and mortality are related to sepsis either from an intra-abdominal or wound complication or from a central venous catheter placed for parental nutrition.

Sources: Peter Mattei, MD. FAAPS, FACS (2011) Fundamentals of Pediatrics Surgery. Springers New York. Prem Puri MS, FRCS, FRCS (Ed)), FACS, Michael E. Hollwarth MD (2006) Pediatric Surgery. Springer Verlag Berlin Heidelberg. The Childrens Hospital of Philadelphia, Available at : http://www.chop.edu/service/fetaldiagnosis-and-treatment/fetal-diagnoses/gastroschisis.html
2013). (Accessed: 14th June