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Most congenital GI anomalies result in some type of intestinal obstruction, frequently manifesting with feeding difficulties, distention, and emesis at birth or within 1 or 2 days. Infants with GI malformations have a high mortality rate, ranging from 10 to 40%; the highest rate occurs in those with congenital diaphragmatic hernia.
A common type of anomaly is atresia, in which a segment of the GI tract fails to form or develop normally. The most common type is esophageal atresia, followed by atresia in the jejunoileal region and in the duodenum.
Immediate management includes bowel decompression (by continuous nasogastric suction to prevent emesis, which can lead to aspiration pneumonia or further abdominal distention with respiratory embarrassment) and referral to a center for neonatal surgery. Also vital are maintenance of body temperature, prevention of hypoglycemia with IV 10% dextrose and electrolytes, and prevention or treatment of acidosis and infections so that the infant is in optimal condition for surgery.
Because about one third of infants with a GI malformation have another congenital anomaly (up to 50% in those with congenital diaphragmatic hernia and 70% in those with omphalocele), they should be evaluated for malformations of other organ systems, especially of the CNS, heart, and kidneys.
Last full review/revision September 2009 by William J. Cochran, MD
Content last modified September 2009
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