PRENATAL CONGENITAL MALFORMATIONS

PRENATAL CONGENITAL MALFORMATIONS

Poor survival with neuroblastomas, diaphragmatic hernias and necrotizing enterocolitis requires efforts during the next few years to reduce mortality rates. These areas will require extensive investigation as to etiology, unique characteristics and better management.

A. Fetal Surgery

Certain lesions such as hydrocephalus, hydroureteronephrosis and diaphragmatic hernias may benefit from intrauterine correction.

B. Fetal Intestinal Obstruction

The fetal gastrointestinal tract (foregut, midgut and hindgut) undergoes ventral folding between 24-28 days' gestation. By the 5-6th wk the stomach rotates to the right and the duodenum occludes by cell proliferation. Recanalization of the duodenum occurs around the 8th wk. The midgut rotation takes place during the 6-11th wk and the final peritoneal closure by 10th wk. The fetal GI tract begins ingestion and absorption of amniotic fluid by the 14th wk. This fluid contributes to 17% effective nutrition; proximally obstructed gut can cause growth retardation. Fetal intestinal obstruction is caused by: failure of recanalization (duodenal atresia), vascular accidents (intestinal atresias), intrauterine volvulus, intussusception, or intraluminal obstruction (meconium ileus). Esophageal obstruction causes polyhydramnios, absent visible stomach and is related to tracheo-esophageal anomalies. Duodenal obstruction seen as two anechoic cystic masses is associated to aneuploidy (trisomy 21) and polyhydramnios. Jejuno-ileal obstruction produces dilated anechoic (fluid-filled) serpentine masses and bowel diameter of 1-2 cm. Large bowel obstruction is most often caused by meconium ileus, Hirschsprung's disease or imperforate anus. The colon assumes a large diameter and the meconium is seen echogenic during sonography. In general the method of delivery is not changed by the intrauterine diagnosis of intestinal obstruction. Timing can be affected if there is evidence of worsening intestinal ischemia (early delivery recommended after fetal lung maturity).