Congenital Anomalies Flashcards
4 different types. Blind esophagus pouch with fistula between esophagus and trachea most common.
Choking/coughing with feeds, cyanosis, respiratory distress/aspiration
Tracheoesophageal fistula
Suction esophageal pouch with NG tube. Do reflux precautions.
Supportive care and surgical correction
Tracheoesophageal fistula
Extrusion of intestine through defect
Gastroschisis
Membrane covered herniation of abdominal contents
Omphalocele
Associated anomalies are common with
Omphalocele
Associated anomalies are rare with
Gastroschisis
Cover abdominal contents with
Moist sterile dressing
Do NG decompression, antibiotics, supportive care, and stabilization by primary closure or staged closure
Abdominal wall defects
With Down syndrome, look for characteristic “double bubble” sign of
Duodenal atresia
Abdominal distention, bilious vomiting, obstipation(failure to pass gas), failure to pass meconium, polyhydramnios
Intestinal atresia (leading to obstruction)
Absence of ganglion cells in the colon leading to narrowing of the aganglionic segment with dilation of the proximal normal colon
Hirschsprung’s
Presents in infancy or first 2 years of life.
Failure to pass meconium, vomiting, abdominal distention, chronic constipation.
Barium enema shows region of marked dilation superior to aganglionic segment
Hirchsprung’s disease
Diagnosed by RECTAL SUCTION BIOPSY at anal verge to look for ganglion cells
Hirschsprung’s disease
Staged procedure > initial diverting colostomy followed by resection with infant is > 6 months old
Hirschsprung’s
Associated with amniotic fluid alpha-fetoprotein
Neural tube defects