Pediatric Gastrointestinal Anomalies

Questions and Answers

Stridor

Diaphragmatic hernia

Upper gastrointestinal series

All of the above Signup and view all the answers

Both A and B Signup and view all the answers

Cyst resection Signup and view all the answers

A right-sided ligament of Treitz Signup and view all the answers

Abdominal x-ray Signup and view all the answers

Beckwith-Wiedemann syndrome Signup and view all the answers

Regular abdominal ultrasound and alpha-fetoprotein level testing Signup and view all the answers

Study Notes

Vascular rings encircle the trachea and/or esophagus, causing compression.
Compression of the trachea presents with stridor.
Compression of the esophagus presents with dysphagia, vomiting, or recurrent food impactions.

Intestinal malrotation is a common cause of bilious emesis in infants, especially those with other congenital anomalies.
Chronic, episodic symptoms occur with intermittent volvulus.
Malrotation with midgut volvulus presents with bilious vomiting in neonates.
Abdominal x-ray may be normal, so an upper gastrointestinal series is required for diagnosis.
The upper gastrointestinal series shows a right-sided ligament of Treitz and a corkscrew duodenum.

Beckwith-Wiedemann syndrome (BWS) is characterized by macrosomia, macroglossia, hemihyperplasia, umbilical hernia/omphalocele, and neonatal hypoglycemia.
Patients with BWS are at increased risk of developing Wilms tumor and hepatoblastoma.
Abdominal ultrasound and alpha-fetoprotein level testing are recommended for patients with BWS.

Biliary cysts may be asymptomatic or present with abdominal pain, a right upper quadrant mass, and/or jaundice.
Cyst resection is necessary to decrease the risk for malignancy.
Biliary cysts can initially present with complications, such as cholestasis and pancreatitis due to distal biliary obstruction.
A biliary cyst should be suspected in a neonate with cholestasis and a mass in the right upper quadrant.

Cholangiocarcinoma is a biliary tract epithelial malignancy that often occurs in those with fibropolycystic liver disease or primary sclerosing cholangitis due to underlying ulcerative colitis.
Most cases present with subacute right upper quadrant pain, weight loss, and signs of biliary obstruction, such as jaundice, cholestatic liver enzyme pattern, and dilation of the intrahepatic or common bile duct.