USMLE (United States Medical Licensing Examination): Step 1: Basic sciences assessment Practice Exam 08
Practice exam for USMLE (United States Medical Licensing Examination): Step 1: Basic sciences assessment.
Question 1
MEDIUMA 2-day-old boy is evaluated for bilious vomiting and abdominal distension that began shortly after birth. Pregnancy and delivery were uncomplicated. Physical examination shows abdominal distension and decreased bowel sounds. Abdominal radiograph shows multiple dilated bowel loops with air-fluid levels. A contrast enema demonstrates a narrow distal rectosigmoid segment with a proximally dilated colon. Rectal suction biopsy shows absence of ganglion cells in the submucosal (Meissner) plexus. Which of the following best explains the underlying mechanism of this condition?
This presentation is consistent with Hirschsprung disease: failure of neural crest cell migration leads to aganglionosis of the distal colon, causing functional obstruction and proximal dilation. Failure of recanalization causes intestinal atresia/stenosis, often presenting with vomiting but not aganglionosis. Malrotation predisposes to volvulus and intermittent obstruction, not distal aganglionosis with transition zone on enema.
Question 1 of 100
Community
Loading ranked answers and discussion...