She passed some meconium on the first day of life. After 24 hrs of life she developed hypochloremia. The UGIS shows a dilated stomach, a normal antrum with a cap and faint insinuation of a pyloric canal. Baby boy born at term with the adjacent abdominal wall defect. CT Scan shows a cystic mass behind the stomach.
Ganglion cells are present on suction rectal biopsy.
A 13 year-old female presented with primary amenorrhea, low abdominal pain and a palpable movable pelvic mass. CT shows multiple cystic cavities in the pelvis. Simple abdominal films demonstrate multiple dilated small bowel loops. At surgery there is a transitional zone 23 cm proximal to ileocecal valve with the proximal dilated ileum filled with tenacious neconium and distal meconium pellets. Simple abdominal film is included.