A neonate develops bile-stained vomiting and progressive abdominal distention, and does not pass meconium over the first two days of life. The anus is patent, and the bowel loops are palpable. Plain radiograph shows bubbly meconium in the right lower quadrant. No localized areas of constriction or other abnormalities are noted. Which of the following is most likely etiologically related to this infant's condition? A. Cystic fibrosis
B. Hirschsprung's disease
C. Meckel's diverticulum
D. Omphalocele
E. Polycystic kidney disease
b?ie agangliosis in colon.
it is A ...this is meconium ileus asso with cystic fibrosis
The correct answer is A. The baby has meconium ileus, which is a manifestation of cystic fibrosis due to the abnormally viscid pancreatic secretions which "get stuck" in the small bowel. Meconium ileus can cause gut perforation with peritonitis and intraperitoneal calcifications (that may be visible on plain film). Meconium ileus complicated by intestinal perforation or formation of fistulas to the bladder or vagina must be treated surgically. Medical treatments for uncomplicated meconium ileus are now available, which use enemas, mucolytic agents, or pancreatic enzymes.
How to differentiate it from B?
In Hirschsprung's disease, distal to the aganlionic area, there will be constriction. "No localized areas of constriction or other abnormalities are noted. " doesn't fit Hirschsprung's disease.