Hirschprungs disease

Question 1

What is Hirschprungs disease (RF and Sx)

Answer 1

absence of ganglions in the myenteric (aubebach) and submucossal (meiners’) tissue -begins at rectum and goes up (variable lenght -75% up to sigmoid), ending in normally invervated dilated colon
can lead to dilated/perforated colon, meconium plug syndrome
RF -Downs, MEN2A, male

presents as failure to pass meconium in 24h
dilated abdomen,
bile stain vomiting
explosive exit of stools when happen
can presents a few weeks later with c.diff enterocolitis

Question 2

ix and mx of hirschprungs disease

Answer 2

AXR useful to identify obstructions
contrast barium enema -dilated distal and narrow proxi
definitive is -FULL THICKNESS rectal biopsy =absent ganglion cells

Mx
1st line -bowel irrigation waiting for surgery
then surgery -endorectal pull through - remove part without ganglions and pull rest down and anastamose
other surgeries available
within 1st week even if some surgeons prefer 2-3 months old