Which of the following is true about Hirschsprung’s Disease?
a) Absence of innervation in the mucosa leads to dilation of the affected areas
b) Autonomic dysfunction leads to constriction of the large intestine
c) Toxic megastomach is a feared complication
d) There is an association between Hirschsprung’s disease and Pierre-Robin sequence
e) It usually does not require surgical intervention
Answer b. Hirschsprung’s disease, or congenital aganglionic megacolon, is a disease of absence of autonomic ganglia in the colon. It may affect a short segment or the entire colon. Importantly, the affected area tends to constrict tightly, leading to development of megacolon (or with bacterial overgrowth, toxic megacolon) in the segment proximal to the affected area without intervention. There is an association between this condition and trisomy 21, Waardenburg syndrome, and other genetic anomalies; there is no known association with the Pierre-Robin sequence.