• Poor unresponsive to initial therapy
  
• Abdominal distension
  
• Dilated bowel loops on plain radiography
  
• Suspected neuropathic bowel or pseudo-obstruction

This is a congenital aganglionosis of the Auerbach plexus and Meissner plexus of a variable length of the bowel, 70% have short segment disease, i.e. distal to the descending colon.

• Neonatal-onset constipation, often with features of bowel obstruction
  
• Delayed passage of meconium (>48 hours) in a term infant
  
• Enterocolitis
  
• Severe constipation with onset in the neonatal period and that is unresponsive to standard therapy, e.g. requiring suppositories or enemas