When to suspect that the newborn has got Hirschsprung’s disease and how to diagnose? What are the procedures for treatment?
• Congenital megacolon
• Colonic aganglionosis
In Hirschsprung’s Disease network of nerves which controls the regular contractions is lacking in the large intestine. It affects about 1 in 5000 newborns. Contractions of large intestine depend on a network of nerves within its wall which produces regular rhythmic contractions in the large intestine to propel the contents of intestine towards the anus which is expelled outside as feces. In Hirschsprung’s disease, the affected part of the intestine cannot contract in a normal fashion. Intestinal contents get collected just proximal to the abnormal part of intestine resulting in enlargement of that part; the so-called megacolon. As the disease is congenital, it is referred as congenital megacolon. The child presents with symptoms of intestinal obstruction.
Typical symptoms are:
• Delayed passage of meconium in infants
• Refusal to feed
• Swollen and distended abdomen
All newborns should pass dark green color feces soon after birth. If the baby has not passed meconium within 48 hours after birth, Hirschsprung’s disease should be suspected. Later on, the child may present with symptoms of intestinal obstruction like bile stained vomiting, refusal to eat, distended abdomen.
Diagnosis is done by
• Rectal biopsy
• Measurement of pressure inside rectum (manometry)
• Barium enema
Treatment: Treatment is done by surgery
The simplest type of surgery is a removal of the abnormal part of the intestine and connecting the anus with normal intestine. In a seriously ill child, abnormal part of the intestine is disconnected and the lower end of the normal intestine is connected to the abdominal wall (a procedure called colostomy) to collect the stool in a bag. When the child is healthy, the colostomy is closed and abnormal part is removed and normal part is connected again to rectum and anus. This procedure is called pull-through procedure.
Severe disease may lead to toxic enterocolitis which is a life-threatening condition. It must be treated promptly.