Hirchprung's disease -a) Is seen in infants and children onlyb) Absence of ganglia in involved segementc) The involved segment is the dilated colond) Bleeding PR is a presenting featuree) Surgery is used in therapy

In Hirchprung's disease the absence of ganglion cells gives rise to a contracted non peristaltic segment with a dilated hypertrophied segment of normal colon above it. Almost all cases present in infancy and childhood. Approximately half are diagnosed in the neonatal period and most of the remainder are diagnosed in early childhood. Schwartz and (9/e p1056) Bailey (24/e, p 1154) write that Hirschprung's ds. can present in adults too. Schwartz writes- "Although Hirschsprung's disease is primarily a disease of infants and children, it occasionally presents later in adulthood, especially if an extremely short segment of the bowel is affected (ultrashort-segment Hirschsprung's disease)." In the newborn period, most common symptoms are abdominal distention and failure to pass meconium. Older patients may experience chronic or intermittent constipation. Occasionally, infants may present with a dramatic complication of Hirschprung's disease called enterocolitis. Surgery is always required. The basic principle is removal of most or all of the aganglionic segment and anastomosing the ganglionic bowel to the rectum Operations done are - Swenson - Duhamel - Soave