Hirchprung’s disease –
Correct Answer: Absence of ganglia in involved segement
Description: Answer 'b' i.e. Absence of ganglia in the involved segment In Hirchprung's disease the absence of ganglion cells gives rise to a contracted non peristaltic segment with a dilated hyperophied 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. Schwaz and (9/e p1056) Bailey (24/e, p 1154) write that Hirschprung's ds. can present in adults too. Schwaz writes- "Although Hirschsprung's disease is primarily a disease of infants and children, it occasionally presents later in adulthood, especially if an extremely sho segment of the bowel is affected (ultrasho-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 - oave
Category:
Surgery
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