Saturday, 24 June 2017

HIRSCHSPRUNG'S DISEASE

The cause is now considered to be a failure of development of ganglion cells in the intramural plexus. This produces a neuromuscular block to the transmission of a peristaltic wave along the colon and the affected segment remains narrow, while the proximial segment becomes enormously dilated and hypertrophied in an attempt to overcome the block.
Symptoms in such cases date from birth and the passage of the first meconium stools is delayed. Constipation is either progressive or occurs in episodes associated with signs of intestinal obstruction. There is progressive abdominal enlargement with faecal and gaseous distension of the colon. Digital examination of the rectum reveals no abnormality and passage is empty. Radiographic examination demonstrates the lower, narrowed segment of rectum and colon, while above it there is enormous dilatation. The diagnosis can be confirmed by establishing the absence of ganglion cells in a biopsy specimen removed through a sigmoidoscope.
Treatment
Treatment is surgical and the abnormal segment is excised. This allows normal movement of the propulsive wave to occur and the colon can be emptied. If the symptoms of obstruction develop at birth, it may be necessary to do a colostomy. The prognosis is excellent.

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