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Paediatrics: Hirschsprung’s disease

The incidence of HSD is 1/5000 live births. It may be familial and associated with trisomy 21.

Hirschsprung’s disease

 

The incidence of HSD is 1/5000 live births. It may be familial and associated with trisomy 21.

   It is caused by a failure of ganglion cells to migrate into the hindgut.

   This defect leads to an absence of co-ordinated bowel peristalsis and functional intestinal obstruction at the junction (‘transition zone’) between normal bowel and the distal aganglionic bowel.

   In 80% of cases the transition zone is in the rectum or sigmoid—short segment disease.

   In 20% of cases the entire colon is involved—long segment disease.

   Occasionally, children with short segment disease present in childhood with chronic constipation.

 

Diagnosis

 

   Usually presents within the first few days of life with low intestinal obstruction, i.e. failure to pass meconium, abdominal distension, and bile-stained vomiting. 99% of normal newborns pass meconium within 24hr of delivery.

 

   AXR: distal intestinal obstruction.

 

   Rectal biopsy: no ganglion cells in the submucosa.

 

Surgical treatment

 

Many surgeons now perform a single stage pull-through in the neonatal period, managing initial intestinal obstruction with rectal washouts, but traditionally a 3-stage procedure is used.

 

   Defunctioning colostomy, with multiple biopsies to confirm the site of the transition zone.

 

   Pull-through procedure to bring ganglionic bowel down to the anus.

 

   Closure of colostomy.

 

Outcome

 

   The long-term results are generally satisfactory with approximately 75% of children acquiring normal bowel control, 15–20% partial control, and 5% who never gain control and may end up with a permanent stoma.

 

Most important complication of HSD is enterocolitis. A dramatic gastroenteritic illness characterized by abdominal distension, bloody watery diarrhoea, circulatory collapse, and septicaemia. Condition usually associated with Clostridium difficile toxin in the stools. Mortality 710%.

 

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