Long term outcome of Hirscuprungs disease in CWMH

Hirschsprung's disease (HD) is characterised by an absence of ganglion cells in the distal bowel and extending proximally for varying distances. The pathophysiology of HD is not fully understood. The aganglionosis is confined to rectosigmoid in 75% of patients, sigmoid, splenic flexure or transverse colon in 15% and total colon along with a short segment of terminal ileum in <IO%. World wide the incidence of HD is estimated to be 1 in 5000 live births. The disease is more common in boys with a male-to-female ratio of 4 : 1. The male prevalence is less evident in long-segment HDi .
The majorities of cases of HD, 80—90% produce clinical symptoms and are diagnosed during the neonatal period. Delayed passage of meconium is one of the important symptoms in neonates with HD. Over 90% of affected patients fail to pass meconium in the first 24 h of life. The symptoms at presentation of HD in the neonatal period are with constipation, abdominal distension and vomiting during the first few days of life. Less than a third of the babies with HD present with diarrhea. Diarrhea in HD is always a symptom of enterocolitis, which remains the as one of commonest cause of mortality in HD ii.
The diagnosis of HD is based on clinical history, radiological studies, anorectal manometry and histological examination of the rectal wall biopsy specimens. It is essential that the infant should not have rectal washouts or even digital examinations prior to contrast enema, to reduce the possibility of false negative diagnosis. A typical case of HD will demonstrate flow of barium from the undilated rectum through a cone-shaped transitional zone into dilated colon.
The diagnosis of HD is confirmed on examination of rectal biopsy specimens. The introduction of histochemical staining technique for the detection of acetylcholinestrase activity in suction rectal biopsy has resulted in a reliable and simple method for the diagnosis of HD. Full thickness rectal biopsy is rarely indicated for the diagnosis of HDlli