Hirschsprung's Disease: a Clinical and Pathologic Study in Iranian Constipated Children

Objective: Hirschsprung's disease (HD) is a complex disorder resulting from absence of ganglion cells in the bowel wall leading to functional obstruction and bowel dilatation proximal to the affected segment. The aim of our study was to evaluate rectal biopsies from constipated children in different age groups to see in which age it is more likely to encounter HD to avoid unnecessary rectal biopsy.
Methods: Records of all children with chronic constipation undergoing a rectal biopsy to exclude HD were obtained from the files of Children's Medical Center in Tehran, Iran. A detailed retrospective demographic review, including age of beginning of signs and symptoms was made of all cases.
Findings: Totally, 172 biopsies were taken from 168 children in a five year period, of which 127 cases (75%) had HD. The mean age of constipated patients at biopsy was 39 months and the mean age of patients with proven HD was 18 months. Males were affected more than females. Congenital anomalies associated with HD were found in 9.6%. In 85 (91%) cases constipation had begun in neonatal period.
Conclusion: Our data supports previous studies that if constipation begins after the neonatal period, the child is unlikely to have HD. In neonates delay in meconium passage is the most important clinical sign of HD.

Keywords
Hirschsprung's Disease; Functional Gastrointestinal Disorders; Constipation; Biopsy; Intestinal Obstruction