BACKGROUND: Hirschsprung's Disease (HD) is a developmental disorder of enteric 
nervous system characterised by the absence of ganglion cells in submucosal 
(Meissner's) and myenteric (Aurbach's) plexuses of distal bowel. The purpose of 
the present study was to observe and report the morphological patterns of 
ganglion related enteric neuronal abnormalities in children presented with 
clinical features of (HD) in a Pakistani population.
METHODS: A total of 92 patients with clinical presentation of HD were enrolled 
between March 2009 and October 2009. Among them, 8 were excluded according to 
the exclusion criteria. After detailed history and physical examination, 
paraffin embedded H and E stained sections were prepared from the serial open 
biopsies from colorectum. The data was analysed using SPSS-17. Frequencies and 
percentages are given for qualitative variables. Non-parametric Binomial 
Chi-Square test was applied to observe within group associations and p<0.05 was 
considered statistically significant.
RESULTS: Among 84 patients, 13 (15.5%) proved to be normally ganglionic whereas 
71 (84.5%) showed ganglion related enteric neuronal abnormalities namely 
isolated hypoganglionosis 9 (12.7%), immaturity of ganglion cells 9 (12.7%), 
isolated hyperganglionosis (IND Type B) 2 (2.8%) and Hirschsprung's disease 51 
(71.8%). Among HD group, 34 (66.7%) belonged to isolated form and 17 (33.3%) 
showed combined ganglion related abnormalities.
CONCLUSIONS: Hirschsprung's disease is common in Pakistani population, followed 
by hypoganglionosis, immaturity of ganglion cells and IND type B. The presence 
of hypertrophic nerve fibres was significant in HD, hyperganglionosis and 
hypoganglionosis, whereas, no hypertrophic nerve fibres were appreciated in 
immaturity of ganglion cell group.