• Nausheen Henna
  • Abdul H Nagi
  • Muhammad A Sheikh
  • Mahmood Shaukat


Background: Hirschsprung’s Disease (HD) is a developmental disorder of enteric nervous systemcharacterised 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 morphologicalpatterns 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 wereenrolled between March 2009 and October 2009. Among them, 8 were excluded according to theexclusion criteria. After detailed history and physical examination, paraffin embedded H and E stainedsections were prepared from the serial open biopsies from colorectum. The data was analysed usingSPSS-17. Frequencies and percentages are given for qualitative variables. Non-parametric BinomialChi-Square test was applied to observe within group associations and p<0.05 was consideredstatistically significant. Results: Among 84 patients, 13 (15.5%) proved to be normally ganglionicwhereas 71 (84.5%) showed ganglion related enteric neuronal abnormalities namely isolatedhypoganglionosis 9 (12.7%), immaturity of ganglion cells 9 (12.7%), isolated hyperganglionosis (INDType B) 2 (2.8%) and Hirschsprung’s disease 51 (71.8%). Among HD group, 34 (66.7%) belonged toisolated form and 17 (33.3%) showed combined ganglion related abnormalities. Conclusions:Hirschsprung’s disease is common in Pakistani population, followed by hypoganglionosis, immaturityof 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 inimmaturity of ganglion cell group.Keywords: Hirschsprung’s disease (HD), hypoganglionosis, immaturity of ganglion cells (IGC),intestinal neuronal dysplasia (IND)


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