J Med Assoc Thai 2002; 85 (2):250

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Congenital Megacolon: Diagnosis Using Size of Submucosal Nerve Trunk as a Criteria
Sangkhathat S Mail, Patrapinyokul S , Tadtayathikom K

Cholinergic hyperinnervation in submucosa of aganglionic segment of Hirschsprung's
disease has been described. However, objective measurement of the nerve fibers was insufficiently
Objectives : To study the amount and size of hypertrophic submucosal nerve trunks in
the aganglionic segment of Hirschsprung's disease, semiquantitatively and evaluate the possibility
of using the size as objective diagnostic criteria.
Material and Method :Thirteen specimens from aganglionic segments from Hirschsprung's
disease and six specimens from the age-matched control subjected without colonic innervation dis-
orders were studied with the S-100 immunohistochemical technic. The submucosal nerve trunks
were counted per ten HPF and measured in width, semiquantitatively. Fiber sizes were stratified into
small (40
micrometers). Maximum fiber
size in each specimen was also recorded.
Results : Average density of submucosal nerve trunk in the aganglionic segment and the
control were 12.5 and 3.4 fibers per ten HPF, respectively. The large nerve trunks were detected in
12 of 13 aganglionic specimens and none was detected in the controls. The maximum fiber size in
aganglionic segments was 78.2 micrometers, compared to 24.1 micrometers in the control group. The
sensitivity and specificity* to set the presence of hypertrophic nerve trunk, larger than 40 micrometers
as a diagnostic criteria for Hirschsprung's disease were 92.3 and 100 per cent, respectively. The data
suggest the use of this size as an objective diagnostic criterion, which may be valuable in rectal
suction biopsy specimens.
Key word : Hirschsprung's Disease, Enteric Nervous System

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