ABNORMAL DISTRIBUTION OF NERVE-TERMINALS IN THE NORMOGANGLIONIC BOWELOF HIRSCHSPRUNGS-DISEASE - A CAUSATIVE FACTOR OF FAILED PULL-THROUGH OPERATIONS

The aim of this study was to examine the innervation pattern of the mu scle layers of normoganglionic pulled-through bowel in patients with H irschsprung's disease (HD) by observing the distribution of nerve term inals comprising neuromuscular junctions and synapses. As a marker for nerve terminals, monoclonal antibody (MAb) 171B5 was used. Pulled-thr ough normoganglionic bowel from 12 patients with HD that was either bi opsied or resected at operation and normal bowel specimens from 7 age- matched controls were labelled using an immuno-histochemical technique with MAb 171B5. In all specimens of the control group and 9 patients of the HD group, numerous neuromuscular junctions were demonstrated in the muscle layers of the normoganglionic bowel and numerous synapses in the myenteric plexuses. However, in the specimens of 3 patients of the HD group abnormal distribution of nerve terminals was seen in the normoganglionic bowel. Two of these 3 patients had abnormal postoperat ive defecation. Based on our study, there appears to be a small number of HD patients who have an innervation disorder of the muscle layers in the proximal, normoganglionic bowel pulled through at surgery, whic h hitherto have been thought to be normal.