[Histopathological diagnosis of Hirschsprung's disease]

The basis of the histological diagnosis of Hirschsprung's disease (HD) is the demonstration of ganglionic nerve cells in the myenteric plexus of the rectum. The deep rectal biopsy with inclusion of colonic muscle fibres can now be replaced by a quantitative study of the cholinergic fibres in the sub-mucosa, which can be stained histochemically by the acetylcholinesterase technique (ACE). This technique only requires an aspiration biopsy which is innocuous and able to be repeated. Provided the disease is limited to the rectum and in the absence of any malformation syndrome and any previous pelvic operation, this technique is reliable with a 3 to 4 p. cent false negative and false positive rate. This technique therefore has an important place in the diagnosis of HD, together with the clinical signs, radiology and recto-manometric findings. This technique also opens the way for promising histochemical studies of intestinal neuromediators.