Anorectal Electromanometry in the Diagnosis of Neuronal Intestinal Dysplasia in Childhood

Abstract

The diagnosis of neuronal intestinal dysplasia (NID) is currently established by histochemical procedures. From October 1, 1981, until July 31, 1990, we submitted a total number of 737 patients under 15 years of age with several distinct colonic and anorectal disorders, to clinical, radiological, electromanometrical, histochemical and histological evaluation. 573 had a clinical diagnosis of chronic constipation, and in 38 of these NID could be demonstrated by histochemical examination. Motivated by our own findings with the anorectal electromanometry in these patients we performed a double-blind prospective study to find out if there are any particular and pathognomonic manometric parameters of NID in childhood. 80% of our patients with NID diagnosed prospectively since April 1st, 1986, had a relaxation of the internal anal sphincter which was not proportional to the volume of rectal distention. Anorectal hyperexcitability was also present in these patients, whereas a statistically highly significant (p less than 0.01) increase of the amplitude of anorectal fluctuations (7.27 +/- 1.12 mmHg) as compared to the values measured in our own patients with functional chronic constipation (2.87 +/- 0.33 mmHg) could be demonstrated. Considering only those patients who simultaneously presented all of the above mentioned electromanometric criteria (e.g. non-proportional relaxation of the internal anal sphincter, anorectal hyperexcitability, increased amplitude of anorectal fluctuations) without an increase of the anorectal pressure profile, we could demonstrate that the correlation between the electromanometric diagnosis of NID and the final histochemical diagnosis was 100%. Nevertheless, under these conditions, 30% (9 out of 30) of patients with NID were not recognized electromanometrically because they had been excluded as false negative cases.(ABSTRACT TRUNCATED AT 250 WORDS)