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)
From October 1, 1981, until December 31, 1991, we performed clinical, radiological, electromanometrical, histochemical and histological diagnostic procedures in 906 patients under 15 years of age, because of several distinct colonic and anorectal disorders. 739 were clinically constipated and in 272 of them hypertonicity of the internal anal sphincter with or without achalasia could be demonstrated by manometry. 121 of these patients were submitted to a posterior, transanal, partially resective internal sphincteromyomectomy, according to a slightly modified classical procedure. Follow-up of our operated patients ranged from 1 month to 9 years, with a mean of 3 years and 2 months and overall satisfactory results in 87.6% based on clinical evaluation. Additionally, 55.4% of the operated patients had early postoperative manometric controls (n = 67) and 77.6% of these (n = 52) accepted a third manometry, in order to evaluate late results of our procedure. We could demonstrate that early postoperative anorectal electromanometry shows a highly significant decrease of the internal anal sphincter pressure correlating with a clinical improvement in 98.5% of these patients (n = 67). Most late postoperative examinations showed recurrent elevation of the manometric internal sphincter pressure parameters, exceeding the mean values of our own normal controls. Nevertheless, clinically 98.1% of these patients had a persistent satisfactory remission of their symptoms, thus not correlating with the manometric findings in these cases. We conclude, that transanal internal sphincter myomectomy is a safe surgical procedure, which leads to satisfactory results in approximately 90% of chronically constipated children with anal sphincter hypertonicity, if very precise indications for operation are considered.
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