The technique of proctocolectomy and formation of an ileal reservoir with ileoanal anastomosis is well described. It is believed that the conservation of a rectal muscular cuff is necessary for continence but no data are available to support this contention. The aims of this study were to describe the clinical and physiological aspects of continence after proctocolectomy and ileal J pouch anastomosis without conservation of a rectal muscular cuff. Eighteen consecutive patients (mean age 37.3 years, 16 ulcerative colitis, two familial polyposis) who underwent proctocolectomy and ileoanal anastomosis on the dentate line were studied 6 months after closure of the loop ileostomy. The 18 patients and eight controls underwent: (a) anal manometry; (b) determination of maximum tolerable volume (MTV); (c) liquid continence test (infusion of NaCl at 60 ml/min for 25 min) with simultaneous measurement of ileal reservoir pressure. The volume evacuated during 5 min after the continence test was also measured. The frequency of bowel actions was (mean +/- s.e.m.) 5.3 +/- 0.4 per 24 h (nocturnal 1.14 +/- 0.26). Seventeen of 18 patients (94 per cent) had normal continence and defaecation; one patient was incontinent. A decrease in resting anal canal pressure (102.5 +/- 4 versus 47.5 +/- 6 cmH2O) was observed after ileoanal anastomosis. A rectoanal inhibitory reflex was elicited in one of the 18 patients (6 per cent). Patients were able to retain 1023 +/- 68 ml saline during the liquid continence test. The percentage evacuation of the ileal reservoir was 61 +/- 4.5 per cent. Correlations were found (P = 0.05) between daily stool frequency and the volume of saline retained during the liquid continence test. It is concluded that conservation of a rectal muscular cuff is not necessary for the achievement of good clinical results.

Evaluation of the rectum by barium enema does not correlate well with rectometrographic studies and is not predictive of bowel function. The purpose of the present study was to describe clinical and functional data in patients with chronic idiopathic constipation, where a megarectum was diagnosed by a rectometrogram. Among 355 patients who underwent rectal elasticity studies, 35 were found to have a megarectum (maximum tolerable volume above 320 ml in women and 440 in men) for which no specific etiology was recognized. They, and a group of 11 healthy controls who were not sensitive to stress, underwent studies of stool frequency, colonic transit time of radiopaque markers, rectal elasticity, and anorectal pressures and reflexes. The elasticity coefficient of the rectal wall was decreased in patients as compared to controls (P less than 0.01). Seven patients had onset of symptoms at birth, with maximum tolerable volume in the rectum between 460 and 900 ml, and all were incontinent for feces. Studies of colonic transit times demonstrated normal function in the right and left colon, but there was rectosigmoid stagnation (transit time of 122 +/- 17 hr vs 8 +/- 2 in stress-free controls; X +/- SE; P less than 0.001). In the other 28 patients (late-onset megarectum), in contrast to the congenital group, there was a marked female preponderance, and their recorded stool frequency (4 +/- 0.7/week) was greater than the recalled frequency (1.4 +/- 0.2/week; P less than 0.001). Only half suffered from fecal incontinence. They did not have a greater rectal capacity when colonic transit times were prolonged (455 +/- 27 ml) than when normal (422 +/- 27). Rectal pressure was similar at the level of conscious sensation of filling, regardless of rectal capacity, suggesting a motor, rather than a sensory, abnormality. The amplitude of the rectoanal inhibitory reflex was decreased (P less than 0.001) as compared to controls, sometimes mimicking the findings of Hirschsprung's disease, but increasing rectal distension always induced a relaxation of the internal anal sphincter. The notion of a megarectum, which tolerates large amounts of fluid without sensation, lacks elasticity, and is accompanied by an abnormal rectoanal inhibitory reflex, provides an explanation for one of the mechanisms of constipation by outlet obstruction.

Dystrophia Myotonica (DM) is a hereditary disease characterized mainly by progressive muscular atrophy and myotonia involving skeletal muscles with progressive muscle wasting and weakness. The electrophysiological feature of the disease is repetitive discharges of striated muscle cell membrane observed after voluntary or induced contractions. Cataract, testiculus atrophy, mental deficiency and fronto-parietal baldness are the more commun extramuscular symptoms of the disease (1, 2, 3). Cardiac muscle is commonly involved and conduction defects are observed. Gastrointestinal smooth muscle may also be involved : oesophagus (4, 5), stomach (6, 7), colon (8) and anal sphincter (9).