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The standard surgical therapy for severe idiopathic constipation is total colectomy with ileorectal anastomosis, but this results in intractable diarrhoea in a third of the patients and recurrent constipation in another 10%. Studies which employ either radio-isotopes or radio-opaque markers permit the delineation of regional delay in colonic transit. Based on these studies, and evidence that the rectum is also abnormal in these patients, we have performed a left hemicolectomy with rectal excision in 2 patients with proven left colonic delay. After 2 and 3 years of follow-up, both patients have normal frequency, transit studies and anorectal physiology studies. Segmental resection based on physiological studies may offer better relief of symptoms with a lower chance of side effects in selected patients.

1. A sucrose-gap technique was used to investigate the neuromodulatory actions of enkephalins on non-adrenergic, non-cholinergic inhibitory junction potentials (IJPs) in the circular muscle of the human large intestine. 2. The native enkephalins, [Leu5]enkephalin (LENK) and [Met5]enkephalin (MENK) caused a concentration-dependent reduction in amplitude of IJPs without a significant effect on the smooth muscle membrane. 3. The actions of LENK and MENK were mimicked by the delta-selective opioid receptor agonists [D-Pen2, D-Pen5]enkephalin (DPDPE) and [D-Ala2, D-Leu5]enkephalin (DADLE). 4. The actions of LENK, MENK and DPDPE were antagonized to similar extents by the delta-selective opioid receptor antagonist ICI 174,864. 5. The mu-selective opioid receptor agonist [D-Ala2, Me Phe, Gly-ol5]enkephalin was approximately 100-fold less potent than any of the native or synthetic enkephalins at reducing the amplitude of the IJP. Dynorphin A and beta-endorphin both had very weak activity. 6. Responses to all of the agonists were inhibited by naloxone. The degree of antagonism of DPDPE or DADLE by naloxone (1 microM) was the same as that of LENK or MENK. 7. Neither MENK nor LENK affected hyperpolarization of the smooth muscle membrane induced by ATP or 5-hydroxytryptamine. Vasoactive intestinal polypeptide (1 pM-1 microM) did not produce any observable responses and this lack of reactivity was not affected by the enkephalins. 8. It is concluded that in the circular muscle of the human colon, LENK and MENK can act on prejunctional delta-opioid receptors to produce inhibition of non-adrenergic, non-cholinergic inhibitory neuromuscular transmission. Possible physiological significance of this prejunctional receptor is discussed.

The distribution in the bowel wall of vasoactive intestinal polypeptide-, neuropeptide Y-, and substance P-containing nerve cell bodies and nerve fibers has been described in human sigmoid colon by immunohistochemical examination. In patients with chronic idiopathic constipation, diverticular disease, and in controls (of tissue taken from patients with carcinoma, from a site distant from the tumor that appeared macroscopically normal), the concentrations of vasoactive intestinal polypeptide, neuropeptide Y, and substance P have been measured by immunoassay in the following preparations of sigmoid colon: mucosa, whole colonic wall with mucosa dissected away, circular muscle, and taenia coli. In idiopathic constipation, the vasoactive intestinal polypeptide content of the whole wall minus mucosa was reduced when compared with controls (P less than 0.05) but was unaltered in the mucosa, circular muscle, and taenia coli. In diverticular disease, the vasoactive intestinal polypeptide content of the mucosa and whole wall minus the mucosal layer was increased when compared with control tissue (P less than 0.05 and P less than 0.02, respectively) but was unaltered in the circular muscle and taenia coli. Substance P and neuropeptide Y levels in all layers of colonic wall were unaltered in these two diseases. The disturbances in the normal neural content of vasoactive intestinal polypeptide in the bowel wall in idiopathic constipation and diverticular disease may initiate or contribute to the functional changes seen in these disorders.

Patients with extensive ulcerative colitis who do not need early surgery have been offered regular examination with the aim of detecting precancerous change (dysplasia) or early colorectal carcinoma. Outpatient visits with clinical examination, sigmoidoscopy, and biopsy were supplemented by two-yearly colonoscopy after the disease course reached 10 years. During the 22 year period from the beginning of 1966 to the end of 1987, 401 patients entered the programme and together contributed 4048 patient-years of observation. Apart from nine patients who left the country, follow up is complete until 1986 or 1987. Colorectal carcinoma developed in 22 patients and, in a further 12, biopsy evidence of precancer, described as severe/high grade dysplasia, was confirmed in a colectomy specimen. The cumulative probability of developing carcinoma was 3% at 15 years, 5% at 20 years, and 9% at 25 years; corresponding figures for precancer or carcinoma, or both were 4%, 7%, and 13%. Five patients died of colorectal carcinoma, two while under regular observation and three after developing carcinoma four to six years after their last attendance. Among the 17 patients who developed carcinoma while under observation, the Dukes stage was A or B in 12. Patients with extensive colitis whose disability does not warrant early surgery have a clinically important cancer risk after the disease has been present for 10 years. Our results suggest that follow up in the manner described reduces the mortality from this complication. Further work is needed to define the optimum method of surveillance and show if it is cost effective.

Long-term parenteral nutrition requires central venous access, often difficult in patients who have had several central venous catheterizations. Therapy may be complicated by thrombosis and sepsis which may further compromise central access. We report five cases illustrating such difficulties and suggest that these patients be referred early to specialist centres where experienced catheter insertion and management results in a greatly reduced incidence of complications.

Rectal sensation is usually assessed using balloon distention. The authors describe a more precise technique that avoids the variables of balloon dynamics and rectal diameter and compliance. Using both methods, rectal sensation was assessed in 13 healthy control women and 26 women with severe idiopathic constipation. Balloon distention in the rectum revealed an elevated sensory threshold (16.9 +/- 4.4 vs. 30.4 +/- 3.1 ml air, controls vs. patients, P = 0.018) and volume required to elicit a call to stool (61.1 +/- 9.1 vs. 97.5 +/- 6.4, P = 0.003) in subjects with severe constipation. The maximum tolerated volume was similar in the two groups. Rectal mucosal electrosensitivity was then tested using a bipolar ring electrode supplying a constant current. This demonstrated an elevated sensory threshold in the constipated subjects (16.3 +/- 3.0 vs. 27.4 +/- 2.1 mAmps, P = 0.005). There was a significant correlation between the sensation thresholds for balloon distention and electrical stimulation for the entire subject group (r = 0.39, P = 0.01). Two other patients with severe constipation after suspected extrinsic nerve damage were also studied and demonstrated a markedly abnormal electrical sensory threshold. Electrical testing avoided the variables inherent in balloon distention and was well tolerated, accurately quantifiable, and reproducible. The raised threshold to electrosensory mucosal testing suggests the presence of a rectal sensory neuropathy in patients with severe idiopathic constipation.

The distribution of serotonin and dopamine beta-hydroxylase was examined in sigmoid colon specimens from patients with severe idiopathic constipation and control patients with carcinoma of the rectum or colon. Specimens were divided into three regions: (a) the mucosa; (b) the myenteric and submucosal plexuses with the longitudinal and circular smooth muscles; and (c) the circular smooth muscle, for biochemical analysis of serotonin and 5-hydroxyindoleacetic acid (total indoles) and noradrenaline. In both groups of patients, serotonin- and dopamine beta-hydroxylase-like immunoreactivity was localized in nerves in the myenteric and submucosal plexuses, and a sparse innervation was observed in the circular muscle. In addition, intense serotonin-like fluorescence was present in a large number of enterochromaffin cells in the mucosa. Total indole levels were significantly increased in the mucosa (p less than 0.02) and circular muscle (p less than 0.05) of the constipated patients. In contrast, no changes in noradrenaline levels were observed in any of the regions studied. Altered levels of total indoles may thus contribute to severe idiopathic constipation. Analysis of biopsy specimens could be a useful tool in clinical diagnosis and future investigations of diseases of the gut.

Visible changes associated with dysplasia or carcinoma have been assessed in 62 colectomy specimens from patients with ulcerative colitis. In 34 colectomy specimens with one or more carcinomas, there were 51 visible carcinomas and 65 macroscopic dysplastic lesions; only one carcinoma was found in flat mucosa. Among 28 specimens with microscopic dysplasia but no carcinoma, eight contained no visible dysplastic lesion, and the remaining 20 contained a total of 40 polypoid or elevated dysplastic areas. In 32 operative specimens removed for long-standing chronic colitis without dysplasia or carcinoma, nine contained visible inflammatory polypoid lesions. On radiological or endoscopic examination of the colon in long-standing colitis close attention should be given to the identification and biopsy of polyps, elevated nodular areas or plaques, strictures, and isolated ulcers. It is likely that colectomy specimens with visible polypoid or elevated dysplastic lesions will contain a carcinoma more often than those removed for dysplasia in flat mucosa.

A double-blind crossover trial compared prednisolone, 40 mg given orally on alternate days, with placebo as a maintenance treatment for ulcerative colitis in remission. In each patient, the study was over two periods of 3 months. Of 24 patients who completed both periods, 11 relapsed while taking placebo but not while taking prednisolone, and 1 relapsed on prednisolone but did not on placebo (p less than 0.01). 1 patient had to stop prednisolone because of hyperglycaemia; other side effects noted were mild. Prednisolone, cautiously used in this way, could be justified for the few patients who relapse frequently despite sulphasalazine.

Seven patients with widespread, active, stricturing, small intestinal Crohn's disease and two with localized disease were treated by a total of 45 strictureplasties. They have been followed up for a mean of 20 months (range, 6 to 30). Two patients had early postoperative complications with enterocutaneous fistulas, one of which may have been related to a strictureplasty. The two patients with localized disease remain well after 16 and 30 months. Of the seven patients with extensive small bowel disease, two are well six and 28 months after surgery. Recurrent symptoms developed in six patients two to six months postoperatively; four of those patients required further surgery. Previous reports of strictureplasty in inactive Crohn's strictures suggest it is a safe operation with good long-term results. Strictureplasty in active Crohn's disease has a much higher recurrence rate of symptoms. Because it is a conservative operation, however, we believe it has a place in the surgical treatment of Crohn's disease.

This study describes the clinical features and management of 80 patients with Crohn's disease affecting initially only the rectum with or without involvement of the sigmoid colon. Most patients were treated medically, and the various measures used are considered; the cumulative probability of completing five years under conservative management was 60% among survivors. The related mortality was very low and confined to patients treated surgically; with 655.24 patient-years of follow-up the number of observed deaths was lower than the number expected from all causes. Since this distribution of the disease is usually seen in older patients and carries a favourable prognosis, it it suggested that it should be considered as a well-defined variant of the condition.

The cancer risk among 229 patients with extensive ulcerative colitis observed during January 1, 1966 to February 29, 1976 is correlated with the length of history and the histological findings in rectal and colonic biopsies. Five patients are known to have developed carcinoma. No carcinoma was observed in 578 patient years of follow-up within 10 years of onset of the colitis, but the risk in the second decade was approximately 1 in 200 patient years and in the third, 1 in 60 patient years. Severe epithelial dysplasia was rare and found in 32 biopsies from 13 patients. No carcinoma has occurred during the period of follow-up in patients without dysplasia. It has not been possible to follow the development of dysplasia in sequential biopsies. Seven patients with consistent severe dysplasia on biopsy have been treated surgically; carcinoma confined to the bowel wall (Dukes' A) was found in 4. A scheme of management for patients with extensive colitis, including regular rectal and colonic biopsies, is proposed. Our results suggest that such a program will isolate a small group of patients who require surgical treatment for established precancerous change or carcinoma with a high likelihood of cure.

Sulfasalazine appears to exert its beneficial effect in colitis by releasing 5-aminosalicylic acid in the colon, but its use can be limited by side effects. Ipsalazide and balsalazide are novel sulfasalazine analogs designed to release 5-aminosalicylic acid and a nontoxic carrier molecule in the gastrointestinal tract. They have a low oral toxicity following single or repeat administration to mouse, rat, and ferret, and balsalazide is not mutagenic in the Ames test. Ipsalazide and balsalazide are split in rat and man, and the urinary and fecal excretion pattern of the 5-aminosalicylic acid released is similar to that of sulfasalazine; the carrier molecules are absorbed to a lesser extent than the sulfapyridine derived from sulfasalazine. These two analogs deserve therapeutic trial.

There is doubt about the timing of surgery for patients with internal fistulas in Crohn's disease. Although immediate operative intervention for all patients has been advocated, such a policy has not always been followed at St. Mark's Hospital. Between 1971 and 1982, 83 internal fistulas were identified in 59 patients with Crohn's disease. Fifty-nine fistulas arose primarily from the small intestine and involved another segment of the bowel, five were between large bowel and duodenum, and three between areas of large bowel. Sixteen fistulas (ten from ileum and six from large bowel) involved the bladder. Thirty-six patients with 54 fistulas underwent immediate surgical treatment. Fifteen patients with 20 fistulas required surgery later. There was one postoperative death among the 51 patients treated surgically and one late death unrelated to the treatment of the fistula. Of the remaining 49 surgically treated patients, 46 were traced and remain well, six after further surgery. Eight patients with nine fistulas (four involving the bladder) were treated at St. Mark's without operation. One later required surgery elsewhere for an enterocutaneous fistula, but the remaining seven patients are well. This study suggests that the presence of an internal fistula, even if it involves the bladder, is not an absolute indication for immediate surgery and that the severity of the symptoms should dictate the treatment policy.

Severe idiopathic constipation affects predominantly young women. We have evaluated the results of colectomy in 44 women with a normal diameter colon and with a median bowel frequency of once per four weeks. The operations were done in one hospital over a 15 year period. After operation 22 patients had normal bowel frequency, 17 diarrhoea (greater than three bowel actions/day), and five persistent or recurrent constipation (interval between bowel actions at least three days). Many patients continued to experience abdominal pain (98% preoperatively v 71% postoperatively); straining and laxative use were still common after surgery. Ten patients needed psychiatric treatment for severe psychological disorders. The preoperative abnormalities of paradoxical contraction of the pelvic floor during straining and impaired rectal evacuation of a water filled balloon did not correlate with the outcome.

In a prospective, randomised clinical trial, 47 patients with severe, acute, non-infective colitis treated with 60 mg intravenous prednisolone daily, received either bowel rest with parenteral nutrition or oral diet. Although those who received 'bowel rest' experienced a reduction in daily stool weight, there were no differences in the operation or mortality rates between the groups. Fourteen of the 27 patients with ulcerative colitis, but none of the 16 patients with Crohn's disease required urgent surgery. Bowel rest did not affect the outcome in severe ulcerative colitis treated with intravenous prednisolone. Ulcerative colitis and Crohn's colitis behaved differently in the acute attack.

Energy absorption from a liquid test meal, intestinal transit rate and water and sodium output over a six hour period were measured in five patients with an ileostomy and 12 patients with the short bowel syndrome, five of whom were on longterm parenteral nutrition. The proportion of total energy absorbed was greatest in the ileostomists (median 87%, range 82-92%), less in short bowel patients not on parenteral nutrition (median 67%, range 59-78%, p less than 0.01) and least in the short bowel group who needed it (median 27%, range 2-63%, p less than 0.01). Transit rate was more rapid in the short bowel patients compared with the ileostomists. A close correlation was observed between percentage energy absorption and the dry weight of the stools/stoma effluent collected during the six hour test period (r = -0.99, p less than 0.001). This simple non-invasive test quantitates the degree of intestinal failure and may be of practical help in management.

Twenty-one women with slow-transit constipation have been treated by bowel resection. All had a normal barium enema, but an increased colonic transit time. Pathological examination of the resected colons showed no evidence of aganglionosis. Some of the patients were treated by segmental colonic resection, anorectal myectomy, anal stretch or internal sphincterectomy but were not helped. Colectomy with ileorectal or caecorectal anastomosis gave the best results; on balance, ileorectal anastomosis seems preferable. Of sixteen patients who underwent colectomy, ten subsequently had normal bowel function and four were markedly improved. The spontaneous bowel frequency in this group rose from 0·3 ± 0·1 (s.e.m.) to 21·7 ± 8·3 weekly (P < 0·001).

Evacuation proctography is a dynamic investigation of rectal expulsion that records the voluntary evacuation of thick barium paste on videotape. Evacuation is a passive phenomenon in a defined zone of the rectum, associated with pelvic floor descent of 3 cm from a resting position of the anorectal junction less than 2 cm above the plane of the ischial tuberosities. The anal canal does not open immediately; it takes about 4.5 sec to open to a maximum diameter of 1.5 cm, with rectal emptying in 11 sec. Anterior rectoceles commonly invert over the anal canal as the rectum collapses in at the end of evacuation.

A technique is described in which a chemical stimulus applied to the mucosa of the right colon is used to assess colonic motor function. Peroral intubation of the right colon was achieved using a fine polyvinylchloride (PVC) tube. Bisacodyl was used to initiate colonic motor activity, and colonic transit was monitored using 99mTc-DPTA and a gamma camera. In normal subjects there was rapid movement of the radiopharmaceutical from the right colon to the rectum. In patients with severe idiopathic constipation, a spectrum of colonic abnormality was observed from slow transit involving the rectum and sigmoid only to slow transit involving the whole colon. The hepatic flexure to rectum transit time for the 'head of the isotope column' in normals ranged from 1-10 minutes (mean 5.3 minutes), whereas in patients the transit time was 14-25 minutes in four patients and radioisotope did not reach the rectum by two hours in three other patients (controls v patients, p less than 0.01). Patients also showed relatively impaired transport of the isotope 'mass'. This technique has shown that the normal colon is capable of rapid effective transport in response to a standard stimulus, and that patients with severe idiopathic constipation have a definable colonic motor disorder.

In the years 1966-80, 303 patients with radiologically extensive ulcerative colitis were followed up regularly, both by clinical assessment and by mucosal biopsies, to detect dysplastic (precancerous) change. Moderate or severe dysplasia (but no carcinoma) was found in the specimen in 8 patients treated surgically. Despite regular surveillance, carcinoma developed in 13 of the 186 patients with a history of disease for ten years or more. Of the total sixteen carcinomas in these 13 patients, eleven were Dukes stage A, three stage B, one stage C, and one was inoperable. There has been 1 death from carcinoma in this series over the fifteen year observation period. The method of follow-up has probably prevented the development of carcinoma in 8 patients and permitted diagnosis of carcinoma at a stage when cure is likely in 11. The incidence of moderate or severe dysplasia and carcinoma in these patients with mild or symptomless extensive colitis shows that either proctocolectomy or careful follow-up should be advised once the duration of symptoms reaches ten years.

The distinction between Hirschsprung's disease and idiopathic megacolon in childhood dates from the classic clinical, radiological, and histological studies of Bodian, Stephens, and Ward. This article describes clinical experience over 15 years of 94 patients in whom megacolon of these two types was recognised for the first time after the age of 10, to illustrate the problems of diagnosis and treatment in later years. Just as it is now recognised that patients with the clinical characteristics of Hirschsprung's disease may have one of several abnormalities of the myenteric plexus, including not only absence of ganglion cells, but also patchy or zonal loss, abnormal neurones or neuronal dysplasia, so idiopathic megacolon may also be a heterogeneous group of cases. This paper suggests on clinical grounds that those patients with idiopathic megacolon whose symptoms start in childhood differ from those whose symptoms develop in later years.

Measurements of bowel width and rectal area were used to define normal limits of size in 50 double-contrast barium enema films. Two types of patient with constipation were defined by comparison with these results. In 18 women with prolonged whole-gut transit time, the measurements were within the normal range. Of 11 men and 9 women with a previous radiologic diagnosis of idiopathic megacolon, all had an enlarged rectum and the abnormal width extended proximally to involve the colon for a variable distance. A width of 6.5 cm at the pelvic brim provides a convenient and discriminating separation of normal from abnormal.

A series of 12 patients with relative sparing of the rectum in ulcerative colitis is analyzed. Half were recorded as having normal sigmoidoscopic appearance and, in every case, double-contrast barium enema showed an apparently normal rectum but an abnormal colon. Rectal biopsy showed changes compatible with ulcerative colitis in all cases, though in four, changes were slight. Thus, complete histologic sparing of the rectum was not observed. In four of six patients treated by colectomy and ileorectal anastomosis, inflammation of the retained rectum required medical or surgical treatment.

Patients with severe idiopathic constipation are almost exclusively women. Common presenting features include lower abdominal pain, irregular and painful menstruation, and a past history of gynaecological surgery. Pelvic ultrasonography was undertaken in 26 patients with severe idiopathic constipation and 21 age matched control women to detect any gynaecological abnormality. No difference was found in ovarian, uterine or pelvic vein morphology or size. The abdominal pain in these patients is related to the bowel disorder, and minor abnormalities of pelvic morphology on ultrasound are probably incidental findings.

Nine women with severe chronic constipation who were unable to expel a water-filled rectal balloon underwent posterior division of the puborectalis muscle. Pre-operative concentric needle electromyography and measurement of the anorectal angle at rest and during straining suggested that the puborectalis muscle failed to relax during attempted defaecation in these patients. Two patients reported improvement after surgery and had normal balloon expulsion after operation. However seven patients reported no benefit from surgery and tests of defaecatory function and anorectal angle did not change. Incontinence for solid stool was not reported following puborectalis muscle division although five patients reported incontinence of flatus, liquid stool and mucus.

The whole gut transit rate has been measured in the follicular and luteal phases of the menstrual cycle in 18 healthy women in whom ovulation was confirmed biochemically. Mean transit time in the follicular phase (45.2 (2.7) h, mean (SEM) was not significantly different from the luteal phase (51.3 (4.1], p = 0.12. Stool weight was also not significantly different in the two phases (132 (7) v 123 (10) g/day). A diary card kept for the entire menstrual cycle revealed no change in bowel frequency or stool consistency during the menstrual, follicular or luteal phases. Under normal physiological conditions the sex hormones do not have a major effect on bowel function.

Stimulation of the rectal mucosa with a bipolar electrode leads to relaxation of the internal anal sphincter. Intraoperative studies in two subjects showed that transmission of the impulse was independent of extrinsic nerves and was interrupted by circular myotomy. Characteristics of the reflex were studied in 11 healthy women and 19 women with severe idiopathic constipation. One control subject and two patients did not tolerate the test. In the remainder the stimulus caused a clearly defined fall in internal sphincter pressure. The mean resting maximum anal canal pressure before stimulation was the same in both groups (90 (10) v 104 (7) cm H2O, p = 0.3, controls v patients). The threshold stimulus for relaxation (12 (2) v 14 (1) mamps, p = 0.5), the maximum percent fall in resting pressure (43 (7) v 46 (4)%, p = 0.7) and the lowest absolute resting pressure produced by stimulation (48 (13) v 49 (6) cm H2O, p = 0.9) were the same in both groups. The stimulus required to achieve maximum relaxation was significantly higher in the patient group (23 (3) v 32 (2) mamps, p = 0.012) suggesting abnormal intrinsic innervation of the sphincter in these patients. Electrical stimulation should not replace balloon distension for routine testing of the rectoanal reflex but it may be useful in quantitative studies.

Motilin, pancreatic polypeptide and gastrin blood concentrations in response to drinking water have been studied in 40 patients with functional bowel disease and compared with results in two groups of healthy control subjects. Patients with slow transit constipation and idiopathic megacolon showed impaired motilin release. Pancreatic polypeptide release was reduced in patients with slow transit constipation, but increased in those with functional diarrhoea. Gastrin release was impaired in all groups complaining of chronic constipation. Circulating motilin, pancreatic polypeptide and gastrin concentrations appear to bear some relationship to intestinal transit time in patients with functional bowel disorders.

The development of an enterocutaneous fistula has frequently been reported as being attended by a high morbidity and mortality, particularly in patients with inflammatory bowel disease who have high output, small bowel fistulas. We report a series of 114 patients who required treatment for 132 fistulas during the period 1976–1981. Fifty-one fistulas originated in the jejunum and ileum, and forty-eight patients had Crohn's disease. The majority of patients required surgical intervention and parenteral nutrition was required in forty-eight cases. Using an integrated programme of nursing, medical and surgical care, the overall healing rate was 90.9 per cent and the mortality 5.3 per cent. With the therapeutic methods currently available, an unacceptably high mortality rate in these patients can be avoided.

Between 1957 and 1985, 886 women with Crohn's disease and an intact distal large bowel were seen at St. Mark's Hospital. Ninety of these patients developed a fistula between the vagina and anus or rectum at an average age of 34 years. The track of the fistula was clearly documented in 80 patients and was extrasphincteric or suprasphincteric in 36, transsphincteric in 42 (high 13, low 29), and superficial in two. Of the 90 patients, 12 (13 percent) were managed throughout without recourse to surgery. Twelve (13 percent) had the fistula laid open or drainage of an abscess as the only surgery. Twelve (13 percent) underwent repair of the fistula and, of these, eight remain symptomatically cured. One has had further symptoms but no surgery while three later underwent proctectomy for rectal disease. In eight patients the colon was removed and the rectum defunctioned and in 34 the rectum was excised as the initial surgery after development of the fistula. The remaining 12 (13 percent) underwent later proctectomy for rectal disease or failed conservative management of the fistula. Extensive colonic involvement, rectal disease, or associated anal lesions were the main reasons for rectal excision in 38 patients. In only ten was the rectovaginal fistula a prominent indication for proctectomy. As medical treatment, repair, or other local surgery were successful in one third of the patients, these options should always be considered in the first instance.

A family with at least six members affected by hollow organ visceral myopathy is described. Patients in the first or second decades of life developed symptoms which included weight loss, nausea and vomiting, abdominal pain and distension, constipation and diarrhoea, and urinary symptoms. The radiological features of the disease consisted of oesophageal aperistalsis, megaduodenum, and variable dilatation of the small and large bowel. Four patients had urinary tract involvement with dilatation of the ureters and/or incomplete bladder emptying. Two patients were severely affected and needed home parenteral nutrition and surgical treatment; others were symptomatic but remained well. The characteristic pathological abnormality was vacuolar degeneration predominantly affecting the longitudinal muscle. The disease in this family appears to be transmitted by autosomal dominant mode of inheritance.

A new technique is described which allows the graphic quantitation of voluntary rectal evacuation. The subject is asked to evacuate 100 ml of barium sulphate paste as rapidly and completely as possible. Using a weight transducer it is possible to determine the maximum emptying rate, time to achieve maximum emptying and proportion of barium evacuated. Normal subjects evacuate quickly and completely. Patients with severe constipation demonstrate a variable evacuation disturbance.

The plain abdominal radiograph is an important investigation in acute colitis, but may fail to demonstrate the state of the colon owing to a lack of intracolonic gas. The extent of the colitis can be demonstrated by introducing air directly into the large bowel; the air also provides sufficient contrast to distinguish a granular from an ulcerated mucosa. The “air enema” may be used as an alternative to an unprepared barium enema. Its accuracy has been established by comparison with an unprepared barium enema in 10 patients with acute colitis.

Acute Crohn's disease of the colon requiring emergency surgery is uncommon, but may be increasing in frequency. Between 1954 and 1981, 215 patients had surgery for acute inflammatory bowel disease at St. Mark's Hospital, and of these 18 had acute Crohn's colitis. There was one postoperative death, and the remaining patients were followed up for an average of 8 years. Ten patients had toxic dilatation, two a toxic dilatation with free perforation, three had perforation without dilatation and in three surgery was required for an acute deterioration. Surgical treatment included proctocolectomy (one), colectomy and ileostomy (fourteen), colectomy and ileorectal anastomosis (two) and defunctioning ileostomy alone (one). Subsequent rectal excision was necessary in ten of sixteen patients. Acute colonic Crohn's disease requiring surgery is less likely than ulcerative colitis to be amenable to restorative surgery despite a policy of rectal conservation.

Patients with severe constipation often do not ‘relax’ their pelvic floor during defaecation. Electromyography of the pelvic floor may reveal inappropriate contraction during defaecation straining, and balloon expulsion, a test of rectal evacuation, may be impaired. Fifteen patients with severe idiopathic constipation and three patients with a megarectum underwent lateral division of the puborectalis muscle and upper half of the external sphincter muscle. Twelve patients had a unilateral division and six patients had both sides divided. Surgery caused a marked reduction in the maximum voluntary squeeze pressure in the anal canal from a pre-operative mean of 90 ± 49 (s.d.) cmH2O to a postoperative mean of 40 ± 29 cmH2O (P < 0·0001). Four patients, three with idiopathic constipation and one with megarectum, experienced symptomatic improvement. Three of these patients had a bilateral division. Improvement did not correlate with a change in the puborectalis electromyography or the ability to expel a balloon. Three patients experienced mild mucus or urge incontinence, but no patient was incontinent for solid stool.

Between 1947 and 1980, 67 patients with carcinoma complicating ulcerative colitis were treated at St Mark's Hospital. The tumours in these patients were compared with those in 4817 patients without colitis seen over the same period. There was a higher proportion of inoperable and high grade tumours in the colitic group but the prognosis was found to be very similar in patients with and without colitis.

171 patients with extensive colitis have been followed up as outpatients for periods up to seven years between 1966 and 1973. The mean length of follow-up was 3.3 years. 7 patients have died compared with the expected mortality of 4.83, giving an excess mortality of 0.003 per patient-year. 3 patients have developed carcinoma of the large bowel; they remain well seven, six, and two years after operation. In all 3, epithelial dysplasia was observed on rectal mucosal biopsy, and no patient in this series has developed carcinoma without this abnormality being present. 11 other patients had epithelial dysplasia on rectal biopsy; 4 of these underwent colectomy without evidence of carcinoma in the operative specimen. 29 patients underwent colectomy without mortality, 7 during a severe attack of colitis, the remainder electively. These results suggest that patients with quiescent extensive colitis can be managed conservatively with relative safety provided that close supervision is maintained.