Long-term follow-up after colectomy and ileorectal anastomosis in familial adenomatous polyposis coli. Is there still a place for the procedure?

Forty-four patients with familial adenomatous polyposis coli treated with colectomy and ileorectal anastomosis were studied. Mean age at operation was 27 years. The mean follow-up period was 10 years (median 8 years). Three patients (7%) developed rectal cancer 1, 4, and 24 years after the initial operation, respectively. Proctectomy with ileostomy was performed in one patient, and 7 patients underwent a conversion to an ileoanal procedure for an increasing number of rectal polyps in the rectum stump. Although frequent bowel actions and episodes of diarrhea were common findings in patients after colectomy and ileorectal anastomosis, almost all patients (96%) were more or less satisfied with their quality of life after the procedure. On the basis of our results and the results reported in the literature, colectomy with ileorectal anastomosis is still the operation of choice in selected patients with familial adenomatous polyposis coli. An initial ileal pouch - anal anastomosis, or a conversion to such a procedure after colectomy and ileorectal anastomosis is indicated, depending on the number and size of rectal polyps.     

The risk of rectal carcinoma following colectomy in ulcerative colitis

In a series of 1439 patients with ulcerative colitis, managed by one of the authors (E.S.R.H.), surgical resection was performed on 374 patients (26 per cent): colectomy, 273 (subtotal colectomy and mucous fistula, 172, colectomy and primary ileorectal anastomosis, 101); proctocolectomy, 61; and miscellaneous procedures, 40. Of the 172 patients undergoing subtotal colectomy and mucous fistula, 93 (54 per cent) subsequently required rectal excision, 33 (19 per cent) had ileorectal anastomosis performed as a second procedure, and in 46 (27 per cent) subsequently developed as a mucous fistula. Two hundred seventy-three patients were at risk for the development of rectal, cancer after subtotal colectomy; ten patients (3.6 per cent) subsequently developed rectal cancer. The cumulative probability of developing rectal cancer after subtotal colectomy reached 17 per cent at 27 years from disease onset. The tumors were more advanced in stage and of higher grade malignancy than those of a parallel general series of patients with rectal cancer uncomplicated by inflammatory bowel disease. Colectomy and ileorectal anastomosis has been successful for most patients. However, the experience of this series highlights the danger of carcinomatous transformation in the retained rectum, the requirement for regular long-term follow-up, the need for markers of precancerous change, and the value, where relevant, of prophylactic proctectomy.     

Predicting polyposis severity by proctoscopy

PURPOSE: Patients with familial adenomatous polyposis need prophylactic colectomy and ileorectal anastomosis or restorative proctocolectomy. Preoperative rectal polyp counts have been used as one factor to determine which operation should be done, triaging patients according to risk of rectal cancer or completion proctectomy after ileorectal anastomosis. This study was designed to examine the reliability of preoperative proctoscopy in predicting familial adenomatous polyposis severity and outcome after ileorectal anastomosis. METHODS: Familial adenomatous polyposis patients were categorized according to preoperative proctoscopy as follows: Group 1, 5 or fewer adenomas; Group 2, 6 to 19 adenomas; Group 3, 20 or more adenomas. Familial adenomatous polyposis severity was defined as mild if there were <1,000 polyps in the colon at colectomy and severe if there were >1,000 polyps. RESULTS: A total of 213 patients were reviewed, 80 in Group 1, 59 in Group 2, and 74 in Group 3. There was no difference among the groups in mean age at presentation. Patients with fewer than five rectal adenomas were predominately females. They rarely had symptoms (22.8 percent), had mostly mild polyposis (86.5 percent), and in 74 of 80 cases underwent ileorectal anastomosis. Only six underwent restorative proctocolectomy. Of those having an ileorectal anastomosis, five needed later proctectomy, none for cancer. Patients with 6 to 19 rectal polyps were a similar group to those with 5 or fewer. Most were asymptomatic (67.8 percent), most had mild polyposis (81.6 percent), and 54 of 59 underwent ileorectal anastomosis (5 had restorative proctocolectomy). Only 3 of the 54 having ileorectal anastomosis needed subsequent proctectomy, 2 for rectal cancer. The patients with 20 or more rectal polyps were different. They usually presented with symptoms (86 percent), the majority (56.6 percent) had severe polyposis, and only 50 percent (37/74) underwent ileorectal anastomosis, the other half having restorative proctocolectomy. Of the 37 patients with an ileorectal anastomosis, 13 needed later proctectomy (35.1 percent), 4 for cancer (10.8 percent). CONCLUSION: Fewer than five rectal adenomas at presentation almost always predicts mild disease, and patients do well after ileorectal anastomosis. Twenty or more adenomas usually means severe disease. Patients with 6 to 19 adenomas are often mildly affected, but their phenotype is less benign than that of patients with fewer than five polyps. Although not foolproof, proctoscopy is a useful test in triaging patients with familial adenomatous polyposis according to disease severity.Read at the meeting of The American Society of Colon and Rectal Surgeons, Boston, Massachusetts, June 24 to 29, 2000.     

Colectomy and Ileorectal Anastomosis is still an Option for Selected Patients with Familial Adenomatous Polyposis

Purpose  The risk of rectal cancer after colectomy and ileorectal anastomosis may be reduced in the last decades, as patients with severe polyposis now have an ileoanal pouch. We have reevaluated the risk of rectal cancer and proctectomy for all causes according to the year of operation. Methods  On the basis of the year of operation in 776 patients with ileorectal anastomosis and 471 pouch patients in Denmark, Finland, Holland, and Sweden, the “pouch period” was defined to start in 1990. Ileorectal anastomosis follow-up data was captured by May 31, 2006. The cumulative risk of rectal cancer and proctectomy was compared before and after 1990 by Kaplan-Meier analysis. Results  In the prepouch period 56/576 patients (10 percent) developed rectal cancer, vs. 4/200 (2 percent) in the pouch period. Neither the cumulative risk of rectal cancer (p = 0.07) nor the cumulative risk of proctectomy (p = 0.17) changed. However, in females the cumulative risk of rectal cancer (p = 0.04) and of proctectomy (p = 0.03) were lower in the pouch period. Conclusions  Since the introduction of the ileoanal pouch rectal cancer has decreased after ileorectal anastomosis, but only statistically significant in females. This indicates that ileorectal anastomosis may still be justified in selected patients with mild adenomatosis, especially in young females. Presented at the meeting of the International Society for Gastrointestinal Hereditary Tumours (InSiGHT), Yokohama, Japan, March 28 to 30, 2007. Reprints are not available.     

Predicting polyposis severity by proctoscopy: how reliable is it?

PURPOSE: Patients with familial adenomatous polyposis need prophylactic colectomy and ileorectal anastomosis or restorative proctocolectomy. Preoperative rectal polyp counts have been used as one factor to determine which operation should be done, triaging patients according to risk of rectal cancer or completion proctectomy after ileorectal anastomosis. This study was designed to examine the reliability of preoperative proctoscopy in predicting familial adenomatous polyposis severity and outcome after ileorectal anastomosis. METHODS: Familial adenomatous polyposis patients were categorized according to preoperative proctoscopy as follows: Group 1, 5 or fewer adenomas; Group 2, 6 to 19 adenomas; Group 3, 20 or more adenomas. Familial adenomatous polyposis severity was defined as mild if there were < 1,000 polyps in the colon at colectomy and severe if there were > 1,000 polyps. RESULTS: A total of 213 patients were reviewed, 80 in Group 1, 59 in Group 2, and 74 in Group 3. There was no difference among the groups in mean age at presentation. Patients with fewer than five rectal adenomas were predominately females. They rarely had symptoms (22.8 percent), had mostly mild polyposis (86.5 percent), and in 74 of 80 cases underwent ileorectal anastomosis. Only six underwent restorative proctocolectomy. Of those having an ileorectal anastomosis, five needed later proctectomy, none for cancer. Patients with 6 to 19 rectal polyps were a similar group to those with 5 or fewer. Most were asymptomatic (67.8 percent), most had mild polyposis (81.6 percent), and 54 of 59 underwent ileorectal anastomosis (5 had restorative proctocolectomy). Only 3 of the 54 having ileorectal anastomosis needed subsequent proctectomy, 2 for rectal cancer. The patients with 20 or more rectal polyps were different. They usually presented with symptoms (86 percent), the majority (56.6 percent) had severe polyposis, and only 50 percent (37/74) underwent ileorectal anastomosis, the other half having restorative proctocolectomy. Of the 37 patients with an ileorectal anastomosis, 13 needed later proctectomy (35.1 percent), 4 for cancer (10.8 percent). CONCLUSION: Fewer than five rectal adenomas at presentation almost always predicts mild disease, and patients do well after ileorectal anastomosis. Twenty or more adenomas usually means severe disease. Patients with 6 to 19 adenomas are often mildly affected, but their phenotype is less benign than that of patients with fewer than five polyps. Although not foolproof, proctoscopy is a useful test in triaging patients with familial adenomatous polyposis according to disease severity.     

Decision analysis in the surgical treatment of patients with familial adenomatous polyposis: a Dutch-Scandinavian collaborative study including 659 patients

BACKGROUND AND AIMS: The choice of colorectal surgery in patients with familial adenomatous polyposis lies between the morbidity of proctocolectomy and ileum-pouch-anal anastomosis (IPAA) and the mortality from rectal cancer after total colectomy and ileorectal anastomosis (IRA). The aims of the present study were: (1) to assess the risk of dying from rectal cancer after IRA, (2) to compare the life expectancy between patients with an IRA and those with an IPAA, and (3) to investigate whether regular endoscopic examination of the rectum leads to detection of cancer at an earlier stage. METHODS: Clinical and pathological data on 659 patients who underwent colectomy and ileorectal anastomosis were collected from four national polyposis registries-that is, in Denmark, Finland, Sweden, and the Netherlands. Data were analysed using survival analysis methods. Decision analysis was used to compare the life expectancy between patients with an IRA and those with an IPAA. RESULTS: A total of 47 patients developed rectal cancer after IRA. The risk of dying from rectal cancer was 12.5% (95% confidence interval 7.1--17.9%) by age 65. Compared with IRA, IPAA would lead to an increase in life expectancy of 1.8 years. Seventy five per cent of patients with rectal cancer had a negative rectoscopy within 12 months before the diagnosis. CONCLUSION: IRA is associated with substantial mortality due to rectal cancer. Follow up examinations of the rectum does not have sufficient preventive effect on morbidity and mortality of rectal cancer.     

Risk of Rectal Cancer in Patients After Colectomy and Ileorectal Anastomosis for Familial Adenomatous Polyposis

PURPOSE: One of the concerns with colectomy and ileorectal anastomosis as a prophylactic procedure for patients with familial adenomatous polyposis is the risk of metachronous rectal cancer, estimated at from 12 to 43 percent. These estimates are based largely on surgeries performed at a time when the only alternative option to ileorectal anastomosis for patients with severe familial adenomatous polyposis was proctocolectomy and ileostomy. This study was designed to test the hypothesis that in the pouch era severe polyposis is now treated by proctocolectomy and ileal pouch-anal anastomosis. Ileorectal anastomosis is performed mostly in mildly affected patients and will therefore carry a very low risk of metachronous rectal cancer. METHODS: Patients undergoing primary prophylactic surgery for familial adenomatous polyposis between 1950 and 1999 were categorized according to the year of their surgery: prepouch era (before 1983) or pouch era (after 1983). Patients undergoing colectomy and ileorectal anastomosis were the focus of the study, and rate of proctectomy and the incidence of rectal cancer were recorded for each group. Data on the severity of the polyposis for each group were abstracted. RESULTS: A total of 197 patients underwent ileorectal anastomosis, 62 in the prepouch era (median follow-up, 212 months; interquartile range, 148 months) and 135 in the pouch era (median follow-up, 60 months; interquartile range, 80 months). Patients in the prepouch era came to surgery at the same median age as those in the pouch era (median age 23.0 years, interquartile ranges 15.5 years for prepouch and 17 years for pouch). Similar proportions of patients in the prepouch era had severe polyposis (49 percent) as in the pouch era (44 percent), although all severely affected patients had an ileorectal anastomosis in the prepouch era vs. 39 percent in the pouch era. Twenty (32 percent) prepouch-era patients underwent proctectomy compared with three (2 percent) pouch-era patients. No pouch-era patient had rectal cancer on follow-up; eight (12.9 percent) prepouch-era patients did. CONCLUSION: Although follow-up is shorter, ileorectal anastomosis for familial adenomatous polyposis performed since 1983 carries a much lower rate of rectal cancer and proctectomy than ileorectal anastomosis performed before this time, when restorative proctocolectomy was not an option. This is related, at least in part, to a greater number of patients with severe polyposis having their rectum initially spared.     

Ileorectal anastomosis--familial adenomatous polyposis.

Familial adenomatous polyposis is a generalized growth disorder. The predominant cause of death is, however, colorectal cancer in the untreated patient. Prophylactic colectomy, whether it be proctocolectomy and ileostomy, colectomy with ileorectal anastomosis, or colectomy with an ileoanal pouch procedure, will dramatically reduce the mortality from large bowel cancer. It is important, however, to realize that because of the generalized nature of this condition and the possibility of developing extracolonic malignancy, the surgical approach chosen should not be considered one that will necessarily cure the disease. Colectomy with ileorectal anastomosis still leaves the potential for rectal cancer. However, death from rectal cancer is distinctly uncommon. The risk of death following ileorectal anastomosis from rectal cancer is actually less than the risk of dying from cancer of the duodenum or from desmoid tumors. Both of these lesions are unpredictable in patients with familial adenomatous polyposis, and usually occur following the patient's prophylactic colectomy. With this low incidence of death from rectal cancer following ileorectal anastomosis it would seem to be debatable whether the complexities of the ileoanal pouch procedure together with its longer recovery and higher complication rate are justified in the routine management of young people with an early diagnosis of familial adenomatous polyposis.     

Risk factors for rectal cancer morbidity and mortality in patients with familial adenomatous polyposis after colectomy and ileorectal anastomosis

PURPOSE: The aims of the study were to investigate the effects of ileorectal anastomosis and the follow-up program on rectal cancer morbidity and mortality and to identify risk factors that predict the fate of the rectal stump. METHODS: One hundred ninety-five patients with familial adenomatous polyposis on whom an ileorectal anastomosis was performed between 1957 and the end of 1995 were included. Median follow-up time was 14 (range, 1-39) years. The cumulative risks of rectal cancer and rectal excision were estimated using survival analysis. RESULTS: Eighteen patients (9.2 percent) developed cancer, 17 in the retained colorectal segment and one on the ileal side of the anastomosis, and nine died of their cancer during the study period. The cumulative rectal cancer morbidity and mortality 20 years after ileorectal anastomosis was 12.1 percent (95 percent confidence interval = 5.7-18.5) and 7 percent (95 percent confidence interval = 2-12), respectively. The cumulative age-dependent risk of rectal cancer was 22.9 percent (95 percent confidence interval = 11.4-34.5) and 25.7 percent (95 percent confidence interval = 13.2-38.2) at the ages of 60 and 70 years, respectively. The corresponding cumulative mortality was 11.1 percent (95 percent confidence interval = 2.9-19.3) at the age of 70 years. Patients with dense polyposis at colectomy had an increased risk for cancer in the retained colorectal segment compared with patients with intermediate or sparse polyposis (P = 0.04). Sixty-six patients (34 percent) had their rectum removed, and the cumulative rectal excision rate 35 years after ileorectal anastomosis was 65.5 percent (95 percent confidence interval = 53-78). CONCLUSION: Patients on whom ileorectal anastomosis was performed had, despite the high rectal excision rate, a substantial risk of developing cancer in the retained colorectal segment, with an ensuing high mortality. Our results indicate that patients with dense polyposis should undergo restorative proctocolectomy as primary operation for familial adenomatous polyposis. In younger patients with intermediate or sparse polyposis and good expected follow-up compliance, ileorectal anastomosis still is an alternative.     

Risk of cancer and secondary proctectomy after colectomy and ileorectal anastomosis in familial adenomatous polyposis

Purpose

The aim of our retrospective study was to review the outcome of patients undergoing colectomy with ileorectal anastomosis (IRA) due to familial adenomatous polyposis (FAP) in Finland during the last 50 years.

Methods

The cumulative risk of rectal cancer and the rate of anus preservation were analyzed. A total of 140 FAP patients with previous colectomy combined with ileorectal anastomosis were included. Kaplan–Meier analysis was performed to evaluate cumulative risks.

Results

Secondary proctectomy was performed for 39 (28 %) of 140 patients. The cumulative risk of secondary proctectomy was 53 % at 30 years after colectomy with IRA. A total of 17 (44 %) secondary proctectomies were performed due to cancer or suspicion of cancer, and another 17 (44 %) secondary proctectomies were performed due to uncontrollable rectal polyposis. During our study, the anus preservation rate in secondary proctectomies was 49 %. The cumulative risk of rectal cancer was 24 % at 30 years after colectomy with IRA. Therefore, the cumulative rectal cancer mortality 30 years after colectomy with IRA was 9 %.

Conclusions

Proctocolectomy and ileal pouch-anal anastomosis (IPAA) should be favored as a primary operation for patients not having technical or medical contraindications for it because colectomy with IRA carried a rectal cancer risk of 13 % with a mortality of 7 % during our study, and because IPAA is likely to succeed better at earlier phase of the disease. Patients with attenuated FAP had no rectal cancer in our study, and they may form a group where IRA should still be the first choice as an exception.     

Prevalence and Morphology of Pouch and Ileal Adenomas in Familial Adenomatous Polyposis

PURPOSE In familial adenomatous polyposis, the long-term risk of pouch polyposis and potential for pouch cancer are unknown. Our aim was to evaluate prospectively the prevalence, nature, and etiology of pouch ileal adenomas with that of nonpouch ileal adenomas in familial adenomatous polyposis.METHODS Sixty patients with familial adenomatous polyposis pouch, 47 familial adenomatous polyposis patients with ileorectal anastomosis, and 20 younger patients with familial adenomatous polyposis who had prophylactic colectomy were examined with videoendoscopy.RESULTS Adenomatous polyps were found in the pouches of 34 patients (57 percent). A total of 362 polyps were identified (range, 0–50 per patient). A logistic regression model confirmed that there was a significant association between the increasing age of the patient and the presence of pouch adenomas (P < 0.02) and the length of follow-up since pouch surgery (P < 0.05). There was no apparent relationship between the development of pouch adenomas and the severity of either colonic or duodenal polyposis and there were no clear genotype or phenotype correlations. Most polyps were tubular adenomas with mild dysplasia, but 11 patients had more advanced histology, including two patients with large villous adenomas. Nonpouch ileal mucosa was spared from visually observed adenomas, with only 1 of 48 (2 percent) patients with ileorectal anastomosis adenomas and 0 of 20 (0 percent) younger, precolectomy patients having terminal ileal adenomas. However, microadenomas were present on random biopsy in 4 percent to 5 percent of nonpouch ileum.CONCLUSION The risk of pouch cancer in familial adenomatous polyposis is unclear, but follow-up periods since surgery remain relatively short. Long-term endoscopic surveillance of familial adenomatous polyposis pouches is thus recommended along with evaluation of potential therapeutic options for pouch adenomas.I. G. Beveridge and C. J. Groves were supported by Cancer Research UK (formerly the Imperial Cancer Research Fund).     

Comparison of bowel function after ileorectal anastomosis for ulcerative colitis and colonic polyposis.

Bowel habit has been studied in a group of 92 outpatients with ileorectal anastomosis for ulcerative colitis and compared with that in a group of 45 outpatients with ileorectal anastomosis for colonic polyposis. Bowel frequency ranged from 1-7 to 8-7 stools per 24 hours with a mean of 4-5 in the colitic group compared with a range of 1-3 to 8-1 stools per 24 hours and a mean of 3-5 in the polyposis group. Eighty-two per cent of the colitic group and 95% of the polyposis group had six or fewer bowel actions per 24 hours. Colitic patients in poor general condition at the time of colectomy seemed more likely to have over six bowel actions per 24 hours than those less severely ill. Urgency of defaecation was rare. Bowel actions every night and occasional faecal incontinence occurred in small but similar percentages of patients in both groups. Fifty-one per cent of patients in the colitic group, as compared with 9% in the polyposis group, were taking regular treatment to improve bowel function. Dietary difficulty was rare after ileorectal anastomosis in both colitic and polyposis groups.     

Sulindac induced regression of colorectal adenomas in familial adenomatous polyposis: evaluation of predictive factors.

BACKGROUND--Sulindac, a non-steroidal anti-inflammatory drug, causes regression of colorectal adenomas in patients with familial adenomatous polyposis (FAP) but the response is variable. Specific clinical factors predictive of sulindac induced regression have not been studied. METHODS--22 patients with FAP were given sulindac 150 mg orally twice a day. Polyp number and size were determined before treatment and at three months. The relation of nine clinical factors to polyp regression (per cent of baseline polyp number after treatment) was evaluated by univariate and multivariate analysis. RESULTS--After three months of sulindac, polyp number had decreased to 45 per cent of baseline and polyp size to 50 per cent of baseline (p < 0.001 and p < 0.01, respectively). Univariate analysis showed greater polyp regression in older patients (p = 0.004), those with previous colectomy and ileorectal anastomosis (p = 0.001), and patients without identifiable mutation of the APC gene responsible for FAP (p = 0.05). With multivariate regression analysis, response to sulindac treatment was associated with previous subtotal colectomy. CONCLUSIONS--Sulindac treatment seems effective in producing regression of colorectal adenomas of FAP patients with previous subtotal colectomy regardless of baseline polyp number and size. Changed sulindac metabolism, reduced area of the target mucosa, or changed epithelial characteristics after ileorectal anastomosis may explain these findings.     

Ileorectal anastomosis for inflammatory bowel disease: 15-year follow-up

Summary A personal series (E.S.R.H.) of 37 patients with inflammatory bowel disease, treated by colectomy and ileorectal anastomosis 15 years or more ago, is reviewed. Twenty-one patients (57 per cent) continue to be in satisfactory condition. Patients subjected to the two-stage operation have a notably lower rate of conversion to ileostomy than those treated by one-stage colectomy. One patient developed a carcinoma of the rectal stump. This 15-year review leads support to the opinion that ileorectal anastomosis has an important place in the treatment of inflammatory bowel disease. Read at the meeting of the American Society of Colon and Rectal Surgeons, Atlanta, Georgia, June 10 to 14, 1979.     

Spontaneous resolution of rectal polyps in patients with familial polyposis following abdominal colectomy and ileorectal anastomosis

One hundred sixteen patients were reviewed after abdominal colectomy and ileorectal anastomosis (IRA) for familial polyposis to determine the rate of postoperative spontaneous regression of rectal polyps. The failure of the IRA procedure was correlated with the preoperative number of rectal polyps and the degree of rectal polyp regression. Spontaneous resolution of rectal polyps occurred in 64 percent of the patients (complete 38 percent, partial 26 percent). In those patients initially having complete resolution, 55 percent redeveloped polyps during follow-up. With a mean follow-up of 9.3 years, seven patients have developed rectal cancer. Rectal cancer development was more common in those patients who had innumerable rectal polyps prior to IRA. Initial polyp regression did not preclude later development of rectal cancer. There were 11 deaths during the follow-up period, but only one of these was from rectal cancer. Abdominal colectomy and IRA is an effective treatment for familial polyposis. Spontaneous regression of polyps occurred in 64 percent of patients, but continuous and complete follow-up is necessary to fulgurate recurrent polyps and to screen for the development of cancer.     

Large Villous Adenomas Arising in Ileal Pouches in Familial Adenomatous Polyposis: Report of Two Cases

A restorative proctocolectomy or ileal pouch procedure is one of the main surgical options for patients with familial adenomatous polyposis. The main premise underlying the recommendation of a pouch procedure rather than an ileorectal anastomosis is that it minimizes the risk of rectal cancer. Several studies have evaluated the risk of developing pouch adenomas. There also have been reports of pouch cancers, although the long-term risk of malignancy cannot yet be quantified. Most pouch polyps reported have been small tubular adenomas with mild dysplasia. A 19-year-old female with familial adenomatous polyposis had a colectomy and ileorectal anastomosis. Progressive rectal polyposis led to a restorative proctocolectomy at aged 38 years. Four years later, a large, 3-cm × 2-cm, villous adenoma was identified in the mid pouch, which was resected endoscopically. A 32-year-old male with familial adenomatous polyposis had a restorative proctocolectomy. Ten years after surgery, pouch endoscopy revealed several large, villous adenomas arising from the pouch mucosa. These advanced polyps may present a significant risk for cancer development and require close endoscopic surveillance. These findings strengthen the recommendation for careful regular endoscopic surveillance of familial adenomatous polyposis pouches and the evaluation of management and treatment strategies for pouch adenomas.     

Outcome of primary and secondary ileal pouch-anal anastomosis and ileorectal anastomosis in patients with familial adenomatous polyposis

PURPOSE: The aim of this study was to present Swedish experiences of the ileal pouch-anal anastomosis in patients with familial adenomatous polyposis from the introduction in 1984. The study also compared the surgical and functional outcome of different anal continence preserving procedures: ileal pouch-anal anastomosis as primary surgery, ileal pouch-anal anastomosis as secondary surgery after colectomy and ileorectal anastomosis, and ileorectal anastomosis alone. METHODS: The material comprises all 120 patients with familial adenomatous polyposis reported to the Swedish Polyposis Registry who had undergone prophylactic colorectal surgery, including those operated on because of colorectal cancer from 1984 until the end of 1996. Anal continence preserving surgery was performed on 102 patients: 20 had ileal pouch-anal anastomosis as primary surgery at a median age of 24.5 years, 39 had ileal pouch-anal anastomosis as secondary surgery at a median age of 34 years, and 43 had ileorectal anastomosis alone, at a median age of 26 years, because 6 of the initially ileorectal anastomosis-operated patients were converted to ileal pouch-anal anastomosis as secondary surgery. Surgical outcome was assessed on the basis of hospital records. A questionnaire was used to evaluate the functional outcome. Fisher's exact probability test was used for statistical analysis. RESULTS: Complications occurred in 51 percent of the patients after ileal pouch-anal anastomosis: 40 percent after ileal pouch-anal anastomosis as primary surgery and 56 percent after ileal pouch-anal anastomosis as secondary surgery. When the previous ileorectal anastomosis was taken into account 67 percent of the patients suffered complications which was significantly more compared with ileal pouch-anal anastomosis as primary surgery. After ileorectal anastomosis, 26 percent had complications which was significantly less compared with all other procedures but ileal pouch-anal anastomosis as primary surgery. No cancer occurred after ileal pouch-anal anastomosis, either in the ileal pouch or in retained rectal mucosa, but two of the patients who had an ileorectal anastomosis developed rectal cancer. One pouch excision was performed compared with ten rectal excisions. Functional outcome did not differ between ileal pouch-anal anastomosis as primary surgery and ileal pouch-anal anastomosis as secondary surgery. However, ileorectal anastomosis-operated patients had significantly better bowel function with regard to nighttime stool frequency, continence and perianal soreness. CONCLUSION: These findings indicate that major advantages of ileal pouch-anal anastomosis are the low excision rate and, so far, no cancer in the ileal pouch. Moreover, the surgical outcome of ileal pouch-anal anastomosis as primary surgery is not significantly different from that of ileorectal anastomosis. However, the good surgical and functional outcome of ileorectal anastomosis, despite the long-range prognosis including rectal cancer and excision risks, has to be taken into consideration when selecting patients with familial adenomatous polyposis for primary surgery.Parts of the functional outcome part of the study were presented at the Leeds Castle Polyposis Group meeting in Noordwijk, the Netherlands, June 4 to 7, 1997.Supported by the Cancer Society in Stockholm and the Karolinska Institute.     

Familial adenomatous polyposis

PURPOSE: Virtually all untreated patients with familial adenomatous polyposis develop colorectal carcinoma. Thus, prophylactic colectomy is indicated. Detractors of ileal pouch-anal anastomosis prefer ileorectal anastomosis for teenagers because of the potential negative impact of ileal pouch-anal anastomosis on quality of life. The aim of this study was to assess the effects on quality of life of ileal pouch-anal anastomosis in teenagers with familial adenomatous polyposis. METHODS: Between 1981 and 1998, 48 teenagers underwent ileal pouch-anal anastomosis for familial adenomatous polyposis. One patient had proctectomy and ileal pouch-anal anastomosis after previous ileorectal anastomosis. A temporary diverting loop ileostomy was established in 42 patients (87.5 percent). One patient had colonic carcinoma diagnosed preoperatively. Two other patients were found to have unsuspected rectal cancer at surgery. Mean follow-up (± standard deviation) in 43 patients was 80.5±42 months. RESULTS: There was no immediate postoperative mortality. Postoperative complications included pelvic sepsis (3 patients; 1 requiring reoperation) and bleeding (1 patient; no surgery required). One patient died of metastatic colonic carcinoma. Ten patients required reoperation, seven had bowel obstruction, one had portal hypertension, and two required an ileostomy. The mean (± standard deviation) daytime and nighttime stool frequency was 4±1.5 and 1±1, respectively. One patient reported daytime and nighttime incontinence, and two patients reported nighttime incontinence only. No patient experienced impotence or retrograde ejaculation. Social, sexual, sport, housework, recreation, family, travel, and work activities were improved or unchanged in 82.5, 87, 80, 90, 80, 92.5, 77.5, and 89 percent of patients, respectively. Three male patients fathered children, and three female patients had a total of six children after normal pregnancies and deliveries. CONCLUSION: The impact of ileal pouch-anal anastomosis on quality of life was favorable in the majority of teenagers. The risk of rectal cancer should be the major consideration before proposing an operation to teenagers with familial adenomatous polyposis.Poster presentation at The American Society of Colon and Rectal Surgeons' 100th Anniversary and Tripartite Meeting, Washington, D.C., May 1 to 6, 1999.     

Does better functional result equate with better quality of life?

PURPOSE: The main impetus for a patient with familial adenomatous polyposis to choose colectomy with ileorectal anastomosis over ileal pouch-anal anastomosis is the better functional result. However, does better functional result necessarily translate into better overall quality of life? Previous studies of other diseases have demonstrated no such correlation. This study was performed to determine whether any relationship exists between functional result and quality of life in patients with familial adenomatous polyposis after ileorectal anastomosis and ileal pouch-anal anastomosis. METHODS: All patients with familial adenomatous polyposis who underwent colectomy with ileorectal anastomosis or proctocolectomy with ileal pouch-anal anastomosis from 1980 to 1998 were studied. Functional data were obtained by questionnaire. Health-related quality of life was assessed by two validated instruments, the SF-36 Physical and Mental Health Summary Scales and the SF-36 Health Survey, which measure physical and mental functioning and eight separate health-quality dimensions, including health perception, physical and social functioning, physical and emotional role limitations, mental health, bodily pain, and energy or fatigue. RESULTS: Data were obtained in 44 of 68 patients, 14 with ileorectal anastomosis and 30 with ileal pouch-anal anastomosis. No differences were demonstrated between the two groups for patient age, mean follow-up time, and mean patient age at operation. Functional results were worse for the ileal pouch-anal anastomosis groupvs. the ileorectal anastomosis group in number of bowel movements per day (7.5vs. 5.2;P<0.05), leakage (43vs. 0 percent;P<0.01), pad usage (17vs. 0 percent;P<0.01), perianal skin problems (33vs. 7 percent;P<0.01), food avoidance (80vs. 43 percent;P<0.01), and inability to distinguish gas (37vs. 7 percent;P<0.01). Results of the health-related quality-of-life surveys, however, demonstrated no difference between the ileal pouch-anal anastomosis and ileorectal anastomosis groups. The Physical and Mental summary scales for the ileal pouchanal anastomosis and ileorectal anastomosis groups were not significantly different (Physical Health Scale, 50.3vs. 50.9; Mental Health Scale, 51.7vs. 49.6), and none of the eight dimensions of the SF-36 health survey demonstrated statistical differences between the ileal pouch-anal anastomosis and ileorectal anastomosis groups. CONCLUSION: Better functional results were not equated with better quality of life in this pilot study. Although patients with the ileorectal anastomosis have better functional results than those withileal pouch-anal anastomosis, the measured health-related quality of life as determined by a validated generic health-related quality-of-life instrument is the same for both groups. These results suggest that all patients with familial adenomatous polyposis might be optimally treated with an ileal pouch-anal anastomosis. More importantly, this study suggests that health-related quality of life should play a greater role in the evaluation of care and treatment in colon rectal surgery.     

A major predictor of cancer following ileorectal anastomosis

Fifty patients with ulcerative colitis managed by colectomy and ileorectal anastomosis had rectal biopsies performed in the period 1967 to 1972. Follow-up information was available on all patients. Thirty-nine patients were reviewed and rectal biopsies performed in the 1980 to 1982 period. Three patients had developed rectal cancer in the period 1975 to 1980, and two rectal cancers were detected in the 1980 to 1982 follow-up period. All cancers occurred in patients with a diagnosis of moderate or severe dysplasia in biopsy specimens from the 1969 to 1972 period. The probability of developing rectal cancer after a diagnosis of moderate or severe dysplasia in this series reached 42 per cent at nine years from diagnosis.