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Different surgical strategies in the treatment of familial adenomatous polyposis: What's the role of the ileorectal anastomosis?
Abstract
Prophylactic (procto-) colectomy is the treatment of choice to reduce the risk of colorectal cancer in FAP patients with multiple adenomas. Because patients present at young age, rectum-sparing surgery is sometimes advocated, so that there is no pelvic dissection with impact on quality of life, preserved pelvic innervation and sexual function and fertility. The main disadvantage of a total colectomy with an ileorectal anastomosis (IRA) is a rectal cancer risk of 50% at the age of 50 years and a cumulative risk of 25.8% after 25 years of follow-up. Therefore, this procedure should be reserved for patients with an unaffected rectum. There should be no discussion to perform a primary IPAA in patients with multiple rectal adenomas (> 20) or those with a severe dysplastic or large (> 3 cm) rectal adenoma or a cancer elsewhere in the colon. A patient with an IRA should undergo yearly follow-up by rectoscopy.
... M ost centers originally employed the straight pull-through technique for patients with ulcerative colitis and familial polyposis or Hirschsprung's disease (1). Coran AG(2) in 1981, used endorectal pull-through for the management of ulcerative colitis. ...
... In our study, the preservation rectum and ileocecal valve with use of reservoirs, stool frequency decreased. Rectumsparing surgery is occasionally recommended, so there is no pelvic manipulation and leads to the improvement of quality of life, with pelvic innervations, and sexual function salvage (1). There are many ways to improve the absorptive function of ileum and reduce bowel movement such as Duhamel-Martin procedure and ileoanal patch (5). ...
Background:
Total colectomy is used in children with total colonic aganglionosis, Ulcerative colitis (UC) and familial adenomatous polyposis (FAP). The purpose of this study was to maintain ileocecal valve and rectal-sparing surgery for the prevention of fecal incontinence in these children.
Methods:
From1990 to 2011, 14 children with diagnosis of UC, FAP and Hirschsprung's disease were operated. Total colectomy was done with the preservation of patch of cecum with ileocecal valve and half of the rectum with ileocecorectal anastomosis. Distal ileum designed as S shape pouch and ileocecal valve were preserved. In Hirschsprung's disease, posterior rectal myotomy was established. The data were collected and analyzed.
Results:
The mean age of the patients was 54 months (ranged from 2 months to 18 years). Ten patients were male. Among 14 patients, Hirschsprung's disease, ulcerative colitis and FAP were seen in 10, 3, and one case, respectively. They were followed up annually. Clinical and endoscopic examinations were performed to evaluate the function of ileocecorectal anastomosis. They followed from 2 to 24 years. At first year, the patients experienced four to six bowel movements during the day and one at night. This frequency decreased over time. The main postoperative complications included recurrent enterocolitis (n=2), perianal fistula (n=2). Only 2 patients were suffering from some degree of fecal soiling.
Conclusion:
The results show that the Ileocecal patch- low rectal anastomosis in total colectomy leads to low complications and prevent fecal frequency and incontinence. It also increases absorptive function of ileum in children.
... Firstly, the pelvic dissection is avoided; hence, potential autonomic nerve injury—that could result in impotence in males and infertility in females—is prevented. Secondly, the anastomotic leakage rate is lower following ileo-rectal anastomosis than after pouch-anal anastomosis [54]. Obviously, the disadvantage of TAC-IRA is the need for frequent surveillance proctoscopy (every 6–12 months) with removal of polyps and the increased risk of rectal cancer following surgery, which is 4% at 5 years, 8% at 15 years, and 25% at 20 years [54, 55]. ...
... Secondly, the anastomotic leakage rate is lower following ileo-rectal anastomosis than after pouch-anal anastomosis [54]. Obviously, the disadvantage of TAC-IRA is the need for frequent surveillance proctoscopy (every 6–12 months) with removal of polyps and the increased risk of rectal cancer following surgery, which is 4% at 5 years, 8% at 15 years, and 25% at 20 years [54, 55]. Sulindac and celecoxib were shown to produce partial regression of polyps; these are feasible treatment options for patients who undergo TAC-IRA [56, 57]. ...
A polyp is defined as any mass protruding into the lumen of a hollow viscus. Colorectal polyps may be classified by their macroscopic appearance as sessile (flat, arising directly from the mucosal layer) or pedunculated (extending from the mucosa through a fibrovascular stalk). Colorectal polyps may also be histologically classified as neoplastic or as non-neoplastic (hyperplastic, hamartomatous, or inflammatory). The neoplastic polyps are of primary importance because they harbor a malignant potential, which represents a stage in the development of colorectal cancer. For this reason, it is essential to identify these polyps at a sufficiently early stage, when a simple outpatient procedure to remove them can interrupt the development of colorectal cancer and prevent disease and death. When invasive carcinoma arises in a polyp, careful consideration must be given to ensuring the adequacy of treatment. Although most neoplastic polyps do not evolve into cancer, it is well accepted that the majority of colorectal carcinomas evolve from adenomatous polyps; the sequence of events leading to this transformation is referred to as the adenoma-to-carcinoma sequence.
The presence of a systemic process that promotes the development of multiple gastro-intestinal polyps is termed ‘polyposis’. Hereditary gastro-intestinal polyposis syndromes account for approximately 1% of all cases of colorectal cancer and are associated with a broad spectrum of extra-colonic tumors. Early detection and accurate classification of these syndromes are essential, in order to initiate a surveillance program for the early detection of cancer. Several polyposis syndromes have been described, each having its own genetic basis and characteristic polyp distribution, clinical presentation, and malignancy risk.
Diagnostic modalities and treatment options for neoplastic polyps—as well as the most prevalent polyposis syndromes—are reviewed below.
... M ost centers originally employed the straight pull-through technique for patients with ulcerative colitis and familial polyposis or Hirschsprung's disease (1). Coran AG(2) in 1981, used endorectal pull-through for the management of ulcerative colitis. ...
... In our study, the preservation rectum and ileocecal valve with use of reservoirs, stool frequency decreased. Rectumsparing surgery is occasionally recommended, so there is no pelvic manipulation and leads to the improvement of quality of life, with pelvic innervations, and sexual function salvage (1). There are many ways to improve the absorptive function of ileum and reduce bowel movement such as Duhamel-Martin procedure and ileoanal patch (5). ...
Background: Total colectomy is used in children with total colonic aganglionosis, Ulcerative colitis (UC) and familial adenomatous polyposis (FAP). The purpose of this study was to maintain ileocecal valve and rectal-sparing surgery for the prevention of fecal incontinence in these children.
Methods: From1990 to 2011, 14 children with diagnosis of UC, FAP and Hirschsprung’s disease were operated. Total colectomy was done with the preservation of patch of cecum with ileocecal valve and half of the rectum with ileocecorectal anastomosis. Distal ileum designed as S shape pouch and ileocecal valve were preserved. In Hirschsprung’s disease, posterior rectal myotomy was established. The data were collected and analyzed.
Results: The mean age of the patients was 54 months (ranged from 2 months to 18 years). Ten patients were male. Among 14 patients, Hirschsprung’s disease, ulcerative colitis and FAP were seen in 10, 3, and one case, respectively. They were followed up annually. Clinical and endoscopic examinations were performed to evaluate the function of ileocecorectal anastomosis. They followed from 2 to 24 years. At first year, the patients experienced four to six bowel movements during the day and one at night. This frequency decreased over time. The main postoperative complications included recurrent enterocolitis (n=2), perianal fistula (n=2). Only 2 patients were suffering from some degree of fecal soiling.
Conclusion: The results show that the Ileocecal patch- low rectal anastomosis in total colectomy leads to low complications and prevent fecal frequency and incontinence. It also increases absorptive function of ileum in children.
Keywords: Total colostomy, Ileocecal patch, Ulcerative colitis, Hirschsprung
... The type of prophylactic surgery to choose for FAP patients is a very old and important Gordian Knot [2][3][4]. IRA is often chosen against the ileal pouch-anal anastomosis to preserve a higher quality of life, balancing with the cancer risk of the rectal stump [5]. ...
We recently read the article by Colletti et al. [...].
Background & aims:
Familial adenomatous polyposis (FAP) is an autosomal dominant disorder that increases risk for colorectal cancer (CRC). We assessed changes in the incidence and prevalence of CRC, and survival times, of patients with FAP participating in the Danish follow-up study.
Methods:
We collected data from the Danish Polyposis Registry, a nationwide, complete registry of patients with FAP that includes clinical information, surgical procedures, follow-up findings, and pathology reports. We compared data between the periods of 1990-1999 and 2000-2017. In 2017, the registry contained 226 families with 721 individuals with FAP. Probands were defined as patients diagnosed based on bowel symptoms, without any knowledge of hereditary bowel disease. Call-up patients were defined as those found to have FAP during screening and due to a diagnosis of FAP in first-degree relatives.
Results:
Although the mean incidence rate of FAP was stable from 1990-1999 (0.19/100,000/year) to 2000-2017 (0.32/100,000/year) (P = .91), the point prevalence increased from 4.86/100,000 in 1999 to 6.11/100,000 by the end of 2017 (P = .005). During 2000-2017, 25 of 72,218 CRC cases were associated with FAP (0.03%)-this was a significant decrease from 1990-1999 (26/30,005 cases; 0.09%) (P = .001). The risk of CRC was significantly higher for probands (n = 191; 61.6%) than call-up cases (n = 5; 1.9%) (P < .001). All CRCs in call-up patients were detected at the diagnosis of FAP (no cases were identified in the follow-up program). The median life expectancy for call-up patients was 72.0 years (95% CI, 63.3-80.7), compared to 55.0 years for probands (95% CI, 51.2-58.8) (P < .001). Therefore, the tracing and follow-up program increased life expectancy by 17.0 years for first-degree family members.
Conclusion:
The Danish Polyposis Registry enables close monitoring of patients with FAP, reducing risk of CRC and prolonging life.
Familial adenomatous polyposis (FAP) is an autosomal dominant inherited syndrome characterized by multiple adenomatous polyps (predisposing to colorectal cancer development) and numerous extracolonic manifestations. The underlying genetic burden generates variable clinical features that may influence operative management. As a precancerous hereditary condition, the rationale of performing a prophylactic surgery is a mainstay of FAP management. The purpose of the present paper is to bring up many controversial aspects regarding surgical treatment for FAP, and to discuss the results and perspectives of the operative choices and approaches. Preferably, the decision-making process should not be limited to the conventional confrontation of pros and cons of ileorectal anastomosis or restorative proctocolectomy. A wide discussion with the patient may evaluate issues such as age, genotype, family history, sphincter function, the presence or risk of desmoid disease, potential complications of each procedure and chances of postoperative surveillance. Therefore, the definition of the best moment and the choice of appropriate procedure constitute an individual decision that must take into consideration patient's preferences and full information about the complex nature of the disease. All these facts reinforce the idea that FAP patients should be managed by experienced surgeons working in specialized centers to achieve the best immediate and long-term results.
The main operative method in familial adenomatous polyposis (FAP) patients is restorative proctocolectomy with "J"-shaped pouch and temporary loop ileostomy. The aim of the study was the analysis of the frequency of the dysplasia and inflammation in the intestinal pouch and prognosis of the clinical course in FAP patients after restorative proctocolectomy.
A group of 165 FAP patients (86 females and 79 males, mean age 22.49 ± 12) subjected to a restorative proctocolectomy in the years 1985-2009 was analyzed. Clinical data coming from follow-up observation in the period of 2004-2009 were evaluated. In all patients, clinical examination and endoscopy with polypectomy and/or biopsy of pouch mucosa were done.
The mean time of pouchitis occurrence after an ileal pouch-anal anastomosis was 6 months. Mean time for low-grade dysplasia was 14 months. The time difference of low-grade dysplasia after the above procedure as compared to pouchitis alone was substantial. Mean time for high-grade dysplasia was 16 months and for neoplasia even 19 months. It was estimated that early pouchitis happening within the first year after surgery occurs in 5% of patients, low-grade dysplasia 4 years later in 7% of cases, high-grade dysplasia 7 years later in around 10% of patients and neoplasia 14 years after surgery in 15% of cases.
In conclusion, the Polyposis Registry encompassing whole country is the best way of controlling FAP patients. The regular lifelong endoscopic monitoring gives the opportunity of the early detection of the dysplasia and can protect against neoplasia.
The American Society of Colon and Rectal Surgeons is dedicated to assuring high quality patient care by advancing the science, prevention, and management of disorders and diseases of the colon, rectum, and anus. The standards committee is composed of Society members who are chosen because they have demonstrated expertise in the specialty of colon and rectal surgery. This Committee was created in order to lead international efforts in defining quality care for conditions related to the colon, rectum, and anus. This is accompanied by developing Clinical Practice Guidelines based on the best available evidence. These guidelines are inclusive, and not prescriptive. Their purpose is to provide information on which decisions can be made, rather than dictate a specific form of treatment. These guidelines are intended for the use of all practitioners health care workers, and patients who desire information about the management of the conditions addressed by the topics covered in these guidelines.
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.
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. 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.
PURPOSE: This study was designed to assess the risk of retained rectal mucosa after proctocolectomy and ileal pouch-anal anastomosis with the double-stapling technique. METHODS: A total of 113 patients underwent proctocolectomy with an ileal pouch-anal reconstruction. In 57 patients the anastomosis between pouch and proximal anal canal was performed using the double-stapling technique. In 26 patients the procedure was carried out without a protecting ileostomy. Of the remaining 31 patients with a proximal ileostomy, 15 underwent endoscopy six weeks postoperatively. Circular biopsies were taken just distal from the pouch-anal anastomosis. RESULTS: Histologic examination revealed rectal mucosa in at least one biopsy in 7 of 15 patients. At follow-up (mean 18 months) no (distal) pouchitis was clinically noticed. In one patient with familial polyposis, a few polyps, distal of the anastomosis, had to be endoscopically removed. CONCLUSIONS: Double-stapled ileal pouch-anal anastomosis has a considerable risk of residual rectal mucosa, because of combined linear transection and circular stapling with bilateral dog-ear formation of rectal mucosa. Residual rectal mucosa did not seem to influence clinical results at follow-up.
The nationwide Danish polyposis register includes all known Danish cases of familial adenomatous polyposis (FAP) and their relatives. By identifying all FAP patients born between 1920 and 1949, we found the frequency of the disease to be 1 in 13,528. By comparing the number of affected and nonaffected offspring born to affected parents during the same period we found the penetrance of the disease for inherited cases to be close to 100% at the age of 40 years. The mutation rate found by the direct method was 9 mutations per million gametes per generation and the proportion of new mutants was estimated to 25%. Fitness for patients between 15 and 29 years was found close to one, while for patients older than 30 the fitness was reduced, but increasing during the three decades (from 0.44 to 0.71) probably because treatment became more widespread and efficient. As we have used the overall fitness in the period, 0.87, to estimate the mutation rate by the indirect method, we found a lower value than by the direct method, namely 5 mutations per million gametes per generation. © 1994 Wiley-Liss, Inc.
PURPOSE: The tradeoff of neoplasia control for better function represented by a stapled ileal pouch-anal anastomosis is still controversial in patients with familial adenomatous polyposis. We compared outcomes after mucosectomy and hand-sewn ileal pouch-anal anastomosis with those after stapled ileal pouch-anal anastomosis in 119 patients with familial adenomatous polyposis who underwent surgery since 1983. METHODS: Age, gender, length of follow-up, complications, quality of life, incontinence, urgency, nighttime and daytime seepage, pad usage, necessity of ileostomy, and incidence of adenomas developing in pouch and anal transitional zone were recorded. RESULTS: There were 42 mucosectomy and 77 stapled patients who were followed up for an average of 5.8 and 3.6 years, respectively, with endoscopic surveillance. There was one postoperative death in the stapled group that prohibited long-term follow-up. Nine of 42 mucosectomy patients developed pouch adenomasvs. 8 of 76 in the stapled group. Six of 42 patients developed adenomas in the mucosectomized anal transitional zone in the mucosectomy group. Twenty-one of 76 patients developed adenomas in the anal transitional zone in the stapled group. All were managed with local procedures or further surveillance. One of 76 patients developed cancer in the residual low rectum; this required further resection. Patients with stapled anastomosis had better outcomes in every category. Differences in incontinence, daytime and nighttime seepage, pad usage, and avoidance of ileostomy were statistically significant. All patients with mucosectomy required ileostomyvs. only 40 of 77 patients with stapled anastomosis. CONCLUSION: Familial adenomatous polyposis patients with stapled ileal pouch-anal anastomosis have better functional outcome and can avoid temporary diversion. This should be balanced against a 28 percent incidence of adenomas in the anal transitional zone.