Evolutionary changes in the pathologic diagnosis after the ileoanal pouch procedure.

PURPOSE: Inadequate initial differentiation between ulcerative colitis and Crohn's disease may lead to a diagnosis of indeterminate colitis. Construction of an ileoanal pouch in these patients may result in significant morbidity and pouch failure when the ultimate diagnosis is Crohn's disease. METHOD: We prospectively studied 543 patients with idiopathic inflammatory bowel disease to determine whether a patient's pathologic diagnosis changed with time and how it affected outcome. RESULTS: Preoperative diagnosis was ulcerative colitis in 499 patients, indeterminate colitis in 42 patients, and Crohn's disease in 2 patients. Prior colectomy was performed in 58 percent of patients with ulcerative colitis and in all patients with indeterminate colitis and Crohn's disease. Postoperatively, the diagnosis changed in 20 patients with ulcerative colitis (13 to indeterminate colitis, 7 to Crohn's disease). Another two patients with indeterminate colitis showed evidence of Crohn's disease in the resected rectal specimen. As patients were followed up, an additional 13 patients were found to have Crohn's disease (5 indeterminate colitis, 8 ulcerative colitis). With the current diagnosis, perineal complications and pouch failure occurred, respectively, in 23 and in 2 percent of patients with ulcerative colitis, in 44 and in 12 percent of patients with indeterminate colitis, and in 63 and in 37 percent of patients with Crohn's disease. Pathologic diagnosis was altered in 35 patients (6 percent) overall, with a 12-fold increase in the diagnosis of Crohn's disease. Only 3 percent of patients with ulcerative colitis compared with 13 percent of patients with indeterminate colitis had a change in diagnosis to Crohn's disease (P = 0.006; Fisher's exact test). CONCLUSION: Pouch-related complications, eventual pouch failure, and discovery of underlying Crohn's disease occurred in a significant number of patients with a diagnosis of indeterminate colitis. Until more accurate diagnostic differentiation is available, caution is advised in recommending the ileoanal pouch procedure to patients with indeterminate colitis.

Gastroduodenal polyps in familial adenomatous polyposis.

BACKGROUND: Malignant degeneration of gastroduodenal polyps has been noted in patients with familial adenomatous polyposis. To evaluate this problem further, patients with familial adenomatous polyposis were contacted and offered upper gastrointestinal tract endoscopy. METHODS: A prospective endoscopic examination was performed in 42 patients. RESULTS: The median age of patients at endoscopy was 35 years. The duration of known familial adenomatous polyposis at the time of endoscopy was 8 years. Polyps were visualized in 21 patients (50%). Gastric polyps were seen in 14 patients (33%), duodenal polyps were seen in 11 patients (26%), and ampullary polyps were seen in 7 patients (17%). Nine patients (43%) had polyps in more than one site. Adenomatous change was noted in 73% of duodenal lesions and in only 14% of gastric polyps. Surgical intervention was required in four patients; one patient had an early ampullary carcinoma, and three patients had severe dysplasia involving the duodenum or ampulla. All four patients had undergone a previous screening examination, results of which were normal in three patients. Compared with other patients, these four patients were older (median age, 58 years; p = 0.02) and had a longer duration of disease (median duration, 25 years; p = 0.002). CONCLUSIONS: All patients with familial adenomatous polyposis require lifelong endoscopic surveillance to detect malignant degeneration, which may appear later in life.

Anal fissure in Crohn's disease: a plea for aggressive management.

PURPOSE: This study was undertaken to identify clinical characteristics, natural history, and results of medical and surgical treatment of anal fissures in Crohn's disease. METHODS: This is a retrospective review of patients with Crohn's disease and anal fissure. RESULTS: Of the 56 study patients, 49 (84 percent) had symptomatic fissures. Fissures were most commonly (66 percent) located in the posterior midline, and 18 patients (32 percent) had multiple fissures. Fissures healed in one-half of patients treated medically. Factors predictive of successful medical treatment included male gender, painless fissure, and acute fissure. Of 15 patients, 10 (67 percent) treated surgically healed. Fissures in seven of eight patients (88 percent) who underwent anorectal procedures healed compared with fissures in only three of seven patients (43 percent) who underwent proximal intestinal resection. In the group of 50 patients with complete follow-up studies, an anal abscess or fistula from the base of an unhealed fissure developed in 13 patients (26 percent). More fissures healed after anorectal surgery (88 percent) than after medical treatment alone (49 percent; P = 0.05) or after abnormal surgery (29 percent; P = 0.03). CONCLUSION: This series documents that unhealed fissures frequently progress to more ominous anal pathologic disease. Judicious use of internal sphincterotomy appears to be safe for fissures unresponsive to medical treatment.

Rediversion after ileal pouch-anal anastomosis. Causes of failures and predictors of subsequent pouch salvage.

PURPOSE: The aim of this study was to understand better the cause and predictability of pouch failure requiring rediversion after ileal pouch-anal anastomosis and to assess the ultimate outcome of patients in a large ileal pouch series who required rediversion. METHODS: Data from 460 patients completing ileal pouch-anal anastomosis at one institution were recorded from both a prospectively accumulated ileal pouch registry and patient medical records. RESULTS: Of 460 patients, 21 (4.6 percent) who underwent ileal pouch-anal anastomosis required rediversion. Five of these patients subsequently had successful restoration of pouch continuity, leaving a permanent failure rate of 16 of 460 patients (3.5 percent). The most common reasons for rediversion were pouch fistula formation (12) and poor functional results (5). Preoperative factors, including age, previous colectomy, and indication for colectomy, did not predict eventual need for rediversion. Patients requiring rediversion had significantly higher rates of postoperative complications (95 vs. 43 percent; P < 0.001). Specifically, this group had a higher rate of postoperative pouch fistula (57 vs. 3.4 percent; P < 0.001). Additionally, a final diagnosis of Crohn's disease significantly predicted the need for rediversion. Permanent pouch failure occurred in 36.8 percent of patients with a final diagnosis of Crohn's disease compared with 1.4 percent of patients with a final diagnosis of ulcerative colitis (P < 0.001). All five salvaged patients had fistula formation in the absence of Crohn's disease. CONCLUSIONS: The overall rate of permanent pouch failure is low. The majority of failures were related to fistula formation associated with Crohn's disease or poor functional results. Pouches complicated by fistulas not associated with Crohn's disease can be salvaged with temporary rediversion.

Treatment of Hirschsprung's disease in the adult. Report of five cases.

PURPOSE: Between 1983 and 1991, five adult patients were diagnosed and treated for Hirschsprung's disease. Mean age was 37 (range, 13-45) years. Three patients had classic Hirschsprung's disease, and two had findings consistent with short segment disease. Each patient had a history of disabling, lifelong constipation. METHODS: Diagnosis was established with the aid of barium enema study, anorectal manometry, and tissue biopsy. RESULTS: Three patients with classic disease underwent resection of diseased bowel, rectal mucosectomy, and anastomosis between the ganglion-containing bowel and anus. All three patients had excellent functional improvement in the perioperative period. Two patients with findings consistent with short segment Hirschsprung's disease were treated by anorectal myectomy. Neither patient obtained lasting relief.

State of the defunctionalized sphincter in patients undergoing ileoanal pouch anastomosis.

PURPOSE: Our aim was to determine manometric status and functional outcome of the ileoanal pouch procedure in a subset of patients with defunctionalized anal sphincters as a result of long-term fecal diversion. METHODS: The anal manometric profiles of 12 patients defunctionalized for one year or more were compared with 26 patients with nondefunctionalized anal sphincters. Functional data were obtained from the Lahey Clinic Ileoanal Pouch Registry. RESULTS: Preoperative manometric data revealed a mean resting pressure of 91.5 mmHg in the nondefunctionalized group vs. 68.7 mmHg in the defunctionalized group; mean squeezing pressure was 171.7 mmHg (nondefunctionalized group) vs. 102.3 mmHg (defunctionalized group); and squeezing pressure volume was 1,283,000 mmHg3 (nondefunctionalized group) vs. 585,000 mmHg3 (defunctionalized group). Functionally both groups had a mean of 6.1 bowel movements in a 24-hour period and could defer defecation for a mean of 2 hours. Leakage occurred in 22 percent of the defunctionalized group and 17 percent of the nondefunctionalized group (P = 0.35). CONCLUSION: Despite physiologic perturbations, the long-term, defunctionalized anal sphincter can adequately support a restorative procedure without regard to timing of pouch creation.

Rectal mucosectomy in the treatment of giant rectal villous tumors.

PURPOSE: Rectal mucosectomy, a technique adapted from restorative proctocolectomy, has been used to treat large rectal villous tumors. We compared morbidity, tumor control, and functional outcome following rectal mucosectomy with the results of more conventional transanal excision and piecemeal snaring and fulguration in patients with large rectal villous tumors. METHODS: We retrospectively reviewed the charts of inpatients who had undergone transanal surgery for villous tumors. RESULTS: Between 1983 and 1993, rectal mucosectomy, transanal excision, and snaring and fulguration were performed, respectively, in 12, 26, and 23 patients with large rectal villous tumors. Tumors treated by rectal mucosectomy had a larger mean diameter (8.5 cm) than those treated by transanal excision or snaring and fulguration (4.5 cm and 4.2 cm, respectively; P < 0.0001, analysis of variance). After a mean follow-up of 47 months, incidence of tumor persistence was 17 percent following rectal mucosectomy, 20 percent following transanal excision, and 40 percent following snaring and fulguration (P = 0.04, chi-squared). Tumor recurrence was 8 percent after rectal mucosectomy compared with 36 and 44 percent, respectively, after transanal excision (P = 0.09, chi-squared) and snaring and fulguration (P = 0.04, chi-squared). Clinically significant postoperative bleeding did not occur after rectal mucosectomy; 17 percent of patients had persistent mild incontinence. CONCLUSIONS: Rectal mucosectomy for villous tumors, a new application of an established technique, is safe and associated with low rates of tumor persistence and recurrence. Rectal mucosectomy may result in mild incontinence and should be reserved for large or circumferential lesions. For smaller lesions, transanal excision results are more reliable tumor eradication than snaring and fulguration.

Biofeedback training is useful in fecal incontinence but disappointing in constipation.

BACKGROUND: Successful biofeedback therapy has been reported in the treatment of fecal incontinence and constipation. It is uncertain which groups of incontinent patients benefit from biofeedback, and our impression has been that biofeedback is more successful for incontinence than for constipation. PURPOSE: This study was designed to review the results of biofeedback therapy at the Lahey Clinic. METHODS: Biofeedback was performed using an eight-channel, water-perfused manometry system. Patients saw anal canal pressures as a color bar graph on a computer screen. Assessment after biofeedback was by manometry and by telephone interview with an independent researcher. RESULTS: Fifteen patients (13 women and 2 men) with incontinence underwent a mean of three (range, 1-7) biofeedback sessions. The cause was obstetric (four patients), postsurgical (five patients), and idiopathic (six patients). Complete resolution of symptoms was reported in four patients, considerable improvement in four patients, and some improvement in three patients. Manometry showed a mean increase of 15.3 (range, -3-30) mmHg in resting pressure and 35.7 (range, 13-57) mmHg in squeezing pressure after biofeedback. A successful outcome could not be predicted on the basis of cause, severity of incontinence, or initial manometry. Twelve patients (10 women and 2 men) with constipation underwent a mean of three (range, 1-14) biofeedback sessions. Each had manometric evidence of paradoxic nonrelaxing external sphincter or puborectalis muscle confirmed by defography or electromyography. All patients could be taught to relax their sphincter in response to bearing down. Despite this, only one patient reported resolution of symptoms, three patients had reduced straining, and three patients had some gain in insight. CONCLUSIONS: Biofeedback helped 73 percent of patients with fecal incontinence, and its use should be considered regardless of the cause or severity of incontinence or of results on initial manometry. In contrast, biofeedback directed at correcting paradoxic external sphincter contraction has been disappointing.

Fertility and sexual and gynecologic function after ileal pouch-anal anastomosis.

PURPOSE: This study was designed to determine the incidence of infertility, gynecologic problems, and sexual dysfunction after ileal pouch-anal anastomosis (IPAA). METHODS: A questionnaire was sent to 206 females who underwent pouch surgery at a single institution from 1980 through 1991. Response rate was 53 percent (110/206). The computerized registry of the 206 females undergoing IPAA at this institution was reviewed to add additional data. RESULTS: Mean age at pouch construction was 32 (range, 14-61) years. Mean time from pouch surgery to survey was 49 (range, 1-132) months. Fifty-seven females had 119 children before pouch surgery, and 23 children were born to 19 females after IPAA (5 vaginal deliveries, 18 Cesarean sections). Eighteen females experienced infertility after IPAA. Thirty patients had persistent dyspareunia. Pelvic cysts developed in 15 patients; 11 patients required surgery. CONCLUSIONS: Although childbirth appears safe, gynecologic problems, such as dyspareunia and formation of pelvic cysts, may be underestimated after IPAA. The effects of IPAA on fertility are still unknown.

Obstruction after ileal pouch-anal anastomosis: a preventable complication?

Small bowel obstruction is a common complication after ileal pouch-anal anastomosis. This review of 460 patients examines the frequency of small bowel obstruction and determines potential risk factors. The leading indication for ileal pouch-anal anastomosis was ulcerative colitis (83 percent). In 142 patients (31 percent), loop ileostomy was rotated 180 degrees to facilitate emptying of the ileostomy. Ninety-four patients (20 percent) had 109 episodes of obstruction. Obstruction occurred after creation of the pouch (40 episodes), closure of the ileostomy (29 episodes), or developed during the subsequent follow-up period (40 episodes). Operative intervention was required in 39 percent of the episodes (7 percent of all patients). At operation, the most common point of obstruction was at closure of the ileostomy (n = 22/42, 52 percent). In 16 of these patients, the ileostomy had been rotated. Multiple risk factors, including age, sex, primary diagnosis, surgeon incidence, pouch type, prior colectomy, steroid usage, stomal rotation, technique of closure of the ileostomy, and prior obstruction, were examined by univariate and multivariate analysis. Of all factors, only stomal rotation was statistically significant (P = 0.0005, chi-squared analysis). Rotation of the loop ileostomy during ileal pouch-anal anastomosis, although an apparent technical refinement, is unnecessary and predisposes to obstruction.

Alteration in intestinal permeability after ileal pouch-anal anastomosis.

PURPOSE: The physiologic changes that occur when the small bowel is used as a reservoir, as in the ileal pouch-anal anastomosis, are poorly understood. Alterations in bowel permeability, which may lead to bacterial translocation that could result in illness or dysfunction of the pouch, may be one such consequence of the pouch procedure. METHODS: Whole-bowel permeability was evaluated in patients with and without the pouch through the use of an orally consumed nonmetabolizable sugar clearance technique. Patients in whom the ileal pouch-anal anastomosis was performed for ulcerative colitis (17 patients) and patients with familial polyposis (7 patients) were compared with normal healthy volunteers (10 patients) and patients with ulcerative colitis with and without curative colectomy and ileostomy (6 and 5 patients, respectively). RESULTS: Measured by this technique, no differences were noted in bowel permeability between the volunteers and patients with ulcerative colitis, even after colectomy and ileostomy (1.7 +/- 0.4 in normal healthy volunteers, 1.8 +/- 0.5 in patients with ulcerative colitis without stoma, and 1.4 +/- 0.2 in patients with ulcerative colitis with ileostomy). The group of patients with an ileal reservoir, however, had a significantly increased index of measured bowel permeability (3.5 +/- 0.5 in patients with ulcerative colitis and 5.1 +/- 0.7 in patients with familial polyposis; P < 0.05 by analysis of variance compared with normal healthy volunteers and patients with ulcerative colitis with or without ileostomy). CONCLUSION: The exact site, cause, and consequence of this possible alteration of bowel permeability are unclear but appear to be related to the presence of the pouch and are not caused by the underlying pathologic diagnosis.

Long-term results of the ileoanal pouch procedure.

Many surgeons consider the ileoanal pouch procedure to be the procedure of choice for patients who require surgery for ulcerative colitis and familial adenomatous polyposis. To determine long-term results, 460 patients (mean +/- SD age, 31 +/- 9 years) who underwent the ileoanal pouch procedure from 1980 through 1991 were prospectively observed by computerized registry. The leading indication for operation was ulcerative colitis (n = 382; 83%). A J-shaped reservoir was created in 434 patients (94%). More than 5 years after ileostomy closure, the mean number of bowel movements was 5.8 +/- 2.2, and 13% of patients had leakage. Most patients (94%) were satisfied with their results. Sixteen patients (3.5%) required recreation of a permanent stoma for pouch failure. Complications (major and minor) occurred in 266 patients (58%) and included obstruction (n = 94; 20%), pouch fistula (n = 26; 6%), anastomotic stricture (n = 40; 9%), anastomosis separation (n = 14; 3%), and pouchitis (n = 83; 18%). Modifications in technique and increased operative experience have significantly decreased the incidence of obstruction (P = .05) and pouch-related complications (P = .004). Despite complications, long-term results are acceptable, and patient satisfaction remains high.

Sphincter-saving alternatives in the management of adenocarcinoma involving the distal rectum. 5-year follow-up results in 40 patients.

We reviewed the treatment outcome in 40 patients undergoing full-thickness local excision (seven patients) or electrocoagulation (33 patients) for adenocarcinoma of the rectum. Patients were followed up for a minimum of 5 years or until death (mean follow-up, 7.6 years). Twenty-two patients (55%) survived 5 years free of disease or were free of disease at the time of death due to other causes following local treatment. Eight (62%) of 13 patients with persistent or locally recurrent disease were successfully treated with additional local therapy, rectal resection, or combined radiation therapy and chemotherapy. Overall, 30 (75%) of 40 patients embarking on a program of local treatment for carcinoma of the rectum survived 5 years free of disease or were free of disease at the time of death due to other causes.

Anastomotic-vaginal fistula after colorectal surgery.

The most feared complication of anterior and low anterior resection is anastomotic dehiscence. Although most leakages remain clinically silent, some may lead to formation of a colovaginal fistula. At the Lahey Clinic Medical Center, the records of nine patients with colovaginal fistula as a complication of colorectal surgery were reviewed to determine clinical characteristics and optimal management. The mean age was 63.7 years (range, 47-72 years). The initial indications for surgery were carcinoma of the rectum (n = 4), diverticular disease (n = 3), and closure of the colostomy after Hartmann's procedure (n = 2). Hysterectomy had been performed earlier in seven patients (78 percent). The end-to-end anastomosis (EEA) stapling device was used in five patients, and four patients had a handsewn anastomosis. The fistula developed within 23 days after surgery and usually originated within 8 cm of the anal verge. Two patients underwent immediate diverting transverse colostomy. None of the seven patients who were initially managed medically had spontaneous closure of the fistula. High fistulas were successfully treated by colorectal resection in two patients, whereas low fistulas healed after transanal repair without colostomy in two patients. These results suggest that previous hysterectomy predisposes to development of a colovaginal fistula after colorectal surgery. Not all patients require fecal diversion. Colorectal resection for high fistulas and transanal repair for low fistulas appear to be viable options for treatment.

Crohn's disease and carcinoma: increasing justification for surveillance?

Carcinoma of the colon that arises in patients with Crohn's disease is being reported with increasing frequency. To help clarify the nature of this association, records of 25 patients with Crohn's disease and colorectal carcinoma seen from 1957 through 1989 were reviewed. One patient had leiomyosarcoma of the rectum, and two patients had the onset of Crohn's disease after the diagnosis and treatment of colorectal carcinoma. Therefore, 22 patients were available for complete retrospective analysis. The median age at diagnosis of Crohn's disease was 37 years (range, 15-67 years), and the median age at diagnosis of carcinoma was 54.5 years (range, 32-76 years). The median duration of symptoms preceding the discovery of colorectal carcinoma was 18.5 years (range, 0-32 years). Carcinoma arose in colonic segments with known Crohn's disease in 77 percent of patients, and six patients (27 percent) had associated colonic mucosal dysplasia. One lesion was classified as Dukes A, nine lesions were Dukes B, five lesions were Dukes C, and seven lesions were Dukes D. Patients with an onset of Crohn's disease before the age of 40 years had primarily Dukes C or D lesions and consequently poor survival. Most patients presented with nonspecific signs and symptoms, with nothing to distinguish the activity of the Crohn's disease from the presence of colorectal neoplasm. Younger patients with long-standing Crohn's disease should be considered for colonic surveillance to permit earlier diagnosis and treatment of potential colorectal carcinoma.

Factors predictive of persistent or recurrent Crohn's disease in excluded rectal segments.

The fate of the excluded rectal segment after surgery for Crohn's colitis remains poorly defined. To determine prognostic factors relating to the fate of the rectal segment, records of 47 patients who underwent creation of an excluded rectal segment were studied. Disease developed in 33 patients (70 percent) in the excluded rectal segment by five years; 24 patients (51 percent) had completion proctectomy by 2.4 years; and 9 patients (19 percent) retained a rectum with disease at a median follow-up period of five years (range, 2-13 years). At a median follow-up time of six years (range, 2-21 years), 14 patients were without clinical disease. The three groups were equivalent with respect to sex, duration of preoperative disease, indication for operation, distribution of disease, and histologic involvement of the proximal rectal margin. The median age of patients in the proctectomy group at diagnosis tended to be younger than that of patients with a retained excluded rectal segment (22, 30, and 31 years for patients having proctectomy, patients with a diseased excluded rectal segment, and patients with a normal excluded rectal segment, respectively). Neither initial involvement of the terminal ileum nor endoscopic inflammatory changes seen in the rectum predicted eventual disease of the excluded rectal segment. However, initial perianal disease complicating Crohn's colitis was predictive of persistent excluded rectal segment disease and often required proctectomy. Therefore, because the presence of perianal disease and Crohn's colitis predicts persistent or recurrent excluded rectal segment disease, primary total proctocolectomy or early completion proctectomy may be indicated in this subgroup of patients.

Indeterminate colitis predisposes to perineal complications after ileal pouch-anal anastomosis.

This study retrospectively evaluated 288 patients who had undergone ileal pouch-anal anastomosis to determine the incidence of perineal complications and to relate these findings to the pathologic diagnosis, with the goal of specifically clarifying the appropriate surgical management of patients with indeterminate colitis. Of these 288 patients, 235 patients (82 percent) had a diagnosis of chronic ulcerative colitis, 18 patients (6 percent) had indeterminate colitis, 6 patients (2 percent) had Crohn's disease, and 29 patients (10 percent) had familial polyposis. All complications occurred at least 6 months after closure of the stoma and required operative therapy. Of 18 patients with indeterminate colitis, 9 patients experienced complications (50 percent) vs. 8 of 235 patients with chronic ulcerative colitis (3 percent), a highly significant difference (P less 0.001). Furthermore, the risk of eventual ileostomy because of perineal complications was 0.4 percent in patients with chronic ulcerative colitis vs. 28 percent in patients with indeterminate colitis (P less than 0.001). We conclude that a diagnosis of indeterminate colitis predisposes the patient undergoing ileal pouch-anal anastomosis to perineal complications, with a resultant high chance of reservoir loss. Ileal pouch-anal anastomosis should be considered with caution in the patient with a diagnosis of indeterminate colitis.

Carcinoma and the ileal pouch-anal anastomosis.

Of 362 patients undergoing ileal pouch-anal anastomosis, 12 (five with chronic ulcerative colitis and seven with familial adenomatous polyposis) had 16 associated carcinomas. Incidental carcinoma was found in four patients who had undergone ileal pouch-anal anastomosis, six patients had known carcinoma, and carcinoma was suspected in two patients with high-grade dysplasia. No tumor was Stage C or D. After a median observation period of 24 months, no evidence of recurrence was documented. Data suggest that patients with carcinoma complicating chronic ulcerative colitis and familial adenomatous polyposis can safely undergo ileal pouch-anal anastomosis; however, it may be prudent to perform resection and later ileal pouch-anal anastomosis after a period of observation and appropriate adjuvant therapy because of the difficulty in intraoperative staging.