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Surgical management of ulcerative colitis.

As long as our understanding of ulcerative colitis is too limited to allow a more specific, disease-targeted treatment, surgery will play an important role in the management of these patients. Careful interdisciplinary evaluation and counselling of patients with ulcerative colitis will permit to achieve the goals on an individual basis with maximum safety. Restorative proctocolectomy with an IPAA has evolved as the procedure of choice among four basic surgical options because it appears to be safe and carries a low mortality. Although the associated morbidity is not negligible, functional results are generally good and patient satisfaction is high.

Colitis, Ulcerative↗

[State of the art: gastroenterologic surgery].

Microcirculation and molecular biology are the hottest topics in modern surgical research. In familial adenomatous polyposis the incidence of carcinoma can be assessed by the localisation of the PAC-gene mutation. Restorative proctocolectomy with ileoanal pouch represents the procedure of choice. The optimal age for the operation varies between 20 and 35 years according to the localisation of the mutation. RT-PCR directed to recently defined surface antigens allows for the sensitive detection of intraoperative tumor cell liberation. Due to tumor cell detection in the systemic circulation the perioperative administration of monoclonal antibodies must be advocated. A preciser definition of lymphogenic tumor spread underlines the importance of systematic lymphadenectomy in resection of the colon. The understanding of microcirculatory disorders has optimized surgical decision-making intra- and perioperatively: function of renal and hepatic microcirculation is a reliable parameter to predict graft quality already intraoperatively and to monitor therapeutic approaches to ischemia/reperfusion injury. Results in the therapy of acute pancreatitis could be improved by operating less and later. Analysis of pancreatic microcirculation resulted in an improvement of ICU-therapy in the early stages of the disease. Transplantation of the liver is limited to hepatocellular carcinoma when its localisation or the residual hepatic function after resection preclude curative excision. In addition liver transplantation should not be carried out in tumors larger than 5 cm or in patients with more than 3 tumor nodules. Liver resection for colorectal metastases is a standard procedure. A second resection of recurrent metastases is advocated since an identical median survival can be achieved compared to the primary resection (32 mo). The surgical treatment of non-colorectal liver metastases is under evaluation and should be restricted to oncological centers. Special aspects of backwashileitis in ulcerative colitis will be outlined concerning timing of colectomy, pouch construction, and follow-up.

Gastrointestinal Diseases↗

[Hereditary colorectal cancer associated with polyposis syndromes].

This study reviews different aspects of hereditary colorectal cancer associated with three polyposis syndromes: familial adenomatous polyposis, juvenile polyposis coli and Peutz-Jeghers syndrome. All these syndromes share some similarities: low incidence, autosomal dominant inheritance, genetic predisposition to colorectal cancer and/or other extracolonic cancers. Classical familial adenomatous polyposis is clinically defined by the presence of hundreds of adenomatous polyps in the colon and rectum, whereas less than 100 polyps are found in attenuated familial adenomatous polyposis. Without prophylactic colectomy, colorectal cancer develops inevitably by the age of 40. Restorative proctocolectomy with ileal anal-pouch anastomosis is the operation of choice in familial adenomatous polyposis. In juvenile polyposis coli, 50-200 hamartomatous polyps are found in the colon, rectum, stomach and small bowel. Life-time cumulative risk for colorectal cancer is estimated to be 50%. Prophylactic colectomy is required only in cases in which endoscopic surveillance is not able to control polyp development. Hereditary mixed polyposis syndrome is a variant form of juvenile polyposis coli, consisting of multiple mixed adenomatous, hyperplastic and hamartomatous polyps. Peutz-Jeghers syndrome is characterized by multiple hamartomatous polyps located in the small bowel, colon and stomach. Small bowel follow through and colonoscopy is advised for surveillance. Surgery is warranted only in cases of polyps larger than 1 cm. The causative genes of these syndromes have been cloned. Molecular genetic testing of affected and at-risk individuals is proposed in order to advise surveillance and management.

Adenomatous Polyposis Coli↗

Complications after ileal pouch-anal anastomosis.

Restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) is currently the procedure of choice for ulcerative colitis patients who require colectomy. Despite its wide acceptance, a variety of long-term complications of the procedure exist that can be severe and even lead to pouch excision. Pouchitis occurs in up to one half of patients after IPAA, but is usually well controlled with medical therapy. A small percentage of patients develop chronic persistent pouchitis, which often requires long-term medical therapy and may result in pouch failure. Fistulas and strictures can also complicate the pouch procedure. In general, patients with Crohn's disease are not usually offered IPAA, because recurrence of disease, fistulas, abscesses, and strictures may lead to a higher incidence of pouch failure. Some ulcerative colitis patients develop complications after IPAA and are subsequently diagnosed with Crohn's disease. These patients may develop refractory fistulas, strictures, and extraintestinal manifestations of inflammatory bowel disease. Neoplastic transformation of the pelvic pouch has also been reported, particularly in patients with chronic pouchitis. Thorough follow-up and endoscopic surveillance with biopsies of the ileal pouch are therefore recommended.

Anastomosis, Surgical↗

Polipoza adenomatoasă familială--care operaţie? [Familial adenomatous polyposis--which operation?].

Familial adenomatous polyposis (FAP) syndromes are well recognized entities that benefit from surgical treatment which should not be delayed. Screening of first degree relatives is important. The aim of removing the colorectal mucosa with significant potential of malignant transformation can be achieved by means of three distinct procedures: panproctocolectomy and ileostomy, subtotal colectomy with ileorectal anastomosis, restorative proctocolectomy with ileoanal anastomosis. In a series of eight patients with FAP we performed mostly subtotal colectomy with ileorectal anastomosis. Of five patients who underwent a form of subtotal colonic resection, one was lost from follow up and two developed carcinoma in the remaining rectal mucosa, which necessitated completion of the resection with proctectomy and permanent ileostomy. Nevertheless, in the increasing number of patients amenable to regular outpatient supervision, there are strong points for recommending sphincter-saving operations.

Adenomatous Polyposis Coli↗

Endoscopical and histological aspects of inflammatory changes in J-pouch mucosa.

The aim of the study was endoscopic and histological evaluation of mucosal inflammatory changes in the ileal reservoir after restorative proctocolectomy with ileoanal anastomosis (IPAA) because of FAP (27 patients) and ulcerative colitis (34 patients). Endoscopic symptoms of pouchitis (edema, granularity, contact bleeding, loss of vascular pattern, mucus, erosions) were found in 13 patients (4 were operated on because of FAP, 9 because of UC). A statistically significant difference (p < 0.0001) was found between endoscopic inflammatory index in UC (4.0 +/- 0.5) and in FAP (1.00 +/- 0.00). The endoscopic changes were correlated with the Moskowitz histological classification, in 12 acute score and in 6 chronic score were above 4 points. In 18 patients histological diagnosis of pouchitis was made (5 in FAP, 13 in UC). Acute pouchitis occurred in 12 patients and chronic in 6. In one patient histological changes resembled Crohn's disease. Regular endoscopic control with histological examination and verification of inflammatory lesions in the ileal reservoir is necessary.

Adenomatous Polyposis Coli↗

Ileal pouch-anal anastomosis operation in children versus adults.

BACKGROUND AND AIMS: To evaluate early results of elective ileal pouch-anal operations in children under the age of fifteen as compared to adult patients operated during the same period by the same surgical team. MATERIAL AND METHODS: Eighty-two patients with ulcerative colitis underwent restorative proctocolectomy during a six-year period, 1991-1996. Twelve of them (16%) were under the age of 15 years. Peroperative and short term postoperative morbidity were compared between the two groups. RESULTS: Covering loop ileostomy was performed on one patient in the juvenile group and 5 (7%) in the adult group. There were no significant differences in postoperative complications between the two groups. Eight (11%) leakages occurred in the adult group as compared to none in the juveniles. The children had a higher incidence of unexplained postoperative fever (50% versus 21%). Seven adults (10%) but no children underwent re-operation for these early complications. CONCLUSIONS: It would appear that a team of experienced gastroenterologic surgeons can operate children as well as adults for ulcerative colitis with comparable early results without additional morbidity.

Adolescent↗

[Immediate and long-term results in ileoanastomosis with reservoir in 335 consecutive cases].

Restorative proctocolectomy with ileal pouch anal anastomosis is the first choice procedure for the treatment of ulcerative colitis and familial adenomatous polyposis. The introduction of the stapled technique has shortened the duration of the procedure and reduced the complication rates. Data on 335 consecutive patients undergoing ileal pouch anal anastomosis for ulcerative colitis (277 pts), Indeterminate colitis (20 pts) and familial adenomatous polyposis (38 pts) between 1984 and 1998 were prospectively collected. Parameters evaluated included diagnosis, surgical technique, functional outcome, early and late complications and their management and results. Twenty-nine patients (8.6%) presented with pelvic sepsis. Twelve patients (3.5%) experienced late perianal fistulas. The pouch failure rate was 3.4%. Six patients required a re-do pouch procedure, with 75.9% preservation of sphincter function. No correlation was found between complication rates and diagnosis. The mean number of stools was 5.2/24 h. The study confirmed the safety and effectiveness of the procedure. In particular, morbidity rates are comparable to those of major abdominal procedures and the long-term functional results are satisfactory. However, a number of technical aspects, such as the anastomosis technique, the need for temporary ileostomy and the treatment of indeterminate colitis, still remain controversial.

Adenomatous Polyposis Coli↗

[Functional outcome after reservoir surgery in ulcerative colitis].

BACKGROUND: The aim of this study was to examine whether the functional result of restorative proctocolectomy for ulcerative colitis deteriorated by postponing follow-up from three to eight years after pouch surgery. MATERIAL AND METHODS: All patients (n = 58) operated for ulcerative colitis from 1984-97, who still had intact reservoir, responded to a questionnaire on defecating pattern, stool leakage, perianal irritation, urinary function, workload, sexual life, social life, and the patient's opinion on outcome. The results for the first 29 patients were compared to last 29 patients operated. Median follow-up was eight for the first 29 patients and three years for the last 29. RESULTS: There were no significant difference in functional outcome and patient opinion after pouch surgery between the first 29 and last 29 patients (n = 58). Respective figures for median 24-hour stool frequency were 7.0 and 6.3, leakage of stool 48% and 38%, ability to defer defecation 86% and 97%, perianal irritation 59% and 48%, use of antidiarrhoeal medication 55% and 52%, and wearing of pad 28% and 38%. Sexual life (n = 57) had improved for 11 (19%) and deteriorated for seven patients (12%). Potency was reduced in five (15%) and ejaculation in four patients (12%), one of whom (3%) had retrograde ejaculation. Three (5%) had received a disability pension and five (9%) had reduced workload. After the pouch operation 48 (91%) felt better and three (6%) felt worse because of frequent bowel emptying (n = 3), perianal eczema (n = 1) and pouch fistula (n = 1). INTERPRETATION: The results do not demonstrate any significant deterioration of functional outcome after pouch surgery for ulcerative colitis by postoperative increase of follow-up from three to eight years.

Adolescent↗

[Surgical treatment of ulcerative rectocolitis: technical aspects of total restorative proctocolitis with S pouch].

Restorative proctocolectomy with an ileal-pouch-anal anastomosis seems to be the treatment of choice for ulcerative colitis. The aim of this study was to discuss a number of technical and functional aspects of the procedure that still appear to be controversial such as the shape of the pouch, the mucosectomy and the type of anastomosis. The authors report on their experience with the surgical treatment of ulcerative colitis with an "S" pouch. The technique reported, however, differs from the original method proposed by Parks and Nicholls in 1978 and the reasons for this surgical choice are discussed. A six-year experience (1993-1999) regarding 35 patients undergoing this approach is reported. No perioperative deaths were observed. The early and long-term complication rates were 8.5% and 11.4%, respectively. The average number of daily evacuations was 4. Mucosectomy affords complete resolution of the disease, while the particular shape of the pouch guarantees good functional results.

Adolescent↗

[Hereditary nonpolyposis colorectal carcinoma (HNPCC): surgical aspects].

1-5% of all patients presenting with colorectal cancer, have an underlying genetic predisposition with an autosomal dominant of pattern of inheritance. Recently the underlying molecular genetic pathway of this syndrome known as HNPCC (hereditary nonpolyposis colorectal cancer) has been characterized: the predisposition is due to a pathogenic mutation in one of the DNA mismatch-repair genes. Colorectal cancers are the leading characterisitic of the syndrom and are frequently encountered at a young age of onset. However, endometrial cancer, stomach cancer, small bowel cancer, urinary tract cancer and skin cancer among others are also inherent to the syndrome. Based on the identification of the underlying molecular genetic pathway, predictive testing has become an option. Once the family-specific underlying pathogenic mutation in one of the DNA mismatch-repair genes has been identified in a proband, at-risk family members may be offered DNA testing with an unequivocal answer to if or if not they have inherited the increased cancer susceptibility. Multiple facets of family DNA testing require a multidisciplinary approach integrating clinicians, geneticists, psychologists, molecular biologist and pathologists. Mutations are identified in a rate of 50-60% of families complying with the Amsterdam criteria for HNPCC. The incidence of recta cancer in HNPCC has as yet not clearly been defined, due to a lack of unequivocal data. In a retrospective analysis (submitted) rectal cancers were encountered in 30% of the HNPCC patients. Half of the patients affected developed metachronous colon cancers. It is essential to address the issue of prophylactic surgery in HNPCC patients presenting with colorectal cancer: Prospective data is required in order to decide which of the options is more beneficial for the HNPCC patient presenting with his first colorectal primary: subtotal colectomy and ileorectal anastomosis versus restorative proctocolectomy. It is evident that apart from the more clinical data quality of life data must be evaluated in this study.

Colectomy↗

Clinico-pathological follow-up after total colectomy and straight ileo-anal anastomosis done for ulcerative colitis.

BACKGROUND: Restorative proctocolectomy is used as surgical treatment for ulcerative colitis. We have earlier documented adaptative changes in the terminal ileum after total colectomy, and straight ileo-anal anastomosis. AIM: To correlate the morphologic and functional changes in the ileal mucosa after total colectomy and hand-sewn straight ileo-anal anastomosis for ulcerative colitis. METHODS: Thirty consecutive patients (age range 15-50 years, 24 men) who had undergone total colectomy, rectal mucosectomy and hand-sewn straight ileo-anal anastomosis for ulcerative colitis were included in the study. These patients were followed up at 3-monthly intervals following surgery for two years and later once every year for a median duration of 9.5 (range 1-17) years. The clinical parameters studied were weight gain, frequency of stools, nature of stools, nocturnal stool frequency and need for antidiarrheal drugs. At each follow-up visit they were subjected to per rectal ileoscopy with ileal biopsy and barium enema. Ileal biopsy was analyzed histologically and histochemically. RESULTS: The clinical features improved over time, with average weight gain of 5 (range 1-7) Kg at one year. Frequency of stools decreased from 8-10 per day to 2-3 per day. The stools became semisolid and there was no need for antidiarrheal drug by the end of one year. All the patients showed adaptative changes in the ileum. The ileal mucosa was completely transformed into colonic type by the end of one year, colonoscopically (spacious lumen characteristic of colon), radiologically (disappearance of ileal characteristics with rectosigmoid-like appearance), histologically (blunting of villi with increase in goblet cells), and histochemically (sialomucin pattern to sulfomucin pattern). CONCLUSIONS: The ileum undergoes adaptative changes with corresponding improvement of clinical parameters over time, after proctocolectomy and ileoanal anastomosis for ulcerative colitis.

Adult↗

[Should liver biopsy be systematic during surgery for ulcerative colitis?].

AIM: The aim of this study was to evaluate the prevalence of primary sclerosing cholangitis and other histological liver abnormalities in patients operated on for ulcerative colitis and to discuss the advantages of performing a systematic liver biopsy during surgery. METHODS: From 1996 to 2001, 21 consecutive patients underwent a restorative proctocolectomy or a reoperation after proctocolectomy for ulcerative colitis. These patients systematically underwent liver biopsy during the procedure. RESULTS: One patient presented with primary sclerosing cholangitis (4.7%). This patient was clinically and biologically asymptomatic. Four patients had steatosis, 8 had non specific inflammation such as small duct cholangitis and 8 had normal liver biopsy. As a result medical treatment was adapted and close surveillance of the live was begun. CONCLUSION: Peroperative liver biopsy identify primary sclerosing cholangitis or other liver diseases in an early diagnosis and help evaluate their stage in order to start appropriate treatment.

Adolescent↗

[Familial adenomatous polyposis: what is new for the clinician?].

Familial adenomatous polyposis (FAP) is a rare autosomal-dominant disease characterized by the development of more than 100 colorectal adenomatous polyps in young adults. In the absence of surgical intervention, colorectal cancer ineluctably develops in all affected patients. Recent progress in the isolation of the gene responsible for the disease allows to detect gene carriers before they present with symptoms attributable to polyps. Moreover, the presence of four or more lesions of congenital hypertrophy of the retinal pigment epithelium is an extracolonic manifestation of FAP allowing presymptomatic screening of this disease. An effective screening programme combined with the elaboration of a registry for FAP and prophylactic colectomy should reduce mortality related to colorectal cancer. Two other extracolonic manifestations of FAP remain major causes of death: abdominal desmoid tumors and duodenal adenocarcinoma. At this time, no effective medical or surgical therapy has been found to cure these lesions. Restorative proctocolectomy with ileal reservoir is another major advance. This procedure is now regarded as the treatment of choice for patients with FAP because radical removal of all premalignant colorectal mucosa eliminates the risk of subsequent development of a colorectal adenocarcinoma.

Adenomatous Polyposis Coli↗

[Familial adenomatous polyposis: long term follow-up of patients after surgical treatment. Prognostic factors].

The purposes of this study are: 1) to evaluate if recent progresses (knowledge of natural history, genetic diagnosis and surgical treatment) have an impact upon the long term follow up of familial adenomatous polyposis (FAP); 2) to assess the prognosis factors that are relative to recent progresses in diagnosis and treatment procedures. A retrospective study of 51 cases was carried out in July 2002 to analyse the following characteristics: phenotype, treatment, operative mortality and morbidity, late complications, especially rectal stump cancer after ileo-rectal anastomosis, duodenal adenomatosis and desmoid tumors. Twenty seven men and 24 women underwent surgery: 11 colo-rectal cancers were present at first step. Initial surgical procedures included 39 total colectomies with ileo-rectal anastomosis (IRA), 6 coloproctectomies with ileo-anal anastomosis (IAA) and 6 coloproctectomies with permanent ileostomy. Operative mortality was nil. Operative morbidity affected 11 patients. The rectum had to be secondary removed in 11 patients with convert in IAA. Duodenal adenomatosis required surgery in 10 patients: 5 surgical local excisions, 4 duodenopancreatectomies and 1 palliative by-pass. Six desmoid tumors were noted during the follow-up. On the whole 3 patients were lost of sight. Nine patients died (19.1%), 4 deaths were in relation with the disease: 1 rectal cancer, 2 duodenal cancers, 1 desmoid tumor necrosis. At the end of the follow up (mean duration: 17 years) 26 IRA and 17 IAA are present with good functional results. This study, according to already published data, suggests that today the risk of death related to colorectal cancer is becoming lower than the risk of death from duodenal cancer and desmoid tumor evolution, particularly since the introduction of the restorative proctocolectomy. The genetic diagnosis is useful in order to determine the choice of surgical procedures.

Adenomatous Polyposis Coli↗

Diagnostic difficulties in pouchitis--experience from a single institution.

The aim of the study was to analyze the clinical, endoscopic and histological signs of pouchitis in patients operated on because of ulcerative colitis (UC) and familial adenomatous polyposis (FAP). Between 1984 and 2002, the Department of Surgery carried out 218 restorative proctocolectomies in patients with UC and 120 in FAP patients. The follow-up to assess the intensity of inflammatory changes included 110 patients: 72 UC and 38 FAP. During the endoscopic examination, samples were taken for routine histological examinations. The histological assessment was based on the so-called Moskwitz's Histological System. Acute pouchitis is diagnosed if the score is > or =4, and chronic condition--if the score is > or =4. We used a modified PDAI scale to analyze the symptoms, taking into account the microscopically detectable histological features of chronic pouchitis. The clinical symptoms of pouchitis were revealed in 28 patients (25.5%), endoscopic symptoms in 36 patients (32.7%), and histological parameters in 48 patients (28-58.3% with > or =4 score of acute pouchitis and 20-41.7% with > or =4 score of chronic pouchitis). The signs of acute and chronic pouchitis were evident in endoscopy and histology, with no clinical features observed in many cases. Patients with symptoms of chronic pouchitis require a continuous follow-up.

Adenomatous Polyposis Coli↗

Familial polyposis coli: clinical manifestations, evaluation, management and treatment.

Familial adenomatous polyposis (FAP) is an autosomal dominant, hereditary colon cancer syndrome that is characterized by the presence of innumerable adenomatous polyps in the colon and rectum. Gardner's syndrome is a variant of FAP, which in addition to the colonic polyps, also presents extracolonic manifestations, including desmoid tumors, osteomas, epidermoid cysts, various soft tissue tumors, and a predisposition to thyroid and periampullary cancers. Mutations of the APC gene are thought to be responsible for the development of FAP, and the location of the mutation on the gene is thought to influence the nature of the extracolonic manifestations that a given patient might develop. Though patients are often asymptomatic, bleeding, diarrhea, abdominal pain and mucous discharge frequently occur. Diagnostic tools include genetic testing, endoscopy, and monitoring for extra-intestinal manifestations. Currently, surgery is the only effective means of preventing progression to colorectal carcinoma. Restorative proctocolectomy with ileal pouch anal anastomosis (RPC/IPAA) with mucosectomy is the preferred surgical procedure, since it attempts to eliminate all colorectal mucosa without the need for an ostomy. Periampullary carcinoma and intra-abdominal desmoid tumors are a significant cause of morbidity and mortality in these patients after colectomy. Frequent endoscopy is needed to prevent the former, while there is no definitive treatment available yet for the latter. The following article presents a case and reviews the evaluation, management and treatment of Gardner's syndrome.

Adenomatous Polyposis Coli↗

[Clinical experiences of five cases with ulcerative colitis and recto- or anovaginal fistula].

Clinical aspects, treatment and outcome of five patients with ulcerative colitis recto- or anovaginal fistula and were studied retrospectively. All patients had total colitis (relapse and remission type) and more than a 5 year history of ulcerative colitis. They all had anorectal complications, such as periproctal abscess, stenosis of fistula. Four patients had total colectomy with an ileal pouch anal canal anastomosis for intractability or dysplasia. One was treated conservatively. Complete closure of fistula was obtained in two patients;in one patient rectum was resected below the fistula and in one patient defect of the vaginal posterior wall was reconstructed by using a gluteal fold flap following colectomy. Recto- or anovaginal fistula complicating ulcerative colitis is rare but may occur in the patients with severe rectal inflammation and they can be managed by restorative proctocolectomy with an ileal pouch anal or anal canal anastomosis.

Adult↗