OBJECTIVE A subgroup of patients with intractable constipation has persistent dilatation of the bowel, which in the absence of an organic cause is termed idiopathic megabowel (IMB). The aim of this systematic review was to evaluate the published outcome data of surgical procedures for IMB in adults. METHODS Electronic searches of the MEDLINE (PubMed) database, Cochrane Library, EMBase, and Science Citation Index were performed. Only peer-reviewed articles of surgery for IMB published in the English language were evaluated. Studies of all surgical procedures were included, providing they were performed on 3 or more patients, and overall success rates were documented. Studies were critically appraised in terms of design and methodology, inclusion criteria, success, mortality and morbidity rates, and functional outcomes. RESULTS A total of 27 suitable studies were identified, all evidence was low quality obtained from case series, and there were no comparative studies. The studies involved small numbers of patients (median 12, range 3-50), without long-term follow-up (median 3 years, range 0.5-7). Inclusion of subjects, methods of data acquisition, and reporting of outcomes were extremely variable. Subtotal colectomy was successful in 71.1%(0%-100%) but was associated with significant morbidity related to bowel obstruction (14.5%, range 0%-29%). Segmental resection was successful in 48.4%(12.5%-100%), and recurrent symptoms were common (23.8%). Rectal procedures achieved a successful outcome in 71% to 87% of patients. Proctectomy, the Duhamel, and pull-through procedures were associated with significant mortality (3%-25%) and morbidity (6%-29%). Vertical reduction rectoplasty (VRR) offered promising short-term success (83%). Pelvic-floor procedures were associated with poor outcomes. A stoma provided a safe alternative but was only effective in 65% of cases. CONCLUSIONS Outcome data of surgery for IMB must be interpreted with extreme caution due to limitations of included studies. Recommendations based on firm evidence cannot be given, although colectomy appears to be the optimum procedure in patients with a nondilated rectum, restorative proctocolectomy the most suitable in those with dilatation of the colon and rectum, and VRR in those patients with dilatation confined to the rectum. Appropriately designed studies are required to make valid comparisons of the different procedures available.

The outcome in 40 patients who underwent colectomy for idiopathic megacolon and megarectum over an 18 year period was evaluated. All patients had a radiologically dilated bowel and a bowel frequency of less than two per week. Twenty two patients had a caecorectal anastomosis, 11 had an ileorectal anastomosis (including one with a previous caecorectal anastomosis and four with a previous sigmoid resection), and seven had a sigmoid resection. The mean (range) age at operation was 35 (17-69) years. All three operations resulted in a normal bowel frequency in more than 80% of patients but no patient with an ileorectal anastomosis experienced recurrent constipation. Thirty four patients experienced pain preoperatively and this was still present in 14 patients postoperatively. One patient died and four required subsequent laparotomy for bowel obstruction. The functional outcome in patients with dilatation of the whole colon and in those with dilatation of the left colon did not differ. Subsequent surgery for constipation was performed in three patients. Colectomy offers good results with few complications in the treatment of idiopathic megacolon, and an ileorectal anastomosis is the preferred operation.

BACKGROUND. The choice between ileal pouch-anal anastomosis (IPAA) and ileorectal anastomosis (IRA) in the treatment of patients with familial adenomatous polyposis remains controversial. The aims of this study were to assess our 10-year experience with proctocolectomy, endoanal mucosectomy, construction of an ileal reservoir pouch, and IPAA in a series of 171 patients with familial adenomatous polyposis and to compare the functional results after IPAA with those after IRA. METHODS. Data from patients treated by IPAA at one institution were prospectively accumulated from October 1983 to October 1993. Medical records of 171 consecutive patients were studied regarding morbidity and functional results. These functional results were compared with those of a series of 23 patients who underwent IRA at the same institution. RESULTS. One patient (0.6%) died after operation. Sixty-two patients (36%) had concomitant colorectal carcinoma, 36 of which tumors were invasive (15 stage A, 13 stage B, and 8 stage C). Forty-six patients (27%) had at least one postoperative complication, with 14 patients requiring reoperation (8%). Twenty-six patients (15%) had obstruction. Seven patients (4%) had pelvic sepsis, and one had transient impotence (0.6%). Only two patients (1%) had a typical episode of pouchitis. The mean follow-up was 29 months (range, 3 to 100 months); 101 patients were monitored for more than 1 year. Little difference was noted between bowel function after IRA and that after IPAA. The mean daytime stool frequency after IPAA was 4.2 with 26% of patients having an average of 1 bowel movement at nighttime, compared with a stool frequency of 3.0 and 13% of patients having night evacuation after IRA. Daytime continence was normal for 98% of patients after IPAA and for all the patients after IRA. Nighttime continence was normal in 96% and 98% of patients, respectively. CONCLUSIONS. Morbidity and functional results after IPAA for familial adenomatous polyposis do not differ from those reported after IRA. For this reason and because of the risk of rectal cancer after ileorectal anastomosis, IPAA with endoanal mucosectomy is our first choice in the treatment of patients with familial adenomatous polyposis.

Eighty-three consecutive patients (38 men, 45 women) underwent colectomy and ileorectal anastomosis (IRA) for Crohn's colitis between 1960 and 1988. The mean age at the time of IRA was 28.5 years after a mean interval of four years from diagnosis. At the time of IRA, 31 patients had proctitis, while 25 had perianal disease. Two patients died postoperatively. Postoperative complications appeared in 21 cases (25.3%) including 7 anastomotic leaks (13.2%). Leakage did not imply IRA compromise and the diverting ileostomy did not decrease the risk of preservation of the ileorectal anastomosis. With a mean follow-up of 8 years after IRA, among the 81 surviving patients, it was necessary to retain the stomy in five, 24 required exclusion or excision of their IRA (10 defunctioning ileostomies, 14 proctectomies) and 52 still had a functioning IRA at follow-up (64.2%). Among the 43 recurrences (53%), 21 underwent reoperation. The mean interval between IRA and recurrence was 2.2 years. The cumulative rate of recurrence reached 47% at 5 years and 57% at 10 years. Fifty percent of the patients still had a functioning IRA and were satisfied. Preoperative ileal lesions affected the functional results of the IRA and the recurrence rate. Development of ileal, rectal or anal disease after IRA significantly increased the risk of exclusion of the rectum but did not require suppression of anal function. Patients under 30 years of age or patients suffering for more than 5 years had poorer functional results and more frequent reoperations at 5 years. Rectal preservation after IRA may be proposed with success to patients with a healthy rectum or with minimal or moderate proctitis, even if there is perianal disease that could be safely treated before IRA. In this last setting, the patient has to be informed of the risk of rectal preservation and the possible risk of requiring ulterior proctectomy.

When conducted as a curative procedure, for tumors with a lower pole at least 7 cm from the anal margin, Babcock's operation can avoid the need for a definitive abdominal colostomy in some patients, without compromising the chances of survival. Postoperative care is often longer and more difficult than after abdomino-perineal amputation, but immediate mortality and long-term survival appear to be totally comparable. Functional results are good in most cases, and though some hygienic and dietary restrictions are often necessary, these constitute a more minor handicap than that of an abdominal colostomy. On the contrary, Babcocks operation should not be used as a palliative measure, as important restrictive postoperative conditions on a patient with a short survival prognosis, to obtain a functional result after several months when he will not be able to benefit from it, appears to be unwarranted.

To evaluate the role of CT colonography (CTC) in the follow-up of patients having received partial colectomy for colorectal cancer. CTC was performed in 72 subjects with history of partial colectomy for colorectal cancer. Colectomy had been performed in the right colon (n = 18), descending colon (n = 15), sigmoid colon (n = 21), and rectum (n = 18). Patients underwent CTC following incomplete conventional colonoscopy due to intolerance to endoscope insertion or luminal stenosis. In 70 cases pneumocolon was obtained through a rectal tube, and in 2 cases through a cutaneous anastomosis. CTC datasets were analyzed in combined 2D and 3D mode. All patients in whom CTC was suggestive for or raised the suspicion of disease recurrence underwent colonoscopy in sedation for confirmation of CTC findings. CTC detected 7 cases of anastomotic stenosis. In 6/7 patients the stenosis was located in the sigmoid colon and in 1/7 patients at the level of the ileo-colic junction in the transverse colon. Out of these cases, four were fibrotic and three were neoplastic stenoses. In none of these cases was the CT appearance of the stenoses specific for disease recurrence, and conventional colonoscopy together with biopsy was necessary in order to characterize such findings. However, sensitivity of CTC in detecting anastomotic stenosis was 100%(7/7). One colonic mass (5 cm largest diameter) was detected in one case at the level of the proximal transverse colon in a patient with left colectomy performed 2 years before. The study of the residual colon showed 3 polyps in three patients (8, 6, and 5 mm, respectively). All CT findings were confirmed and characterized by conventional colonoscopy. In all cases the residual colon was entirely visualized by CTC with a completion rate of 100%. CTC is a feasible and minimally invasive method for full exploration of the colon after surgical resection allowing detection of cancer recurrence, metachronous disease, and distant metastases in one single study, and represents a valid alternative to conventional colonoscopy in this patient population.

BACKGROUND The aim of this study is to explore the long-term outcome and the colon and sphincter function in children undergoing the Swenson's operation for Hirschsprung's disease (HD). METHODS Three hundred forty-six children (266 males and 80 females) undergoing Swenson's operation for HD for 8 to 20 years were followed up. Barium enema and defecography, total and segmental colonic transit time with the simplified radioopaque markers, and the anorectal vector manometry were used. RESULTS Stooling patterns were fair in most patients, and the functional examinations were abnormal in few cases. CONCLUSIONS The long-term outcome is satisfactory in most of the children undergoing the Swenson's operation for HD, but it is still not as good as what the surgeons have expected. This is probably because of the abnormal colonic motility and/or the dysfunctions of the internal anal sphincter.

BACKGROUND: The purpose of this study was to evaluate the technical difficulties and complications of a transanal pull-through for Hirschsprung's disease. MATERIAL AND METHODS: This report was based on a multicentric retrospective study of 65 cases. Pull-through procedures were transanal Swenson or Soave procedures in 26 and 39 cases, respectively. RESULTS: Evaluation of the aganglionic level, peri-rectal dissection, and anastomosis were the three steps in the procedure where surgeons encountered difficulties. Such difficulties led to serious complications in 3 cases. A patient with a colon biopsy before the pull-through procedure had a postoperative pneumoperitoneum requiring a second laparoscopy for suture and washing. Another patient had peritonitis due to anastomotic leakage. Finally, a difficult rectal dissection in a neonate led to a urethral injury requiring secondary urethral repair. Only 41 of the 65 patients had no abdominal scars (63 %). CONCLUSION: We considered the transanal pull-through for Hirschsprung's disease to be a reliable technique. Nevertheless, it requires an urethral stent, precise dissection, careful anastomosis and selected indications in order to avoid major complications.

OBJECTIVE To investigate the diagnosis and surgical treatment of adult Hirschsprung disease (AHD). METHODS Clinical data of 10 patients with AHD undergoing operation from May 1985 to May 2005 were analyzed retrospectively. RESULTS There were 7 males and 3 females with an age ranged from 14 to 40 years. All the cases had constipation, and were diagnosed by barium enema. Aganglionosis was located in distal sigmoid and rectum in 2 cases, in rectum in 7 cases, unknown in one case. Colostomy was performed in one, Ikeda s operation in 6, Rehbein operation in two,modified Swenson operation in one. After radical operation,7 patients had excellent continence function, one had good function, one had poor function. CONCLUSIONS The diagnosis of adult HD mainly depends on the history of constipation, barium enema and manometry examination. The pull-through procedures are effective surgical treatments for adult HD. The operation type should be selected individually.

OBJECTIVE To investigate the feasibility and short- term effect of injecting botulinum toxin A through anorectum to treat Hirschsprung disease. METHODS Eight cases with Hirschsprung disease were proven pathologically or diagnosed by barium enema. At the clock point of 3,6,9 under supine posture,a total dose of 1.5 U/ kg of botulinum toxin A was injected into the internal anal sphincter and the submucosa of rectum. The pressure of anus- rectum was measured before operation and 1 month,1 year after operation,respectively. Barium enema examination was carried out within 3-6 months after operation,then once a year during followed- up. RESULTS All of the patients recovered well without operative or postoperative complications. In the first year,all patients could defecate spontaneously without constipation and abdominal distention. Three cases had constipation again and slight abdominal distention 1 year after operation,but the symptoms were relieved after anus dilatation. The resting pressure of anus- rectum was lower 1 month after operation than that before operation (P< 0.01) in 8 cases,and still lower 1 year after operation (P< 0.05)in 6 cases. Barium enema examination within 3-6 months after operation revealed that the dilated colon shrank. CONCLUSION Botulinum toxin A injection is a new,feasible and safe method for Hirschsprung disease,but the long- term efficacy needs to be further studied.

AIM To assess the effectiveness of and complications associated with metallic stent placement for treatment of anastomotic colonic strictures. METHODS A 46-year-old man undergoing two procedures of surgery for perforation of descending colon due to a traffic accident presented with pain, abdominal distention, and inability to defecate. Single-contrast barium enema radiograph showed a severe stenosis in the region of surgical anastomosis and the patient was too weak to accept another laparotomy. Under fluoroscopic and endoscopic guidance, we placed two metallic stents in the stenosis site of the anastomosis of the patient with anastomotic colonic strictures. RESULTS In this case of postsurgical stenosis, the first stent relieved the symptoms of obstruction, but stent migration happened on the next day so an additional stent was required to deal with the stricture and relieve the symptoms. CONCLUSION This case confirms that metallic stenting may represent an effective treatment for anastomotic colonic strictures in the absence of other therapeutic alternatives.

BACKGROUND/PURPOSE The authors analyzed the results of a modified entirely mechanical Duhamel pull-through for the treatment of Hirschsprung' disease or type B intestinal neuronal dysplasia. The aim of the follow-up was to evaluate results of a Duhamel procedure entirely performed with the use of staplers. METHODS Fifty-six patients were followed up to detect complications and outcome. Patients were evaluated clinically, and, when symptoms were present, a suction biopsy with histochemical analysis, an x-ray contrast enema, or an endoscopy was performed. RESULTS Early complications developed in 4 cases (7%) including a case of leakage (1.8%). No patient experienced recurrence. During a mean follow-up of 49 months, further complications occurred in 16 other patients: 4 adhesive obstructions requiring surgery, 3 strictures, 5 chronic bleeding, 4 fecaloma formation, 2 urinary problems, and 13 postoperative enterocolitis. Four patients (7%) had more than 3 passages of liquid stools per day, and 3 of them who had an ileal pull-through had constant soiling. Thirty-seven children (68.5%) had 1 to 2 bowel movements per day and were continent. Seven (13%) had less than 3 bowel movements per week. Three children who underwent total colectomy were incontinent (5.5%). Three more children (5.5%) have not yet reached the age for continence. Forty-nine parents (87%) judged the outcome satisfactory. CONCLUSIONS Follow-up of mechanical Duhamel pull-through showed an acceptable long-term outcome. An increased risk of incontinence is present in case of ileal pull-through, whereas constipation is more frequent after partial colon resection, regardless of the type of intestinal neuronal malformation.

The experience of the Pediatric Surgical Service of the Materno Infantil University Hospital from Málaga on surgical treatment of the Hirschsprung disease by means of a modified technique of Swenson and Rehbein operations is presented. MATERIAL AND METHODS: Between 1992 and 2001 25 patients were operated of a rectosigmoidectomy. Of them, 23 were diagnosed of Hirschsprung's disease, one suffered a rectal angiodisplasia and the other one presented with a rectal stenosis secondary to a previous rectosigmoidectomy. In all of them a transabdominal rectosigmoidectomy with coloanal end to end anastomosis by means of a circular intraluminal stapler was performed. In 10 of them (group A), a modified Rehbein operation with intraabdominal anastomosis was performed. In the remaining 15 patients (group B), a modified Swenson operation with exteriorization of the aganglionic colon through the anus and extrabdominal anastomosis was performed. RESULTS: The postoperative course was evaluated by measuring the postoperative fasting time and the first spontaneous deposition. The medium hospital stay was of 9 days, nevertheless in 16 patients (64%) was lower than 7 days. The postoperative complications are presented. It consists in 1 anastomotic leakage (4%), postoperative enterocolitis 1 case (4%) and transient anastomotic stenosis in 4 patients (16%). All of them were treated with conservative treatment except one case of stenosis which needed a sphincterotomy. CONCLUSIONS: The rectosigmoidectomy and coloanal end to end anastomosis with endoluminal stapler is a safe and easy to do technique to treat the Hirschsprung's disease allowing a deep rectal resection which is very difficult to achieve by manual suture. The anastomosis is located in and extraperitoneal position, with a minimum risk of peritoneal involvement in case of anastomotic leakage. The patients presented a fast recovery, a minimum of complications and good functional result.

Among Gastrointestinal Duplications, colonic duplications are the less common. The case presented here consist of a duplication of the transverse colon, difficult to diagnose, which had abdominal distension as the main symptom. A 4-year-old child was referred to the Unity of Pediatric Surgery, Hospital de Base, Brasilia, DF with a history of progressive abdominal distension. Plain X-Rays of the abdomen demonstrated a large fecaloma, which demanded removal. A Barium Enema was done suggesting Congenital Megacolon. A rectal biopsy was performed under general anesthesia, demonstrating normal ganglion cells. Medical treatment was instituted for chronic constipation in the Pediatric Gastroenterology clinic. The patient returned three months later with the same complaints. A new rectal biopsy was done; normal ganglion cells were described, ruling out Hirschsprung's disease. The parents were told to insist on the medical treatment diets. Four years later the patient was seen in the Emergency Room with signs and symptoms of low intestinal obstruction. Exploratory Laparotomy was undertaken as an emergency and the findings were complete volvulus of the large bowel involving the transverse colon up to the splenic flexure, demonstrating a large duplication of the transverse colon. A resection of the duplication and end-to-end colonic anastomosis was performed with an uneventful postoperative care. Discharged on excellent conditions.

Enterovesical fistula is a relatively uncommon complication of colorectal and pelvic malignancies, diverticulitis, inflammatory bowel disease, radiotherapy, and trauma in Asian countries. A case of vesico-ileosigmoidal fistula and a literature review of this disease in Japan are presented. A 70-yr-old male was referred with complaints of urinary pain and pneumaturia. On admission, urinary tract infection and pneumaturia were presented. A barium enema demonstrated multiple diverticulum in his sigmoid colon and the passage of contrast medium into the bladder and ileum. Under the diagnosis of vesico-ileosigmoidal fistula due to suspected diverticulitis of the sigmoid colon, sigmoidectomy and partial resection of the ileum with partial cystectomy were performed. The histopathology revealed diverticulosis of the sigmoid colon with diverticulitis and development of a vesico-ileosigmoidal fistula. No malignant findings were observed. Until the year 2000, a total of 173 cases of vesico-sigmoidal fistula caused by diverticulitis had been reported in Japan. Pneumaturia and fecaluria are the most common types, presenting symptoms in 63% of the cases. Computed tomography, with a sensitivity of 40% to 100%, is the most commonly used diagnostic study. For patients with vesico-sigmoidal fistula, resection of the diseased sigmoid colon and partial cystectomy with primary anastomosis are the safest and most acceptable procedures, leading to the best results.