Stapled restorative proctocolectomy in children with refractory ulcerative colitis

Objective

The aim of this study was to review the results after stapled restorative proctocolectomy among children with refractory ulcerative colitis.

Patients and Methods

Clinical records of 16 consecutive children with refractory ulcerative pancolitis undergoing colectomy and stapled straight ileoanal anastomosis at a median age of 8.3 years (range, 3.1-14.9 years) were reviewed.Periodical clinical examinations and endoscopies with biopsies above (terminal ileum) and below (columnar cuff) the anastomosis were carried out during follow-up. Median follow-up after bowel restoration lasted 5.3 years (range, 1.2-9.6 years).

Results

Two major complications occurred (12.5%), 1 episode of sepsis treated conservatively and 1 bowel perforation proximal to the anastomosis treated with a temporary diverting ileostomy. All the anastomoses were functional at the end of the study. The columnar cuff averaged 2.6 cm in length and presented signs of persistent inflammation (cuffitis) in 94% of children. Inflammation responded poorly to any medical treatment but was symptomatic in 1 case only. Ileal inflammation was detected endoscopically in 31% of patients and histologically in 62.5%. No case of dysplasia or cancer was recorded. At final follow-up, children had an average of 7.1 ± 3.1 bowel movements per day; full daytime and nighttime continence were achieved in 87.5% and 62.5% of cases, respectively. A severe inflammation of the columnar cuff was associated with an increased risk of nighttime incontinence.

Conclusions

Stapled ileoanal anastomosis in children with pancolitis is associated with low morbidity. Refractory cuffitis persists in almost all patients but is mostly asymptomatic, although it could be associated with nighttime incontinence.     

Complications following restorative proctocolectomy in children

Background

Adverse outcomes following restorative proctocolectomy (RPC) in adults have been attributed to steroid exposure and use of hand-sutured anastomoses. This study analyses complications in children undergoing RPC.

Methods

This study is a retrospective review of all children undergoing RPC in an English regional center over a 10-year period. The main outcome measure was defined as a complication within 30 days of surgery. Logistic regression analysis was used with possible explanatory variables (eg, steroid use, indication for surgery, weight and height z scores, hematologic indices, degree of blood loss, and use of laparoscopic surgery).

Results

Sixty (33 female) patients underwent RPC at a median age of 13.5 years. Of these, 16 had an operative complication and 17 had a late complication. Only severe acute colitis with inability to induce remission as an indication for surgery was significant in predicting operative complications (odds ratio, 6.8 [95% confidence interval, 1.2-37]; P = .03).

Conclusions

Severe acute colitis resistant to medical therapy but not steroid use or hand-sutured anastomoses appears to be a risk factor for complication. This differs from the adult experience.     

Incidence and outcome of complications following restorative proctocolectomy

BACKGROUND: Complications were analyzed in 296 patients with ulcerative colitis who underwent restorative proctocolectomy. METHODS: In 96.3% of patients, the pouch was anastomosed using the double stapling method. A total of 44.6% of patients underwent restorative proctocolectomy in 1 stage without ileostomy and 53% in 2 stages. Complications were divided into 2 stages: early (within 1 month) and late (after 1 month); moreover, the annual incidences were calculated, mean onset time, and pouch survival rate. RESULTS: The overall incidence of complications was 52.7%. Early complications (13.2%) occurred significantly less often than late complications (46.3%) (P < .05). Thirty-five (17.7%) of 198 complications required surgery. The cumulative 5- and 10-year pouch survival rate was 99%, respectively. CONCLUSIONS: The rate of complications after restorative proctocolectomy was almost equivalent to that in other reports, but the pouch survival rate was very high.     

Female infertility following restorative proctocolectomy

Aim Females of child‐bearing age have been reported to have a two to three‐fold increase in infertility after restorative proctocolectomy (RPC). This study aimed to assess aspects of infertility and pregnancy. Method A postal questionnaire was sent to 790 females who had undergone primary RPC in two tertiary centres. Infertility, the number and outcome of pregnancies, delivery method and the use of fertility treatments were determined. Results Three hundred and six (38.5%) females responded (median age 47.9 years at follow up; 35.3 years at the time of RPC). Eighty‐two per cent (n = 250) had ulcerative colitis. Forty‐five per cent (n = 138) had conceived prior to RPC, 5.2% (n = 16) conceived both before and after RPC, 5.5% (n = 17) conceived after RPC only and 44.1% (n = 135) had never conceived. Females delivering before RPC had significantly more vaginal deliveries than those conceiving after (pre‐RPC 69.6%, n = 96 vs post‐RPC 35.3%, n = 6; P = 0.001). Fifty‐seven patients stated they had attempted to conceive after RPC, with 25 (45.5%) being successful. Eighteen females had been referred to a fertility specialist, of whom 16 received in vitro fertilization (IVF). Four (30.7%) females conceived using IVF. Conclusion While RPC is known to be associated with infertility, only a small proportion of patients are referred for fertility management. IVF outcomes and success rates after RPC are similar to the general population. Patients are more likely to have a Caesarean section following RPC.     

Vitamin B12 deficiency following restorative proctocolectomy.

OBJECTIVE: Restorative proctocolectomy (RP) involves terminal ileal resection and formation of a small bowel reservoir that predisposes to bacterial overgrowth. It was anticipated that these patients would be at risk of vitamin B12 deficiency. METHOD: Vitamin B12 levels were measured sequentially in 171 patients who underwent RP. Prospective results were obtained from all 20 patients undergoing pouch formation after the commencement of the study. Further results were obtained retrospectively from case notes and computerized laboratory records of the 151 patients who underwent RP prior to the commencement of the study and these were correlated with the results of follow-up samples taken prospectively from the same patients after the commencement of the study. The median age of the patients was 40 years (range: 13-67) and the median duration of follow up was 5.4 years (range: 1-12). Patients with an abnormally low serum B12 level underwent both a Schilling and a hydrogen breath test. Eight of these patients were then treated with oral vitamin B12. RESULTS: Abnormally low serum B12 levels were found in 25% of patients. Forty per cent of our patient group had three or more sequential B12 measurements and of these, 66% showed steadily declining B12 levels. Ninety-four per cent of patients with low B12 had a normal Schilling test and were negative for bacterial overgrowth. CONCLUSION: Subnormal vitamin B12 levels develop in almost one-quarter of patients after pouch surgery. The exact mechanism for B12 deficiency in these patients is uncertain. In the majority of patients undergoing RP, vitamin B12 levels fall on sequential measurement. Serum B12 levels should be measured during follow up and pouch patients with subnormal B12 levels, should see them successfully restored to a normal value after treatment with oral B12 replacement therapy.     

Ulcerative colitis and coexisting colorectal cancer: Recurrence rate after restorative proctocolectomy

Background: The association between mucosal ulcerative colitis (MUC) and adenocarcinoma is well established. Methods: Records of patients who had undergone restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) from 1983 through 1992 were examined. Of these, 604 had MUC and 27 (4.3%) had MUC with coexisting cancer. Patients were surveyed annually for recurrent disease. Pouch function and quality of life were evaluated with a questionnaire and physical examination. Results: The duration of disease was longer (p=0.001) in patients with cancer (16.1±8.0 years) than in those without cancer (9.1±7.1 years), although the mean age at diagnosis of MUC was the same. Of the 27 patients, 20 had colon cancer and seven had rectal cancer. Multicentricity was found in seven (25.9%) patients. Using the TNM staging classification, 14 patients (51.8%) had stage 1 cancer, eight (29.6%) had stage 2, four (14.8%) had stage 3, and one (3.8%) had stage 4. The patient with stage 4 cancer died 5 months after surgery and was excluded from the follow-up analysis. During a mean follow-up time of 4.3±2.6 years, cancer recurred in two of the remaining 26 patients (7.7%). In one patient, a local recurrence was found 8 months after surgery, and distant metastases were found in the other patient 35 months after surgery. Both recurrences were in patients with colon cancer. Two of the 26 patients died; one death was related to cancer recurrence (3.8%). Pouch function is good to excellent in all surviving patients. Conclusions: Restorative proctocolectomy for patients with MUC and coexisting colorectal cancer can be performed with a favorable prognosis and function. It is appropriate for curative intent, given that an adequate margin without tumor is obtained.Presented at the 47th Annual Cancer Symposium of The Society of Surgical Oncology, Houston, Texas, March 17–20, 1994.     

Technique of laparoscopic-assisted total proctocolectomy and ileal pouch anal anastomosis in children and adolescents: a single center's 8-year experience

Background

Laparoscopic techniques have been applied to restorative proctocolectomy since the early 2000s. We have employed a technique for laparoscopic-assisted total proctocolectomy (TPC) and ileal pouch anal anastomosis (IPAA) for the treatment of children with ulcerative colitis (UC).

Methods

A total of 68 children underwent laparoscopic-assisted TPCs at our institution from June 2003 to February 2011. The patients ranged from 5 to 22 years of age. We progressively modified our surgical approach in positioning, equipment use, and operative technique.

Results

A total of 68 children underwent laparoscopic-assisted TPC within the 8-year time period. Two major complications occurred (2/68 = 3%) consisting of 1 duodenal injury and 1 small bowel injury. There were 6 minor complications (6/68 = 9%), including 5 stoma revisions and 1 port site hernia. There were no superficial wound infections. There were no reported cases of pouch fistulas, anastomotic breakdown, or neurovascular injuries.

Conclusions

Our surgical technique is safe and effective in treating children with UC.     

腹腔镜辅助次/全结-直肠切除的临床应用

目的探讨腹腔镜辅助次／全结一直肠切除术的安全性及可行性。方法2003年3月～2005年11月。行腹腔镜辅助次／全结一直肠切除术8例。年龄33—65岁，中位数44岁，包括结一直肠多发肿瘤1例、溃疡性结肠炎3例、家族性息肉病2例，慢传输性便秘2例。行回肠储袋一肛管吻合3例、回肠储袋一直肠吻合3例、盲肠一直肠吻合2例。预防性回肠造瘘5例。结果8例均在腹腔镜协助下完成，无中转开腹，无死亡病例。手术时间5．5—7．5h，中位数6h。术中出血量150—400ml，中位数200ml，无术中、术后输血。术后48h进食。8例随访4～31个月，中位数25个月。腹腔内残余感染1例（溃疡性结肠炎），吻合口狭窄1例（家族性息肉病）。结论腹腔镜辅助次／全结一直肠切除术能够安全、有效进行。     

A systematic stapling defect – a potential cause of 'anastomotic' leak after restorative proctocolectomy

Objective When constructing ileal‐anal pouches with staples, a novel potential cause of anastomotic leak was recently identified at the apex of the pouch. This study was performed to assess this stapling defect. Method Careful inspection of the ileal pouch staple lines was made in eight consecutive pouch constructions. Pouch construction was further evaluated using pig small bowel. Results When constructing ileal‐anal pouches with staples, a novel potential cause of anastomotic leak was recently identified at the apex of the pouch. This defect was present in seven of eight consecutive ileo‐anal J‐pouches. It was repaired by direct suturing and no clinical or radiological leaks were identified in these patients. In the pig model, the same defect was found in five of five stapled constructions. It was avoiding in five of five cases by limiting the amount of bowel placed into the stapler on the initial firing. Conclusion This defect may be related to stapler design. It can be avoided by using less than the full staple line on the initial firing of the stapler or by using an alternative device. Surgeons creating ileal pouches using staplers, or indeed any use of this particular stapler, need to be aware of this potential for a defect in the staple line and should take steps to avoid the defect or repair it depending on the circumstances.     

A selective approach to restorative proctocolectomy without ileostomy: a single centre experience

Objective A single surgeon series on complications and functional outcomes following restorative proctocolectomy (RPC) is presented. Method An ethically‐approved database was used to collect data on all patients undergoing RPC at a single institution. Patient demographics, operative details, complications and functional outcomes were assessed. The impact of ileostomy omission on outcomes was also assessed. Results Two hundred patients undergoing RPC between 1987 and 2006 were included. There were 122 (61.0%) males and the mean age at surgery was 37.6 years. A J pouch was constructed in 199 (99.5%) patients and an ileostomy omitted in 160 (80.0%). Since adopting a selective policy after the 36th consecutive patient in the series, only 9 (5.5%) patients have had an ileostomy constructed at the time of pouch construction. Complications occurred in 112 (56.3%) patients, with anastomotic stricture (20.6%) and pouchitis (28.6%) being the most common. Anastomotic stricture was more common in those patients receiving an ileostomy (43.6%vs 15.0%, P < 0.001), as were pouch‐cutaneous fistulae (5.1%vs 0.6%, P = 0.039) and pelvic sepsis (15.4%vs 5.0%, P = 0.023). Functional outcomes were good, with median 24‐h stool frequency of five motions at 1 year. There was increased urgency to defaecate which in part may be due to a significant decline in the use of antidiarrhoeal medication during follow up. Conclusions Selective omission of a covering ileostomy in most cases can produce good results following RPC with no increase in the risk of septic complications or pouch failure, and a decreased risk of anastomotic stricture, with maintenance of good function in the majority.     

Pouchitis may increase the risk of dysplasia after restorative proctocolectomy in patients with ulcerative colitis.

Aim Dysplasia of the pouch mucosa after restorative proctocolectomy is rare. The aim of this study was to establish whether there is a correlation between pouchitis and dysplasia. Method A group of 276 patients treated for ulcerative colitis by restorative proctocolectomy between 1984 and 2009 was analysed. The presence or absence of pouchitis and dysplasia within the pouch was evaluated. Results Inflammation was diagnosed in 66 (23.9%) patients, low‐grade dysplasia in five (1.8%), high‐grade dysplasia in three (1.1%), and cancer in one patient (0.4%). The prevalence of low‐grade dysplasia was significantly higher in patients with inflammation than in those without (P < 0.04). High‐grade dysplasia was significantly more frequent in pouchitis than in non‐inflamed pouches (P < 0.01). Logistic regression analysis suggested that the occurrence of mucosal inflammation increased the risk of low grade dysplasia. Conclusion Patients with chronic pouchitis are at risk of dysplasia and require surveillance of the pouch.     

Elimination of mucosectomy during restorative proctocolectomy in patients with ulcerative colitis may provide better results in low-volume centers

BACKGROUND: To compare the outcomes of hand-sewn and double-stapling techniques among ulcerative colitis patients undergoing restorative proctocolectomy at a center that has limited experience with restorative proctocolectomy. METHODS: Forty-four patients with ulcerative colitis were divided into two groups according to the anastomosis techniques: hand sewing and double stapling. Postoperative early and late complications, postoperative hospital stay, and long-term functional results were compared. RESULTS: Pelvic sepsis (9% versus 36%, P = 0.03), operation time (median 240 minutes versus 270 minutes, P = 0.01), postoperative hospital stay (median 9 days versus 12 days, P = 0.04), and night incontinence (42% versus 80%, P = 0.07) were less common in the double-stapling group. CONCLUSIONS: We recommend the double-stapling technique to centers that do not have extensive experience with restorative proctocolectomy for ulcerative colitis. This technique provides a good postoperative course in most patients and provides satisfactory long-term results. The results of the double-stapling group were similar to the results of the high-volume centers.     

Risk of dysplasia and adenocarcinoma following restorative proctocolectomy for ulcerative colitis

OBJECTIVE: Restorative proctocolectomy (RPC) with or without mucosectomy is the treatment of choice for most patients with ulcerative colitis (UC) requiring surgery. The ileal mucosa in the reservoir and the anorectal columnar epithelium below the ileo-anal anastomosis are at risk of neoplastic transformation. METHOD: The literature has been reviewed to identify patients developing this complication and an attempt has been made to develop a rational follow-up policy based on the data available. RESULTS: Dysplasia in the ileal reservoir is rare. It is associated with histological type C changes, sclerosing cholangitis and unremitting pouchitis in the ileal mucosa and to the presence of sclerosing cholangitis. Nine patients who have developed adenocarcinoma in the residual anorectal mucosa and seven in the reservoir have been reported in the literature. A further hitherto unreported patient treated by the authors brings the total to 17 patients. Twelve of these had histopathological data on either dysplasia or carcinoma in the original operative specimen. The time intervals from the onset of UC and from the RPC to the development of cancer were 120-528 (median 246) and 16-216 (median 60) months respectively. Cancer appeared to be related to the duration of disease rather than to the interval from RPC. In all the reported patients the interval from the onset of UC was 10 years. CONCLUSION: Based on these data a surveillance programme should begin at 10 years from the onset of disease. Patients with dysplasia or carcinoma in the original specimen, those with type C ileal mucosal changes and patients with sclerosing cholangitis should be selected for surveillance. This will involve multiple biopsies of the ileal reservoir and the anorectal mucosa below the ileo-anal anastomosis.     

Meta-analysis of short-term and long-term outcomes of J, W and S ileal reservoirs for restorative proctocolectomy

OBJECTIVE: The choice of ileal pouch reservoir has been a contentious subject with no consensus as to which technique provides better function. This study aimed to compare short- and long-term outcomes of three ileal reservoir designs. METHOD: Comparative studies published between 1985 and 2000 of J, W and S ileal pouch reservoirs were included. Meta-analytical techniques were employed to compare postoperative complications, pouch failure, and functional and physiological outcomes. Quality of life following surgery was also assessed. RESULTS: Eighteen studies, comprising 1519 patients (689 J pouch, 306 W pouch and 524 S pouch) were included. There was no significant difference in the incidence of early postoperative complications between the three groups. The frequency of defecation over 24 h favoured the use of either a W or S pouch [J vs S: weighted mean difference (WMD) 1.48, P < 0.001; J vs W: WMD 0.97, P = 0.01]. The S pouch was associated with an increased need for pouch intubation (S vs J: OR 6.19, P = 0.04). The use of a J pouch was associated with a significantly higher prevalence of use of anti-diarrhoeal medication (J vs S: OR 2.80, P = 0.01; J vs W: OR 3.55, P < 0.001). CONCLUSION: All three reservoirs had similar perioperative complication rates. The S pouch was associated with the need for anal intubation. There was less frequency and less need for antidiarrhoeal agents with the W rather than the J pouch.     

Long-term cancer risk of the anorectal cuff following restorative proctocolectomy assessed by p53 expression and cuff dysplasia

OBJECTIVE: Restorative proctocolectomy (RP) for ulcerative colitis (UC) retains a 'cuff' of columnar rectal epithelium that has unknown risk of malignant change. Markers of malignant potential in UC include aberrant p53 expression and dysplasia. We undertook a prospective study comprising serial surveillance biopsy and assessed the occurrence of aberrant p53 expression, epithelial dysplasia and carcinoma in the retained anorectal cuff following stapled RP. METHOD: A total of 110 patients who underwent stapled RP for UC between 1988 and 1998 were followed up by cuff surveillance biopsies under general anaesthesia. Histological samples were analysed by a specialist colorectal pathologist for the presence of rectal mucosa, dysplasia and carcinoma. Immunohistochemistry for p53 expression was performed for each most recent cuff biopsy. Median follow-up was 56 months (12-145) and median time since diagnosis of UC was 8.8 years (2-32). RESULTS: Rectal mucosa was obtained from the cuff in 65% of biopsies. No overt carcinomas developed during the follow-up period and there was no dysplasia or carcinoma in any cuff biopsy. The p53 overexpression was identified in 38 specimens (50.6%), but was also identified in controls (3/3 colitis, 3/3 ileal pouch and 6/6 stapled haemorrhoidectomy donuts). CONCLUSION: The lack of carcinoma and dysplasia in the columnar cuff epithelium specimens is reassuring. The lack of stabilized p53 and absence of a relationship between p53 stabilization and dysplasia up to 12 years after pouch formation suggests neoplastic transformation is a rare event. Furthermore, p53 expression was not useful in surveillance of cuff biopsies from patients who have undergone RP for UC and the search should continue for alternative predysplastic markers. These data suggest that in patients who do not have high-grade dysplasia or colorectal cancer at the time of RP, cuff surveillance in the first decade after pouch formation is unnecessary. However, we consider regular cuff surveillance biopsies should continue for patients with high-grade dysplasia, whether or not there was a carcinoma in the original colectomy specimen.