Laparoscopic restorative proctocolectomy with ileal pouch-anal anastomosis for Peutz-Jeghers syndrome with synchronous rectal cancer

We report on a patient diagnosed with PeutzJeghers syndrome(PJS) with synchronous rectal cancer who was treated with laparoscopic restorative proctocolectomy with ileal pouch-anal anastomosis(IPAA). PJS is an autosomal dominant syndrome characterized by multiple hamartomatous polyps in the gastrointestinal tract, mucocutaneous pigmentation, and increased risks of gastrointestinal and nongastrointestinal cancer. This report presents a patient with a 20-year history of intermittent bloody stool, mucocutaneous pigmentation and a family history of PJS, which together led to a diagnosis of PJS. Moreover, colonoscopy and biopsy revealed the presence of multiple serried giant pedunculated polyps and rectal adenocarcinoma. Currently, few options exist for the therapeutic management of PJS with synchronous rectal cancer. For this case, we adopted an unconventional surgical strategy and ultimately performed laparoscopic restorative proctocolectomy with IPAA. This procedure is widely considered to be the first-line treatment option for patients with ulcerative colitis or familial adenomatous polyposis. However, there are no previous reports of treating PJS patients with laparoscopic IPAA. Since the operation, the patient has experienced no further episodes of gastrointestinal bleeding and has demonstrated satisfactory bowel control. Laparoscopic restorative proctocolectomy with IPAA may be a safe and effective treatment for patients with PJS with synchronous rectal cancer.

Pouchitis and pre-pouch ileitis developed after restorative proctocolectomy for ulcerative colitis:A case report

We report a case of pouchitis and pre-pouch ileitis, and inflammation in the neo-terminal ileum proximal to the pouch, developed after restorative proctocolectomy for ulcerative colitis. A 35-year old female presented with fever and abdominal pain five weeks after ileostomy closure following proctocolectomy. Computed tomography showed collection of feces in the pouch and proximal ileum. A drainage tube was placed in the pouch perianally, and purulent feces were discharged. With antibiotic treatment, her symptoms disappeared, but two weeks later, she repeatedly developed fever and abdominal pain along with anal bleeding. Pouchscopy showed mucosal inflammation in both the pouch and the pre-pouch ileum. The mucosal cytokine production was elevated in the pouch and pre-pouch ileum. With antibiotic and corticosteroid therapy, her symptoms were improved along with improvement of endoscopic inflammation and decrease of mucosal cytokine production. The fecal stasis with bacterial overgrowth is the major pathogenesis of pouchitis and pre-pouch ileitis in our case.

Postoperative changes of manometry after restorative proctocolectomy in Korean ulcerative colitis patients

To investigate the changes of postoperative anal sphincter function and bowel frequency in Korean patients with ulcerative colitis (UC). METHODSA total of 127 patients with UC who underwent restorative proctocolectomy (RPC) during 20 years were retrospectively analyzed. The parameters of anal manometry and bowel frequency were compared according to the 6-mo intervals until 24 mo postoperatively. Manometry was used to measure the maximal squeezing pressure (MSP) and maximal resting pressure (MRP). RESULTSMSP decreased after surgery until 6 mo (157 to 142 mmHg); thereafter, it improved and was recovered to and maintained at the preoperative value at 12 mo postoperatively (142-170 mmHg, P < 0.001). Although the decreased MRP (65 to 56 mmHg) improved after 18 mo (62 mmHg), it did not completely recover to the preoperative value. The decreased rectal capacity after surgery (90 to 82 mL) gradually increased up to 150 mL at 24 mo. Although bowel frequency showed significant gradual decreases at each interval, it was stabilized after 12 mo postoperatively (6.5 times/d). CONCLUSIONPostoperative changes of manometry and bowel frequency after restorative proctocolectomy in Korean patients with UC were not different from those in Western patients with UC.

Role of ileostomy in restorative proctocolectomy

Restorative proctocolectomy(RP) is the treatment of choice in patients affected with refractory ulcerative colitis or familial adenomatous polyposis.Surgery in elective settings is often performed in 2 stages,fashioning an ileostomy which is closed 2-3-mo later.It is still debated whether omitting ileostomy could offer advantages in the management of patients undergoing RP.

Cumulative incidence and risk factors for pouch adenomas associated with familial adenomatous polyposis following restorative proctocolectomy

BACKGROUND The emergence of restorative total proctocolectomy has significantly reduced the lifetime colorectal cancer risk associated with familial adenomatous polyposis(FAP).However,adenomas may develop in the ileal pouch over time and may even progress to carcinoma.We evaluated the cumulative incidence,time to development,and risk factors associated with ileal pouch adenoma.AIM To evaluate the cumulative incidence,time to development,and risk factors associated with pouch adenoma.METHODS In this retrospective,observational study conducted at a tertiary center,95 patients with FAP who underwent restorative proctocolectomy at our center between 1989 and 2018 were consecutively included.The mean follow-up period was 88 mo.RESULTS Pouch adenomas were found in 24(25.3%)patients,with a median time of 52 mo to their first formation.Tubular adenomas were detected in most patients(95.9%).There were no high-grade dysplasia or malignancies.Of the 24 patients with pouch adenomas,13 had all detected adenomas removed.Among the 13 patients who underwent complete adenoma removal,four(38.5%)developed recurrence.Among 11(45.8%)patients with numerous polyps within the pouch,seven(63.6%)exhibited progression of pouch adenoma.The cumulative risks of pouch adenoma development at 5,10,and 15 years after pouch surgery were 15.2%,29.6%,and 44.1%,respectively.Severe colorectal polyposis(with more than 1000 polyps)was a significant risk factor for pouch adenoma development(hazard ratio,2.49;95% confidence interval:1.04-5.96;P=0.041).CONCLUSION Pouch adenomas occur at a fairly high rate in association with FAP after restorative proctocolectomy,and a high colorectal polyp count is associated with pouch adenoma development.

Hepatobiliary manifestations following two-stages elective laparoscopic restorative proctocolectomy for patients with ulcerative colitis:A prospective observational study

BACKGROUND Hepatobiliary manifestations occur in ulcerative colitis(UC)patients.The effect of laparoscopic restorative proctocolectomy(LRP)with ileal pouch anal anastomosis(IPAA)on hepatobiliary manifestations is debated.AIM To evaluate hepatobiliary changes after two-stages elective laparoscopic restorative proctocolectomy for patients with UC.METHODS Between June 2013 and June 2018,167 patients with hepatobiliary symptoms underwent two-stage elective LRP for UC in a prospective observational study.Patients with UC and having at least one hepatobiliary manifestation who underwent LRP with IPAA were included in the study.The patients were followed up for four years to assess the outcomes of hepatobiliary manifestations.RESULTS The patients'mean age was 36±8 years,and males predominated(67.1%).The most common hepatobiliary diagnostic method was liver biopsy(85.6%),followed by Magnetic resonance cholangiopancreatography(63.5%),Antineutrophil cytoplasmic antibodies(62.5%),abdominal ultrasonography(35.9%),and Endoscopic retrograde cholangiopancreatography(6%).The most common hepatobiliary symptom was Primary sclerosing cholangitis(PSC)(62.3%),followed by fatty liver(16.8%)and gallbladder stone(10.2%).66.4%of patients showed a stable course after surgery.Progressive or regressive courses occurred in 16.8%of each.Mortality was 6%,and recurrence or progression of symptoms required surgery for 15%.Most PSC patients(87.5%)had a stable course,and only 12.5%became worse.Two-thirds(64.3%)of fatty liver patients showed a regressive course,while one-third(35.7%)showed a stable course.Survival rates were 98.8%,97%,95.8%,and 94%at 12 mo,24 mo,36 mo,and at the end of the follow-up.CONCLUSION In patients with UC who had LRP,there is a positive impact on hepatobiliary disease.It caused an improvement in PSC and fatty liver disease.The most prevalent unchanged course was PSC,while the most common improvement was fatty liver disease.

Surgical treatment of ulcerative colitis in the biologic therapy era

Recently introduced in the treatment algorithms and guidelines for the treatment of ulcerative colitis,biological therapy is an effective treatment option for patients with an acute severe flare not responsive to conventional treatments and for patients with steroid dependent disease.The reduction in hospitalization and surgical intervention for patients affected by ulcerative colitis after the introduction of biologic treatment remains to be proven.Furthermore,these agents seem to be associated with increase in cost of treatment and risk for serious postoperative complications.Restorative proctocolectomy with ileal pouch-anal anastomosis is the surgical treatment of choice in ulcerative colitis patients.Surgery is traditionally recommended as salvage therapy when medical management fails,and,despite advances in medical therapy,colectomy rates remain unchanged between 20% and 30%.To overcome the reported increase in postoperative complications in patients on biologic therapies,several surgical strategies have been developed to maintain long-term pouch failure rate around 10%,as previously reported.Surgical staging along with the development of minimally invasive surgery are among the most promising advances in this field.

Gardner's syndrome:Genetic testing and colonoscopy are indicated in adolescents and young adults with cranial osteomas:A case report

We present a case of a 25-year-old female with diagnosed familial adenomatous polyposis and elevated carcinoembryonic antigen with negative family history. The suspicion of Gardner's syndrome was raised because extirpation of an osteoma of the left temporo-occipital region was made 10 years ago. Restorative procto-colectomy and ileal pouch anal anastomosis was made but histology delineated adenocarcinoma of the rectum (Dukes C stage). We conclude that cranial osteomas often precede gastrointestinal manifestations of familial adenomatous polyposis or Gardner's syndrome and such patients should be evaluated with genetic testing followed by colonoscopy if results are positive to prevent the development of colorectal carcinoma. If the diagnosis is positive all family members should be evaluated for familial adenomatous polyposis.

Surgical treatment of familial adenomatous polyposis: Dilemmas and current recommendations

Familial adenomatous polyposis (FAP) is an autosomal dominant inherited syndrome characterized by multiple adenomatous polyps (predisposing to colorectal cancer development) and numerous extracolonic manifestations. The underlying genetic burden generates variable clinical features that may influence operative management. As a precancerous hereditary condition, the rationale of performing a prophylactic surgery is a mainstay of FAP management. The purpose of the present paper is to bring up many controversial aspects regarding surgical treatment for FAP, and to discuss the results and perspectives of the operative choices and approaches. Preferably, the decision-making process should not be limited to the conventional confrontation of pros and cons of ileorectal anastomosis or restorative proctocolectomy. A wide discussion with the patient may evaluate issues such as age, genotype, family history, sphincter function, the presence or risk of desmoid disease, potential complications of each procedure and chances of postoperative surveillance. Therefore, the definition of the best moment and the choice of appropriate procedure constitute an individual decision that must take into consideration patient&#x02019;s preferences and full information about the complex nature of the disease. All these facts reinforce the idea that FAP patients should be managed by experienced surgeons working in specialized centers to achieve the best immediate and long-term results.

Anal transition zone in the surgical management of ulcerative colitis

Preservation of the anal transition zone has long been a significant source of controversy in the surgical management of ulcerative colitis.The two techniques for restorative proctocolectomy and ileal pouch anal anastomosis(RPC IPAA) in common practice are a stapled anastomosis and a handsewn anastomosis;these techniques differ in the amount of remaining rectal mucosa and therefore the presence of the anal transition zone following surgery.Each technique has advantages and disadvantages in long-term functional outcomes,operative and postoperative complications,and risk of neoplasia.Therefore,we propose a selective approach to performing a stapled RPC IPAA based on the presence of dysplasia in the preoperative endoscopic evaluation.

Pouchitis

While restorative proctocolectomy with ileal pouch-anal anastomosis has significantly improved the quality of life in patients with underlying ulcerative colitis who require surgery,complications can occur.Pouchitis as the most common long-term complication represents a spectrum of disease processes ranging from acute,antibiotic-responsive type to chronic antibiotic-refractory entity.Accurate diagnosis using a combined assessment of symptoms,endoscopy and histology and the stratification of clinical phenotypes is important for treatment and prognosis the disease.The majority of patients respond favorably to antibiotic therapy.However,management of chronic antibiotic-refractory pouchitis remains a challenge.

Endoscopic closure instead of surgery to close an ileal pouch fistula with the over-the-scope clip system

An ileal pouch fistula is an uncommon complication after an ileal pouch anal anastomosis. Most patients who suffer from an ileal pouch fistula will need surgical intervention. However, the surgery can be invasive and has a high risk compared to endoscopic treatment. The over-the-scope clip(OTSC) system was initially developed for hemostasis and leakage closure in the gastrointestinal tract during flexible endoscopy. There have been many successes in using this approach to apply perforations to the upper gastrointestinal tract. However, this approach has not been used for ileal pouch fistulas until currently. In this report, we describe one patient who suffered a leak from the tip of the "J" pouch and was successfully treated with endoscopic closure via the OTSC system. A 26-year-old male patient had an intestinal fistula at the tip of the "J" pouch after an ileal pouch anal anastomosis procedure. He received endoscopic treatment via OTSC under intravenous anesthesia, and the leak was closed successfully. Endoscopic closure of a pouch fistula could be a simpler alternative to surgery and could help avoid surgeryrelated complications.

Risk factors for postoperative stoma outlet obstruction in ulcerativecolitis

BACKGROUND Current medical treatments can achieve remission of ulcerative colitis (UC).Surgery is required when potent drug treatment is ineffective or when coloncancer or high-grade dysplasia develops. The standard procedure is restorativeproctocolectomy (RPC) with ileal pouch-anal anastomosis, commonly performedas two- or three-stage RPC with diverting ileostomy. Postoperative stoma outletobstruction (SOO) is frequent, but the causes are not well known.AIM To identify the risk factors for SOO after stoma surgery in patients with UC.METHODS We retrospectively reviewed the files of 148 consecutive UC patients whounderwent surgery with stoma construction. SOO was defined as small bowelobstruction symptoms and intestinal dilatation just below the penetrating part ofthe stoma on computed tomography. Patients were divided into two groups:Those who developed SOO within 30 d after surgery and those who did not.Patient characteristics, intraoperative parameters, the stoma site, and rectusabdominis muscle thickness were collected. Moreover, we identified the patientswho repeatedly developed SOO. Univariate and multivariate analyses wereperformed to identify risk factors for SOO and recurring SOO.RESULTS Eighty-nine patients who underwent two-stage RPC were included betweenJanuary 2008 and March 2020. Postoperatively, SOO occurred in 25 (16.9%)patients after a median time of 9 d (range 2-26). Compared to patients withoutSOO, patients with SOO had a significantly higher rate of malignant tumors ordysplasia (36.0% vs 17.1%, P = 0.032), lower total glucocorticoid dose one monthbefore surgery (0 mg vs 0 mg, P = 0.026), higher preoperative total protein level(6.8 g/dL vs 6.3 g/dL, P = 0.048), higher rate of loop ileostomy (88.0% vs 55.3%, P= 0.002), and higher maximum stoma drainage volume (2300 mL vs 1690 mL, P =0.004). Loop ileostomy (OR = 6.361;95%CI 1.322–30.611;P = 0.021) and maximumstoma drainage volume (OR = 1.000;95%CI 1.000–1.001;P = 0.015) wereconfirmed as independent risk factors for SOO. Eighteen patients with SOO weretreated conservatively without recurrence (sSOO group). Seven (28.0%) patientsrepeatedly developed SOO (rSOO group) during the observation period. Asignificant difference was observed in the rectus abdominis muscle thicknessbetween the two groups (sSOO 9.3 mm, rSOO 12.7 mm, P = 0.006). Musclethickness was confirmed as an independent risk factor for recurring SOO (OR =2.676;95%CI 1.176-4.300;P = 0.008).CONCLUSION In this study, high maximum stoma drainage volume and loop ileostomy areindependent risk factors for SOO. Additionally, among patients with a thickrectus abdominis muscle, the risk of SOO recurrence is high.

Adenocarcinomas after Prophylactic Surgery for Familial Adenomatous Polyposis

The incidence of familial adenomatous polyposis (FAP) is one in 7,000 to 12,000 live births. Virtually, all surgically untreated patients with FAP inevitably develop colorectal-cancer in their lifetime because they carry the adenomatous polyposis coli gene. Thus prophylactic proctocolectomy is indicated. Surgical treatment of FAP is still controversial. There are however, four surgical options: ileorectal anastomosis, restorative proctocolectomy with ileal pouch-anal anastomosis, proctocolectomy with ileostomy, and proctocolectomy with continent-ileostomy. Conventional proctocolectomy options largely lie between colectomy with ileorectal anastomosis or ileal pouch-anal anastomosis. Detractors of ileal pouch-anal anastomosis prefer ileorectal anastomosis because of better functional results and quality of life. The functional outcome of total colectomy with ileorectal anastomosis is undoubtedly far superior to that of the ileoanal pouch;however, the risk for rectal cancer is increased by 30%. Even after mucosectomy, inadvertent small mucosal residual islands remain. These residual islands carry the potential for the development of subsequent malignancy. We reviewed the literature (1975-2012) on the incidence, nature, and possible etiology of subsequent ileal-pouch and anal transit zone adenocarcinoma after prophylactic surgery procedure for FAP. To date there are 24 studies reporting 92 pouch-related cancers;15 case reports, 4 prospective and 5 retrospective studies. Twenty three of 92 cancers (25%) developed in the pouch mucosa and 69 (75%) in anal transit zone (ATZ). Current recommendation for pouch surveillance and treatment are presented. Data suggest lifetime surveillance of these patients.

Hepatobiliary manifestations of ulcerative colitis:an example of gut–liver crosstalk

The interaction between inflammatory bowel disease(IBD)and hepatobiliary manifestations represents a classic example of liver–gut crosstalk.The importance of liver–gut crosstalk in IBD is demonstrated in the pathogenesis and outcome of primary sclerosing cholangitis(PSC)in IBD patients.Immunoglobulin G4-associated cholangitis(IAC),which has recently been described in UC patients,may also illustrate the significance of gut–liver interaction in these patients.Presence of these hepatobiliary manifestations influences the outcome of associated IBD,in particular ulcerative colitis(UC),and vice versa.The pathogenesis of PSC is postulated to be related to gut inflammation in IBD that results in inflammation in the portal tracts(the‘leaky gut’).Enterohepatic circulation of lymphocytes from the gut to the liver is also of potential relevance to PSC pathogenesis and outcomes.The presence of PSC and gut inflammation in IBD influences the course and outcomes of both diseases.Further research is required,to understand the mutual effect of liver–gut crosstalk in the outcomes of UC patients,and highlights the importance of an interdisciplinary approach—involving gastroenterologists,hepatologists,advanced endoscopists and liver transplant surgeons—in the management of these patients.

Association between gastro-intestinal symptoms and menstruation in patients with ileal pouches

Background and aims:Gastro-intestinal(GI)symptoms are often experienced by healthy women during menstruation.An increased frequency of GI symptoms during menses has also been reported in women with irritable bowel syndrome or inflammatory bowel disease(IBD);however,IBD patients with restorative proctocolectomy and ileal pouch-anal anastomoses(IPAA)have not been studied.We aimed to examine the association between GI symptoms before and during menses in patients with IPAA,and to assess factors for exacerbation of GI symptoms in those patients.Methods:Adult women recorded in the Pouchitis Registry were invited to participate in a mailed survey.Participants reported on GI symptoms 1–5 days prior to-(pre-menses)and during the days of their menses in recent months.Demographic and clinical variables were obtained through the survey and chart review.Results:One hundred and twenty-eight(21.3%)out of 600 women with IPAA responded to the survey questionnaire.Fortythree(33.5%)were excluded for reasons including post-menopausal(n=25),hysterectomy(n=14)and use of contraceptives(n=4).Abdominal pain(P=0.001),diarrhea(P=0.021),and urgency(P=0.031)were more commonly reported during menses than pre-menses by the participants.Only a history of painful menses was significantly associated with increased GI symptoms during menses for patients with ileal pouch(odds ratio=5.67;95%confidence interval:1.41–22.88;P=0.015).Conclusion:GI symptoms such as abdominal pain,diarrhea,and urgency are commonly associated with menses in patients with ileo-anal pouch.Painful menses may be associated with worsening of GI symptoms.

Auto-inflammatory diseases in ileal pouch patients with NOD2/CARD15 mutations

Pouchitis is common in ulcerative colitis patients undergoing total proctocolectomy with ileal pouch-anal anastomosis,and chronic antibiotic-refractory pouchitis occurs in a subgroup of the patients.Auto-inflammatory diseases are characterized by systemic inflammation,manifesting as periodic fever,rash,arthritis,and serositis.We describe two cases with ulcerative colitis and an ileal pouch,who presented with extra-intestinal manifestations and genetic features atypical for inflammatory bowel disease alone.Case 1 had a spectrum of clinical manifestations including refractory pouchitis,intermittent fevers,polyarthralgia,and pericarditis.Case 2 presented with oral ulcers,migratory oligoarthritis,and periodic papular rash.Genetic testing in both cases revealed mutations of the NOD2/CARD15 gene,including the IVS8þ158 mutation commonly detected among patients with NOD2-associated auto-inflammatory disease.Both of the patients demonstrated clinical improvement of these diverse systemic complaints following treatment with immunosuppressive and anti-inflammatory therapies.

Vedolizumab in the treatment of Crohn’s disease of the pouch

Background:Our recent study showed the efficacy and safety of vedolizumab in the treatment of chronic antibioticrefractory pouchitis.However,there are no published studies on its efficacy and safety in Crohn’s disease(CD)of the pouch.The aim of this study was to assess the efficacy and safety of vedolizumab in those patients.Methods:This case series included all eligible patients with CD of the pouch from our prospectivelymaintained,IRB-approved Pouchitis Registry from 2015 to 2017.Disease activity in pouch patients can bemonitored using the modified Pouchitis Disease Activity Index(mPDAI).mPDAI is the 18-point pouchitis disease activity index consisting of three principal component scores:symptom(range,0–6 points),endoscopy,(range 0–6 points),and histology(range,2–6 points).Pre-and post-treatment(minimum 6 months)pouchoscopy and clinical visits were used to calculate mPDAI.Results:A total of 12 patients were included in this study,who had restorative proctocolectomy with ileal pouch anal anastomosis for medically refractory ulcerative colitis(UC).The mean age at the time of pre-colectomy diagnosis of UC was 25.0611.5 years.The mean current age was 41.0612.1 years,nine(75.0%)were female,three(25.0%)had smoked and eight(66.7%)had used anti-tumor necrosis factor agents prior to vedolizumab use.The mean duration of vedolizumab use was 1.066.4 years.There was a significant reduction in mPDAI symptom subscores after vedolizumab therapy(3.5061.93 vs 5.0860.79,P=0.015).The pre-and post-treatment mean endoscopy subscores were 1.2561.36 and 0.9161.50 in the afferent limb(P=0.583);2.5861.68 and 2.2762.05(P=0.701)in the pouch body;and 2.6761.93 and 2.0962.12(P=0.511)in the cuff,respectively.None of the patients experienced side effects throughout the vedolizumab therapy.Conclusion:The findings of our study suggests that vedolizumab appears to be effective and safe in reducing the symptoms in patients with CD of the pouch.