Atrophy and Neoplastic Transformation of the Ileal Pouch Mucosa in Patients With Ulcerative Colitis and Primary Sclerosing Cholangitis

INTRODUCTION: Patients with ulcerative colitis and primary sclerosing cholangitis have an increased risk of developing carcinoma both in the bile ducts and in the colon. PURPOSE: To investigate whether this patient group also has an increased risk of developing atrophy and neoplasia in the ileal pouch mucosa after construction of a pelvic pouch with an ileoanal anastomosis or a continent Kock ileostomy. METHODS: Flexible video endoscopic examinations of the ileal pouch were performed in 16 patients with ulcerative colitis and primary sclerosing cholangitis and in 16 matched patients with ulcerative colitis without sclerosing cholangitis. Biopsies were sampled from different locations in the pouch for histologic assessment of mucosal atrophy and dysplasia and for flow cytometric DNA analysis assessing chromosomal aberrations. RESULTS: The patients with sclerosing cholangitis developed moderate or severe atrophy in the pouch significantly more often (P < 0.01). Persistent severe mucosal atrophy was revealed in eight patients with sclerosing cholangitis and only in two controls. One patient with sclerosing cholangitis had high-grade dysplasia in multiple locations. Low-grade dysplasia was assessed in three patients with sclerosing cholangitis and in two of the controls. DNA aneuploidy was displayed in three patients, all with sclerosing cholangitis and dysplasia. All patients with neoplastic transformation had a pouch with ileoanal anastomosis and a long pouch duration (> 8 years). CONCLUSION: Patients with ulcerative colitis and primary sclerosing cholangitis with an ileal reservoir are more prone to developing mucosal atrophy in the pouch and seem to have a higher risk of neoplastic transformation in the pouch mucosa than patients with ulcerative colitis without sclerosing cholangitis.     

Intestinal Mucosa-Associated Microflora in Ulcerative Colitis Patients Before and After Restorative Proctocolectomy with an Ileoanal Pouch

Purpose  This study was designed to identify the mucosa-associated microflora in patients with severe ulcerative colitis before and after restorative proctocolectomy with ileoanal pouch construction in comparison with historic controls. Methods  Ten patients with a diagnosis of ulcerative colitis were evaluated. Mucus was collected during colonoscopy from all segments of the colon and terminal ileum before surgery, and from the ileal pouch two and eight months after ileostomy closure. The prevalence and mean concentration of the mucosa-associated microflora were compared over time and with historic controls. Results   Veillonella sp was the most prevalent bacterium in patients and controls. Klebsiella sp was significantly more prevalent in the ileum of controls, was not found in patients with ulcerative colitis, and after proctocolectomy returned to values found in controls. Some bacteria such as Enterobacter sp, Staphylococcus sp (coag-), Bacteroides sp (npg), Lactobacillus sp, and Veillonella sp had higher mean concentrations in the ileal pouch of patients after surgery than in controls. Conclusion  No bacterium was identified that could be exclusively responsible for the maintenance of the inflammatory process. The mucosa-associated microflora of patients with ulcerative colitis underwent significant changes after proctocolectomy with ileal pouch construction and returned to almost normal values for some bacteria. Presented at the meeting of the International Society of University Colon & Rectal Surgeons, Budapest, Hungary, June 6 to 10, 2004.     

Adenocarcinoma Arising from the Rectal Stump Eleven Years after Excision of an Ileal J-Pouch in a Patient with Ulcerative Colitis: Report of a Case

Adenocarcinomas in relation to the ileal J-pouch after restorative proctocolectomy for ulcerative colitis have been recently reported with increasing frequency. All previously reported cases have occurred in patients with their ileal pouch in situ. We report a case of adenocarcinoma in the anal canal 11 years after removal of a failed ileal J-pouch. Mucosectomy had been performed at the restorative proctocolectomy. The anus had been left in place at the pouch excision because of severe fibrosis in the pelvis. If it is decided to remove an ileal pouch permanently, a total abdominoperineal excision should be performed, particularly in patients with risk factors for cancer development. Reprints are not available.     

Intermittent Torsion of a Megapouch: Report of a Case

We report an unusual presentation of pouch dysfunction because of excessive pouch enlargement or “mega pouch” and probable torsion. The patient presented with abdominal pain that was positional. Contrast-enhanced computerized tomography showed pouch anastomotic staples extending into the right upper quadrant. At operation, gross pouch enlargement with dilation of the afferent ileum was confirmed. Reduction pouch-plasty resulted in pain resolution and maintenance of satisfactory function.     

Primary Sclerosing Cholangitis and Inflammatory Bowel Disease: A Review

Primary sclerosing cholangitis is a disease affecting around 0.006–0.016% of the population. Of these, around 75% have concomitant inflammatory bowel disease (IBD) according to the most recent epidemiological studies. Several theories have been proposed regarding the pathogenesis of primary sclerosing cholangitis (PSC). These include changes in the function of cholangiocytes, effects of the gut microbiome, association with specific human leukocyte antigen haplotypes and dysregulation of the immune system. However, these do not explain the observed association with IBD. Moreover, there are considerable differences in the frequency and outcomes between patients with PSC and ulcerative colitis compared with PSC and Crohn’s disease. The aim of this review is to appraise the most recent studies that have contributed to the epidemiology, advances in the pathophysiology, and characterization of important clinical aspects of the association of PSC and IBD.     

Laparoscopic Ileal Pouch-Anal Anastomosis in Patients with Chronic Ulcerative Colitis and Primary Sclerosing Cholangitis: A Case-Matched Study

Purpose This study was designed to compare short-term outcomes after laparoscopic ileal pouch-anal anastomosis with those of open ileal pouch-anal anastomosis in patients with both sclerosing cholangitis and ulcerative colitis. Methods Sixteen patients with sclerosing cholangitis and ulcerative colitis undergoing laparoscopic ileal pouch-anal anastomosis were matched with 16 open ileal pouch control subjects by sex, American Society of Anesthesiologists’ score, age, and body mass index. Results Operative mortality was zero. Operative time was longer in the laparoscopic group (500 ± 125.8 vs. 381.8 ± 60.9 minutes, P = 0.03). Thirty-day complications were not significantly different between groups (laparoscopic 25 percent vs. open 43.7 percent, P = 0.26). Length of stay was significantly shorter in the laparoscopic group (5.3 ± 1.3 days vs. 9.9 ± 3.3 days open, P < 0.001). Average return of gastrointestinal function was 2.5 days in the laparoscopic group and 4.8 days in the open group (P = 0.001). Time to soft diet was three days in the laparoscopic group and six days in the open group (P < 0.001). All patients were alive and all pouches were intact at last follow-up. Conclusions Laparoscopic ileal pouch-anal anastomosis is feasible with apparent safety in patients with primary sclerosing cholangitis, resulting in shorter duration of hospital stay and quicker return of gastrointestinal function compared with the open procedure with no difference in perioperative complications, reoperations, and readmissions.     

Successful Low-Dose Chemotherapy Using Vinblastine and Methotrexate for the Treatment of an Ileoanal Pouch Mesenteric Desmoid Tumor: Report of a Case

The purpose of this report was to describe the first known case of ileoanal pouch salvage by a low-dose regimen of vinblastine and methotrexate chemotherapy for the treatment of desmoid tumor arising from the mesentery of the ileoanal pouch in a patient who had undergone ileal pouch-anal anastomosis for familial adenomatous polyposis. Mesenteric desmoid tumor involving the ileoanal pouch in a 28-year-old female was treated with vinblastine and methotrexate biweekly for 12 months and monthly for 12 months in an outpatient unit. The desmoid tumor response to the treatment was assessed at routine intervals by physical examination and magnetic resonance imaging. Desmoid tumor was successfully treated with a low-dose regimen of vinblastine and methotrexate chemotherapy without significant side effects, and function of the ileoanal pouch was fully preserved. Magnetic resonance imaging showed a decrease in desmoid tumor size and cellularity, and changes consistent with fibrosis. This is a unique case highlighting the possibility of ileoanal pouch salvage by low-dose combination chemotherapy using vinblastine and methotrexate in a familial adenomatous polyposis patient with mesenteric desmoid tumor.     

Ileal Pouch Perforation in Pregnancy: Report of a Case and Review of the Literature

Acute abdominal pain, especially in the presence of free intra-abdominal air, in a pregnant patient who has previously undergone ileal pouch-anal anastomosis may be a result of acute perforation of the pouch. The case of a 30-year-old multigravida with an uncomplicated pregnancy at 27 weeks gestation who was admitted to the perinatal ward for preterm labor is described. The patients past medical history was significant for an ileal pouch-anal anastomosis for ulcerative colitis. Forty-eight hours after admission, she acutely developed generalized abdominal pain with associated dyspnea. A spiral CT of the chest to rule out pulmonary embolus revealed free intra-abdominal air. An emergency cesarean section was performed, which resulted in a viable female infant. The etiology for the free air was a perforation of the ileal pouch secondary to adhesions to the posterior part of the uterus. Perforation of the pouch has been reported secondary to a variety of factors. However, no cases of ileal pouch perforation in pregnancy have been reported. Adhesions that involve the pouch and an enlarged contracting uterus may result in perforation of the pouch. Awareness and knowledge of this potential complication may lead to earlier recognition and optimal management.     

Parking the Pouch: Pouch Salvage After Anastomotic Leak Following Restorative Proctocolectomy. Report of a Case

Anastomotic leak is a feared complication after restorative proctocolectomy with formation of an ileal pouch. We describe the use of a technique that is appropriate for profound anastomotic failure in the immediate postoperative period, which will aid in controlling sepsis and may allow salvage of the pouch. A 59-year-old man who failed medical treatment underwent restorative proctocolectomy and ileal pouch-anal anastomosis as a single-stage procedure. The patient developed an anastomotic leak that was not controlled by defunctioning stoma formation. Further surgery was undertaken and the pouch was exteriorized as a mucous fistula. A redo pouch-anal anastomosis was performed 12 months after the original procedure. The patient has good functional outcome with complete continence. Anastomotic leak after restorative proctocolectomy and ileal pouch-anal anastomosis often can be managed by conservative or local procedures. Laparotomy may be required rarely, but this subgroup is associated with pouch failure in up to half of the patients. Awareness that the ileal pouch-anal anastomosis can be taken down and the pouch temporarily parked in the abdominal cavity may persuade surgeons to retain a pouch with the knowledge that the acute pelvic sepsis after an anastomotic leak can be safely treated.     

Squamous-Cell Carcinoma Developing After an Ileoanal Pouch Procedure: Report of a Case

PURPOSE This study was designed to report a new variant of a rare but serious complication of restorative proctocolectomy.METHODS We present a 47-year-old female who underwent restorative proctocolectomy after 16 years of disease. Twenty-five years after her pouch procedure, she underwent pouchoscopy for fever and poor pouch function. A suspicious mass was biopsied and pathology indicated squamous metaplasia. On referral, a mass could be palpated above the anorectal ring. Biopsy of the mass was read as invasive squamous carcinoma in the background of normal intestinal mucosa. This represents the twelfth reported case of carcinoma arising in a pouch, but the first report of a squamous carcinoma, as all previous reports had been of adenocarcinoma.RESULTS The patient has undergone chemoradiation. Response to therapy, functional status, and biopsy after treatment will determine whether the patient will be able to salvage the pouch.CONCLUSIONS Diligence and vigilance with regard to active follow-up, and a high index of suspicion, are required to prevent this from becoming a more frequently seen problem.Reprints are not available.     

Perineal Adenocarcinoma Thirty Years After Proctocolectomy: Report of a Case

This report describes an adenocarcinoma arising in the perineum 30 years after two-stage total proctocolectomy for ulcerative colitis. This is one of the longest intervals reported between resection and presentation with cancer and also the only case in which the tumor is clearly free of association with existing bowel. The mass originally presented as a perineal mucocele, an unusual complication of proctocolectomy for ulcerative colitis, and this is the first such mucocele to demonstrate malignant transformation. Reprints are not available.     

Invasive Mucormycosis in a Patient With Liver Cirrhosis: Case Report and Review of the Literature

Introduction

Cutaneous Mucormycosis is a rare opportunistic infection caused by Zygomycetes class of fungi that is often fatal, requiring aggressive local control as well as systemic therapy. Few cases of mucormycosis were described in patients with liver cirrhosis, mostly rhino-orbital. To our knowledge, only two cases of upper extremity involvement was reported in cirrhosis while a few cases were reported in the post-transplant setting. We report herein the third case of upper extremity mucor infection in the setting of liver cirrhosis.

Case Presentation

We described a rare case of forearm infection originating in a traumatic intravenous access portal in a 25 year-old woman with liver cirrhosis secondary to autoimmune hepatitis.

Discussion

She developed acute on chronic liver failure during the last trimester of pregnancy, which was terminated. Painful, erythematous lesion was noted on her right forearm in the area of intravenous access, which later became necrotic. Extensive debridement was done and histopathological examination confirmed the diagnosis of mucormycosis. The patient started on Amphotericin B. Her condition continued to deteriorate and ended up with above elbow amputation followed by right shoulder disarticulation. She died two days later due to multi-organ failure. In conclusion, forearm mucromycosis in liver cirrhosis can be fatal.     

Ulcerative Colitis Accompanied with Sarcoidosis and Dermatomyositis: Report of a Case

We report a rare case of ulcerative colitis with sarcoidosis and dermatomyositis. A 33-year old woman had a ten-year history of ulcerative colitis and dermatomyositis. After nine-year maintenance treatment with sulphasalazine and steroids, she underwent a proctocolectomy and ileal pouch-anal anastomosis because of deterioration of ulcerative colitis. One year after the operation, she noticed some subcutaneous masses in the right forearm and left lower leg. A total biopsy of the mass was performed, and noncaseous epithelioid granulomas were found in the specimen. Chest roentgenogram and chest computed tomography showed hilar adenopathy and a diffuse granular shadow. The diagnosis of sarcoidosis was made. Concomitant development of ulcerative colitis and sarcoidosis may not be incidental, and there may be a common factor in the two diseases. Ulcerative colitis also may be a part of a systemic disorder associated with abnormal immune reactions. Complications by disorders associated with other immunologic abnormalities, as noted in this patient, should be kept in mind in the management of ulcerative colitis. Presented at the Digestive Disease Week (DDW)-Japan 2005 meeting, Kobe, Hyogo, Japan, October 5 to 8, 2005.     

A Case Report: First Case of Filtration Leukocytapheresis for a Patient of Aortitis Syndrome Associated with Ulcerative Colitis

Abstract: An 18‐year‐old woman was treated with leukocytapheresis (LCAP) for her combined ulcerative colitis (UC) and aortitis syndrome (AS). Because a close relationship between these two diseases has been suspected based on their etiological and/or pathological findings, we had hypothesized that LCAP, which has satisfactory effects on inflammatory bowel disease such as UC and Crohn's disease might be effective for both her UC and her AS. After informed consent, LCAP therapy was performed once a week for a total of 7 times. Endoscopic remission of the UC was observed. Even though there were no significant improvements in her subjective symptoms of AS such as side‐neck pain and dizziness, objective evidence of improvement was obtained when the patient's condition was compared before and after LCAP by angiography, angio‐magnetic resonance imaging, and the plethysmogram of her fingertips. These results suggest that LCAP may be valuable as a new adjunct therapy for AS.     

Adenocarcinoma Developing at an Ileostomy: Report of a Case and Review of the Literature

Primary adenocarcinoma of a permanent ileostomy is a rare and unusual complication. We report a case of primary adenocarcinoma arising at an ileostomy site 46 years after total proctocolectomy for Crohn’s colitis. In addition, we performed a literature search and found 36 such cases reported. Based on the results of this case and literature review, we concur with the previously reported theory that the etiology of this phenomenon is likely the result of colonic metaplasia in the ileal mucosa, which eventually progresses to carcinoma. Common presenting symptoms include a bleeding, friable mass, difficulty fitting the stomal appliance, and bowel obstruction. Once confirmed by biopsy, appropriate surgical en bloc excision and stomal relocation is the mainstay of therapy. Lymph node metastasis occurs in 19 percent of patients and survival is at least 85 percent. Adjuvant therapy may be of additional benefit. Patient education is important for early detection as the lesion typically appears an average of 27 years after the original operation.