Surgical outcome in patients with primary sclerosing cholangitis undergoing ileal pouch–anal anastomosis: A case-control study

doi:10.1016/j.surg.2005.07.014

Surgery

Volume 138, Issue 4, October 2005, Pages 631-639

https://doi.org/10.1016/j.surg.2005.07.014 Get rights and content

Background

The outcome of restorative proctocolectomy in the setting of chronic ulcerative colitis complicated by primary sclerosing cholangitis (PSC) is not clear. The purpose of this study was to determine the surgical outcome, risk of dysplasia/cancer, morbidity/mortality, long-term results, and functional and quality of life results in patients with inflammatory bowel disease (IBD) and PSC who underwent restorative proctocolectomy with ileal pouch–anal anastomosis and compare them in a case-matched study.

Methods

Patients with PSC-associated IBD undergoing restorative proctocolectomy between 1983 and 2002 were included in the study. This study group was matched for age, gender, diagnosis, duration of disease, anastomosis technique, and proximal diversion to a cohort of IBD patients with no associated PSC who underwent restorative proctocolectomy during the same period of time. Postoperative morbidity, incidence of neoplasia/cancer in the resected specimen, pouchitis, pouch failure, long-term mortality, and 5-year survival rates were compared between the groups. The functional and quality of life records were prospectively collected and compared between the groups. For each group, matched Kaplan-Meier survival analysis was also conducted comparing 5-year survival between the 2 cohorts, matching for diagnosis, duration of disease, age, gender, anastomosis type, and proximal diversion.

Results

Sixty-five patients with PSC and IBD underwent restorative proctocolectomy with ileal pouch–anal anastomosis during the study period. Two hundred sixty IBD patients with no associated PSC who matched with the outlined criteria comprised the control group. The follow-up period was 68 ± 50 months for the PSC group and 102 ± 62 months for the control group. A higher incidence of cancer (14% vs 5%, P = .02) and dysplasia in the resected specimen (40% vs 7%, P < .001), an associated increased risk of postoperative pelvic sepsis (14% vs 5%, P = .02), and higher long-term mortality (35% vs 4%, P < .001) were found in the PSC group compared with control group with no associated PSC. The majority, 13 of 23 (57%), of the deaths in the PSC group were a result of liver disease. Five-year survival for the PSC group was significantly poorer than the 5-year survival for the control group with no associated PSC. No significant differences were found in functional and quality of life results between the groups in the short- and long-term follow-up periods.

Conclusions

PSC-associated IBD patients after restorative proctocolectomy have a higher risk of neoplasia/cancer in the resected specimen, postoperative pelvic sepsis, and higher long-term mortality. Functional and quality of life remains similar in IBD patients after restorative proctocolectomy with or without associated PSC in the follow-up. However, patients with IBD and PSC have a significantly poorer survival than patients with no associated PSC after restorative proctocolectomy.

Section snippets

Materials and methods

By using our institutional review board–approved pelvic pouch database, we identified the patients with PSC and IBD who underwent RP/IPAA between 1983 and 2002. This study group was matched for age, gender, diagnosis, time to diagnosis, anastomosis technique, and proximal diversion to a cohort of IBD patients with no associated PSC who underwent RP during the same period of time. The pelvic pouch database provided the information on patients' demographics, disease history, dysplasia/cancer,

Results

From 1983 to December 2002, 2557 RPs and IPAAs were performed at the Cleveland Clinic Foundation Colorectal Surgery Department. During this period, there were 65 IBD patients with associated PSC who underwent RP/IPAA, and this was designated as the study cohort. Patients in the study group were compared with a cohort of patients with IBD with no associated PSC disease (N = 260) who underwent RP/IPAA during the same period of time. The results of the matching criteria are listed in Table I.

Discussion

The outcome of RP/IPAA in UC patients with associated PSC has not been well-established. Orthotopic liver transplantation (OLTx) is currently the curative treatment for PSC, but the surgical management of patients who have both UC and PSC can be difficult. The results of our current study suggest that the risk of postoperative pelvic sepsis, cancer/neoplasia in the resected specimen, and long-term mortality after RP/IPAA is higher in IBD patients with associated PSC in comparison with IBD

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Cited by (65)

Primary Sclerosing Cholangitis–Associated Pouchitis: A Distinct Clinical Phenotype
2022, Clinical Gastroenterology and Hepatology
Patients with primary sclerosing cholangitis (PSC) commonly undergo ileal pouch-anal anastomosis (IPAA) for medically-refractory ulcerative colitis (UC) or colorectal dysplasia. Pouchitis develops more frequently in patients with PSC, potentially leading to increased morbidity. We aimed to assess clinical characteristics and treatment outcomes for pouchitis in patients with PSC compared to a matched, non-PSC cohort.
All patients with PSC who underwent IPAA and were diagnosed with pouchitis (PSC-pouchitis) were identified. A matched cohort composed of non-PSC patients who underwent IPAA for UC and subsequently developed pouchitis (UC-pouchitis) was developed. Relevant demographic, clinical, endoscopic, histologic, and treatment data were collected and compared between groups.
Of those with PSC-pouchitis (n=182), 53.9% and 46.1% underwent IPAA for medically-refractory disease and dysplasia, respectively, compared to 88.7% and 11.3% in the UC-pouchitis group (P < .001). Patients with PSC-pouchitis were more likely to develop chronic pouchitis (68.1% vs 34.1%; P < .001), have moderate-to-severe pouch inflammation (54.9% vs 32.4%; P < .001), and prepouch ileitis (34.1% vs 11.5%; P < .001) compared to UC-pouchitis. Of those with PSC-pouchitis, 50.6% and 17.6% developed chronic antibiotic-dependent or antibiotic-refractory pouchitis, respectively, compared to 25.8% and 7.7% with UC-pouchitis. There was no difference in treatment response between the two groups with use of thiopurines, anti-tumor necrosis factor agents, and newer biologics.
PSC-associated pouchitis presents with a unique clinical phenotype, characterized by increased risk of chronic pouchitis, moderate-to-severe pouch inflammation, prepouch ileitis, and less response to conventional antimicrobial therapy.
Diagnosis and classification of ileal pouch disorders: consensus guidelines from the International Ileal Pouch Consortium
2021, The Lancet Gastroenterology and Hepatology
Restorative proctocolectomy with ileal pouch–anal anastomosis is an option for most patients with ulcerative colitis or familial adenomatous polyposis who require colectomy. Although the construction of an ileal pouch substantially improves patients' health-related quality of life, the surgery is, directly or indirectly, associated with various structural, inflammatory, and functional adverse sequelae. Furthermore, the surgical procedure does not completely abolish the risk for neoplasia. Patients with ileal pouches often present with extraintestinal, systemic inflammatory conditions. The International Ileal Pouch Consortium was established to create this consensus document on the diagnosis and classification of ileal pouch disorders using available evidence and the panellists' expertise. In a given individual, the condition of the pouch can change over time. Therefore, close monitoring of the activity and progression of the disease is essential to make accurate modifications in the diagnosis and classification in a timely manner.
Genetic and Environmental Considerations for Inflammatory Bowel Disease
2019, Surgical Clinics of North America
Citation Excerpt :
In contrast, patients with primary sclerosing cholangitis (PSC) and UC who undergo IPAA have rates of pouchitis (63%–75%41) compared with UC patients without PSC.39,41 It has been suggested that pouchitis may be more common in PSC patients due to their higher risk of postoperative complications, such as pelvic sepsis, which increases risk of chronic inflammation.42 The selective development of pouchitis based on indication for IPAA suggests that stasis of intestinal contents in the small intestine does not fully explain the inflammatory changes that occur.
The cleveland classification of ileal pouch disorders
2018, Pouchitis and Ileal Pouch Disorders: A Multidisciplinary Approach for Diagnosis and Management
While restorative proctocolectomy with ileal pouch-anal anastomosis, the standard of surgical treatment for patients with ulcerative colitis or familial adenomatous polyposis who require proctocolectomy. However, various adverse mechanical, inflammatory, functional, neoplastic, and metabolic conditions can occur postoperatively. Each category consists of multiple phenotypes. Commonly used diagnostic tools including clinical assessment, endoscopy, histology, imaging, and manometry. Accurate diagnosis and differential diagnosis are crucial for the prevention of disease progression and associated complications and guidance of proper treatment.
Extraintestinal manifestations and concurrent autoimmune disorders in pouch patients
2018, Pouchitis and Ileal Pouch Disorders: A Multidisciplinary Approach for Diagnosis and Management
Restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) remains the treatment of choice for patients with medically refractory ulcerative colitis (UC) or UC with dysplasia. There are risks of complications following IPAA including chronic pouchitis. Chronic pouchitis is associated with extraintestinal manifestations and autoimmune disorders, suggesting overlap in the disease pathogenesis. Pouchitis encompasses a spectrum based on the response or lack of response to antibiotic therapy. There is also emerging evidence of autoimmunity in a subgroup of patients with pouchitis, including patients with concurrent primary sclerosing cholangitis, patients with high serum immunoglobulin G4, or infiltration of immunoglobulin G4-expressing plasma cells in the pouch mucosa. It appears that in a subgroup of patients with chronic antibiotic refractory pouchitis (CARP), there is association with autoimmune factors. It is unclear whether the antibodies play any role in the pathogenesis of CARP or result of an epiphenomenon. However, the presence of these antibodies is useful as biomarkers in identifying patients with immune-mediated pouchitis (IMP), who are thereby more likely to be resistant to standard antibiotic therapy and more likely to respond to immunosuppressive therapies. Further studies are needed to determine if IMP and “non-IMP” types of CARP are distinct clinical entities, and if they are associated with differing clinical outcomes and response to medical therapies.
Risk factors for chronic inflammatory disorders of ileal pouch
2018, Pouchitis and Ileal Pouch Disorders: A Multidisciplinary Approach for Diagnosis and Management
Restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) has become the surgical procedure of choice for the majority of patients with ulcerative colitis who fail medical therapy or develop neoplasia. The IPAA procedure with reestablishment of gastrointestinal continuity significantly improves patients’ quality of life. However, adverse sequelae of inflammatory and noninflammatory conditions related to the pouch can occur postoperatively. Chronic pouchitis, Crohn’s disease (CD) of the pouch and cuffitis are the three most common inflammatory disorders following ileal pouch construction. Each of these disorders has been shown to be associated with different risk factors. In this chapter, we describe the risk factors related to chronic pouchitis, CD of the pouch and cuffitis in detail.

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: Presented at the 62nd Annual Meeting of the Central Surgical Association, Tucson, Arizona, March 10-12, 2005.

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Central Surgical AssociationSurgical outcome in patients with primary sclerosing cholangitis undergoing ileal pouch–anal anastomosis: A case-control study

Background

Methods

Results

Conclusions

Section snippets

Materials and methods

Results

Discussion

Am J Gastroenterol

Gastroenterology

Gastroenterology

Mayo Clin Proc

Autoimmun Rev

Primary sclerosing cholangitis: natural history, prognostic factors and survival analysis

Hepatology

Long-term functional outcome and quality of life after stapled restorative proctocolectomy

Ann Surg

Central Surgical Association
Surgical outcome in patients with primary sclerosing cholangitis undergoing ileal pouch–anal anastomosis: A case-control study