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

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.