IntroductionIleal-pouch anal anastomosis (IPAA) is a widely preferred surgical intervention in patients with medically refractory ulcerative colitis, familial adenomatous polyposis, and indeterminate colitis. Concerns have been raised regarding the impact of connective tissue disorders on the outcomes of IPAA owing to potential alterations in collagen metabolism. To date, there is a lack of studies assessing the feasibility, long-term viability, and pouch function of IPAA in patients with connective tissue disorders. We hypothesized that the presence of a connective tissue disorder was associated with an increased risk of pouch failure. MethodsData were collected from a prospectively maintained pouch database and chart review. Patients with connective tissue disorders were propensity score matched in a 1:5 ratio with control pouch patients without connective tissue disorders. A comparative analysis of the demographics, long-term complications, functional outcomes, and QoL was conducted. ResultsNineteen patients with connective tissue disorders were matched with 95 control pouch patients. Postoperative diagnoses included ulcerative colitis (82.5%), indeterminate colitis (7%), constipation (5.3%), Crohn's disease (4.4%), and familial adenomatous polyposis (1%). The connective tissue disorders identified included unspecified diffuse connective tissue disorders (26.3%), Ehlers-Danlos syndrome (15.7%), Sjögren's syndrome (15.7%), and others (31.2%). Patients with connective tissue disorders were more likely to have pouch leaks (OR 4.8, 95% CI: 1.54,15, p=0.007), pouchitis (OR 3.02, 95% CI: 1.09, 8.37, p=0.034), pouch revisions (OR 3.83, 95% CI: 1.31,11.2, p=0.014), and pouch failure (OR 3.55, 95% CI: 1.25, 10.1, p=0.017). When questioned, patients with connective tissue diseases were more likely to have work restrictions (OR 4.68, 95% CI: 0.74, 6.38, p=0.005) and a worse quality of life (std. estimate -0.15, 95% CI: -0.28,-0.19, p=0.025). ConclusionPatients with connective tissue diseases are more likely to be affected by pouch-related complications and pouch failure and report worse quality of life. Physicians treating patients with these disorders should counsel patients appropriately and be aware of the elevated risks of leaks, pouchitis, and pouch failure.
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