Background: The association between primary sclerosing cholangitis (PSC) and ulcerative colitis (UC) often mandates their contemporaneous management. Orthotopic liver transplantation (OLTX) has emerged as the only curative therapy for PSC, and total proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the definitive treatment for refractory UC. The published experience to date describing IPAA after OLTX has been limited; we sought to examine outcomes associated with proctocolectomy-IPAA after OLTX. Materials and Methods: We reviewed our multi-institutional experience performing proctocolectomy-IPAA for UC after OLTX for PSC. Results: Twenty-two patients underwent proctocolectomy-IPAA for UC after OLTX for PSC at four academic medical centers between 1989 and 2006. No perioperative complications or allograft dysfunction were observed. During a median follow-up of 52 months, complications have included transient dehydration (n=6), chronic pouchitis (n=2), recurrent PSC (n=2), small bowel obstruction (n=2), and pouch-anal anastomotic stricture (n=1). Median 24-h stool frequency was 5, and fecal continence was reported as satisfactory by all patients. Conclusions: This multi-institutional experience suggests that proctocolectomy-IPAA can be performed safely after OLTX. Management strategies should include optimization of small bowel length during pouch and ileostomy construction, vigorous postoperative hydration, early ileostomy closure, and careful monitoring for pouchitis.
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