Proctocolectomy for ulcerative colitis after liver transplantation for primary sclerosing cholangitis

Sclerosing cholangitis is a chronic cholestatic liver disease of unknown etiology. Immunologic and genetic factors may be involved in the pathogenesis of the disease, characterized by fibrosis involving bile ducts, which can progress to biliary cirrhosis and cholangiocarcinoma (8%-30%). Sclerosing cholangitis is frequently associated with inflammatory bowel diseases, especially ulcerative colitis (60%-80%), which may require a proctocolectomy with ileal pouch anal anastomosis if there is no response to therapy or the appearance of colonic carcinoma.

Among 1629 liver recipients from 1990 to 2008, 47 (2.9%) had sclerosing cholangitis as the cause of cirrhosis. Forty two percent of these also had associated inflammatory bowel disease with 34% displaying ulcerative colitis. Eight patients died after liver transplantation (OLT) in the absence of recurrence of sclerosing cholangitis. Among the remaining 39 patients, 17 (43.6%) developed recurrent of sclerosing cholangitis; three required re-OLT, and among these three patients, one developed another recurrence. After OLT, ulcerative colitis persisted in an active state in eight patients, requiring proctocolectomy with ileal pouch-anal anastomosis for three patients (median time after OLT was 78.6 months).

One of the three patients who had proctocolectomy had an immediate complication, a pelvic hematoma, which required a surgical approach. One patient developed acute pouchitis 15 months after OLT, medically treated with antibiotics and corticoids. Histology of the colectomy specimen demonstrated colorectal cancer in two patients (pT3N0 and pT2N0) and high-grade dysplasia in the remaining subjects. All patients displayed a cure of their colonic disease (median follow-up 14 months) despite two patients developing recurrence of the liver disease.

Proctocolectomy with ileal pouch anal anastomosis is safe in patients who underwent OLT for sclerosing cholangitis in association with ulcerative colitis. If not surgically treated, patients may receive immunosuppression to prevent rejection and disease recurrence, avoiding at the same time the occurrence of "de novo" neoplasms. Mammalian target or rapamycin inhibitors may have an important role but this must be established with randomized controlled trials.