Proctocolectomy for ulcerative colitis after liver transplantation for primary sclerosing cholangitis.

BACKGROUND: 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. PATIENTS AND METHODS: 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). RESULTS: 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. CONCLUSION: 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.

Liver transplantation for erythropoietic protoporphyria: report of a case with medium-term follow-up.

A case of liver transplantation is described in a 35-year-old male with hepatic failure due to erythropoietic protoporphyria. Data regarding protoporphyrin levels in erythrocytes and faeces, before and after transplantation, seem to indicate that, in this case, protoporphyrin overproduction was, in part, due to liver synthesis. Four years after surgery, the patient is completely free of skin photosensitivity. Liver function tests are normal and there are no significant protoporphyrin deposits in the new liver. However, recurrence of the disease in the long-term cannot be excluded, since erythrocyte protoporphyrin levels have remained elevated after liver transplantation.

[Critical evaluation of preoperative instrumental staging of rectal tumors. Research on the adequate use of US, CT and MR]. / Valutazione critica della stadiazione strumentale preoperatoria dei tumori del retto. Ricerca di un impiego adeguato di US, TC e RM.

Both traditional exams (rectal exploration, rectoscopy, barium enema, CEA) and advanced imaging (31 US, 40 CT and 11 MR) were performed for preoperative evaluation of rectal carcinoma in order to assess the accuracy of radiological imaging in the T and N staging. The results obtained have not been considered satisfactory and it is felt that US, CT and MR should not be employed routinely for rectal staging. Indeed accuracy of US, CT and MR is respectively 64%, 75% and 81% in the T evaluation and 64%, 70% and 64% in the N staging. In order to evaluate the effective usefulness of these three latter imaging techniques a double therapeutical choice was proposed. The first treatment option was suggested on the basis of traditional staging while a second choice was given considering US, TC and MR data also. Operatory findings subsequently allowed a definitive judgement on the influence of the different techniques on treatment selection. US has furnished useful data that could have allowed us to modify treatment in one case while in 5 other cases diagnostic error would have influenced treatment negatively. CT was useful in 5 cases while in 7 cases it would have influenced treatment choice negatively. MR would have been useful in one case and harmful in another. It is concluded that only patients with large neoplasms (stages T3 and T4) benefit from CT and MR staging with the exception of those cases that have tumors above the peritoneal fold or in strict relation with the sphincter structures. US was useful only in evaluating relations of neoplasms of the anterior rectal wall with nearby pelvic structures.