The neuropathology of intestinal failure and small bowel transplantation

The aim of this study was to elucidate the neuropathological substrate of i ntestinal failure before and after small bowel transplantation (SBT). Retro spective analysis of complete autopsy or brain biopsy specimens of 17 patie nts with intestinal failure (12 children and 5 adults) were studied. Patien ts were divided into two groups. Group I (transplanted group; n = 13) inclu ded those patients who underwent intestinal transplantation under tacrolimu s and steroids immunosuppressive therapy. Group II (control group) included 4 children with intestinal failure who were candidates for SET and died wh ile awaiting an intestinal allograft. Central nervous system (CNS) abnormal ities were seen in 92% of the SET recipients and in 100% of SET candidates. The neuropathological lesions of SET recipients included: (a) vascular les ions: global brain ischemia, infarcts, intracranial hemorrhage and edema (7 children/2 adults; 69%); (b) cerebral atrophy (6 children; 46%); (c) Alzhe imer type II gliosis (5 children/4 adults; 69%); (d) infection (3 patients; 23%) due to cytomegalovirus (1 child), Aspergillus fumigatus (1 adult) and progressive multifocal leukoencephalopathy (PML)-like (1 adult); (e) Epste in-Barr virus-related cerebral post-transplant lymphoproliferative disorder (2 children; 15%); and (f) central pontine and extrapontine myelinolysis ( 1 child; 7.5%). The neuropathological lesions of SET candidates were Alzhei mer type II astrocytosis (4 patients), vascular changes (4 patients), brain atrophy (4 patients) and cerebral candidiasis (1 patient). CNS vascular, m etabolic and infectious pathology are significant causes of morbidity and m ortality in patients suffering intestinal failure, both before and after SE T. Brain atrophy was a frequent finding and may be related to nutritional a nd developmental inadequacy of long-term total parenteral nutrition.