Chronic rejection with sclerosing peritonitis following pediatric intestinal transplantation

Abstract

Intestinal transplantation is considered the usual treatment for patients with permanent intestinal failure when parenteral nutrition has failed. Chronic rejection is a complication difficult to diagnose because of the scarcity and lack of specificity in the symptoms and the characteristics of typical histological findings. We report the case of a four-yr-old patient who received an isolated intestinal transplant. After developing a chronic rejection he presented an intestinal obstruction secondary to a sclerosing peritonitis that required the surgical removal of the graft.

Sclerosing peritonitis, a rare complication after intestinal transplant. Report of one case successfully treated with adjustment of immunosuppression

“…In our case, in concordance with the literature, diffuse infiltration of the intestinal wall was observed. Vasculopathy was not observed in all the cases reviewed, being, however, described as a concomitant feature of SP . Indeed, some of the reports describe the occurrence of SP as an end stage of chronic rejection , whereas other cases clearly were not associated with chronic rejection , indicating that also in the setting of IT several causes may contribute to the occurrence of SP.…”

“…SP is a rare entity that may arise after IT, consisting of progressive fibrosis and degeneration of the intestinal wall and mesenteries, clinically insidious and associated with high mortality . Here, we report a first case of SP occurring after IT that has been successfully treated with a combination of immunosuppressive drugs usually used for the post‐IT management.…”

“…Published data show that the prevalence rates in the general population are low, between 0.54% and 0.9% . So far, SP cases related to IT have been treated with partial intestinal resection or enterectomy leading to retransplantation . In some of these cases, adjustment of immunosuppression including corticosteroid boluses as palliative treatment was used, but in all cases, surgical resections were performed to solve the intestinal obstruction with high mortality associated with the occurrence of SP.…”

Clinical Allograft Rejection Syndromes in Intestinal Transplantation

“…Encapsulating peritoneal sclerosis has also been described in a few cases, although this finding is usually evident only in the operating room [38]. Most studies of chronic rejection have focused on the histology of explants after death or graft enterectomy [17,37].…”

Pathology of Pediatric Gastrointestinal and Liver Disease

“…Another striking feature that has been reported primarily in pediatric intestinal grafts is the development of a sclerosing or fi brosing peritonitis, wherein the serosa and subserosa of the entire allograft are markedly fi brotic and thickened with extensive areas of hyalinization and associated infl ammation (Noguchi Si et al 2002 ;Macedo et al 2005 ;Ramos et al 2007 ;Ruiz et al 2003 ). The clinical presentation is usually vomiting and features of bowel obstruction with abdominal pain.…”