Posttransplant encapsulating peritoneal sclerosis: presentation of cases and review of the literature

Background: Encapsulating peritoneal sclerosis (EPS) is a rare but serious complication of peritoneal dialysis (PD). EPS almost exclusively occurs in patients treated longer than 3-5 years on PD. The more severe clinical features of EPS may develop if PD is discontinued (patient transferred to hemodialysis or transplanted). Methods: All PD patients diagnosed with EPS after transplantation were identified, and their data were compared with those of non-EPS PD patients transplanted in our unit between 1994 and 2010. Results: Four EPS cases were diagnosed among 157 transplanted PD patients. Mean renal replacement therapy and PD-only duration were 103.8 and 83.5 months in EPS and 26.5 and 22.2 months in non-EPS patients, respectively. All EPS patients (n = 4) required high-volume dialysis, 2 received icodextrin, 3 were high transporters, 1 had recurrent intraperitoneal bleeding, 4 received beta-blockers and 3 had peritonitis incidents. All required surgical intervention within 1-3 months after kidney transplantation (KT). Diagnosis of EPS was based on clinical symptoms, surgery, radiologic and histopathology findings. Treatment consisted of adhesiolysis (all), parenteral nutrition (PN) (3/4) and tamoxifen (all). One patient died 49 months after EPS diagnosis. Conclusions: First, bowel obstruction symptoms in long-term PD patients undergoing KT may suggest EPS. Second, long-term PD patients showing features of technique failure are at high risk of EPS after KT. Third, adhesiolysis, PN and tamoxifen are the available treatment options in EPS patients post KT. And finally, referral