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Encapsulating peritoneal sclerosis in children on chronic PD: a survey from the European Paediatric Dialysis Working
Rukshana Shroff1, Constantinos J Stefanidis, Varvara Askiti
1Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. rukshana.shroff@gosh.nhs.uk
Insights
Encapsulating peritoneal sclerosis (EPS) in children on peritoneal dialysis (PD) is as common as in adults, but less deadly. Longer dialysis, frequent peritonitis, and ultrafiltration failure signal potential EPS in pediatric PD patients.
Area of Science:
- Pediatric Nephrology
- Gastroenterology
- Dialysis Complications
Background:
- Encapsulating peritoneal sclerosis (EPS) is a rare but serious complication of peritoneal dialysis (PD).
- Limited data exists on pediatric EPS, necessitating further investigation into its prevalence and outcomes in children.
- This study addresses the scarcity of information regarding EPS in pediatric PD patients.
Purpose of the Study:
- To determine the prevalence of EPS in children undergoing PD.
- To identify risk factors associated with EPS development in pediatric patients.
- To describe the clinical outcomes and mortality associated with pediatric EPS.
Main Methods:
- A 10-year survey of chronic PD patients from January 2001 to December 2010 across 14 European dialysis units.
- Inclusion of pediatric patients on chronic PD.
- Data collection on EPS diagnosis, patient demographics, dialysis vintage, peritonitis rates, and clinical outcomes.
Main Results:
- The prevalence of EPS in European children on PD was 1.5% (8.7 per 1000 patient-years).
- Patients with EPS had significantly longer PD vintage (median 5.9 years) and higher peritonitis rates compared to non-EPS patients.
- Ultrafiltration failure and bowel obstruction were common presenting symptoms, with 3 deaths and 8 patients on hemodialysis at follow-up.
Conclusions:
- The prevalence of pediatric EPS is comparable to adults, but mortality is significantly lower.
- A high index of suspicion for EPS is crucial in children with prolonged dialysis, high peritonitis rates, and ultrafiltration failure.
- Early diagnosis and management are vital for improving outcomes in pediatric EPS.
Background:
Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis (PD) that is associated with significant morbidity and mortality in adults. There are scarce data for children. We performed a 10-year survey to determine the prevalence, risk factors and outcome for EPS in children.
Methods:
Chronic PD patients in 14 dialysis units participating in the European Paediatric Dialysis Working Group between January 2001 and December 2010 were included in this study.
Results:
Twenty-two cases of EPS were reported (prevalence 1.5%; 8.7 per 1000 patient-years on PD). Median PD vintage was 5.9 (1.6-10.2) in EPS and 1.7 (0.7-7.7) years in the remainder of the PD population (P<0.0001). EPS patients had a significantly higher peritonitis rate than non-EPS patients (P=0.2). EPS was diagnosed while the child was on PD in 17 (77%), after conversion to haemodialysis (HD) in 3 and after transplantation in 2. Fifteen of 17 (88%) developed ultrafiltration (UF) failure. The median interval between UF failure and presentation with bowel obstruction was 2.8 (0.02-5.8) months. Twenty (91%) had clinical and radiological signs of bowel obstruction. Enterolysis was performed in 14 and 19 received immunosuppression or tamoxifen. Nine required parenteral nutrition. At final follow-up 4.8 (1.3-8.7) years after EPS diagnosis, 3 patients died, 11 had a functioning transplant and 8 were on HD.
Conclusions:
The prevalence of EPS in European children on PD is comparable with that of adult PD patients, but mortality from paediatric EPS is significantly lower. A high index of suspicion is required for the diagnosis of EPS in children with longer dialysis duration, a high peritonitis rate and UF failure.
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