Surgical management of Encapsulating Peritoneal Sclerosis (EPS) in children: international case series and literature review

Abstract

Background: Encapsulating Peritoneal Sclerosis (EPS) is a rare phenomenon in paediatric patients with kidney failure treated with peritoneal dialysis (PD). This study highlights clinical challenges in the management of EPS, with particular emphasis on peri-operative considerations and surgical technique.

Methods: Retrospective analysis of all paediatric patients with EPS treated at the Manchester Centre for Transplantation.

Results: Four patients were included with a median duration of 78 months on PD. All patients had recurrent peritonitis (> 3 episodes), and all had symptoms within three months of a change of dialysis modality from PD to haemodialysis or transplant. In Manchester, care was delivered by a multi-disciplinary team, including surgeons delivering the adult EPS surgical service with a particular focus on nutritional optimisation, sepsis control, and wound management. The surgery involved laparotomy, lavage, and enterolysis of the small bowel + / - stoma formation, depending on intra-abdominal contamination. Two patients had a formal stoma, which were reversed at three and six months, respectively. Two patients underwent primary closure of the abdomen, whereas two patients had re-look procedures at 48 h with secondary closure. One patient had a post-operative wound infection, which was managed medically. One patient's stoma became detached, leading to an intra-abdominal collection requiring re-laparotomy. The median length of stay was 25 days, and patients were discharged once enteral feeding was established. All patients remained free of recurrence with normal gut function and currently two out of four have functioning transplants.

Conclusions: This series demonstrates 100% survival and parenteral feed independence following EPS surgery. Post-operative morbidity was common; however, with individualised experience-based decision-making and relevant additional interventions, patients made full recoveries. Health and development post-surgery continued, allowing the potential for transplantation. A higher resolution version of the Graphical abstract is available as Supplementary information.

Keywords: Encapsulating Peritoneal Sclerosis; Kidney failure; Peritoneal dialysis; Surgery.

Fig. 1

Pre-operative CT-scan of Case 2 showing profuse turbid ascites, thickening of the intestinal wall, intestine tethered to the spinal column, but no peritoneal calcifications

Fig. 2

Pre-operative CT scan of Case 4 showing a large encapsulated abdominal fluid collection with associated peripheral peritoneal calcification

Fig. 3

Intra-operative picture of index procedure at the first inspection of the abdomen (Case 4), demonstrating bloodstained ascites and fibrous encasement of the small bowel

Fig. 4

Intra-operative picture at 48 h post-index procedure (Case 4), showing viable small bowel loops and healthy abdominal wall tissue for safe closure