Abstract
BackgroundEncapsulating 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.MethodsRetrospective analysis of all paediatric patients with EPS treated at the Manchester Centre for Transplantation.ResultsFour 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.ConclusionsThis 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.Graphical abstractA higher resolution version of the Graphical abstract is available as Supplementary information
Highlights
Encapsulating Peritoneal Sclerosis (EPS) is a rare but potentially devastating diagnosis in paediatric patients with kidney failure treated with peritoneal dialysis (PD) [1]
A high index of suspicion should be maintained in patients on long-term PD (> 5 years) who present with abdominal symptoms, malnutrition and raised inflammatory markers [17]
The overall prevalence of EPS paediatric patients is reported to be low at 1.5% to 1.9% across the three registry studies [14,15,16]
Summary
Encapsulating Peritoneal Sclerosis (EPS) is a rare but potentially devastating diagnosis in paediatric patients with kidney failure treated with peritoneal dialysis (PD) [1]. Characteristic features of EPS include chronic inflammation and thickening of the peritoneum with encapsulation of bowel loops and formation of a pathognomonic fibrotic cocoon [5]. It has a spectrum of symptoms, ranging from general malaise, weight loss, and anorexia to eventual bowel obstruction, manifesting with abdominal pain, vomiting, and constipation [6]. 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. Health and development post-surgery continued, allowing the potential for transplantation
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