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.

中文翻译：

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儿童包膜性腹膜硬化症 (EPS) 的手术治疗：国际病例系列和文献综述。

背景 包裹性腹膜硬化症 (EPS) 在接受腹膜透析 (PD) 治疗的肾衰竭儿科患者中是一种罕见的现象。本研究强调了 EPS 管理中的临床挑战，特别强调围手术期注意事项和手术技术。方法 回顾性分析在曼彻斯特移植中心治疗的所有 EPS 儿科患者。结果 包括 4 名患者，PD 的中位持续时间为 78 个月。所有患者都有复发性腹膜炎（> 3 次），并且在透析方式从 PD 改变为血液透析或移植后的三个月内都有症状。在曼彻斯特，护理由一个多学科团队提供，包括提供成人 EPS 手术服务的外科医生，特别关注营养优化，脓毒症控制和伤口管理。根据腹腔内的污染情况，手术涉及小肠 + / - 造口的剖腹手术、灌洗和肠溶术。两名患者有一个正式的造口，分别在三个月和六个月时被逆转。两名患者接受了一次腹部闭合术，而两名患者在 48 小时进行了二次闭合手术。一名患者出现术后伤口感染，已接受药物治疗。一名患者的造口脱落，导致需要再次剖腹探查的腹腔内收集物。中位住院时间为 25 天，一旦建立肠内营养，患者即可出院。所有患者均未复发，肠道功能正常，目前四分之二的患者进行了功能性移植。结论 本系列表明 EPS 手术后 100% 的存活率和肠外喂养独立性。术后并发症很常见；然而，通过基于经验的个体化决策和相关的额外干预，患者完全康复。手术后的健康和发育仍在继续，有可能进行移植。图形摘要的更高分辨率版本可作为补充信息。