Department of Child Healthy, Pediatric Nephrology Unit, Royal Hospital, Muscat, Oman.
Pediatric Residency Training Program, Oman Medical Specialty Board, Muscat, Oman.
Pediatr Transplant. 2024 Nov;28(7):e14867. doi: 10.1111/petr.14867.
Encapsulating peritoneal sclerosis (EPS) is an uncommon complication of long-term peritoneal dialysis (PD). Despite its rarity, EPS significantly increases morbidity and mortality in patients undergoing prolonged peritoneal dialysis. In children on PD, the incidence of EPS ranges from 0.5% to 7.3%.
We present the case of a 13-year-old Omani boy diagnosed with end-stage kidney disease (ESKD) secondary to steroid-resistant nephrotic syndrome due to diffuse mesangial sclerosis at the age of 2 years. He was started on automated peritoneal dialysis (APD) on the same year and experienced four episodes of peritonitis, which were treated successfully with intraperitoneal (IP) antibiotics. In January 2023, he developed intermittent abdominal pain and chronic constipation, which progressed to daily vomiting, reduced oral intake, and weight loss. He later developed subacute intestinal obstruction which was managed conservatively. A CT scan of the abdomen revealed findings consistent with EPS. Following the diagnosis of EPS, peritoneal dialysis (PD) was discontinued, and the patient transitioned to hemodialysis. Treatment for EPS began with steroids and Tamoxifen. Subsequently, he underwent deceased donor kidney transplantation and was started on multiple immunosuppressive medications. During subsequent follow-up appointments, he was maintained on total parenteral nutrition (TPN) along with a soft diet. His overall condition improved significantly, enhancing his quality of life.
This case highlights the risk of encapsulating peritoneal sclerosis (EPS) in patients undergoing long-term peritoneal dialysis. Transitioning to hemodialysis and kidney transplantation, combined with targeted treatments such as steroids and Tamoxifen, significantly improved the patient's condition and quality of life. Early diagnosis and intervention are crucial for effective management of EPS in children.
包裹性腹膜硬化症（EPS）是长期腹膜透析（PD）的一种罕见并发症。尽管其发病率较低，但在接受长期腹膜透析的患者中，EPS 显著增加了发病率和死亡率。在接受 PD 的儿童中，EPS 的发病率范围为 0.5%至 7.3%。
我们报告了一例 13 岁的阿曼男孩病例，他因弥漫性系膜硬化症导致类固醇耐药性肾病综合征，于 2 岁时被诊断为终末期肾病（ESKD）。同年，他开始接受自动化腹膜透析（APD），并经历了 4 次腹膜炎发作，均通过腹腔内（IP）抗生素成功治疗。2023 年 1 月，他出现间歇性腹痛和慢性便秘，随后发展为每日呕吐、减少口服摄入和体重减轻。他后来出现亚急性肠梗阻，通过保守治疗进行了管理。腹部 CT 扫描显示符合 EPS 的发现。在诊断为 EPS 后，停止了 PD，并将患者转为血液透析。EPS 的治疗开始使用类固醇和他莫昔芬。随后，他接受了已故供体肾移植，并开始使用多种免疫抑制药物。在随后的随访预约中，他接受了全胃肠外营养（TPN）和软食。他的整体状况显著改善，提高了他的生活质量。
本病例强调了长期腹膜透析患者发生包裹性腹膜硬化症（EPS）的风险。转为血液透析和肾移植，并结合类固醇和他莫昔芬等靶向治疗，显著改善了患者的病情和生活质量。早期诊断和干预对于儿童 EPS 的有效管理至关重要。
