Challenging differential diagnosis, Rare disease
Ali I. Al-Lawati, Maha Al Shaibi, Ghaitha Al Mahruqi, Titus Augustine, Zia Moinuddin, Meerah Al Hinai, Rana Al Moqbali, Hani Al Qadhi
Department of Nephrology, Sultan Qaboos University Hospital, Muscat, Oman
Am J Case Rep 2020; 21:e925341
Available online: 2020-08-24
Encapsulating peritoneal sclerosis (EPS) is a rare, life-threatening, and serious complication of long-term peritoneal dialysis (PD). No evidence-based management strategy has been established until now. Surgical management, including enterolysis and excision of the sclerotic and obstructing adhesions, should be considered as soon as conservative management fails to work. We report a case of EPS soon after transplantation in a patient with end-stage kidney disease who had been on long-term PD.
CASE REPORT: A 26-year-old man had been found to have advanced chronic kidney disease secondary to glomerulonephritis on pre-employment investigation. He was on continuous ambulatory PD for 5 years, after which he underwent a living donor renal transplant from his full HLA-matched sibling. He did well postoperatively, with excellent graft function. One month after transplantation, he repeatedly presented to our Emergency Department with signs and symptoms of complete small-bowel obstruction. Computed tomography of the abdomen showed features of small-bowel obstruction secondary to interloop adhesions. The patient was initially managed conservatively; however, as his condition continued to deteriorate, an exploratory laparotomy was carried out. Operative findings were suggestive of early EPS localized to the terminal ileum. Total enterolysis along with peritonectomy was performed along with resection of the diseased and obstructing terminal ileum. The patient did well, and he was discharged home day 10 postoperatively.
CONCLUSIONS: EPS remains a serious and fatal complication of long-term PD. Early definitive diagnosis, treatment, and ultimately surgical intervention may be required to prevent the morbidity and mortality associated with this condition.
Keywords: Kidney Transplantation, Peritoneal Dialysis, peritoneal fibrosis, Tissue Adhesions