Surgical Treatment of Medically Refractory Encapsulating Peritoneal Sclerosis in a Patient After Kidney Transplant.

Encapsulating peritoneal sclerosis is a rare but highly morbid disease process in patients with end-stage kidney disease on peritoneal dialysis. Surgical management has been described in patients with encapsulation of bowel causing obstruction. Here, we describe a case of surgical management in a patient following kidney transplant with medically refractory ascites and lower extremity edema.

Weekly intraperitoneal steroid infusion to treat recurrent ascites in a patient with early stage of peritoneal dialysis-related encapsulating peritoneal sclerosis: A case report.

Encapsulating peritoneal sclerosis (EPS) is a rare and potentially fatal complication of long-term peritoneal dialysis (PD). EPS-induced large volume and recurrent ascites represents a challenging condition. We report a 51-year-old man with kidney failure treated with PD for 13 years who eventually developed early stage of EPS accompanied with poor intake and recurrent ascites. After management including discontinuing PD and switching to haemodialysis, as well as oral steroids and tamoxifen administration, the patient had refractory ascites. An intervention of weekly intraperitoneal steroid infusion with methylprednisolone was implemented for a year. Gradually, we observed a reduction in ascites drainage, an improvement of clinical symptoms and the patient's nutritional status. The PD catheter was successfully removed as there was no recurrence of ascites. Intraperitoneal corticosteroid administration represents a new intervention for patients with early stage of EPS and recurrent ascites after PD cessation.

Pathophysiology of encapsulating peritoneal sclerosis: lessons from findings of the past three decades in Japan.

Encapsulating peritoneal sclerosis (EPS), a condition with a high mortality rate, is a serious complication of peritoneal dialysis (PD). In Japan, EPS became a central issue in the clinical setting during the mid-90s and the beginning of this century. However, following the introduction of biocompatible neutral PD solutions containing lower levels of glucose degradation products, the incidence and clinical severity of EPS has been greatly lessened. During the past three decades, the etiology of EPS has been elucidated by findings obtained by peritoneal biopsy, laparoscopy, and surgical intervention. Accumulating findings suggest the need for a paradigm change on the nature of EPS pathophysiology; notably, EPS appears not to reflect peritoneal sclerosis per se, but rather the formation of a neo-membrane as a biological reaction to peritoneal injury. This narrative review looks back on the history of EPS in Japan, and discusses EPS pathophysiology, the impact of neutral PD solution on peritoneal protection, and a future novel diagnostic approach, ultra-fine endoscope, for the identification of patients at high risk of EPS.

Sclerosing Encapsulating Peritonitis: A Rare Cause of Bowel Obstruction.

Sclerosing encapsulating peritonitis (SEP), also referred to as abdominal cocoon syndrome, is a rare cause of bowel obstruction characterized by a thickened fibrous peritoneum that encapsulates the intestines. The exact etiology is idiopathic but may be associated with long-term peritoneal dialysis (PD). In the absence of risk factors for adhesive disease, preoperative diagnosis can be difficult and may require operative intervention or advanced imaging to diagnose. Thus, the inclusion of SEP in the differential diagnosis for bowel obstruction is essential for early detection. Existing literature is focused on renal disease as an origin, but it can be multifactorial. Here, we discuss a case of sclerosing encapsulating peritonitis in a patient without known risk factors.

Encapsulating peritoneal sclerosis: Your questions answered.

Encapsulating peritoneal sclerosis (EPS) is a clinical syndrome hallmarked by the formation of a fibrous cocoon encapsulating the bowel resulting in morbidity and mortality. EPS is most frequently associated with peritoneal dialysis (PD), particularly with its discontinuation. While EPS is one of the most feared complications of PD, the majority of patients receiving PD will not go on to develop EPS. Risk factors for development include time on PD, some types of peritonitis and discontinuation of PD. Owing to its rarity, much of the knowledge of EPS comes from case series and registries and treatments are extrapolated from low-quality evidence. Malnutrition is a significant driver of mortality, and nutritional support is critical in management. We present a case of EPS and frequently asked questions including the definition, diagnosis, epidemiology, pathophysiology, risk factors, role of infection, management and roles of nutrition and surgery.

A case report of encapsulating peritoneal sclerosis followed by cesarean section: Clinical diagnosis and treatment experience.

RATIONALE: Encapsulating peritoneal sclerosis (EPS), also known as abdominal cocoon syndrome, is an uncommon condition that typically presents with features of bowel obstruction. EPS followed by cesarean section is extremely rare. Intestinal obstruction caused by EPS lacks specificity and poses clinical difficulties for diagnosis and treatment. We present the case of a patient with recurrent intestinal obstruction followed by cesarean section, and the diagnosis of EPS was confirmed intraoperatively. The patient recovered well postoperatively, and achieved satisfactory therapeutic results. Management of this condition tests the surgeon's knowledge and experience of the disease. PATIENT CONCERNS: A 27-year-old woman with recurrent abdominal pain and distention accompanied by reduced anal discharge and defecation there months. The patient had a history of cesarean section 4 months earlier and recovered well after operation. She had no other history of abdominal surgery or diseases. On examination, a 10-cm long transverse incision was made in the lower abdomen, and marking on the intestinal movements were observed in the left mid-abdomen. A long, soft lump with good mobility was touched in the left lower abdomen. The abdominal computed tomography and small bowel barium meal examination revealed incomplete intestinal obstruction. DIAGNOSIS: Incomplete small bowel obstruction due to abdominal adhesions after the cesarean section was initially considered. INTERVENTIONS: After conservative treatment, the symptom of intestinal obstruction still recurred. Thus, we decided to perform a surgery of repeated decortication of fibrous peritoneal membranes. OUTCOMES: The operation successfully released the intestinal obstruction and abdominal pain, postoperative course recovered smoothly. LESSONS: After cesarean section could develop EPS. Intestinal obstruction caused by EPS lacks specificity and poses clinical difficulty in diagnosis and treatment. The management of this condition tests the surgeon's knowledge and experience, and surgery is an effective treatment measure.

Peritoneal effluent MicroRNA profile for detection of encapsulating peritoneal sclerosis.

BACKGROUND: Encapsulating peritoneal sclerosis (EPS) is a catastrophic complication of peritoneal dialysis (PD) with high mortality. Our aim is to develop a novel noninvasive microRNA (miRNA) test for EPS. METHODS: We collected 142 PD effluents (EPS: 62 and non-EPS:80). MiRNA profiles of PD effluents were examined by a high-throughput real-time polymerase chain reaction (PCR) array to first screen. Candidate miRNAs were verified by single real-time PCR. The model for EPS prediction was evaluated by multiple logistic regression and machine learning. RESULTS: Seven candidate miRNAs were identified from the screening of PCR-array of 377 miRNAs. The top five area under the curve (AUC) values with 5 miRNA-ratios were selected using 127 samples (EPS: 56 vs non-EPS: 71) to produce a receiver operating characteristic curve. After considering clinical characteristics and 5 miRNA-ratios, the accuracies of the machine learning model of Random Forest and multiple logistic regression were boosted to AUC 0.97 and 0.99, respectively. Furthermore, the pathway analysis of miRNA associated targeting genes and miRNA-compound interaction network revealed that these five miRNAs played the roles in TGF-ß signaling pathway. CONCLUSION: The model-based miRNA expressions in PD effluents may help determine the probability of EPS and provide further therapeutic opinion for EPS.

Abdominal cocoon syndrome: Rare cause of intestinal obstruction-Case report and systematic review of literature.

BACKGROUND: Abdominal cocoon or sclerosing encapsulating peritonitis is an uncommon condition in which the small bowel is completely or partially encased by a thick fibrotic membrane. Our study presents a case of sclerosing encapsulating peritonitis and conducts a literature review. METHODS: A bibliographic research was conducted. Our research comprised 97 articles. Gender, age, symptoms, diagnostic procedures, and treatment were all included in the database of patient characteristics. CASE PRESENTATION: A 51-year-old man complaining of a 2-day history of minor diffuse abdominal pain, loss of appetite, and constipation was presented in emergency department. Physical examination was indicative of intestinal obstruction. Laboratory tests were normal. Diffuse intraperitoneal fluid and dilated small intestinal loops were discovered on computed tomography (CT). An exploratory laparotomy was recommended, in which the sac membrane was removed and adhesiolysis was performed. He was discharged on the tenth postoperative day. RESULTS: There were 240 cases of abdominal cocoon syndrome in total. In terms of gender, 151 of 240 (62.9%) were male and 89 of 240 (37%) were female. Ages between 20 and 40 are most affected. Symptoms include abdominal pain and obstruction signs. For the diagnosis of abdominal cocoon syndrome, CT may be the gold standard imaging method. The surgical operation was the treatment of choice in the vast majority of cases (96.7%). Only 69 of 239 patients (28.9%) were detected prior to surgery, and CT was applied in these cases. CONCLUSION: Abdominal cocoon is a rare condition marked by recurrent episodes of intestinal obstruction. Surgical therapy is the most effective treatment option.

Effluent decoy receptor 2 as a novel biomarker of peritoneal fibrosis in peritoneal dialysis patients.

BACKGROUND: Peritoneal fibrosis (PF) is a common complication of peritoneal dialysis (PD), but a specific and sensitive biomarker for PF is lacking. The present study aimed to determine the use of effluent decoy receptor 2 (eDcR2) as a biomarker for PF in PD patients. METHODS: PD patients (n = 248) were recruited, and peritoneal specimens were collected at PD initiation (n = 30) and cessation (n = 33). Enzyme-linked immunoassay was used to measure eDcR2 and the eDcR2 appearance rate (eDcR2-AR) was calculated. The levels of DcR2 mRNA and protein were determined. The correlation of eDcR2 level with peritoneal function, histological parameters and DcR2 expression were analysed. Receiver operating characteristic (ROC) analysis was used to assess the diagnostic performance of eDcR2 for PF, which was defined as a submesothelial thickness 150 µm or more. Co-localisation of DcR2 with a mesothelial marker, fibroblast markers and fibrotic markers were determined. RESULTS: The eDcR2-AR level correlated with PD duration, D/P Cr values, peritoneal Kt/V and peritoneal injury scores, especially submesothelial thickness (r = 0.638, p < 0.001). DcR2 was primarily expressed in peritoneal fibroblasts, and co-localised with α-SMA, vimentin, collagen I and fibronectin, but not with E-cadherin. Peritoneal DcR2 expression had a positive correlation with eDcR2-AR. ROC analysis indicated eDcR2 had an area under the curve of 0.907 for detection of PF (sensitivity: 78.6%, specificity: 100%) and the best cut-off value was 392.5 pg/min. CONCLUSION: The eDcR2-AR level is a potential biomarker for assessing PF in PD patients. Effluent DcR2 was mainly derived from peritoneal fibroblasts and DcR2-positive cells may accelerate PF, suggesting that it may be a potential therapeutic target.

Encapsulating peritoneal sclerosis: from early diagnosis to successful kidney transplantation.

Encapsulating peritoneal sclerosis is an uncommon but serious complication of peritoneal dialysis. In most cases, the symptoms appear after peritoneal dialysis withdrawal, which hampers its diagnosis. We present the case of a 44-years-old Caucasian male who had been on peritoneal dialysis for 6 years and 3 months and was switched to hemodialysis due to ultrafiltration failure. During his last months on peritoneal dialysis, he developed anorexia and asthenia, which were initially attributed to dialysis inadequacy. After hemodialysis induction, the patient developed abdominal pain, increased abdominal volume, obstipation alternating with diarrhea, and weight loss. Computed tomography showed de novo ascites. A diagnosis of early encapsulating peritoneal sclerosis was considered, and treatment was promptly initiated with nutritional support, oral prednisolone, and tamoxifen for one year. The patient progressed with resolution of the symptoms. One month after the end of the treatment, he underwent a successful kidney transplant and remain without any major intercurrences. A high level of clinical suspicion is crucial for the early diagnosis of encapsulating peritoneal sclerosis as the disease can be fatal in advanced stages. This case highlights that with early treatment, kidney transplantation can be successfully performed after an episode of encapsulating peritoneal sclerosis.

Risk factors and clinical outcomes of encapsulating peritoneal sclerosis: A case-control study from China.

BACKGROUND: Encapsulating peritoneal sclerosis (EPS) is an uncommon, but serious complication in patients with continuous ambulatory peritoneal dialysis (PD) who have a considerable mortality rate. This study aimed to identify risk factors and outcomes of EPS in Chinese patients on PD. METHODS: Sixteen patients on PD who met the International Society for Peritoneal Dialysis criteria for diagnosis of EPS in the First Affiliated Hospital of Sun Yat-Sen University from 1997 to 2018 were included. Patients without EPS were matched for age, sex and the duration of PD and selected at a 1:3 ratio for the controls. A case-control study was conducted to analyse the clinical profile and risk factors associated with EPS in patients. RESULTS: The prevalence of EPS in patients on PD in our centre was 0.55%. The percentage of EPS significantly increased with the duration of PD. In univariate regression analysis, a history of peritonitis (odds ratios (OR): 2.83; 95% confidence interval (CI): 0.82-9.68; p = 0.08), peritoneal glucose exposure (OR: 1.12; 95% CI: 1.03-1.22; p < 0.01) and a high peritoneal transport status (OR: 14.70; 95% CI: 1.85-117.02; p < 0.01) were associated with EPS in patients on PD. However in the multivariate model, only a high peritoneal transport status (adjusted odds ratios (aOR): 13.65; 95% CI: 1.69-109.96; p = 0.01) was independently associated with EPS. CONCLUSION: The rate of EPS significantly increases with the duration of PD. Progressive peritoneal dysfunction, especially a high peritoneal transport status, is associated with a higher risk of EPS in this population.

A Matrix Metalloproteinase-2-Based Nomogram to Assess the Risk of Encapsulating Peritoneal Sclerosis in Peritoneal Dialysis Patients.

BACKGROUND: Encapsulating peritoneal sclerosis (EPS) is a rare but serious complication of peritoneal dialysis (PD). So far, there is no biomarker-based prediction tool available for EPS. Matrix metalloproteinase-2 (MMP-2) is a protein involved in the breakdown of the extracellular matrix, and the effluent MMP-2 can be a potential biomarker of EPS. This study is aimed at developing a nomogram for EPS based on effluent MMP-2 levels. Patients and Methods. We enrolled 18 EPS patients and 90 gender-matched PD patients without EPS in this cross-sectional case-controlled study. The effluent MMP-2 levels and possible risk factors for EPS were analyzed using multivariable logistic regression, and a nomogram was developed. The nomogram was validated using 200 bootstrap resamples to reduce overfit bias. RESULTS: The effluent MMP-2 levels in EPS patients were significantly higher than those in normal PD patients (p < 0.001, Manny-Whitney U test). Effluent MMP-2 levels and PD duration were independently associated with EPS risks (p < 0.001 and p = 0.001) in multivariate logistic regression. A nomogram based on MMP-2 levels and PD duration was proposed. The AUC of MMP-2 was 0.824, and the AUC of the nomogram was 0.907 (p = 0.05). CONCLUSION: A nomogram based on effluent MMP-2 levels and PD duration may predict EPS with high accuracy.

Recent Understanding of Peritoneal Pathology in Peritoneal Dialysis Patients in Japan.

The thin peritoneum covering the peritoneal cavity has been used as a dialysis membrane for peritoneal dialysis (PD) because it is highly vascularized and has a large body surface area. However, it has been reported that peritoneal membranes affected by peritonitis, as well as those exposed to the nonphysiological high glucose levels containing PD dialysate, may undergo histological and functional changes. Patients undergoing PD may experience encapsulating peritoneal sclerosis (EPS), which is a life-threatening serious complication of PD that can significantly impair activities of daily living. The incidence of EPS was 1.4-7.3% of maintenance PD patients in the 1980s. The incidence has improved to 1.0% after a neutral dialysate became the standard PD treatment in Japan. Furthermore, the pathogenesis of EPS is uncertain although its onset may be explained by the "two-hit theory," in which some factors leading to impairment had an additive effect on simple peritoneal sclerosis. The evaluation of histopathological findings has shown the impact of the neutral dialysate on peritoneal deterioration as well as its role in the development of functional changes. In the present report, we discuss the advances in the understanding of peritoneal deterioration based on histological and macroscopic evaluations of the peritoneum of patients undergoing PD. We also discuss the recent treatment for PD patients.

A case of cyclic hemoperitoneum in a hemodialysis patient: Encapsulated peritoneal sclerosis complicated by endometriosis and fungal peritonitis.

Encapsulated peritoneal sclerosis (EPS) is a rare, but frequently fatal, long-term complication of peritoneal dialysis. Endometriosis is a common gynecological problem but hemoperitoneum due to endometriosis has been reported to be extremely rare in hemodialysis (HD) patients. A 25-year-old female HD patient was admitted to our clinic with nausea, vomiting, abdominal pain, and weight loss for last 3 months. Candida tropicalis and Candida glabrata were isolated in the fungal cultures from peritoneal fluid. Her abdominal computerized tomography scan has shown irregular peritoneal calcifications, diffuse peritoneal thickening, dilatation of the small bowel loops, and cocoon formation which all were typical for EPS. Hemoperitoneum was reported to recur for four times with intervals suggesting menstrual cycles. Her peritoneal biopsy, along with the signs of EPS, has also revealed the presence of endometriosis. The patient died with symptoms of septic shock in the first year of EPS diagnosis.

A Successful Treatment of Encapsulating Peritoneal Sclerosis in an Adolescent Boy on Long-term Peritoneal Dialysis: A Case Report.

Encapsulating peritoneal sclerosis (EPS) is a rare life-threatening complication associated with peritoneal dialysis (PD). EPS is characterized by progressive fibrosis and sclerosis of the peritoneum, with the formation of a membrane and tethering of loops of the small intestine resulting in intestinal obstruction. It is very rare in children. We present a case of a 16-year-old adolescent boy who developed EPS seven years after being placed on continuous ambulatory peritoneal dialysis (CAPD) complicated by several episodes of bacterial peritonitis. The diagnosis was based on clinical, radiological, intraoperative and histopathological findings. The patient was successfully treated with surgical enterolysis. During a 7-year follow-up, there have been no further episodes of small bowel obstruction documented. He still continues to be on regular hemodialysis and is awaiting a deceased donor kidney transplant. EPS is a long-term complication of peritoneal dialysis and is typically seen in adults. Rare cases may be seen in the pediatric population and require an appropriate surgical approach that is effective and lifesaving for these patients.

Encapsulating Peritoneal Sclerosis: A Case Report and Literature Review.

BACKGROUND 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.