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.

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.

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.

Clinical risk factors and outcomes of massive ascites accumulation after discontinuation of peritoneal dialysis.

Background: Encapsulating peritoneal sclerosis (EPS) is a serious complication of peritoneal dialysis (PD), with high morbidity and mortality that requires an early diagnosis for effective treatment. PD withdrawal and bacterial peritonitis are important triggers for the onset of EPS. However, few studies have focused on cases of PD withdrawal without a clinical diagnosis of peritonitis, cirrhosis, or carcinomatosis. We aimed to compare the clinical characteristics and computed tomography (CT) images of patients with or without ascites in such situations and assess clinical outcomes in terms of mortality.Methods: Our retrospective review included 78 patients who withdraw PD between January 2000 and December 2017.Results: Ten patients had ascites, and 68 did not have a significant intra-abdominal collection. The ascites group had a significantly longer PD duration (months; 134.41 [range, 35.43-181.80] vs. 32.42 [733-183.47], p < 0.001) and higher peritoneal membrane transport status based on the dialysate-to-plasma ratios of creatinine (0.78 ± 0.08 vs. 0.68 ± 0.11, p = 0.009) and glucose (0.27 ± 0.07 vs. 0.636 ± 0.08, p = 0.001) than the control group. CT parameters, including peritoneal calcification, thickness, bowel tethering, or bowel dilatation, were not all present in each patient with ascites and EPS. During the 12-month study period, the ascites group had a higher risk for developing EPS (70% vs. 0%, p < 0.001) and a higher 12-month all-cause mortality (30% vs. 0%, p = 0.002).Conclusions: Ascites accumulation was not rare after PD discontinuation. A longer PD duration and high peritoneal membrane transport status could predict subsequent ascites accumulation. Furthermore, patients with ascites were at a higher risk of EPS.

[Encapsulant peritonitis]. / Une péritonite encapsulante.

INTRODUCTION: Encapsulating peritonitis is a rare but severe chronic fibrotic condition related to the development of a white fibrous membrane surrounding the digestive tract. Idiopathic forms have been described, however the disease is most often secondary to peritoneal dialysis or more rarely to surgery. Treatment is difficult and not codified. CASE REPORT: We report here the observation of a 36-year-old patient whose diagnosis of encapsulating peritonitis was made after a long sub-occlusive history, eight years after a gastric ulcer perforation. DISCUSSION: We discuss the possible etiologies and we present a focus on this rare and little-known entity.

Abdominal cocoon syndrome: A rare cause of acute abdomen syndrome.

BACKGROUND: A rare cause of acute abdomen or intestinal obstruction, the abdominal cocoon syndrome is also described in the literature as sclerosing peritonitis or sclerosing encapsulating peritonitis. Abdominal cocoon is characterized by the total or partial wrapping of the abdominal organs by a fibrous membrane. Although it is usually observed in young women, the etiology is unknown. The diagnosis is usually made during laparotomy. In this case series, we aimed to present seven patients diagnosed with abdominal cocoon syndrome during operation. METHODS: The records of patients who underwent laparotomy for abdominal pain and/or intestinal obstruction in our hospital and diagnosed as abdominal cocoon during operation between January 2012 and November 2018 were retrospectively reviewed. The demographic characteristics of the patients, etiologic factors, surgical procedures, operative findings and follow-up of the patients were recorded. RESULTS: Four out of seven patients who were operated for abdominal cocoon were male and 3 of them were female. The median age of patients was 61 (57-63) years in male and 39.6 (28-49) years in female. Six of the patients were operated in emergency conditions with the diagnosis of an acute abdomen or ileus. One of the patients was operated with the diagnosis of an intra-abdominal mass in elective conditions. In five out of seven patients, all of the small intestines were wrapped with a fibrous collagen capsule, while two of the patient intestines were partially wrapped with a fibrous collagen capsule. Four of the patients had no underlying disease, while one of the patients had Familial Mediterranean Fever (FMF), one had Endometriosis and one had beta-blocker medication. One patient who had small bowel necrosis and septic peritonitis were observed during the operation and died post operative 6th days. Postoperative complications were not observed in the follow-up of other patients and reoperation was not required due to recurrence. CONCLUSION: Abdominal cocoon is a condition that is usually diagnosed during operation in patients that were operated for reasons, such as the acute abdomen or intestinal obstruction. When the diagnose delayed, death can be seen due to small bowel necrosis and septic complications. High clinical suspicion and radiological imaging are important in the preoperative diagnosis. Treatment is required adhesiolysis and excision of the fibrous membranes.

Early Detection of Imminent Encapsulating Peritoneal Sclerosis: Free Water Transport, Selected Effluent Proteins, or Both?

BACKGROUND: No diagnostic tool or methodology is currently available for early detection of imminent encapsulating peritoneal sclerosis (EPS). The objective of this study was to investigate the predictive value of free water transport (FWT) and construct a panel of peritoneal effluent proteins for EPS alone or in combination with FWT. These parameters could be incorporated in the follow-up of peritoneal dialysis (PD) patients. METHODS: A case-control study, nested in a longitudinal PD patient cohort, was conducted. Time-specific areas under the receiver operating characteristic (ROC) curve were calculated for FWT and effluent biomarkers at a lag time up to 3 years before EPS diagnosis. Free water transport was combined with appearance rates (AR) of biomarkers to assess their clinical validity. RESULTS: Free water transport volume and AR of effluent biomarkers were investigated in 11 EPS patients and 34 long-term PD patients. Diagnostic performance was best for FWT (area under the curve [AUC] 0.94) followed by plasminogen activator inhibitor (PAI-1) AR. Throughout, diagnostic panels of FWT and AR of cancer antigen 125 (CA125), interleukin-6 (IL-6), or (PAI-1) yielded specificity estimates above 84%. The combination of FWT and PAI-1 AR identified the largest proportion of EPS patients at 1 year prior to diagnosis (sensitivity 100%, specificity 94%). CONCLUSION: Measurement of FWT is simple and has the highest predictive value for imminent EPS. The addition of effluent biomarkers provides an all-round insight into the state of the peritoneum. Our data indicate that combining FWT with either PAI-1, CA125, or IL-6 has the highest specificity. This is required to avoid unnecessary discontinuation of PD treatment.

Encapsulating sclerosing peritonitis : two rare observations and literature review.

Sclerosing encapsulating peritonitis (SEP) is a rare and little known pathological entity. It is a chronic fibro-inflammatory disease of the peritoneum, resulting in the formation of a thick fibrous membrane, which engages partially or totally the abdominal organs. Clinical and radiological signs make the diagnosis difficult to establish preoperatively. We present two original observations of SEP illustrating different clinical presentations, diagnostic and therapeutic means. His diagnosis requires a peritoneal biopsy. The treatment is not completely established. Surgical treatment is difficult, involving viscerolysis and multiple incisions of the fibrous membrane. Despite the current progress in therapeutic management, the prognosis remains pejorative, with significant mortality.

Encapsulating peritoneal sclerosis with steroid-resistant massive ascites successfully treated by peritoneal lavage.

Encapsulating peritoneal sclerosis (EPS) is the most serious complication of long-term peritoneal dialysis (PD). EPS is diagnosed by clinical symptoms (abdominal pain, nausea, vomiting, diarrhea, and anorexia.) and image study (intestinal expansion, peritoneal thickening and calcification, and ascites.). Steroid therapy and surgery are recommended as the treatment of EPS. Here, we report a case of EPS with steroid-resistant massive ascites successfully treated with peritoneal lavage. A 59-year-old female with end-stage kidney disease secondary to hypertension was started on PD in 2003. Due to recurrent exit-site infection and two episodes of peritonitis, she was transferred to hemodialysis (HD), and her PD catheter was removed in 2011. In February 2012, six months after discontinuation of PD, she was found to have massive ascites on abdominal computerized tomography (CT). The patient was diagnosed to have EPS and was started on prednisolone. Despite eight months of prednisolone therapy, the ascites did not decrease. Therefore, the PD catheter was inserted again, and she was started on daily peritoneal lavage from September 2012. After four months of daily peritoneal lavage, her ascites disappeared in January 2013. The PD catheter was removed in July 2013. Steroid treatment was completed in May 2014, and there has been no recurrence of ascites since then. The evaluation of ascites by abdominal CT is important in a patient on long-term PD. Since EPS may appear any time after the discontinuation of PD, it is important to start screening abdominal CT shortly after the discontinuation of PD. Steroid-resistant massive ascites can be successfully treated with peritoneal lavage.

The use of computed tomography in the diagnosis of abdominal cocoon.

BACKGROUND: Literature on the diagnosis of abdominal cocoon using computed tomography (CT) outside the setting of continuous ambulatory peritoneal dialysis is sparse. METHODS: We did a retrospective analysis of contrast enhanced CT done for patients treated for abdominal cocoon. The clinical features, radiological findings, underlying etiology and outcomes were recorded. RESULTS: Of the 22 patients analyzed, 19 had tuberculosis, 2 had malignancy and 1 was idiopathic. The basis of diagnosis of cocoon was CT in 18 and CT and surgery in 4. Types 1, 2 and 3 cocoon were found in 3 (13.63%), 6 (27.27%) and 13 (59.09%) respectively. The bowel findings were clumped loops in 21 (95%), inter-bowel fluid in 13 (59%), bowel wall thickening/enhancement in 6 (27%) and stricture in 1 (5%). Peritoneal thickening and nodularity were seen in 14 (64%) and 2 (9%) while omental thickening, nodularity and mass in 9 (41%), 5 (21%) and 1 (5%). Cauliflower sign was seen in 14 (64%), concertina pattern in 5 (23%) and Bottle Gourd sign in 6 (23%) patients. Of 12 with imaging done during episode of IO, 11 (92%) had cauliflower sign, 4 (80%) had concertina appearance and 6 (100%) had bottle gourd sign. Post treatment weight gain with anti-tubercular therapy was seen in 14 (63.63%), resolution of ascites in 12 (54.54%) and 2 patients had complete resolution of cocoon on repeat imaging. CONCLUSION: CT is a valuable tool for pre-operative diagnosis of abdominal cocoon. The classical described signs are seen more frequently in patients with IO.

Sclerosing peritonitis post liver transplantation: a rare condition where surgery is an important treatment option.

BACKGROUND: Sclerosing peritonitis (SP) is a rare but potentially fatal complication following orthotopic liver transplantation (OLT). The definitive surgical management is via a laparotomy peritonectomy and enterolysis procedure, but this carries risks particularly in the immunosuppressed transplant patient population. The natural history of SP is known from a handful of case reports and series, which mostly report de novo cases arising early on following OLT. The aim of this study was to identify all cases of de novo SP following OLT and the outcomes of management. METHODS: Cases of SP post OLT were identified from the Australian National Liver Transplantation Unit (New South Wales) database of all 1393 adult patients. RESULTS: Three cases of SP were diagnosed between 2 and over 9 years post-transplantation. Two patients proceeded to laparotomy and a peritonectomy and enterolysis procedure of the cocooned bowel. The third was managed conservatively due to a relatively indolent course and their medical co-morbidities. CONCLUSION: SP should be considered in the differential diagnosis in patients post OLT presenting with symptoms of bowel obstruction, even years following transplantation. Surgery may be required in order to achieve a satisfactory outcome in some cases.

Effective Remedy for Encapsulating Peritoneal Sclerosis with Ureteroileal Fistula.

Encapsulating peritoneal sclerosis (EPS), treated with surgical enterolysis as a final option, may become refractory to surgical intervention due to intraperitoneal complications. We report the case of a 59-year-old man presenting with EPS who underwent enterolysis at the age of 50, following 15 years of peritoneal dialysis (PD) and peritonitis. During the patient's first surgery, complete surgical enterolysis could not be performed due to severe intestinal adhesions with a deteriorated/calcified small bowel. Six months after the surgery, the obstructive bowel symptoms occurred several times a year. Nine years later, the patient suffered cystitis-like symptoms and fecal discharge from the urinary meatus. The patient was subsequently diagnosed with EPS recurrence with ureteroileal fistula between the right ureter and ileum. During the second surgical intervention, we conducted a divided jejunostomy, as surgical enterolysis and fistulectomy were unachievable due to severe ileal adhesion with calcified capsule and inflammation. Thereafter, symptoms reduced dramatically and oral intake became possible. Three years following surgery, the patient's condition is improved, with no evidence of EPS recurrence or cystitis-like symptoms. Although EPS with ureteroileal fistula is extremely rare, we propose that jejunostomy may be an effective treatment option for patients with EPS refractory to surgical enterolysis or intestinal bypass due to intraperitoneal complications.

[Encapsulating peritoneal sclerosis]. / La péritonite sclérosante et encapsulante.

Encapsulating peritoneal sclerosis is a rare but devastating complication of long-term peritoneal dialysis with a high mortality rate. The incidence is between 0.5 and 2.5%, decreasing with time. PSE is defined as a clinical syndrome with signs of gastrointestinal obstruction, inflammation parameters, radiological and macroscopic changes. The duration of treatment and the cessation of peritoneal dialysis are the main risks. About 75% occured in patients on hemodialysis or after kidney transplantation. Morphological alterations are disappearance of mesothelial layer, submesothelial fibrosis, interstitial sclerosis and vasculopathy. Ultrafiltration failure, fast transport status of the peritoneal membrane and loss of sodium sieving, the most powerful predictor, are the functional abnormalities. Biomarkers in peritoneal effluent include cancer antigen 125, interleukin-6. The pathophysiology is probably a consequence of a multiple-hit process in which expression of growth factors and cytokines play a central role. Medical strategies (corticosteroids, tamoxifen) in association with parenteral nutrition and/or surgery (enterolysis) are discussed. Prevention is the use of physiological peritoneal dialysis solutions, icodextrine instead of high glucose concentration solutions and peritoneal lavage after peritoneal dialysis stopping.

Diagnosis and Treatment of 26 Cases of Abdominal Cocoon.

BACKGROUND AND AIMS: Abdominal cocoon (AC) is a rare abdominal disease with nonspecific clinical features, and it is difficult to be diagnosed before operation and hard to be treated in clinical practice. The aim of this study is to investigate the diagnosis and treatment of AC. METHODS: The clinical manifestations, findings during surgery, treatments, and follow-up results of 26 cases of AC were retrospectively studied from January 2001 to January 2015. RESULTS: All of 26 cases were diagnosed as AC definitely by laparotomy or laparoscopic surgery. Their clinical findings were various, with 7 intestines obstructed with bezoars and 4 intestines perforated by spiny material. Based on the existence of the second enterocoelia, all cases were categorized into 2 types: type I is absent of second enterocoelia (18 cases, 69.23%), while type II shows second enterocoelia (8 cases, 30.77%). Twenty cases (12 were type I and 8 were type II) underwent membrane excision and careful enterodialysis to release the small intestine entirely or partially, while the other 6 cases (all were type I) did not. In addition, all patients were treated with medical treatment and healthy diet and lifestyle. Finally, most of the patients recovered smoothly. CONCLUSIONS: AC can be categorized into two types; surgery is recommended for type II and part of type I with severe complications, but sometimes conservative therapy might be appropriate for type I. Laparoscopic surgery plays an important role in the diagnosis and treatment of AC. Furthermore, favorite health education, healthy diet and lifestyle are of significance in patients' recovery.