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Query: UMLS:C0021843 (bowel obstruction)
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Encapsulating peritoneal sclerosis (EPS) is recognized as a serious complication of continuous peritoneal dialysis. A preliminary diagnosis of EPSis usually based on clinical signs and symptoms, which commonly include abdominal pain, nausea, vomiting, anorexia, abdominal fullness, an abdominal mass, bowel obstruction, and radiologic findings, including abdominal roentgenogram, contrast studies, ultrasound studies, and computed tomography. The diagnosis is confirmed by laparoscopy or laparotomy showing the characteristic gross thickening of the peritoneum enclosing some or all of the small intestine in a cocoon of opaque tissue. A variety of therapeutic approaches to EPS have been reported. This review discusses medical treatment of EPS and includes an overview of the clinical features and diagnostic aspects of the condition.
Perit Dial Int 2005 Apr
PMID:Encapsulating peritoneal sclerosis--a clinician's approach to diagnosis and medical treatment. 1630 Feb 70

Encapsulating peritoneal sclerosis (EPS) is a life-threatening complication of peritoneal dialysis (PD). The overall prevalence of EPS in Japanese PD patients is 2.3%. Among patients on PD for less than 5 years, the rate is 0.9%; among patients on PD for 5 - 10 years, the rate is 3.8%; and among patients on PD for >10 years, it is 11.5%. Thus, the longer the treatment duration, the higher the prevalence of EPS. Encapsulating peritoneal sclerosis does not result solely from the natural progression of peritoneal sclerosis. A "second hit" event, such as bacterial peritonitis, abdominal bleeding, or abdominal surgery may be needed to trigger the onset of EPS in the face of advanced peritoneal sclerosis. To prevent development of EPS, PD treatment is replaced by other treatments when patients reached high-transport status. Peritoneal lavage and prednisolone administration have been reported to be effective in preventing or stopping the progress of EPS. When bowel obstruction has occurred, total enterolysis to remove the fibrous capsule from the bowel is indicated. To maximize overall quality of life, patients with endstage renal disease (ESRD) should have the choice to make use of all the treatment modalities available: PD, hemodialysis (HD), and transplantation. Furthermore, the development of truly biocompatible PD equipment--including peritoneal catheters, solutions, and systems--are desirable to extend PD treatment for the long-term. The cost of individual products could decrease significantly if PD use were to increase to 30% from 10% among ESRD patients worldwide. As practitioners, we have to further improve the technical survival rate and functional duration of PD treatment so that adequate peritoneal function can be maintained for 10 years in at least 40% of PD patients. The goal is to place PD on par with HD using high-flux dialysis membranes and ultrapure dialysis solution.
Perit Dial Int 2005 Apr
PMID:Peritoneal dialysis in Japan: the issue of encapsulating peritoneal sclerosis and future challenges. 1630 Feb 76

Sclerosing peritonitis is a rare complication of peritoneal dialysis (PD). In encapsulating peritoneal sclerosis (EPS), the most severe form of the disease, the intestine is entrapped in a fibrous tissue, causing intestinal obstruction. Patients are typically seriously ill, with evidence of infection and requirement for parenteral nutrition. A mortality rate of 73% has been reported. There is no established medical treatment and surgery has offered variable results. Our unit provides renal replacement therapy for a population of about 2 million. The prevalent population of PD patients averages 110. The cumulative PD population since January 1993 is 643, with an EPS prevalence of 0.6%. Influenced by the first case reported by Allaria in 1999 suggesting benefit of tamoxifen in treating EPS, we have treated with tamoxifen the four consecutive cases of EPS that have presented since 1999. All 4 patients have survived and recovered intestinal function. ALL showed prior evidence of peritoneal dysfunction with ultrafiltration failure and were characterized by long duration of PD therapy rather than multiple episodes of peritonitis. We conclude that tamoxifen is a highly promising therapy in EPS, hitherto a usually fatal condition. This description of its efficacy in acutely ill patients with EPS complements its possible prophylactic use in patients with the earlier and milder disease, sclerosing peritonitis. A high index of clinical suspicion for sclerosing peritonitis is desirable, perhaps facilitated by routine screening of at-risk patients.
Perit Dial Int
PMID:Four consecutive cases of peritoneal dialysis-related encapsulating peritoneal sclerosis treated successfully with tamoxifen. 1662 21

Since 1993, we have performed enterolysis for encapsulating peritoneal sclerosis (EPS) in 86 patients. Five patients died after surgery, but the remaining 81 patients achieved symptomatic improvement. However, some of the surviving patients developed symptoms of intestinal obstruction after several months, and EPS recurrence remains our greatest challenge. In the present study, we identified factors related to recurrence by clinically comparing 47 recurrent and nonrecurrent patients that we were able to follow for more than 2 years after surgery. In the 47 patients we followed, 11 (23.4%) experienced recurrence, and 10 of those patients underwent a second surgery. Four patients needed a third surgery. The mean time to recurrence was 15.2 months, and all patients with recurrence, except one, experienced their recurrence within 20 months. We observed no differences between the recurrent and nonrecurrent patients in follow-up period, age, history of peritoneal dialysis, use or nonuse of steroids after surgery, thickness of the submesothelial compact zone, or inflammatory cell infiltration. However, the number of microvessels was significantly higher in the recurrent patients. Our surgical experience with EPS shows recurrence in approximately 23% of patients. The results of the present study suggest that peritoneal microvascular hyperplasia is a factor involved in recurrence, and that patients with recurrence may have an irreversible pathologic condition and may experience repeated recurrences.
Adv Perit Dial 2006
PMID:Experience of 100 surgical cases of encapsulating peritoneal sclerosis: investigation of recurrent cases after surgery. 1698 41

Since the first peritoneal dialysis (PD) patients with encapsulating peritoneal sclerosis (EPS) were reported in 1980, EPS has been considered primarily a fatal complication. The incidence of EPS in PD patients has been reported to be 2.5%, with a negative effect of increasing PD duration (which also augments mortality). Because EPS occurs after withdrawal from PD in more than half of all cases, strict monitoring is necessary when a long-term PD patient is withdrawn from PD. Maintaining patients on standard PD with conventional solutions for more than 8 years is associated with a substantial risk of EPS development. Treatment appropriate to the disease stage is most important in EPS. Basic therapeutic strategies for EPS include the appropriate use of steroids. If bowel obstruction persists, laparotomy and enterolysis should be performed to achieve a complete cure. It is now recognized that EPS need not be a fatal complication of PD.
Perit Dial Int 2007 Jun
PMID:Encapsulating peritoneal sclerosis: prevention and treatment. 1755 21

Encapsulating peritoneal sclerosis (EPS) is a rare but serious complication in patients on peritoneal dialysis (PD). We describe a cluster of 13 EPS cases occurring in 2 university hospitals in The Netherlands. Most of these cases were diagnosed after recent kidney transplantation, when the patients developed severe symptoms of bowel obstruction. This accumulation raised the question as to whether other than known risk factors, such as duration of PD treatment, could be involved in the development or course of EPS after transplantation. According to various publications, EPS has been diagnosed often after withdrawal from PD, suggesting that cessation in itself may be a risk factor. In addition, transplantation-related management should be considered to play a role, including the use of the profibrotic calcineurin inhibitors and the trend to reduce the load of corticosteroids in treatment regimes. To identify risk factors, further multicenter studies are required, paying special attention to alterations in immunosuppressive treatment regimens as well as PD prescriptions, including PD fluid characteristics. Transfer from PD to hemodialysis should be under serious consideration in patients eligible for kidney transplantation as soon as there are indications of ultrafiltration failure.
Perit Dial Int
PMID:Posttransplant encapsulating peritoneal sclerosis: a worrying new trend? 1798 19

Encapsulating peritoneal sclerosis (EPS) is an intestinal obstruction syndrome in which peritoneal deterioration and intraperitoneal inflammation result in intestinal adhesions, which are covered with a fibrin capsule and cause bowel obstruction. The widespread use of peritoneal dialysis (PD) has been associated with an increase in the number of patients with this life-threatening complication. For this reason, some negative comments have been made about PD therapy. However, recent clinical studies have elucidated the pathogenesis of EPS and proposed therapeutic strategies. Currently, these facts are known: EPS occurs in 2.5% of all patients (3.18/1000 patient-years). A longer duration of PD is associated with a higher incidence of EPS and a poorer prognosis, indicating the involvement of peritoneal deterioration in the development of EPS. Development of EPS involves some kind of infection. Development of EPS frequently occurs after PD withdrawal and catheter removal. Peritoneal lavage after PD withdrawal delays, but cannot prevent the development of EPS. Timely administration of steroids is effective. Surgical adhesiolysis is the optimal treatment to relieve bowel obstructions, but does not exclude the potential for re-adhesions, requiring various ingenious gastro-intestinal surgical techniques.
Perit Dial Int 2008 Jun
PMID:Recommendation of the surgical option for treatment of encapsulating peritoneal sclerosis. 1855 57

We performed total enterolysis in 130 patients with encapsulating peritoneal sclerosis (EPS) between 1993 and 2007. The postoperative survival rate was 93.1%. However, 33 of the patients (25.4%) required additional surgery for recurrent bowel obstruction. To prevent recurrent bowel obstruction, we investigated various techniques following total enterolysis. In 7 patients, we employed the splinting method, in which the intestinal tract is fixed for 1 week after surgery by the insertion of a long intestinal tube. In 3 of the patients, recurrence was detected within 6 months after surgery. We therefore ceased using splinting. From April 2007, we performed the Noble plication procedure, in which intestine-to-intestine suturing is performed to prevent recurrent bowel obstruction, in 17 patients. None of those patients experienced a recurrence during 8 months of follow-up. In 7 patients showing marked calcification or repeated recurrence, we performed anastomosis of the superior jejunum and transverse colon after adhesiotomy. In 5 patients, excluding 2 with recurrence, improvement was achieved. Total enterolysis for EPS relieved bowel obstruction in most patients. However, after surgery, bowel obstruction recurred in some cases. Thus, strategies to reduce recurrence should be established. Currently, we use total enterolysis and Noble plication as our standard techniques. Further basic and clinical studies regarding EPS prevention and treatment should be conducted.
Adv Perit Dial 2008
PMID:Surgical techniques for prevention of recurrence after total enterolysis in encapsulating peritoneal sclerosis. 1898 1

Encapsulating peritoneal sclerosis is a devastating condition in long-term peritoneal dialysis patients. Animal models have employed chemical insults to simulate its pathology and have provided insights into its pathophysiology, which appears to include inflammation, angiogenesis, and fibrosis. Monitoring of biomarkers and interruption of molecular pathways have provided potential interventions to slow or prevent the disease process. However, there remain many questions concerning the trigger that alters chronic peritoneal inflammation in peritoneal dialysis to severe sclerosis, peritoneal adhesions, and bowel obstruction. Further advances in therapy will likely require an effective means of an early diagnosis through related biomarkers, which in turn will require further advances in the understanding of the pathogenesis of this disease process.
Perit Dial Int 2008 Nov
PMID:Pathogenesis and treatment of encapsulating peritoneal sclerosis: basic and translational research. 1900 33

Encapsulating peritoneal sclerosis (EPS) is a rare but devastating complication. The patients at high risk for EPS include those on peritoneal dialysis (PD) for more than 8 - 10 years and patients with high peritoneal transport and frequent peritonitis. Effective medical treatment with tamoxifen and immunosuppressives (a steroid with or without azathioprine or mycophenolate) has been reported. Surgical enterolysis is needed when irreversible intestinal obstruction occurs. In experienced hands, mortality can be reduced to a few percentage points, but the recurrence rate is very high. Noble plication has been reported to reduce the recurrence rate. Accelerated EPS development may possibly occur after conversion from PD to HD.
Perit Dial Int 2009 Feb
PMID:Encapsulating peritoneal sclerosis--medical and surgical treatment. 1927 Feb 21


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