Encapsulating Peritoneal Sclerosis Research Articles

Peritoneal Dialysis-Induced Encapsulating Peritonitis: Diagnostic and Therapeutic Challenges in Women with Benign Gynecological Pathology

Background: Peritoneal sclerosis (PS) and its most severe form, encapsulating PS (EPS), are rare entities that can occur in various procedures (liver transplantation, intraperitoneal chemotherapy) or secondary to medications (beta-blockers); however, PS or EPS typically occur in patients undergoing peritoneal dialysis as a form of renal function substitution. Medical or surgical treatments can be applied, but morbidity and mortality have high rates. This condition typically presents clinically as an intestinal obstruction caused by the inclusion of the intestinal loops in the peritoneal fibrous membrane. Methods: Herein, we present data from a single tertiary surgery center that has dedicated teams for patients receiving dialysis. Over 12 years, we analyzed a group of 63 patients admitted for catheter replacement/removal or for acute surgical pathology. In five cases (7.9%), we diagnosed EPS. Two patients with EPS presented with atypical abdominal pathologies requiring emergency surgery: one case of hemoperitoneum caused by a ruptured ovarian cyst and one case of uterine fibroids and metrorrhagia. Results: The definitive diagnoses were established intraoperatively and by analyzing the morpho-pathological changes in the peritoneum. The possible intraoperative challenges included laborious dissection, difficulties in restoring the correct anatomical landmarks, an increased duration of the surgical intervention and a high rate of incidents and accidents. Conclusions: The aim of the present study was to emphasize the possibility of other surgical pathologies overlapping with EPS, increasing the complexity of the surgical intervention.

Extracellular Vesicles of Patients on Peritoneal Dialysis Inhibit the TGF-β- and PDGF-B-Mediated Fibrotic Processes.

Among patients on peritoneal dialysis (PD), 50-80% will develop peritoneal fibrosis, and 0.5-4.4% will develop life-threatening encapsulating peritoneal sclerosis (EPS). Here, we investigated the role of extracellular vesicles (EVs) on the TGF-β- and PDGF-B-driven processes of peritoneal fibrosis. EVs were isolated from the peritoneal dialysis effluent (PDE) of children receiving continuous ambulatory PD. The impact of PDE-EVs on the epithelial-mesenchymal transition (EMT) and collagen production of the peritoneal mesothelial cells and fibroblasts were investigated in vitro and in vivo in the chlorhexidine digluconate (CG)-induced mice model of peritoneal fibrosis. PDE-EVs showed spherical morphology in the 100 nm size range, and their spectral features, CD63, and annexin positivity were characteristic of EVs. PDE-EVs penetrated into the peritoneal mesothelial cells and fibroblasts and reduced their PDE- or PDGF-B-induced proliferation. Furthermore, PDE-EVs inhibited the PDE- or TGF-β-induced EMT and collagen production of the investigated cell types. PDE-EVs contributed to the mesothelial layer integrity and decreased the submesothelial thickening of CG-treated mice. We demonstrated that PDE-EVs significantly inhibit the PDGF-B- or TGF-β-induced fibrotic processes in vitro and in vivo, suggesting that EVs may contribute to new therapeutic strategies to treat peritoneal fibrosis and other fibroproliferative diseases.

Role of endothelial hyaluronan in peritoneal membrane transport and disease conditions during peritoneal dialysis

Peritoneal membrane dysfunction in peritoneal dialysis (PD) is primarily attributed to angiogenesis; however, the integrity of vascular endothelial cells can affect peritoneal permeability. Hyaluronan, a component of the endothelial glycocalyx, is reportedly involved in preventing proteinuria in the normal glomerulus. One hypothesis suggests that development of encapsulating peritoneal sclerosis (EPS) is triggered by protein leakage due to vascular endothelial injury. We therefore investigated the effect of hyaluronan in the glycocalyx on peritoneal permeability and disease conditions. After hyaluronidase-mediated degradation of hyaluronan on the endothelial cells of mice, macromolecules, including albumin and β2 microglobulin, leaked into the dialysate. However, peritoneal transport of small solute molecules was not affected. Pathologically, hyaluronan expression was diminished; however, expression of vascular endothelial cadherin and heparan sulfate, a core protein of the glycocalyx, was preserved. Hyaluronan expression on endothelial cells was studied using 254 human peritoneal membrane samples. Hyaluronan expression decreased in patients undergoing long-term PD treatment and EPS patients treated with conventional solutions. Furthermore, the extent of hyaluronan loss correlated with the severity of vasculopathy. Hyaluronan on endothelial cells is involved in the peritoneal transport of macromolecules. Treatment strategies that preserve hyaluronan in the glycocalyx could prevent the leakage of macromolecules and subsequent related complications.

Renal hyperparathyroidism- a risk factor in the development of encapsulating peritoneal sclerosis.

Encapsulating peritoneal sclerosis (EPS) is a rare complication of prolonged peritoneal dialysis (PD) exposure, characterised by peritoneal thickening, calcification, and fibrosis ultimately presenting with life-threatening bowel obstruction. The presence or role of peritoneal calcification in the pathogenesis of EPS is poorly characterised. We hypothesise that significantly aberrant bone mineral metabolism in patients on PD can cause peritoneal calcification which may trigger the development of EPS. We compared the temporal evolution of bone mineral markers during PD in EPS patients with non-EPS long-term PD controls. Linear mixed model and logistic regression analysis were used to compare four-monthly serum levels of calcium, phosphate, parathyroid hormone, and alkaline phosphatase (ALP) over the duration of PD exposure in 46 EPS and 46 controls (PD, non-EPS) patients. EPS patients had higher mean calcium (2.51 vs. 2.41 mmol/L) and ALP (248.00 vs. 111.13 IU/L) levels compared with controls (p=0.01 and p<0.001 respectively, maximum likelihood estimation). Logistic regression analysis demonstrated that high serum calcium and phosphate levels during PD were associated with a 4.5 and 2.9 fold increase in the risk of developing EPS respectively. High levels of calcium and phosphate in patients on PD were identified to be risk factors for EPS development. Possible reasons for this may be an imbalance of pro-calcifying factors and calcification inhibitors promoting peritoneal calcification which increases peritoneal stiffness. Mechanical alterations may trigger, unregulated fibrosis and subsequent development of EPS. Improved management of secondary hyperparathyroidism during PD may ultimately diminish the EPS risk.

Melatonin inhibits the formation of chemically induced experimental encapsulating peritoneal sclerosis through modulation of T cell differentiation by suppressing of NF-κB activation in dendritic cells

Encapsulating peritoneal sclerosis (EPS) is a severe complication of peritoneal dialysis (PD). Surgery is a therapeutic strategy for the treatment of complete intestinal obstruction. However, complete intestinal obstruction in long-term PD results in high mortality and morbidity rates after surgery. Immunopathogenesis participates in EPS formation: CD8, Th1, and Th17 cell numbers increased during the formation of EPS. The anti-inflammatory and immunomodulatory effects of melatonin may have beneficial effects on this EPS. In the present study, we determined that melatonin treatment significantly decreases the Th1 and Th17 cell populations in mice with EPS, decreases the production of IL-1β, TNF-α, IL-6, and IFN-γ, and increases the production of IL-10. The suppression of Th1 and Th17 cell differentiation by melatonin occurs through the inhibition of dendritic cell (DC) activation by affecting the initiation of the NF-κB signaling pathway in DCs. Our study suggests that melatonin has preventive potential against the formation of EPS in patients with PD.

Magnesium inhibits peritoneal calcification as a late-stage characteristic of encapsulating peritoneal sclerosis

Peritoneal calcification is a prominent feature of the later stage of encapsulating peritoneal sclerosis (EPS) in patients undergoing long-term peritoneal dialysis (PD). However, the pathogenesis and preventive strategy for peritoneal calcification remain unclear. Peritoneum samples from EPS patients were examined histologically. Peritoneal calcification was induced in mice by feeding with an adenine-containing diet combined with intraperitoneal administration of lipopolysaccharide and a calcifying solution containing high calcium and phosphate. Excised mouse peritoneum, human mesothelial cells (MeT5A), and mouse embryonic fibroblasts (MEFs) were cultured in calcifying medium. Immunohistochemistry confirmed the appearance of osteoblastic differentiation-marker-positive cells in the visceral peritoneum from EPS patients. Intraperitoneal administration of magnesium suppressed peritoneal fibrosis and calcification in mice. Calcifying medium increased the calcification of cultured mouse peritoneum, which was prevented by magnesium. Calcification of the extracellular matrix was accelerated in Met5A cells and MEFs treated with calcification medium. Calcifying medium also upregulated osteoblastic differentiation markers in MeT5A cells and induced apoptosis in MEFs. Conversely, magnesium supplementation mitigated extracellular matrix calcification and phenotypic transdifferentiation and apoptosis caused by calcifying conditions in cultured MeT5A cells and MEFs. Phosphate loading contributes to the progression of EPS through peritoneal calcification and fibrosis, which can be prevented by magnesium supplementation.

Chylous ascites: A warning sign of life-threatening encapsulated peritoneal sclerosis in patient recently transferred to haemodialysis.

Chylous ascites: A warning sign of life-threatening encapsulated peritoneal sclerosis in patient recently transferred to haemodialysis.

Prediction of surgical outcomes in severe encapsulating peritoneal sclerosis using a computed tomography scoring system

Encapsulating peritoneal sclerosis (EPS) is a rare and potential lethal complication of peritoneal dialysis characterized by bowel obstruction. Surgical enterolysis is the only curative therapy. Currently, there are no tools for predicting postsurgical prognosis. This study aimed to identify a computed tomography (CT) scoring system that could predict mortality after surgery in patients with severe EPS. This retrospective study enrolled patients with severe EPS who underwent surgical enterolysis in a tertiary referral medical center. The association of CT score with surgical outcomes including mortality, blood loss, and bowel perforation was analyzed. Thirty-four patients who underwent 37 procedures were recruited and divided into a survivor and non-survivor group. The survivor group had higher body mass indices (BMIs, 18.1 vs. 16.7kg/m2, p=0.035) and lower CT scores (11 vs. 17, p<0.001) than the non-survivor group. The receiver operating characteristic curve revealed that a CT score of ≥15 could be considered a cutoff point to predict surgical mortality, with an area under the curve of 0.93, sensitivity of 88.9%, and specificity of 82.1%. Compared with the group with CT scores of <15, the group with CT scores of ≥15 had a lower BMI (19.7 vs. 16.2kg/m2, p=0.004), higher mortality (4.2% vs. 61.5%, p<0.001), greater blood loss (50 vs. 400mL, p=0.007), and higher incidence of bowel perforation (12.5% vs. 61.5%, p=0.006). The CT scoring system could be useful in predicting surgical risk in patients with severe EPS receiving enterolysis.

#5664 PERITONEAL DIFFUSE PODOPLANIN EXPRESSION IS ASSOCIATED WITH SEVERE ARTERIOLOPATHY IN CHILDREN ON PERITONEAL DIALYSIS

Abstract Background and Aims Podoplanin is a transmembrane glycoprotein binding chemokines and modulating inflammatory reactions. In healthy peritoneum, it is expressed in mesothelial cells and in lymphatic endothelium, expression beyond these structures (diffuse podoplanin staining, DPS) has been observed in adult patients with encapsulating peritoneal sclerosis (EPS). Method Parietal peritoneal tissues from 71 children on PD fluids with low glucose degradation product (GDP) content underwent digital histomorphometry. DPS and pathomechanisms of peritoneal membrane transformation and vasculopathy were hierarchically clustered. A panel of 13 peritoneal cell markers was selected and tissue sections were stained with metal conjugated antibodies and acquired using imaging mass cytometry (IMC; Hyperion Imaging System®). After single-cell segmentation, spillover correction, and normalization, cell types were identified using manual gating to train a random forest classifier for final cell type identifications using published workflows. Single-cell analysis was used to characterize peritoneal cells with positive podoplanin signals. Results DPS was present in 22% of the PD patients, all devoid of any clinical, radiological or histological feature of EPS. DPS positive patients were younger, had higher dialytic glucose exposure (median 103 vs. 154 g/m²/day, p = 0.002), and lower peritoneal arteriolar lumen to vessel (L/V) ratio, i.e. exhibited more arteriolar lumen narrowing. PD duration [median 20 (14.5, 48) months] and peritonitis incidence were similar as in non-DPS patients. In multivariable analysis, glucose exposure, PD duration, epithelial-to-mesenchymal transformed (EMT) cells and lower patient body surface area (BSA) independently predicted the presence of DPS. DPS and lower mesothelial surface coverage predicted lower arteriolar L/V ratio. In subgroups matched for BSA, PD duration and dialytic glucose exposure, DPS patients had a lower L/V ratio, (0.44 ± 0.13 vs. 0.31 ± 0.18, p = 0.003) and higher submesothelial CD45 leucocyte, CD68 macrophage and EMT cell count. Submesothelial lipopolysaccharide and hyaluronic acid receptor CD44 were increased in DPS positive patients, hyaluronan synthase 1 and 2 were similar. Among DPS positive patients, DPS intensity grade was higher in those with history of bacterial or fungal peritonitis (7 out of 15). Two patients had three and four peritonitis episodes respectively, their DPS pattern did not differ from patients with history of only one peritonitis episode. Hierarchical clustering demonstrated highest similarity of DPS positive areas with CD68 positive areas. In IMC analysis of DPS positive areas, highest podoplanin signals were derived from M2 classified macrophages (CD68+CD163+) and fibroblastic cells [αSMA positive/non-endothelial/non-macrophage (αSMA+PROX1-CD31-CD68-CD163-)], signals derived from M1 classified macrophages (CD68+CD163-) were lowest. Conclusion Peritoneal DPS in pediatric patients on low-GDP PD is associated with lower BSA, higher dialytic glucose exposure, history of infectious peritonitis and peritoneal inflammatory and EMT cell invasion, and predicts obliterating peritoneal vasculopathy. We identified M2 macrophages as major source of extra-lymphatic peritoneal podoplanin accumulation, suggesting their role in severe peritoneal transformation.

NPHP3-Related Disease: A Possible Risk Factor for Developing Encapsulating Peritoneal Sclerosis.

Encapsulating peritoneal sclerosis (EPS) presents a rare but serious complication of chronic peritoneal dialysis (PD) which is associated with significant morbidity and mortality (Stefanidis CJ et al., Pediatr Nephrol 2014; 29: 2093–2103). In paediatric patients, there are only several previously published studies (Vidal E et al., Nephrol Dial Transplant 2013; 28: 1603–1609; Ekim M et al., Nephrology (Carlton) 2005; 10: 341–343), but the prevalence (8.7 per 1000 patient-years on PD) seems similar as in the adult population (Shroff R et al., Nephrol Dial Transplant 2013; 28: 1908–1914). On the other hand, the general outcome of paediatric patients is better with lower mortality compared to adults (Shroff R et al., Nephrol Dial Transplant 2013; 28: 1908–1914).

Inkapsulirajuća peritoneumska skleroza - retka i ozbiljna komplikacija peritoneumske dijalize - prikaz slučaja

Introduction: End-stage renal disease is a terminal illness with a glomerular filtration rate of less than 15 mL/min. when must start one of the methods of renal replacement therapy: hemodialysis, peritoneal dialysis or kidney transplantation. Peritoneal dialysis is based on the processes of diffusion and osmosis, while the transport of substances takes place through the peritoneal membrane. The advantages of peritoneal dialysis compared to hemodialysis include longer-term preservation of residual renal function, preservation of blood vessels and a better quality of life for the patient. Complications of peritoneal dialysis can be infectious, metabolic and mechanical. Encapsulating peritoneal sclerosis (EPS) is a life-threatening complication of peritoneal dialysis (PD) treatment. The incidence of EPS is variable between 0.7 and 3.7%, while it increases with the length of PD treatment (0.7-6.4% after 5 years to as much as 17.2% after 15 years). The diagnosis of EPS is based on clinical symptoms. and signs as well as specific, but not pathognomonic, radiological and pathohistological findings. Features of IPS on computed tomography (CT) images are thickening and calcification of the peritoneal membrane, thickening of the intestinal wall, narrowing and dilatation of the in-testine, as well as "trapped" fluid collections. Case report: A 60-year-old patient, who has been treated with peritoneal dialysis for 4 years, was hospitalized due to severe gastrointestinal complaints. Due to clinical findings a peritoneal equilibration test was performed, which determined that it was a moderately fast transporter. Based on the findings of the computed tomography, the diagnosis was confirmed. Treatment is started tamoxifen and prednisone was started, the response to therapy was positive. Despite the control of sclerosing peritonitis, death occurred because of cardiac decompensation. Conclusion: Early diagnosis is necessary to start specific treatment in a timely manner and, if possible, preserve peritoneal dialysis as a treatment method and patient survival.

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

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. 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. Incomplete small bowel obstruction due to abdominal adhesions after the cesarean section was initially considered. After conservative treatment, the symptom of intestinal obstruction still recurred. Thus, we decided to perform a surgery of repeated decortication of fibrous peritoneal membranes. The operation successfully released the intestinal obstruction and abdominal pain, postoperative course recovered smoothly. 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.

The Role of Enterolysis and Tamoxifen in Encapsulating Peritoneal Sclerosis: A Successful Case

Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis which mortality can reach 50%. The usual treatment involves suspension of the technique, medical treatment, and surgical enterolysis. We report the case of a 36-year-old female who undergone continuous ambulatory peritoneal dialysis without complications for 10 years. She presented at the emergency department with abdominal pain and a diagnosis of peritonitis was assumed. The patient developed a systemic inflammatory response, and her abdominal computer tomography (CT) scan showed multiple parietal calcifications. After multiple broad-spectrum antibiotics, an undetermined febrile syndrome persisted, and parenteral nutrition was started. An EPS diagnosis was established and treatment with tamoxifen was proposed. Exploratory laparoscopy was decided, and the intraoperative findings were consistent with EPS. The patient was submitted to extensive enterolysis. A clinical improvement led to a discharge after 60 days of hospitalization. After more than 30 months with tamoxifen, she remains on hemodialysis and doing well. There are no randomized controlled trials to guide EPS management and the following procedures are more difficult to decide, who makes these reports important to the reflection. We describe a case of successful treatment of EPS, with a very severe presentation. The management with enterolysis and tamoxifen contributed decisively to this outcome.

C allele in transforming growth factor-β1 rs1800471 gene polymorphisms might indicate a protective feature in encapsulating peritoneal sclerosis development.

Peritoneal fibrosis may progress in peritoneal dialysis (PD) patients to a fatal clinical condition called encapsulating peritoneal sclerosis (EPS). Transforming growth factor (TGF)-β plays a pivotal role in the pathogenesis of peritoneal fibrosis. We aimed to investigate the association among polymorphisms in the gene encoding TGF-β1, which were -509C/T (rs1800469), +869T/C (rs1982073), and +915G/C (rs1800471) in EPS patients. A total of 16 PD patients who were clinically and radiologically diagnosed with EPS were enrolled and 22 age- and gender-matched PD patients were selected as the non-EPS group. G allele frequency at the rs1800471 gene polymorphism was significantly higher in the EPS group than non-EPS group (p=0.005). Interestingly, the non-EPS group patients had CC or CG polymorphisms. C allele in TGF-β1 rs1800471 gene polymorphisms might indicate a protective feature in EPS development. Knowing the presence of polymorphism may be effective in selecting renal replacement therapy in patients.

Risk factors for Encapsulating Peritoneal Sclerosis in patients undergoing peritoneal dialysis: A meta-analysis.

Encapsulating Peritoneal Sclerosis (EPS) is the most serious complication of long-term peritoneal dialysis (PD), which considerably reduces the patient's quality of life, leading to patients discontinuing PD. Considering these negative effects, it is necessary to systematically review and determine the risk factors of EPS. The PubMed, Embase, Web of Science, Cochrane Library, and China Biology Medicine (CBM) were searched from their inception to January 1st, 2022, and the bibliographies from the citations of relevant articles were manually searched. The ROBINS-I (Risk of Bias in Non-randomized studies of Interventions) tool was used to evaluate the risk of bias of included studies. Ten studies involving 12595 participants were included in this meta-analysis. The results revealed that a younger age at PD onset (MD = -7.70, 95% CI, -11.53~-3.86), a higher transporter (MD = 0.13, 95% CI, 0.09~0.18), a longer PD duration (SMD = 1.15, 95% CI, 0.68~1.61), a longer peritonitis duration (MD = 12.66, 95% CI, 3.85~21.47), and history of glomerulonephritis (OR = 1.42, 95% CI, 1.02~1.97) were significant risk factors for EPS. However, sex, use of icodextrin, the number of peritonitis episodes, and history of multicystic kidney disease did not affect the risk of EPS. This review provides a scientific basis for further understanding the etiology of PD-related EPS and improving prevention strategies. More high-quality studies are necessary to validate this paper's findings.

Refractory Peritonitis and Small Bowel Ileus: A Case of Encapsulating Peritoneal Sclerosis Secondary to Mycobacterium abscessus Peritonitis.

IntroductionComplications of peritoneal dialysis (PD) such as exit-site infections and PD peritonitis are common reasons for admission to General Internal Medicine wards. Culprit organisms range from typical skin flora to rarer complicated atypical organisms such as non-tuberculous mycobacteria. Encapsulating peritoneal sclerosis (EPS) is a rarer complication of PD characterized by peritoneal inflammation, ileus and fibrosis with high morbidity, few management options, and poor prognosis.Case DescriptionWe report the case of a patient with a history of end-stage renal disease on peritoneal dialysis who presented with undifferentiated peritonitis and ileus refractory to standard antimicrobial therapy. Subsequent ascitic cultures were positive for Mycobacterium abscessus, and CT imaging was consistent with EPS. To date, EPS secondary to M. abscessus peritonitis has not previously been described.Discussion and ConclusionThis report describes the diagnostic process and treatment offered to this patient and his treatment outcomes over 8 months. It highlights the importance of prompt identification of patients at risk, timely eradication of high-risk pathogens, and transition to haemodialysis to limit morbidity and improve patient prognosis.LEARNING POINTSEncapsulating peritoneal sclerosis secondary to Mycobacterium abscessus peritonitis has not been described to date. M. abscessus and other non-tuberculous mycobacterial organisms should be suspected in peritoneal dialysis patients who present with refractory peritonitis and ileus.Encapsulating peritoneal sclerosis is an inflammatory phenomenon characterized by peritoneal inflammation, fibrosis and bowel ileus in the setting of peritoneal dialysis or recurrent infectious insults with high-risk organisms. Diagnosis depends on characteristic imaging coupled with compatible clinical symptoms.Treatment involves transition to haemodialysis, infection eradication, immunosuppression and anti-hormonal treatment (e.g., tamoxifen). Despite these options, prognosis remains very poor.

POS-683 ENCAPSULATING PERITONEAL SCLEROSIS (EPS) : WITHIN A 20 YEARS EXPERIENCE

Encapsulating Peritoneal Sclerosis (EPS) is a rare but a serious complication mostly seen in patients on peritoneal dialysis (PD). EPS is characterized by thickening and fibrosis of the peritoneal membrane with formation of a fibrous cocoon encapsulating the bowels.

POS-673 A DUAL TREATMENT WITH TAMOXIFEN AND CORTICOSTEROID LEADING TO A MIRACULOUS RESULT for A PATIENT WITH THE DIAGNOSIS OF ENCAPSULATING PERITONEAL SCLEROSIS: A CASE REPORT

Encapsulating peritoneal sclerosis (EPS) is a debilitating condition characterized by a fibro collagenous membrane encasing the small intestine, leading to relapsing small bowel obstructions. It is a rare and life-threatening condition that also affects general and nutritional state as well as the quality of life.

Intra-Peritoneal Pseudocyst as a Rare Complication After Peritoneal Dialysis: The Use of a Staged Surgical Approach in Management.

Despite its numerous benefits, peritoneal dialysis (PD) can rarely result in dangerous and even life-threatening complications, including peritonitis, hernias, encapsulating peritoneal sclerosis (EPS), and rarely peritoneal pseudocysts. Herein, we present a rare case of a giant intra-peritoneal pseudocyst that presented four months following the discontinuation of a 5-year course of complicated PD. Despite the initially successful drainages, the patient's symptoms continued to recur, and the imaging findings were concerning for underlying neoplastic processes. As such, a staged surgical approach was performed, starting with a diagnostic laparoscopy and was subsequently followed with cyst excision and marsupialization to the peritoneal cavity. While previous reports of such rare pseudocyst have been documented in the literature as a complication of PD, to our knowledge, this is the second case of pseudocyst formation to occur months after the discontinuation of PD therapy. This case emphasizes the importance of close follow-up in PD patients and showcases how a staged surgical approach can be utilized to accurately diagnose and manage such complicated cases.

Surgical management of Encapsulating Peritoneal Sclerosis (EPS) in children: international case series and literature review

BackgroundEncapsulating Peritoneal Sclerosis (EPS) is a rare phenomenon in paediatric patients with kidney failure treated with peritoneal dialysis (PD). This study highlights clinical challenges in the management of EPS, with particular emphasis on peri-operative considerations and surgical technique.MethodsRetrospective analysis of all paediatric patients with EPS treated at the Manchester Centre for Transplantation.ResultsFour patients were included with a median duration of 78 months on PD. All patients had recurrent peritonitis (> 3 episodes), and all had symptoms within three months of a change of dialysis modality from PD to haemodialysis or transplant. In Manchester, care was delivered by a multi-disciplinary team, including surgeons delivering the adult EPS surgical service with a particular focus on nutritional optimisation, sepsis control, and wound management. The surgery involved laparotomy, lavage, and enterolysis of the small bowel + / − stoma formation, depending on intra-abdominal contamination. Two patients had a formal stoma, which were reversed at three and six months, respectively. Two patients underwent primary closure of the abdomen, whereas two patients had re-look procedures at 48 h with secondary closure. One patient had a post-operative wound infection, which was managed medically. One patient’s stoma became detached, leading to an intra-abdominal collection requiring re-laparotomy. The median length of stay was 25 days, and patients were discharged once enteral feeding was established. All patients remained free of recurrence with normal gut function and currently two out of four have functioning transplants.ConclusionsThis series demonstrates 100% survival and parenteral feed independence following EPS surgery. Post-operative morbidity was common; however, with individualised experience-based decision-making and relevant additional interventions, patients made full recoveries. Health and development post-surgery continued, allowing the potential for transplantation.Graphical abstractA higher resolution version of the Graphical abstract is available as Supplementary information