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

BACKGROUND/PURPOSE: 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. METHODS: 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. RESULTS: 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. 16.7 kg/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. 16.2 kg/m2, p = 0.004), higher mortality (4.2% vs. 61.5%, p < 0.001), greater blood loss (50 vs. 400 mL, p = 0.007), and higher incidence of bowel perforation (12.5% vs. 61.5%, p = 0.006). CONCLUSION: The CT scoring system could be useful in predicting surgical risk in patients with severe EPS receiving enterolysis.

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

BACKGROUND: Encapsulating 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. METHODS: Retrospective analysis of all paediatric patients with EPS treated at the Manchester Centre for Transplantation. RESULTS: Four 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. CONCLUSIONS: This 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. A higher resolution version of the Graphical abstract is available as Supplementary information.

Surgical Outcomes in Patients with Abdominal Cocoon: Series of 15 Patients.

BACKGROUND: Abdominal cocoon (AC) or sclerosing encapsulating peritonitis is an uncommon cause of intestinal obstruction. Surgical intervention is warranted in patients with persistent pain or intestinal obstruction. METHODOLOGY: A retrospective analysis of patients operated for AC was performed. Clinical presentation, radiological data, postoperative outcomes (Ryles tube (RT) removal, duration of hospital stay, enterocutaneous fistula, requirement for re-exploration and mortality) were retrieved and analyzed. RESULTS: Fifteen patients of abdominal cocoon required surgical intervention for various indications. The mean age was 34.46 years (13-60), and 11 (73.3%) were males. Intermittent abdominal pain was present in 14 (93.3%) followed by recurrent subacute intestinal obstruction (SAIO) in 11 (73.3%). Three patients presented with intestinal perforation. Of the 14 patients with preoperative computed tomography, radiological diagnosis was possible in five patients. The mean duration for surgery was 159 min (60-360 min). Membrane encasement was complete in 9/15 and partial in 6/15 patients. Adhesiolysis was done in all patients (complete-10/15 and partial-5/15). Mean duration for RT removal and hospital stay was 4.3 and 12.3 days, respectively. Recurrence of SAIO was observed in three patients, and one patient needed re-exploration for the same. One patient developed postoperative enterocutaneous fistula requiring surgical intervention. Overall mortality in the study was 13.3% (2/15). Four patients had underlying tuberculosis, and the rest were idiopathic. CONCLUSION: Etiology of AC is not known in majority of patients. Persistent pain and recurrent SAIO are the most common indications for surgery. This morbidity associated with surgery can be reduced by meticulous dissection techniques and appropriate peri-operative care.

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.

Clinical signs of fibrosis in small intestinal neuroendocrine tumours.

BACKGROUND: In patients with small intestinal neuroendocrine tumours (SI-NETs), serotonin and other cytokines released from tumour cells may induce fibrosis, leading to carcinoid heart disease and abdominal fibrotic reactions. The aim of this study was to assess the prevalence, clinical complications and management of this reaction in the abdomen. METHODS: This was a retrospective cohort study of patients with SI-NETs diagnosed between 1985 and 2015. Clinical data, outcomes, radiological findings, and surgical and radiological interventions were reviewed. RESULTS: A total of 824 patients were diagnosed with SI-NETs in the study interval. Clinically significant abdominal signs and symptoms of fibrosis occurred in 36 patients. Of these, 20 had critically symptomatic central mesenteric fibrosis causing obstruction of mesenteric vessels, and 16 had retroperitoneal fibrosis causing obstructive uropathy with hydronephrosis. Extensive fibrosis causing mesenteric vessel obstruction and/or obstructive uropathy was more often associated with symptomatic and advanced disease encompassing lymph node metastases in the mesenteric root, para-aortic lymph node metastases, as well as liver metastases and peritoneal carcinomatosis. Palliative intervention in terms of superior mesenteric vein stenting or resection of central mesenteric metastases and/or percutaneous nephrostomy and J stent treatment was beneficial in the majority of the patients. CONCLUSION: Extensive abdominal fibrosis associated with clinically significant symptoms of intestinal ischaemia and/or obstructive uropathy was linked to advanced disease in patients with SI-NETs. Prompt recognition and minimally invasive intervention was effective in disease palliation.

Surgical Management of Complications with Peritoneal Dialysis.

This report reviews the most common surgical interventions and complications of chronic peritoneal dialysis (PD) patients. Based on the current knowledge as well as our experience we detail the role of these surgical procedures. We supplement the reported knowledge in the field with our own experience in this area. The areas discussed include early complications such as surgical wound hemorrhage, bleeding from the catheter, intestinal perforation and urinary bladder perforation, dialysate leakage through the wound, as well as late complications including catheter kinking or occlusion, retention of fluid in the peritoneal recess, hernias and hydrothorax, and encapsulating peritoneal sclerosis. We also briefly cover the surgical aspects of exit-site infection and peritonitis. An understanding by nephrologists of the role for surgical intervention in PD patients will improve their care and outcomes.

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.

The enigma of primary and secondary encapsulating peritoneal sclerosis.

BACKGROUND: Encapsulating Peritoneal Sclerosis (EPS) describes a variety of diseases that are frequently confused with different names and different etiopathogeneses. The aim of this article is to report personal experience of focusing on correct classification and the status of current diagnosis and treatment. METHODS: A retrospective analysis was performed. Age, sex, ethnic origin, past medical history, symptoms and their duration, radiological tools and signs, laboratory tests, preoperative diagnosis, surgical approach, intraoperative findings, pathological findings, hospital stay, morbidity and mortality were studied. RESULTS: A total of seven patients, including six males and one female, aged from 24 to 72 years were observed. Four patients had recurrent abdominal colic pain for 3 months, 1, 2 and 9 years; two patients also reported recurrent attacks but without any specification of the duration. All seven patients presented at the emergency department with abdominal pain that was mainly diffused over the entire abdomen. Six patients were submitted to a CT scan. Only in two patients was the diagnosis of EPS made preoperatively. All seven patients were submitted to open surgery. The hospital stay was between 4 and 60 days. One patient had morbidity, and one patient died of MOF. CONCLUSIONS: Currently, the correct identification of EPS is more easily possible than in the past, but the diagnosis is still a challenge. Surgery must be performed as soon as possible to avoid a poorer quality of life.

A role for everolimus in post-transplant encapsulating peritoneal sclerosis: first case report.

Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis (PD) that carries a high morbidity and mortality. The 'two hit theory' suggests that long term deterioration of the peritoneum combined with intraperitoneal inflammation is needed in the pathogenesis of EPS. For unclear reasons, post transplantation EPS is being increasingly reported in patients previously on PD. To date, there is no proven effective therapy with an absence of randomised controlled trials. Individual case reports and small case series have reported on the use of tamoxifen and corticosteroids for medical management of EPS. The use of everolimus has been reported in a single case, and never in the setting of renal transplantation. Here, we present the first case of post-transplant encapsulating peritoneal sclerosis treated successfully with a combination of everolimus, tamoxifen, low dose corticosteroid and surgery.

Successful surgical approach for a patient with encapsulating peritoneal sclerosis after hyperthermic intraperitoneal chemotherapy: a case report and literature review.

BACKGROUND: Encapsulating peritoneal sclerosis (EPS) is a rare surgical complication that can occur after intraperitoneal treatment. It is also a serious and potentially fatal complication of continuous ambulatory peritoneal dialysis. The present report describes a case of surgically treated EPS that probably occurred as a complication of hyperthermic intraperitonal chemotherapy (HIPEC). CASE PRESENTATION: A 39-year-old man required sigmoidectomy for serosal invasive advanced sigmoid colon cancer. HIPEC with oxaliplatin, 5-fluorouracil and mitomycin C were given as adjuvant therapy. Subsequently, intestinal obstruction developed at 15 months postoperatively, and the patient was hospitalized. Abdominal computed tomography showed a dilated small intestine enveloped by a thickened membrane. We found no evidence of peritoneal recurrence, but exploratory surgery revealed EPS, probably caused by HIPEC. We peeled the capsule off of the intestine. The patient's postoperative course was uneventful, and sufficient nutritional intake after surgery was noted. Seven months after surgery, he is well with no recurrence. CONCLUSION: The surgical treatment via peritonectomy and enterolysis for postoperative EPS appears safe and effective. A diagnosis of EPS should be considered when intestinal obstruction does not show improvement with conservative treatment in patients who have undergone HIPEC, provided the possibility of peritoneal cancer recurrence is excluded.

[Rare case of systemic lupus erythematosus with encapsulating peritoneal sclerosis during hemodialysis].

A 57-year old male patient was admitted to our hospital because of severe vomiting and abdominal pain with massive ascites. He had been diagnosed as mixed connective tissue disease in 1997 and lupus nephritis ISN III (A/C) + V in 2003. Treatment was started with intravenous steroid pulse therapy combined with an immunosuppressant resulting in improvement of his proteinuria and serological activity. In 2008, the disease activity flared and he was admitted to our hospital with nephrotic syndrome. Hemodialysis was unavoidable, despite treatment with intravenous steroid pulse therapy and plasma exchange. We continued to treat him with oral prednisolone and tacrolimus. However, for personal reasons, he terminated tacrolimus treatment and massive ascites remained because of insufficient hemodialysis. Since the end of 2011, he suffered repeated abdominal pain with ileus and encapsulating peritoneal sclerosis (EPS) was detected. In February 2013, he underwent synechotomy for EPS. Here, we present a rare case of EPS in a hemodialysis patient.

Encapsulating Peritoneal Sclerosis 43 Years after Distal Gastrectomy for Early Gastric Cancer.

We herein report a case of encapsulating peritoneal sclerosis (EPS) in a patient without chronic kidney disease after gastrectomy. A 69-year-old man underwent distal gastrectomy for early gastric cancer at 25 years old. After 43 years, he developed bowel obstruction and underwent enterolysis of the encapsulated small intestine. A pathological examination of the capsular membranes revealed inflammation, foam, and giant cells that destroyed foreign substances. The patient was discharged 1.5 months later. Foreign body reactions to surgical instruments used in gastrectomy are considered a cause of EPS. EPS due to foreign body reactions to surgical instruments should also be considered in such cases.

Idiopathic encapsulating peritoneal sclerosis presenting with abdominal mass.

A man in his late 30s presented with a history of recurrent colicky abdominal pain, bilious vomiting and intermittent mass formation in the lower abdomen. The mass was mobile, non-tender and fluctuant, and appeared in the right iliac fossa during episodes of pain and disappeared once the pain subsided. Contrast-enhanced CT (CECT) scan revealed a thick membrane-like structure covering the clumped small bowel loops, suggestive of an abdominal cocoon. A midline laparotomy was carried out with extensive adhesiolysis, and a membrane incision was performed. The final histopathological diagnosis was primary encapsulating peritoneal sclerosis. Encapsulating peritoneal sclerosis of idiopathic origin is rare and typically presents as an acute or subacute intestinal obstruction. A CECT scan is the diagnostic modality of choice, with a thick peritoneal membrane covering the small bowel loops being the hallmark sign. Surgical intervention is the preferred treatment for idiopathic cases, while medical management may address secondary causes.

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.

Abdominal cocoon syndrome (sclerosing encapsulated peritonitis) causing small bowel occlusion in a patient with gastric bypass.

A rare condition, sclerosing encapsulating peritonitis, is characterized by a fibrotic membrane forming over the bowels, leading to intestinal obstruction. In this case of a 56-year-old male patient with a history of laparoscopic gastric bypass, a computed tomography scan showed findings indicative of the condition. Extensive adhesiolysis was performed, and biopsies confirmed the presence of fusiform cells (D2-40 positive on immunochemistry) resembling fibroblasts, within dense collagenous peritoneal tissue sheets, typical of sclerosing encapsulating peritonitis. The prevalence of this condition is uncertain, and diagnosis typically requires a peritoneal biopsy due to the nonspecific clinical presentation.

La peritonitis esclerosante encapsulada es una condición rara caracterizada por una membrana fibrótica que se genera sobre las asas intestinales causando cuadros de oclusión intestinal. Se presenta el caso de un paciente varón de 56 años con antecedente de derivación gastroyeyunal por laparoscopia que presenta oclusión intestinal. Se realizó tomografía computada que evidenció sitio de transición previo al sitio de anastomosis. Se realizó de anastomosis extensa y toma de biopsias. Histológicamente se observó engrosamiento de la membrana peritoneal, células fusiformes (D2-40 positivo en inmunohistoquímica) similares a fibroblastos con láminas de colágeno peritoneal denso. La peritonitis esclerosante encapsulada es una patología de prevalencia desconocida. El cuadro clínico es inespecífico y el diagnóstico definitivo es por patología con biopsia peritoneal.

Post-transplantation encapsulating peritoneal sclerosis without inflammation or radiological abnormalities.

BACKGROUND: Post-transplantation encapsulating peritoneal sclerosis (EPS) causing bowel obstruction has been identified as a serious complication after kidney transplantation in patients previously treated with peritoneal dialysis. Systemic inflammation and abnormalities on an abdominal computed tomography (CT) scan are important hallmarks of EPS. To our knowledge, this is the first report of a case being diagnosed with late-onset post-transplantation EPS without systemic inflammation or abnormalities on a CT scan which could only be diagnosed by laparotomy. CASE PRESENTATION: A 59-year old female presented because of symptoms of bowel obstruction 33 months after kidney transplantation. The patient had a 26-month history of peritoneal dialysis before her first kidney transplantation and was treated with peritoneal dialysis for 4 years before undergoing a second kidney transplantation. Physical examination was unremarkable and laboratory tests showed no signs of systemic inflammation (C-reactive protein <1 mg/L). An abdominal CT scan did not reveal any abnormalities fitting the diagnosis of EPS, except a "feces sign". Given the severity of the progressive symptoms, a diagnostic laparotomy was performed, visualizing a classical EPS. Total peritonectomy and enterolysis were performed, leading to restoration of peristalsis. CONCLUSION: EPS may occur several years after kidney transplantation in the absence of inflammation and typical radiological abnormalities. Obtaining a diagnosis of post-transplantation EPS is challenging, however, a low threshold for surgical exploration in case of high clinical suspicion and negative findings on the CT scan is mandatory.

[Surgical treatment for idiopathic abdominal cocoon].

OBJECTIVE: To investigate diagnosis and treatment of abdominal cocoon. METHODS: Clinical data of patients received treatment for abdominal cocoon from January 2000 to January 2011 was retrospectively analyzed. RESULTS: A total of 67 patients underwent treatment in our hospital were analyzed, the preoperatively diagnosis rate was only 47.8% (32/67). Patients who received preoperatively nutrition support have a lower postoperative complication (8/27 vs.13/20, χ(2) = 5.815, P < 0.05) and patients with less extent of intestine involved had a lower early postoperative inflammatory ileus (EPII) rate (9/25 vs. 1/22, χ(2) = 6.912, P < 0.05) when compared with large extent. CONCLUSIONS: Appropriate perioperative management play an important role in the prognosis of abdominal cocoon. The main treatment is surgery while preoperatively nutrition support can reduce postoperative complications.

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.

Encapsulating peritoneal sclerosis associated with abnormal liver development in a young dog.

A 6-month-old male miniature pinscher dog developed chronic ascites, formation of fibrous membrane covering the abdominal organs, and numerous adhesions between the intestinal loops. The membrane and adhesions were surgically removed twice, but the dog died 1 month after initial presentation. Necropsy revealed recurrence of the membrane and intestinal adhesions. The fibrous membrane was composed of a thick layer of mature collagenous connective tissue, covered by immature collagenous connective tissue with mild lymphoplasmacytic infiltration, prominent neovascularization, and fibrin exudation. Similar fibrotic lesions were observed in the serosa of the liver, spleen, stomach and intestines. These findings are consistent with encapsulating peritoneal fibrosis reported in humans and dogs. The dog also had a maldeveloped liver, which is characterized by disorganized hepatic lobules and disarranged hepatic cords.