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.

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.

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.

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.

Imaging pearls and differential diagnosis of encapsulating peritoneal sclerosis: Emphasis on computed tomography.

Encapsulating peritoneal sclerosis (EPS) is a severe peritoneal fibrotic reaction most frequently identified as a complication of peritoneal dialysis. EPS is a complex condition whose management requires multidisciplinary input from radiologists, gastroenterologists, nephrologists, surgeons, and dietitians. EPS carries significant morbidity and mortality, primarily due to bowel obstruction that results in intestinal failure, malnutrition, and sepsis. The nondialysis causes of EPS include tuberculous peritonitis, prior abdominal surgery, beta-blocker medication use, and endometriosis. The clinical symptoms of EPS are nausea, vomiting, and abdominal pain, all of which appear to be associated with bowel obstruction. The diagnosis of EPS needs three pillars to be met: clinical features, radiological evaluation, and histopathological analysis. The disease is frequently progressive and can be fatal. Computed tomography is the gold standard imaging modality for the detection of peritoneal abnormalities and encapsulation of bowel loops by thick adhesions or fibrosis (cocooning). Computed tomography also aids in making a differential diagnosis. Unfortunately, the diagnosis of EPS is often delayed because clinical findings are not specific and may resemble other peritoneal diseases. Radiologists should be familiar with the clinical impacts and related imaging features of EPS and realize when to seek them to facilitate timely and proper treatment.

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.

Encapsulated Peritoneal Sclerosis in an Adolescent With Kidney Transplant After Long-Term Peritoneal Dialysis.

Encapsulated peritoneal sclerosis is a rare complication of long-term peritoneal dialysis that has a high rate of morbidity and mortality. We present an 18-year-old female patient who was first diagnosed with renal failure at 8 years of age and who had 7 years of peritoneal dialysis and then hemodialysis before kidney transplant from a deceased donor. Before transplant, the patient developed encapsulated peritoneal sclerosis and was treated with tamoxifen and steroids. Three years after transplant, the patient presented with complaints of vomiting, abdominal pain, and abdominal distension and was again diagnosed with encapsulated peritoneal sclerosis. The patient required excretory paracentesis, pulse steroid treatment for 3 days, and treatment with methylprednisone and tamoxifen, which resulted in regression of signs and symptoms. Factors such as long-term peritoneal dialysis, a history of bacterial peritonitis, and use of high-concentration dialysate may cause encapsulated peritoneal sclerosis, but symptoms can recur after transplant, as shown in our patient. Thus, it is important to recognize that encapsulated peritoneal sclerosis may cause graft loss due to the various complications that it can cause.

Concurrent development of encapsulating peritoneal sclerosis and calciphylaxis in a patient with peritoneal dialysis for end-stage renal disease.

Both encapsulating peritoneal sclerosis (EPS) and calciphylaxis are rare but severe complications involving patients with end-stage renal disease. In this report, we discuss a unique case of a 73-year-old female patient who had undergone 8 years of peritoneal dialysis for IgA nephropathy and concurrently developed these two synchronous complications within 3 months of each other. Diagnosis and management of both conditions were discussed in detail as well as the possible association between the two. With surgical treatment for EPS and measures to minimise bone mineral disorder abnormalities, both complications have been successfully managed to date.

Encapsulated peritoneal sclerosis.

Encapsulating peritoneal sclerosis (EPS) also known as abdominal cacoon is a rare cause of acute or subacute small bowel obstruction. It is characterized by total or partial encasement of the small bowel within a thick fibrocollagenous membrane which may be formed in response to prolonged, repetitive, and severe insult to the peritoneal mesothelium. This is frequently seen in the setting of peritoneal dialysis. However other causes may include chronic inflammation. We present a case of EPS in a male with infrequent abdominal pain, nausea and fever.

Abdominal cocoon in children: A case report and review of literature.

BACKGROUND: Abdominal cocoon or "encapsulating peritoneal sclerosis" (EPS) is an uncommon and rare cause of intestinal obstruction. Only a few cases have been reported in paediatric patients. Typically, EPS is described as the primary form in young adolescent girls from tropical and subtropical countries because of viral peritonitis due to retrograde menstruation or a history of peritoneal dialysis. Most patients are asymptomatic or present with abdominal pain, which is likely to occur secondary to subacute bowel obstruction. Findings at imaging, such as ultrasound, computed tomography, and magnetic resonance imaging, are often nonspecific. When diagnosed, EPS is characterized by total or partial encasement of the bowel within a thick fibrocollagenous membrane that envelopes the small intestine in the form of a cocoon because of chronic intraabdominal fibroinflammatory processes. The membrane forms a fibrous tissue sheet that covers, fixes, and finely constricts the gut, compromising its motility. CASE SUMMARY: We present a case of EPS in a 12-year-old boy 8 wk after primary surgery for resection of symptomatic jejunal angiodysplasia. There was no history of peritoneal dialysis or drug intake. CONCLUSION: In this report, we sought to highlight the diagnostic, surgical, and histopathological characteristics and review the current literature on EPS in paediatric patients.

[Idiopathic sclerosing encapsulating peritonitis: a case report]. / La péritonite encapsulante sclérosante idiopathique: à propos d´un cas.

Sclerosing encapsulating peritonitis is a very rare pathological entity. It is a chronic fibroinflammatory disease affecting the peritoneum and leading to the formation of diffuse egg-shell-shaped fibrous capsule which totally or locally encapsulate the abdominal viscera, in particular the digestive tract. Clinical signs are little specific and misleading. Medical imaging, including computed tomography, can help clinicians to make a diagnosis, by highlighting a thin peritoneal membrane encompassing an agglutination of digestive loops. Secondary types (postperitoneal dialysis, tuberculosis, medications, postintraperitoneal chemotherapy) are quite common, however idiopathic sclerosing encapsulating peritonitis is very rare and few cases have been reported in the literature. We here report the case of a 53-year-old woman with idiopathic sclerosing encapsulating peritonitis.

Encapsulating peritoneal sclerosis caused by cell-free and concentrated ascites reinfusion therapy: a case report.

BACKGROUND: Encapsulating peritoneal sclerosis (EPS) is a rare condition in which the small intestine is covered by an inflammatory fibrocollagenous membrane; the exact etiology of EPS is unclear. Herein, we report the case of our patient who underwent hemodialysis and cell-free and concentrated ascites reinfusion therapy (CART) and was diagnosed with EPS. CASE PRESENTATION: A 64-year-old Japanese man visited our emergency department with a chief complaint of abdominal pain. He had a medical history of cirrhosis due to hepatitis C for 25 years. He had undergone partial resection of the small intestine 2 years earlier for an incarcerated hernia. One year earlier, he experienced renal failure due to hepatorenal syndrome and started hemodialysis three times a week and CART twice a month. Physical examination of the abdominal wall revealed a lack of peristalsis of the intestinal tract and strong tenderness on palpation. Because hernia of the small intestine was found on computed tomography, we suspected strangulation ileus, requiring emergency operation. When the abdomen was opened, the entire small intestine was found to be wrapped in a fibrous membrane and constricted by it. The patient was diagnosed with EPS; hence, during surgery, the fibrous membrane was excised, resulting in decompression of the intestinal tract and subsequent recovery. CONCLUSIONS: EPS is thought to be related to various elements, but no case of EPS induced by CART has been reported to date. EPS should be considered in the differential diagnosis of small bowel obstruction in patients undergoing CART for refractory ascites.

Long-term outcomes in patients with encapsulating peritoneal sclerosis managed with nutritional support.

BACKGROUND: Little is known about long-term survivors with encapsulating peritoneal sclerosis (EPS). Published literature focuses on patients managed surgically. We describe our experience of the long-term outcomes in patients with EPS conservatively managed with nutritional support alone. METHODS: This is a single-centre retrospective observational study of patients who had survived for ≥5 years since diagnosis. EPS survivors were invited for review of symptoms, nutritional assessment and evaluation of quality of life. Radiological progression was assessed based on serial computed tomography (CT) scores for each patient. RESULTS: A total of 23 patients with a diagnosis of EPS for at least 5 years were identified, with 18 patients alive at the time of the study. Of these 18 patients, 10 patients transferred to haemodialysis (HD) and 8 patients received kidney transplants. Commonest symptoms were nausea (91%) and vomiting (73%). Mean body mass index for patients was within the ideal and healthy range, with only 11% suffering from continued weight loss. In all, 70% EPS survivors on HD received nutritional support compared to 15% of those with transplants; 17% required ongoing parenteral nutrition. Of the 11 patients with serial CT scans at least 4 years apart, 10 had an increase in radiological score for EPS but with no apparent correlation to clinical outcomes. There were no significant differences in the reported quality of life between EPS survivors on HD and those transplanted, with self-rated health status equivalent to that reported for the general end-stage kidney disease (ESKD) population. CONCLUSION: Long-term survival following EPS managed conservatively with nutritional support is feasible, with the majority no longer requiring nutritional support and having a quality of life similar to other patients with ESKD.

Massive recurrent hemoperitoneum with encapsulating peritonitis: another enigmatic clinical feature of endometriosis.

OBJECTIVE: To describe the clinical characteristics and laparoscopic findings of a very uncommon presentation of a patient with endometriosis. DESIGN: Video presentation of case report (Canadian Task Force classification III). (The institutional review board of the Hospital Naval Pedro Mallo, Buenos Aires, Argentina, has ruled that approval was not required for the publication of this case report.) SETTING: Hospital. PATIENT(S): Thirty-two-year-old woman with endometriosis presenting with hemorrhagic ascites. INTERVENTION(S): We demonstrate the laparoscopic appearance of the peritoneal organs in the presence of massive hemoperitoneum and encapsulating peritonitis and also describe the diagnosis and management options of an uncommon clinical presentation of endometriosis. The patient is a 32-year-old woman, gravida 0, who presented with abdominal pain and ascites. Initially, she underwent exploratory laparotomy with drainage of 5 liters of ascites and excision of endometrial peritoneal implants. She then presented 4 months later with sudden worsening abdominal pain and distention, weight gain, bloating, and shortness of breath. A diagnostic laparoscopy was performed with the findings of over 10 liters of dark hemoperitoneum and diffuse pelviperitonitis with loose necrotic, easy to remove, dense peritoneal tissue. Patient was started on triptorelin acetate with great response. MAIN OUTCOME MEASURE(S): Resolution of the symptomatology secondary to hemorrhagic peritonitis. RESULT(S): Clinical improvement of symptomatology of a patient with endometriosis and hemorrhagic ascites. CONCLUSION(S): Endometriosis can have different clinical presentations. Endometriosis should be a differential diagnosis in women of reproductive age presenting with massive hemorrhagic ascites. Hemorrhagic ascites, considered an exceedingly rare clinical course of endometriosis, represents a challenge to the surgeon who is unfamiliar with this condition. Bilateral oophorectomy is the definitive treatment, but conservative therapy is indicated for women of childbearing age. Diagnostic laparoscopy with drainage of hemoperitoneum is a feasible option to obtain a pathology-confirmed diagnosis in patients presenting with hemoperitoneum secondary to pelvic endometriosis. Awareness of this condition will prevent unnecessary aggressive resection, as is commonly performed when the condition is confused with ovarian cancer.