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World Journal of Gastroenterology Jul 2018Encapsulating peritoneal sclerosis (EPS) is a debilitating condition characterized by a fibrocollagenous membrane encasing the small intestine, resulting in recurrent... (Review)
Review
Encapsulating peritoneal sclerosis (EPS) is a debilitating condition characterized by a fibrocollagenous membrane encasing the small intestine, resulting in recurrent small bowel obstructions. EPS is most commonly associated with long-term peritoneal dialysis, though medications, peritoneal infection, and systemic inflammatory disorders have been implicated. Many cases remain idiopathic. Diagnosis is often delayed given the rarity of the disorder combined with non-specific symptoms and laboratory findings. Although cross-sectional imaging with computed tomography of the abdomen can be suggestive of the disorder, many patients undergo exploratory laparotomy for diagnosis. Mortality approaches 50% one year after diagnosis. Treatment for EPS involves treating the underlying condition or eliminating possible inciting agents (. peritoneal dialysis, medications, infections) and nutritional support, frequently with total parenteral nutrition. EPS-specific treatment depends on the disease stage. In the inflammatory stage, corticosteroids are the treatment of choice, while in the fibrotic stage, tamoxifen may be beneficial. In practice, distinguishing between stages may be difficult and both may be used. Surgical intervention, consisting of peritonectomy and enterolysis, is time-consuming and high-risk and is reserved for situations in which conservative medical therapy fails in institutions with surgical expertise in this area. Herein we review the available literature of the etiology, pathogenesis, diagnosis, and treatment of this rare, but potentially devastating disease.
Topics: Glucocorticoids; Humans; Intestinal Obstruction; Intestine, Small; Parenteral Nutrition, Total; Peritoneal Dialysis; Peritoneal Fibrosis; Peritoneum; Peritonitis; Recurrence; Sclerosis; Tamoxifen; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 30065556
DOI: 10.3748/wjg.v24.i28.3101 -
Internal Medicine (Tokyo, Japan) Dec 2023
Topics: Humans; Abdomen; Intestinal Obstruction; Peritonitis
PubMed: 37062740
DOI: 10.2169/internalmedicine.1743-23 -
Kidney Medicine Sep 2022
PubMed: 36061982
DOI: 10.1016/j.xkme.2022.100524 -
Sultan Qaboos University Medical Journal May 2016Sclerosing encapsulating peritonitis (SEP) is a rare chronic inflammatory condition of the peritoneum with an unknown aetiology. Also known as abdominal cocoon, the... (Review)
Review
Sclerosing encapsulating peritonitis (SEP) is a rare chronic inflammatory condition of the peritoneum with an unknown aetiology. Also known as abdominal cocoon, the condition occurs when loops of the bowel are encased within the peritoneal cavity by a membrane, leading to intestinal obstruction. Due to its rarity and non-specific clinical features, it is often misdiagnosed. The condition presents with recurrent episodes of small bowel obstruction and can be idiopathic or secondary; the latter is associated with predisposing factors such as peritoneal dialysis or abdominal tuberculosis. In the early stages, patients can be managed conservatively; however, surgical intervention is necessary for those with advanced stage intestinal obstruction. A literature review revealed 118 cases of SEP; the mean age of these patients was 39 years and 68.0% were male. The predominant presentation was abdominal pain (72.0%), distension (44.9%) or a mass (30.5%). Almost all of the patients underwent surgical excision (99.2%) without postoperative complications (88.1%).
PubMed: 27226904
DOI: 10.18295/squmj.2016.16.02.003 -
World Journal of Gastroenterology Jan 2015To review the literature on idiopathic sclerosing encapsulating peritonitis (SEP), also known as abdominal cocoon syndrome. (Review)
Review
AIM
To review the literature on idiopathic sclerosing encapsulating peritonitis (SEP), also known as abdominal cocoon syndrome.
METHODS
The PubMed, MEDLINE, Google Scholar, and Google databases were searched using specific key words to identify articles related to idiopathic SEP. These key words were "sclerosing encapsulating peritonitis," "idiopathic sclerosing encapsulating peritonitis," "abdominal cocoon," and "abdominal cocoon syndrome." The search included letters to the editor, case reports, review articles, original articles, and meeting presentations published in the English-language literature from January 2000 to May 2014. Articles or abstracts containing adequate information about age, sex, symptom duration, initial diagnosis, radiological tools, and surgical approaches were included in the study. Papers with missing or inadequate data were excluded.
RESULTS
The literature search yielded 73 articles on idiopathic (primary) SEP published in 23 countries. The four countries that published the greatest number of articles were India (n = 21), Turkey (n = 14), China (n = 8) and Nigeria (n = 3). The four countries that reported the greatest number of cases were China (n = 104; 53.88%), India (n = 35; 18.13%), Turkey (n = 17; 8.80%) and Nigeria (n = 5; 2.59%). The present study included 193 patients. Data on age could be obtained for 184 patients (range: 7-87 years; mean ± SD, 34.7 ± 19.2 years), but were unavailable for nine patients. Of the 184 patients, 122 were male and 62 were female; sex data could not be accessed in the remaining nine patients. Of the 149 patients whose preoperative diagnosis information could be obtained, 65 (43.6%) underwent operations for abdominal cocoon, while the majority of the remaining patients underwent operations for a presumed diagnosis of intestinal obstruction and/or abdominal mass. Management information could be retrieved for 115 patients. Of these, 68 underwent excision + adhesiolysis (one laparoscopic); 24 underwent prophylactic appendectomy in addition to excision + adhesiolysis. Twenty patients underwent various resection and repair techniques along with excision + adhesiolysis. The remaining three patients were managed with antituberculosis therapy (n = 2) and immunosuppressive therapy (n = 1).
CONCLUSION
Idiopathic SEP is a rare disorder characterized by frequently recurring bouts of intestinal obstruction. Surgical therapy is the gold standard management strategy.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Female; Humans; Intestinal Obstruction; Male; Middle Aged; Peritoneal Fibrosis; Predictive Value of Tests; Recurrence; Treatment Outcome; Young Adult
PubMed: 25593498
DOI: 10.3748/wjg.v21.i2.675 -
Indian Journal of Pathology &... 2024Sclerosing encapsulating peritonitis also known as cocoon abdomen is a rare chronic inflammatory condition of the peritoneum in which the bowel loops are encircled by a...
Sclerosing encapsulating peritonitis also known as cocoon abdomen is a rare chronic inflammatory condition of the peritoneum in which the bowel loops are encircled by a membrane (cocoon formation) within the peritoneal cavity leading to intestinal obstruction. It can be primary (idiopathic) or secondary (chemotherapy, beta-blockers, peritoneal dialysis, shunts, tuberculosis, systemic lupus erythematosus, etc.). The symptomatology report includes recurrent episodes of abdominal pain and vomiting. We present here a case of a 32-year-old male who presented with complaints of being unable to pass stools, vomiting (3-4 times), and abdomen pain for 4 days. This case is considered worth mentioning due to its rarity, lack of identification of secondary causes, and diminutive mention of histopathological aspect.
Topics: Male; Humans; Adult; Peritonitis; Intestinal Obstruction; Peritoneum; Vomiting
PubMed: 38358217
DOI: 10.4103/ijpm.ijpm_1228_21 -
World Journal of Gastroenterology May 2012Abdominal cocoon, the idiopathic form of sclerosing encapsulating peritonitis, is a rare condition of unknown etiology that results in an intestinal obstruction due to... (Review)
Review
Abdominal cocoon, the idiopathic form of sclerosing encapsulating peritonitis, is a rare condition of unknown etiology that results in an intestinal obstruction due to total or partial encapsulation of the small bowel by a fibrocollagenous membrane. Preoperative diagnosis requires a high index of clinical suspicion. The early clinical features are nonspecific, are often not recognized and it is difficult to make a definite pre-operative diagnosis. Clinical suspicion may be generated by the recurrent episodes of small intestinal obstruction combined with relevant imaging findings and lack of other plausible etiologies. The radiological diagnosis of abdominal cocoon may now be confidently made on computed tomography scan. Surgery is important in the management of this disease. Careful dissection and excision of the thick sac with the release of the small intestine leads to complete recovery in the vast majority of cases.
Topics: Humans; Intestinal Obstruction; Peritonitis; Prognosis; Sclerosis
PubMed: 22563185
DOI: 10.3748/wjg.v18.i17.1999 -
Frontiers in Veterinary Science 2022This retrospective case series describes imaging findings in seven dogs and two cats with a presumptive diagnosis of sclerosing encapsulating peritonitis (SEP) between...
This retrospective case series describes imaging findings in seven dogs and two cats with a presumptive diagnosis of sclerosing encapsulating peritonitis (SEP) between 2014 and 2021. Peritoneal effusion was present in all animal patients. Sonographically, echogenic fluid with or without echogenic intraperitoneal septations, gathered or corrugated bowel loops, and abdominal lymphadenomegaly were suggesting an inflammatory process and the presence of adhesions. Gathering of the bowel with abdominal distension and/or signs of intestinal obstruction were major findings on radiographs. Abdominal fat stranding was an additional finding in animals undergoing a CT examination. Previous surgery, pregnancy, and the presence of a perforating foreign body were potential predisposing causes in 4/9 animals. Peritonitis was septic in 4/9 animals. As SEP is a rare condition but life threatening, this detailed description of imaging findings in a short case series can be useful for a presumptive diagnosis and surgical planning.
PubMed: 35754547
DOI: 10.3389/fvets.2022.891492 -
Cureus Jan 2023Sclerosing encapsulating peritonitis is a rare chronic inflammatory condition often with unknown origins. We report a case of an abdominal cocoon or sclerosing...
Sclerosing encapsulating peritonitis is a rare chronic inflammatory condition often with unknown origins. We report a case of an abdominal cocoon or sclerosing encapsulating peritonitis, which was suspected to be a result of bowel obstruction. Tuberculosis peritonitis was also suspected. However, the exact diagnosis was unclear, and it was diagnosed as an idiopathic abdominal cocoon. The patient's history is of clear relevance in this diagnosis, and this report will be of interest to clinicians attending to cases of bowel obstruction.
PubMed: 36721711
DOI: 10.7759/cureus.34322