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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 nonspecific clinical features,...
Context in source publication
Context 1
... Other theories proposed for the aetiopathogenesis of primary SEP include developmental disorders related to vascular anomalies and omental hypoplasia. 2,16,19 In contrast, secondary SEP is associated with several causes and is therefore more common [ Table 2]. The predominant cause of secondary SEP is PD, due to both its frequency worldwide and the associated peritoneal inflammation that the dialysis fluid induces. ...
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Rationale:
Sclerosing encapsulated peritonitis (SEP) is a rare chronic peritoneal inflammation with unknown etiology, and is also known as abdominal cocoon. This occurs when the intestinal annulus is enveloped in the peritoneal cavity, resulting in intestinal obstruction. Its preoperative diagnosis and treatment strategy remains a challenge.
Pati...
Citations
... It is also further divided into primary (idiopathic) or secondary SEP. [16] Primary abdominal cocoon disease stems from embryonic body curling, abnormal mesoderm differentiation, and intestinal dorsal mesenteric dysplasia. This form is often associated with anatomical abnormalities such as the absence of the omentum, gastrocolic ligament, visceral transposition, intestinal or colonic malrotation, cryptorchidism, hernia, and other related conditions [17]. ...
... SEP classification is based on anatomical involvement, categorized into types I, II, and III, depending on whether it partially, totally, or also involves adjacent abdominal contents, respectively [26] (Fig. 4). A comprehensive systematic review conducted by Machado et al. [16], encompassing 118 patients, disclosed a distribution of 43 % for type I, 31 % for type II, and 25 % for type III SEP. This observation aligns with the prevailing trend in the literature, indicating a higher prevalence of type I and type II presentations. ...
... Diagnostic imaging assumes a pivotal role in the assessment of SEP. Small-bowel follow-through studies unveil a distinctive concertina-like arrangement of bowel loops, and an air-fluid level on erect abdominal X-ray, indicative of bowel obstruction [16]. Ultrasonography (US) reveals a distinctive "cauliflower" appearance of bowel with a narrow base, along with a "trilaminar" appearance, particularly accentuate through the use of high-resolution US proves [18]. ...
Introduction: Abdominal Cocoon Syndrome (ACS), or Sclerosing Encapsulating Peritonitis (SEP) is a
rare cause of intestinal obstruction in which the bowel and internal abdominal organs are wrapped with a
fibrocollagenous cocoon
-like encapsulating membrane. While cocooning of the abdomen primarily
manifests in individuals undergoing Peritoneal Dialysis (PD), it has also been reported to occur
spontaneously. Remarkably rare, SEP may present with complete mechanical bowel obstruction in select
cases.
Case presentation: We hereby report a case of an 87
-year
-old female patient with a prior history of
abdominal surgery, who presented to our emergency department with a clinical picture of complete small
bowel obstruction. Clinical and radiological data were suggestive of a strangulated midline hernia,
prompting a therapeutic laparotomy. The surgical exploration revealed the encasement of the small bowel
loops within a thick fibrocollagenous membrane. Efficient resolution was achieved through skillful
adhesiolysis and the meticulous excision of the fibrocollagenous membrane.
Discussion: SEP is more prevalent in men, with a higher incidence observed in tropical and subtropical
countries. While the precise pathophysiology remains elusive, it is hypothesized that subclinical
intraabdominal inflammation gives rise to the formation of a dense fibrocollagenous membrane. This
membrane encapsulates intraperitoneal organs, ultimately leading to intestinal obstruction. Patients
typically present with a recurrent history of small bowel obstruction, notably in the absence of prior
abdominal surgery. Abdominal CT scan with experienced radiologist interpretation can aid in preoperative
diagnosis. In cases where non
-operative management fails and recurrent obstructions persist, surgical
adhesiolysis stands as the well
-established gold standard.
Conclusion: SEP is a rare abdominal disease, posing challenges for preoperative diagnosis. Laparotomy
plays an important role in its diagnosis and treatment. The primary objective of the surgical intervention is
to release the encapsulation of the bowel and safeguard the optimal functioning of the small intestines as
much as possible.
... According to the Peritoneal Dialysis Society, it is defined as a syndrome that occurs continuously, intermittently, or repeatedly with symptoms of intestinal obstruction caused by adhesions of a diffusely thickened peritoneum [2]. Sometimes it involves the stomach, colon, liver, and spleen, causing intestinal occlusion at different levels [2,3]. It is a very rare disease that presents in the patient with a wide variety of symptoms and clinical signs. ...
... CESP is a chronic inflammatory condition that causes peritoneal thickening due to the formation of a membrane of collagen and fibrous tissue that surrounds the loops of the small intestine. According to the studies carried out, CESP can be classified as primary (idiopathic) or secondary [3,4]. ...
... On the other hand, extensive endometriosis and the role of prostaglandin E2 and its relationship with the protease-activated receptor (PAR) have been described in women [4,5]. This can be classified into three categories based on the extension of the peritoneal membrane over the loops of intestine: type I refers to only partially covering a part of intestinal loops, type II covers the entire loops of the small intestine, and type III involves the entire loops of the small intestine and other organs (appendix, cecum, ascending colon, stomach, liver, and ovaries) [3]. ...
Chronic encapsulated sclerosing peritonitis (CESP) is a very rare cause of intestinal obstruction that has been associated with certain chronic conditions. It is characterized by the thickening of the peritoneum, generating a membrane that covers the intestinal loops and prevents their adequate mobilization. Most cases present as a surgical emergency, leading to a diagnosis during surgery; however, imaging studies can support the pre-surgical diagnosis. Treatment is based on the clinical context of the patient, based on medical management with corticosteroids in a stable patient, or surgical management when it presents as an acute complication. The morbidity and mortality associated with this condition are high and epidemiological data are scarce. There is still a lack of studies to describe the associated demographic data, diagnosis, and treatment.
... It is also further divided into primary (idiopathic) or secondary SEP. [16] Primary abdominal cocoon disease stems from embryonic body curling, abnormal mesoderm differentiation, and intestinal dorsal mesenteric dysplasia. This form is often associated with anatomical abnormalities such as the absence of the omentum, gastrocolic ligament, visceral transposition, intestinal or colonic malrotation, cryptorchidism, hernia, and other related conditions [17]. ...
... SEP classification is based on anatomical involvement, categorized into types I, II, and III, depending on whether it partially, totally, or also involves adjacent abdominal contents, respectively [26] (Fig. 4). A comprehensive systematic review conducted by Machado et al. [16], encompassing 118 patients, disclosed a distribution of 43 % for type I, 31 % for type II, and 25 % for type III SEP. This observation aligns with the prevailing trend in the literature, indicating a higher prevalence of type I and type II presentations. ...
... Diagnostic imaging assumes a pivotal role in the assessment of SEP. Small-bowel follow-through studies unveil a distinctive concertina-like arrangement of bowel loops, and an air-fluid level on erect abdominal X-ray, indicative of bowel obstruction [16]. Ultrasonography (US) reveals a distinctive "cauliflower" appearance of bowel with a narrow base, along with a "trilaminar" appearance, particularly accentuate through the use of high-resolution US proves [18]. ...
Introduction: Abdominal cocoon syndrome (ACS), or sclerosing encapsulating peritonitis (SEP) is a rare cause of intestinal obstruction in which the bowel and internal abdominal organs are wrapped with a fibrocollagenous cocoon-like encapsulating membrane. While cocooning of the abdomen primarily manifests in individuals undergoing peritoneal dialysis (PD), it has also been reported to occur spontaneously. Remarkably rare, SEP may present with complete mechanical bowel obstruction in select cases. Case presentation: We hereby report a case of an 87-year-old female patient with a prior history of abdominal surgery, who presented to our emergency department with a clinical picture of complete small bowel obstruction. Clinical and radiological data were suggestive of a strangulated midline hernia, prompting a therapeutic laparotomy. The surgical exploration revealed the encasement of the small bowel loops within a thick fibrocollagenous membrane. Efficient resolution was achieved through skillful adhesiolysis and the meticulous excision of the fibrocollagenous membrane. Discussion: SEP is more prevalent in men, with a higher incidence observed in tropical and subtropical countries. While the precise pathophysiology remains elusive, it is hypothesized that subclinical intraabdominal inflammation gives rise to the formation of a dense fibrocollagenous membrane. This membrane encapsulates intraperitoneal organs, ultimately leading to intestinal obstruction. Patients typically present with a recurrent history of small bowel obstruction, notably in the absence of prior abdominal surgery. Abdominal CT scan with experienced radiologist interpretation can aid in preoperative diagnosis. In cases where non-operative management fails and recurrent obstructions persist, surgical adhesiolysis stands as the well-established gold standard. Conclusion: SEP is a rare abdominal disease, posing challenges for preoperative diagnosis. Laparotomy plays an important role in its diagnosis and treatment. The primary objective of the surgical intervention is to release the encapsulation of the bowel and safeguard the optimal functioning of the small intestines as much as possible.
... Despite extensive research, the precise etiology of SEP remains enigmatic. SEP is classified into primary or secondary types based on etiology and pathogenesis [7]. Primary SEP, also known as idiopathic SEP or ACS, denotes instances where alternative causative factors are excluded or when the phenomenon defies explanation within the patient's medical context. ...
... This regional correlation hints at a distinctive trend within the epidemiological landscape, suggesting a potentially heightened incidence or recognition of SEP cases in Asian populations. This noteworthy observation corresponds to previously documented reports, underscoring a particular focus on Asia in the prevalence and incidence of SEP cases [4,7,10,11]. ...
... The rarity of ACS often leads to underdiagnosis, with most patients presenting recurrently with abdominal discomfort, tenderness, nausea/vomiting, intestinal distention, and abdominal masses [7]. Manifestations vary across acute, subacute, and chronic settings, frequently accompanied by weight loss and malnutrition. ...
BACKGROUND
Abdominal cocoon syndrome (ACS) represents a category within sclerosing encapsulating peritonitis, characterized by the encapsulation of internal organs with a fibrous, cocoon-like membrane of unknown origin, resulting in bowel obstruction and ischemia. Diagnosing this condition before surgery poses a challenge, often requiring confirmation during laparotomy. In this context, we depict three instances of ACS: One linked to intestinal obstruction, the second exclusively manifesting as intestinal ischemia without any obstruction, and the final case involving a discrepancy between the radiologist and the surgeon.
CASE SUMMARY
Three male patients, aged 53, 58, and 61 originating from Northern Thailand, arrived at our medical facility complaining of abdominal pain without any prior surgeries. Their vital signs remained stable during the assessment. The diagnosis of abdominal cocoon was confirmed through abdominal computed tomography (CT) before surgery. In the first case, the CT scan revealed capsules around the small bowel loops, showing no enhancement, along with mesenteric congestion affecting both small and large bowel loops, without a clear obstruction. The second case showed intestinal obstruction due to an encapsulated capsule on the CT scan. In the final case, a patient presented with recurring abdominal pain. Initially, the radiologist suspected enteritis as the cause after the CT scan. However, a detailed review led the surgeon to suspect encapsulating peritoneal sclerosis (ACS) and subsequently perform surgery. The surgical procedure involved complete removal of the encapsulating structure, resection of a portion of the small bowel, and end-to-end anastomosis. No complications occurred during surgery, and the patients had a smooth recovery after surgery, eventually discharged in good health. The histopathological examination of the fibrous membrane (cocoon) across all cases consistently revealed the presence of fibro-collagenous tissue, without any indications of malignancy.
CONCLUSION
Individuals diagnosed with abdominal cocoons commonly manifest vague symptoms of abdominal discomfort. An elevated degree of clinical suspicion, combined with the application of appropriate radiological evaluations, markedly improves the probability of identifying the abdominal cocoon before surgical intervention. In cases of complete bowel obstruction or ischemia, the established norm is the comprehensive removal of the peritoneal sac as part of standard care. Resection with intestinal anastomosis is advised solely when ischemia and gangrene have been confirmed.
... Due to the low incidence of sclerosing peritonitis, there is no expert agreement on whether the treatment of choice should be surgical or conservative; according to a 2011 paper, no respective clinical trials have compared the different therapeutic options, and experience is confined to case reports [1]. However, according to a 2016 review article, individuals with minor abdominal symptoms should be managed conservatively with bowel rest, nasogastric decompression, and either enteral or parenteral nutrition [4]. We describe a successful conservative approach that includes canceling surgery as soon as the diagnosis is suspected and, subsequently, initiating inpatient total parenteral nutrition. ...
... Sclerosing encapsulating peritonitis can be described as primary (idiopathic) or secondary. Primary SEP, also known as abdominal cocoon syndrome, is divided into three categories based on the degree of encasement by the membrane [4]. Primary SEP is idiopathic, meaning it has no known etiology. ...
... According to a 2016 review article, individuals with minor abdominal symptoms should be managed conservatively with bowel rest, nasogastric decompression, and either enteral or parenteral nutrition, with a drug therapy that may include tamoxifen, steroids, colchicine, azathioprine, and mycophenolic acid in those who failed to respond to a conservative approach [4]. Despite the initial severe picture of obstruction in this patient, we decided to take a conservative strategy, which justified maintaining her away from the operation theater for a high-mortality adhesiolysis procedure. ...
An uncommon cause of intestinal obstruction is an abdominal cocoon, also known as sclerosing encapsulating peritonitis (SEP). We present the case of a 24-year-old female peritoneal dialysis patient who presented with a picture of complete intestinal obstruction. After reviewing the patient's medical history and acquiring relevant laboratory and imaging data, the decision was made to proceed with surgery. Intraoperatively, however, she had a picture of sclerosing peritonitis. The decision was to terminate the surgery and to take a conservative approach, including total parenteral nutrition. Her condition improved, obstruction was resolved, and she was discharged home in good clinical condition. Sclerosing peritonitis should be considered a possible etiology that can be managed conservatively in any peritoneal dialysis patient with intestinal obstruction.
... Sclerosing Encapsulating Peritonitis (SEP) leads to sclerosis membrane development and cocoon formation [16]. The etiology of SEP is assumed to be recurrent sub-clinical peritonitis [16]. ...
... Sclerosing Encapsulating Peritonitis (SEP) leads to sclerosis membrane development and cocoon formation [16]. The etiology of SEP is assumed to be recurrent sub-clinical peritonitis [16]. Condition is characterized by a dense, greyish-white fibrotic membrane encasing the small bowel and other abdominal organs [16]. ...
... The etiology of SEP is assumed to be recurrent sub-clinical peritonitis [16]. Condition is characterized by a dense, greyish-white fibrotic membrane encasing the small bowel and other abdominal organs [16]. SEP can be primary or secondary; primary SEP is also known as abdominal cocoon while secondary SEP may be developed due to peritoneal dialysis (PD) or abdominal TB [16]. ...
Peritonitis is an infection with a substantial source of morbidity and death. The mortality rate is 10% to 60%. Its etiology may be infection of bacteria, viruses, or fungi. The objective of the current systematic review is to identify the causes of peritonitis and discuss available treatment options. A systematic review was conducted from the literature from January 2012 to December 2022. More than 60 articles were downloaded; after abstracting relevant information from the studies and assessing quality, data was synthesized and presented by PRISMA flow diagram. The most common cause was bacterial infection; followed by fungal and viral infections. Reported organisms were E. coli, Klebsiella spp., Streptococcus spp., M. tuberculosis, C. trachomatis, Pseudomonas spss., C. albicans, C. glabrata, C. krusei, Cryptococcus spp., and Aspergillus spp., and Feline-infectious-corona-virus. Empiric antibiotics therapy covers broad-spectrum antibacterials; antifungal and surgical interventions are treatment options. The acutely ill patient requires combined medical and surgical methods; culture sensitivity is highly advisable to reduce the chances of failure.
... Other lesions such as ascites, intestinal obstruction, abdominal calcifications, and lymphadenopathy may be present [6]. Some other diseases that may give similar clinical and radiological presentation of SEP include peritoneal tuberculosis, peritoneal mesothelioma or pseudomyxoma, and congenital peritoneal encapsulation [7,8,9]. ...
Sclerosing encapsulating peritonitis (SEP) is an unusual fibroinflammatory disease of the peritoneum marked by the development of a fibrous membrane enveloping generally the small intestines. The knowledge around this subject is not completely understood. And the etiology can be either idiopathic or secondary to several diseases, treatments, and/or medications. We present a case of a 52-year-old man suffering from atypical clinical symptoms including recurrent abdominal ascites and intestinal obstruction. An abdominal computed tomography showed findings typical of SEP. Therefore, the patient benefited from exploratory laparotomy, which confirmed the diagnosis of idiopathic SEP. Postoperatively, he again had an episode of bowel obstruction, but this was controlled with steroids. Diagnosis of SEP is a real challenge to surgeons, gastroenterologists, and radiologists. And imagery is very helpful to make the diagnosis. Consequently, it is imperative that all hospital practitioners should distinguish between this lesion and other etiology of acute peritonitis.
... There are known factors that can lead to secondary ACS, and the most common cause is reported to be continuous ambulatory peritoneal dialysis [3] . Other causes include abdominal TB infection, recurrent peritonitis, previous abdominal surgery, peritoneal shunts, abdominal sarcoidosis, SLE, prolonged beta-blocker treatment, and rarely B-cell lymphoma [5] . None of those factors existed in our case, which was then diagnosed a primary/ idiopathic ACS. ...
Introduction and Importance
Abdominal cocoon syndrome (ACS), as a rare cause of mechanical intestinal obstruction, can be divided into primary/idiopathic vs. secondary type. The primary ACS is often asymptomatic and only diagnosed in exploratory laparotomy. The major treatment of surgery can be challenging. Since the gut wall and peritoneum are densely adhered, gut perforation might occur during adhesiolysis. Thus, it is important to have an experienced surgeon to perform the surgery.
Case presentation
We present a primary ACS case of a 50-year-old man. The patient demonstrated an unbearable upper abdominal pain upon admission. A CT scan showed a severe bowel obstruction. An exploratory laparotomy was indicated, leading to the diagnosis of ACS, which was considered idiopathic after ruling out secondary factors. An adhesiolysis was performed successfully. Note that the entire intestine measured was only 2.1 meters during the surgery. There was no post-surgical complication. The patient was recovered uneventfully.
Clinical Discussion
The etiology of primary ACS is unknown. The incidence is comparatively low and considered equal between men and women. As a rare cause of gut obstruction, the suspicion of the diagnosis should be strengthened. Surgery including adhesiolysis and bowel resection remains the major treatment. If adhesiolysis fails, bowel resection will be inevitable. The knowledge and experience of surgeon will be tested.
Conclusion
The etiology of primary ACS should be further explored. And the differential diagnosis of bowel obstruction should cover ACS in order for the surgeon to be prepared before surgery.
... Encapsulating peritoneal sclerosis (EPS), also called sclerosing encapsulating peritonitis (SEP), is a rare condition characterized by a thickened fibrous peritoneum encasing the abdominal organs and first described in human medicine (1), often presenting with ascites. In human medicine, peritoneal dialysis induced EPS is most common and abdominal surgery or foreign body can cause EPS (2). Idiopathic EPS is also reported in some cases in human medicine (2). ...
... In human medicine, peritoneal dialysis induced EPS is most common and abdominal surgery or foreign body can cause EPS (2). Idiopathic EPS is also reported in some cases in human medicine (2). In veterinary medicine, EPS is categorized as either primary (idiopathic) or secondary (3,4) as in human. ...
A 13-year-old neutered male Korean short-hair cat presented with anorexia, lethargy, and a severely distended abdomen, suggestive of ascites. Abdominocentesis yielded serosanguineous fluid. A subsequent diagnostic workup, including blood tests, ascitic fluid analysis, imaging studies [radiography, ultrasound, and computed tomography (CT)], and histopathological examination, was performed to identify the underlying cause. Imaging studies revealed characteristics of encapsulating peritoneal sclerosis (EPS) such as peritoneal thickening, fat stranding, and calcification. During laparotomy, fibrous membranes encapsulating the abdominal organs and ascites were observed, and multiple calcified regions were detected on the abdominal wall. Histopathological analysis confirmed the diagnosis of poorly differentiated invasive malignant neoplasms, which were further classified as carcinomatosis based on positive cytokeratin and negative vimentin immunohistochemistry results. To our knowledge, this is the first report of sclerosing peritoneal carcinomatosis with osseous metaplasia in a cat.
... While there is debate over the optimal management of patients with symptomatic, nonobstructive SEP, surgery remains the main line of management in patients presenting with bowel obstruction 1,4 . The surgical principles consist of exploratory laparotomy with complete excision of the peritoneal capsule and interloop adhesiolysis. ...
INTRODUCTION: Scelerosing encapsulating peritonitis (SEP), also known as abdominal cocoon syndrome, represents a rare cause of small bowel obstruction. CASE PRESENTATION: Herein we report an uncommon case of small bowel obstruction caused by SEP in a 30-year-old male with no prior surgical history who presented to the emergency department. Patient was diagnosed with SEP preoperatively using CT scan and underwent an explorative laparotomy with extensive adhesiolysis. Patient symptoms resolved postoperatively and was discharged in a good condition. DISCUSSION: Scelerosing encapsulating peritonitis has higher prevalence in males than in female, and higher incidences in tropical and subtropical countries. The exact mechanism of the disease in not well understood, but subclinical intra-abdominal inflammation is theorized to result in a thick fibrocollagenous membrane encapsulating intra-peritoneal organs that leads to intestinal obstructive disease. The disease is categorized into primary and secondary SEP depending on the etiological factor. It is also further divided into 3 types according to the extent of the peritoneal membrane encasement observed intra-operatively. Patients often present with recurrent history of small bowel obstruction in the absence of prior abdominal surgery. Computed tomography of the abdomen with experienced radiologist interpretation can aid in preoperative diagnosis. Despite several trials of medical management, surgical treatment remains the gold standard. CONCLUSION: Scelerosing encapsulating peritonitis, is a rare cause of small bowel obstruction.The exact pathogenesis is not well understood. The main line of treatment is surgical adhesiolysis and excision of the intra-abdominal fibrocollagenous membrane.
