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Primary Sclerosing Encapsulating Peritonitis: A Rare Case of Intestinal Obstruction
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Himangsu Sarma1, Hariharan Ramesh1, John M Manipadam1, Rasmi Unnikrishnan2 Departments of 1Surgical Gastroenterology and 2Pathology, Lakeshore Hospital and Research Centre, Kochi, Ernakulam-682040, Kerala, India. |
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Corresponding Author:
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Dr Himangsu Sarma Email: himangsu59@gmail.com |
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Received:
17-MAR-2025 |
Accepted:
02-JUN-2025 |
Published Online:
25-JUN-2025 |
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Abstract
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Background: Sclerosing Encapsulating Peritonitis (SEP), also known as abdominal cocoon, is a rare chronic inflammatory condition characterized by encasement of bowel loops within a thick fibro-collagenous membrane. It may be idiopathic or secondary to known risk factors such as peritoneal dialysis, infections, or abdominal surgeries. Due to its rarity and non-specific symptoms, SEP is frequently misdiagnosed, often recognized only intraoperatively. Case Report: We report a case of a 76-year-old male with no prior medical or surgical history, who presented in 2022 and again in 2023 with recurrent episodes of abdominal pain, vomiting, and constipation. Initial conservative management provided temporary relief, but symptoms recurred. Imaging suggested small bowel obstruction with clumped bowel loops. Exploratory laparotomy revealed a dense fibrous membrane encasing the intestines, consistent with SEP. Histopathology confirmed chronic inflammation without evidence of secondary causes. Postoperatively, the patient developed paralytic ileus, which was managed conservatively with nutritional support. He was discharged in stable condition and remained well at follow-up. Conclusion: This case highlights the importance of considering idiopathic SEP in the differential diagnosis of unexplained intestinal obstruction, especially in patients without identifiable risk factors. Early surgical intervention can be curative, though postoperative complications like ileus may prolong recovery. |
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Keywords :
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Abdominal Pain, Constipation, Laparotomy, Paralytic Ileus, Vomiting.
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Introduction
Sclerosing Encapsulating Peritonitis (SEP), also known as abdominal cocoon, is a rare condition characterized by the encasement of small bowel loops within a dense, fibrous peritoneal membrane. Although its exact pathogenesis remains unclear, several secondary causes have been proposed, including chronic peritoneal dialysis, prior abdominal surgeries, peritonitis, use of certain medications (notably beta-blockers), and intra-abdominal malignancy. However, in some cases, no identifiable etiology is found, and these are categorized as primary or idiopathic SEP. Given its rarity and non-specific presentation, SEP poses a diagnostic challenge and is often identified intraoperatively. We report a case of idiopathic SEP in an elderly male, managed successfully with surgical intervention, and discuss relevant literature.
Case Report
A 76-year-old male with no history of prior abdominal surgery, chronic illness, or long-term medication use presented in July 2022 with a four-day history of intermittent colicky abdominal pain, vomiting, and constipation. There were no associated symptoms of fever, jaundice, or rectal bleeding. On examination, he was hemodynamically stable with a distended, tender abdomen. Laboratory investigations were within normal limits, except for elevated C-reactive protein and serum creatinine levels. Initial management included intravenous fluids and empirical antibiotics. Contrast-enhanced computed tomography (CECT) of the abdomen revealed dilated, clumped jejunal loops (up to 4.5 cm) located in the midline, with normal-calibre ileum and large bowel, and no ascites or lymphadenopathy [Fig.1a]. The patient improved with conservative treatment and was discharged in stable condition. Six months later, in January 2023, he was readmitted with similar symptoms. This time, a soft, non-tender, mobile midline abdominal mass was palpable. Repeat CECT showed a thick, hyperdense membrane encasing clumped, dilated small bowel loops with surrounding mesenteric fat stranding, raising suspicion for abdominal tuberculosis [Fig.1b]. Due to persistent symptoms, exploratory laparotomy was performed. Intraoperatively, a thick fibrous membrane encasing both small and large bowel was noted, consistent with an abdominal cocoon [Fig.2]. Adhesiolysis and prophylactic appendectomy were performed. Histopathological examination revealed chronic inflammation with no granulomas; Ziehl-Neelsen stain and mycobacterial culture were negative for tuberculosis [Fig.3]. Postoperatively, the patient developed paralytic ileus with high nasogastric output and required total parenteral nutrition, followed by naso-jejunal feeding. He gradually improved and was discharged in a stable condition. At 18-month follow-up, he remained symptom-free except for occasional constipation, with no recurrence of intestinal obstruction.
Discussion
Sclerosing Encapsulating Peritonitis (SEP), also known as abdominal cocoon, is a rare chronic inflammatory disorder characterized by a fibrous membrane encasing bowel loops, leading to intestinal obstruction. First described by Foo et al. [ 1] and earlier termed "peritonitis chronica fibrosa incapsulata" by Owtschinnikow [ 2], SEP is more commonly reported in females from tropical and subtropical regions, though idiopathic cases have been noted in other demographics [ 3]. SEP is classified as primary (idiopathic) or secondary. While primary SEP has no identifiable cause, it is thought to involve cytokine-driven fibroblast activation and neo-angiogenesis [ 4]. Secondary SEP is associated with conditions such as peritoneal dialysis, tuberculosis, autoimmune disorders, malignancy, and prior abdominal interventions [ 3]. Hypoxia-induced processes, including epithelial–mesenchymal transition and activation of thrombin and protease-activated receptors, may contribute to membrane formation [ 4]. Clinical presentation can range from incidental findings to recurrent bowel obstruction. Laboratory tests are often unremarkable, while CECT is the most useful imaging modality. The “cocoon sign” clumped bowel loops encased by a thick membrane is considered characteristic [4]. Although elevated IgG4 levels and IL-6 have been proposed as diagnostic markers [5], such investigations were not feasible in our case. Definitive diagnosis is typically made intraoperatively, supported by histopathology revealing dense fibrosis with minimal inflammation [4]. Management depends on symptom severity. Mild cases may respond to nutritional support and immunomodulators [6], but surgery remains the standard for obstructive cases. Our patient underwent adhesiolysis and membrane excision, with postoperative ileus managed conservatively. The absence of recurrence over eighteen months suggests a favourable outcome.
Conclusion
Sclerosing Encapsulating Peritonitis is an uncommon and often underdiagnosed cause of intestinal obstruction. While imaging may suggest the diagnosis, confirmation is usually surgical and histological. Management should be individualized based on severity, with surgery being definitive in symptomatic cases. This case is notable for its idiopathic presentation in an elderly male and reinforces the importance of considering SEP in unexplained bowel obstruction.
Contributors: HS: manuscript writing, patient management; HR, JMM: manuscript editing, patient management; RU: pathological inputs into the manuscript. HS will act as a study guarantor. All authors approved the final version of this manuscript and are responsible for all aspects of this study. Funding: None; Competing interests: None stated.
References - Foo KT, Ng KC, Rauff A, Foong WC, Sinniah R. Unusual small intestinal obstruction in adolescent girls: The abdominal cocoon. Br J Surg. 1978;65:427-430.
- Owtschinnikow PJ. Peritonitis chronica fibrosa incapsulata. Arch für Klinische Chirurgie. 1907;83:623-634.
- Mishra S, Chaudhary K, Tyagi S, Mishra P, Misra V. Primary sclerosing encapsulating peritonitis. Indian J Pathol Microbiol. 2024;67(1):182-184.
- Pintar T, Tavcar M, Šušteršic A, Volavšek M. Primary sclerosing encapsulating peritonitis: a case report. J Med Case Rep. 2023;17(1):318.
- Osama MA, Roy S, Rawat S, Rao S. IgG4 related sclerosing encapsulating peritonitis with cocoon formation: an unusual and undescribed presentation. Indian J Pathol Microbiol. 2022;65(3):683-685.
- Sivakumar J, Brown G, Galea L, Choi J. An intraoperative diagnosis of sclerosing encapsulating peritonitis: a case report. J Surg Case Rep. 2020;2020(9):rjaa329.
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Article Statistics |
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Sarma H, Ramesh H, Manipadam JM, Unnikrishnan RPrimary Sclerosing Encapsulating Peritonitis: A Rare Case of Intestinal Obstruction.JCR 2025;15:47-49 |
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Sarma H, Ramesh H, Manipadam JM, Unnikrishnan RPrimary Sclerosing Encapsulating Peritonitis: A Rare Case of Intestinal Obstruction.JCR [serial online] 2025[cited 2025 Jul 26];15:47-49. Available from: http://www.casereports.in/articles/15/2/Primary-Sclerosing-Encapsulating-Peritonitis.html |
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