Sclerosing encapsulating peritonitis: Report of a case and review of literature

Hussam Al Trabulsi; Tala Muassess; Salman Yousuf Guraya

doi:10.4103/abhs.abhs_59_22

CASE REPORT

Year : 2023 | Volume : 2 | Issue : 1 | Page : 45-48

Sclerosing encapsulating peritonitis: Report of a case and review of literature

Hussam Al Trabulsi¹, Tala Muassess¹, Salman Yousuf Guraya²
¹ Department of Surgery, Medcare Hospital, Dubai, United Arab Emirates
² Clinical Sciences Department, College of Medicine, University of Sharjah, Sharjah, United Arab Emirates

Date of Submission	27-Oct-2022
Date of Decision	08-Nov-2022
Date of Acceptance	12-Nov-2022
Date of Web Publication	05-Dec-2022

Correspondence Address:
Dr. Hussam Al Trabulsi
Department of Surgery, Medcare Hospital, Dubai
United Arab Emirates

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/abhs.abhs_59_22

Abstract

Sclerosing encapsulating peritonitis (SEP) is a rare clinical condition that occurs secondary to a triggering factor such as peritoneal dialysis. The condition entails the encasement of a part of or entire small bowel in a fibro-collagenous membrane, causing subacute, acute, or chronic bowel obstruction. This case report presents a middle-aged man with recurring episodes of vague abdominal pain. The clinical examination and investigations showed small-bowel obstruction mostly focused around the previous area of mesh placement for umbilical hernia repair. The abdominal exploration showed a fibrous sac encasing the small-bowel loops, which necessitated adhesiolysis. Most of the fibrous encapsulating membrane was removed. The patient made an uneventful recovery. The treating surgeons for patients with vague manifestations of small obstruction should have a high index suspicion for rare causes of abdominal pain such as SEP.

Keywords: Abdominal pain, peritoneal dialysis, sclerosing encapsulating peritonitis, small-bowel obstruction, surgical exploration

How to cite this article:
Al Trabulsi H, Muassess T, Guraya SY. Sclerosing encapsulating peritonitis: Report of a case and review of literature. Adv Biomed Health Sci 2023;2:45-8

How to cite this URL:
Al Trabulsi H, Muassess T, Guraya SY. Sclerosing encapsulating peritonitis: Report of a case and review of literature. Adv Biomed Health Sci [serial online] 2023 [cited 2023 Feb 6];2:45-8. Available from: http://www.abhsjournal.net/text.asp?2023/2/1/45/362697

Background

Chronic abdominal pain remains a diagnostic challenge for physicians and other health-care professionals.[1] Occasionally, a definitive diagnosis is not established even after extensive evaluation by physical evaluation, laboratory tests, and multimodal imaging. The causes of chronic abdominal pain are far ranging; The causes of chronic abdominal pain are far ranging; intestinal and colonic angina, celiac disease, endometriosis, functional dyspepsia, gallstones, and gastritis. [2,3] A careful history focused physical examination, and laboratory and imaging tools essentially aid the diagnostic accuracy of physicians. However, there remain a substantial number of cases where a concrete diagnosis and management plan are not well-defined, and an exploratory laparotomy is offered to establish the diagnosis.

One of the rare causes of chronic abdominal pain is sclerosing encapsulating peritonitis (SEP), which primarily manifests with recurring episodes of partial intestinal obstruction due to the kinking and compression of small intestines within the encasing membrane.[4] The patient's history helps raise clinical suspicion and guide the diagnostic process, including the choice of imaging and steps of management.[3] SEP, also known as abdominal cocoon, is a rare chronic inflammatory condition of the peritoneum with an unknown etiology.[5] Patients with SPE mostly present with multiple events of small-bowel obstruction and can be idiopathic or secondary. Some of the known predisposing factors of SPE include peritoneal dialysis, abdominal tuberculosis, and COVID-19. [6,7]

Due to its nonspecific clinical features and scarcity, SEP is often misdiagnosed or missed altogether. This case report presents a middle-aged man with nonclassical manifestations of SEP where the final diagnosis was established during the abdominal exploration.

Case Report

A 44-year-old male presented to the Emergency Department of Medcare Hospital, Dubai, United Arab Emirates, with recurrent episodes of abdominal pain over a period of 1 month. The abdominal pain was associated with constipation and hard stools for 1 week. On examination, a hard mass was felt in the middle of the abdomen. The patient's past surgical history included a laparoscopic umbilical hernia with mesh placement. The mesh had an uncoated medium-weight monofilament polypropylene on the anterior side with an absorbable hydrogel barrier on its posterior side. An ultrasound of the abdomen showed a diffuse gaseous distension of the small bowel. Contrast-enhanced computed tomography (CT) scan of the abdomen showed dilated proximal small-bowel loops, mostly clustered together in the mid-abdomen [Figure 1]. Several dilated and walled-off small-bowel loops in the left hemiabdomen were also noted.

Figure 1: Contrast-enhanced CT scan of the abdomen with a huge central abdominal mass (arrows) of loops of the small bowel encased by a well-defined membrane. CT: Computed tomography.

Click here to view

The patient was admitted in the surgical unit, and a conservative management was ensued. This approach did not improve the patient's condition. Finally, a diagnostic laparoscopy was performed, which showed distended bowel loops, a membrane encapsulating multiple bowel loops, and fluid in the pelvis. Thereupon, a decision was taken to convert to an exploratory laparotomy through a midline incision. The operative findings included a dense fibrous sac encasing small-bowel loops [Figure 2]. Multiple longitudinal and transverse incisions were performed on the capsule to release the bowel loops. Adhesiolysis was then performed, as the membrane was closely attached to the small-bowel wall. Eventually, most of the encapsulating membrane was removed [Figure 3]. The histopathological examination of the encapsulating sac showed a fibrocollagenous tissue wall exhibiting chronic inflammation comprising lymphocytes. The plasma cells with proliferating capillaries and stromal hyalinization were also noted. The patient made a smooth and uneventful recovery, and he was doing well on his follow-up.

Figure 2: An operative view with a dense fibrous sac encasing small-bowel loops.

Click here to view

Figure 3: The encapsulating membrane of the sclerosing encapsulating peritonitis mass after opening the encasement membrane.

Click here to view

Discussion

SEP entails the encasement of bowel loops within the peritoneal cavity by an inflammatory membrane. Primary SEP is a rare idiopathic condition, whereas secondary SEP is more common as it is associated with several causes, including peritoneal dialysis, abdominal tuberculosis, autoimmune disease like familial Mediterranean fever, and drug use such as beta-blockers.[8] Primary SEP is classified according to the extent of encasement by the fibrocollagenous membrane, with Type I involving the encasement of a part of the small intestines, and Type II the complete intestine, whereas the membrane encases the appendix, cecum, ascending colon, stomach, liver, and ovaries in addition to the complete small intestine in Type III.[9] On the other hand, secondary SEP is classified into either systematically induced or locally induced disease. Certain drugs are commonly found to be the cause of secondary SEP, including beta-adrenergic blocking agents. Other systematic triggers of secondary SEP are protein S deficiency, and autoimmune diseases like systemic lupus erythematosus, and infection like abdominal tuberculosis, whereas locally induced SEP can be due to peritoneal dialysis, abdominal trauma, liver transplantation, and ventriculoperitoneal shunt. [10,11]

Most patients with SEP remain asymptomatic, but when symptomatic, the clinical course presents with intermittent and partial intestinal obstruction,[12] presenting with nonspecific symptoms such as fever, loss of appetite, nausea, crampy abdominal pain, and altered bowel habits. However, as the disease progresses, patients present with a more obvious bowel obstruction, with occasional tell-tale signs of a painless soft abdominal mass and ascites. A high clinical index of suspicion is crucial in the diagnosis of SEP. The diagnostic modalities include abdominal X-ray, ultrasonography, and CT scan which invariably demonstrate dilated bowel loops, air-fluid levels, and peritoneal fluid.[13] However, a contrast-enhanced CT is the most reliable imaging test for the diagnosis of SEP, which shows a characteristic finding of small-bowel segment conglomerated in the midline, surrounded by a thick, contrast-free capsule. CT scan may also show intestinal obstruction, ascites, lymphadenopathy, mesenteric thickening, or peritoneal calcifications.

The management of SEP depends on the severity of symptoms.[6] Patients with mild abdominal symptoms should be managed conservatively; Patients with mild abdominal symptoms should be managed conservatively, which entails bowel rest, gastric decompression and parenteral nutrition. As the patients may have multiple episodes of incomplete obstruction, with resultant appetite loss, malnutrition, and weight loss, it is vitally important to establish nutritional support. This has been shown to be a statistically independent factor for preventing postoperative complications.[14] The candidates for surgical management include those presenting with bowel obstruction or not responding to conservative treatment. The approach is usually through a midline laparotomy since laparoscopy carries a significant risk of bowel injury during trocar insertion and membrane separation and resection. The surgical management consists of excising the membrane completely, which may require multiple transverse and longitudinal incisions to allow the stripping of the membrane and return of underlying intestines to normal length. A similar surgical approach was followed in our care as well. Theoretically, a thorough cleaning and removal of foreign bodies from the peritoneal cavity can potentially reduce the risk of SEP. However, the occurrence of SEP remains a scientific mystery.

Finally, the characteristic histopathological features of the encapsulating membrane include fibroconnective tissue proliferation, inflammatory infiltration, and dilated lymph vessels. In our case, the histopathological examination showed fibrocollagenous tissue with features of chronic inflammation comprising lymphocytes and plasma cells, with proliferating capillaries and stromal hyalinization.

Conclusion

Our case report presents an adult male with SEP, caused by an inflammatory reaction to a monofilament polypropylene mesh with an absorbable hydrogel barrier placed during a previous laparoscopic umbilical hernia repair. A high index of clinical suspicion, combined with the appropriate use of imaging modalities, is necessary to reach the diagnosis of SEP.

Authors' contributions

All authors equally and substantially contributed to the conceptualization, data collection, write-up, and the final execution of this case report. All authors take responsibility for the academic integrity of this research.

Ethical statement

An ethical approval was obtained from the hospital administration for the publication of this case report.

Data availability statement

The data can be furnished, if requested.

Declaration of patient consent

Informed consent was obtained from the patient for the publication of this case report.

Conflict of interests

SG is an editor of the Advances in Biomedical and Health Sciences Journal. No conflict of interests declared.

References

1.	McGarrity TJ, Peters DJ, Thompson C, McGarrity SJ. Outcome of patients with chronic abdominal pain referred to chronic pain clinic. Am J Gastroenterol 2000;95:1812-6.
2.	Zhao J, Samaan JS, Toubat O, Samakar K. Laparoscopy as a diagnostic and therapeutic modality for chronic abdominal pain of unknown etiology: A literature review. J Surg Res 2020;252:222-30.
3.	Mol FM, Jansen CH, Dijk WV, Eerten PV, Scheltinga MR, Roumen RM. Factors predicting outcome after anterior neurectomy in patients with chronic abdominal pain due to Anterior Cutaneous Nerve Entrapment Syndrome (ACNES). Surgery 2019;165:417-22.
4.	Machado NO. Sclerosing encapsulating peritonitis: Review. Sultan Qaboos Univ Med J 2016;16:e142-51.
5.	George C, Al-Zwae K, Nair S, Cast JE. Computed tomography appearances of sclerosing encapsulating peritonitis. Clin Radiol 2007;62:732-7.
6.	Liberale G, Sugarbaker PH. Sclerosing encapsulating peritonitis as a potential complication of cytoreductive surgery and HIPEC: Clinical features and results of treatment in 4 patients. Surg Oncol 2018;27:657-62.
7.	Mofti AH, Ghabashi FA, Sadagah MM, Ibrahim MA, Altowairqi AM, Zabidi AH, et al. Sclerosing encapsulating peritonitis following recovery from COVID-19 Pneumonia. Cureus 2021;13:e19306.
8.	Yusuf MH. Sclerosing encapsulating peritonitis: A rare cause of intestinal obstruction. Cureus 2021;13:e15291.
9.	Mekann Bouv-Hez M, Charissoux A, Bouvier S, Mathonnet M, Christou N. From abdominal pain to a diagnosis of primary sclerosing encapsulating peritonitis and its management, a case report. Acta Chir Belg 2022;122:420-3.
10.	Hajjar R, Debroux É, Richard C, Plasse M, Loungnarath R. Sclerosing encapsulating peritonitis presenting as acute-on-chronic small-bowel obstruction in a patient with history of peritoneal carcinomatosis. J Surg Case Rep 2018;2018:rjy082.
11.	Park JI, Jung BH, Chung YK. Sclerosing encapsulating peritonitis after living donor liver transplantation. HPB 2022;24:S563.
12.	Zhang Z, Zhang M, Li L. Sclerosing encapsulating peritonitis: Three case reports and review of the literature. J Int Med Res 2020;48:1-6.
13.	López Grove R, Heredia Martínez A, Aineseder M, de Paula JA, Ocantos JA. Sclerosing encapsulating peritonitis: Imaging findings in an uncommon entity. Radiologia (Engl Ed) 2019;61:388-95.
14.	Kumar A, Ramakrishnan TS, Sahu S, Mishra KB. Idiopathic sclerosing encapsulating peritonitis – Is a preoperative diagnosis possible? Report of three cases. Surg Today 2009;39:610-4.