Surgical management of sclerosing encapsulating peritonitis (SEP) secondary to tuberculosis: A case report and review of the literature.

Written informed consent was obtained from the patient for publication and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

A 20-year-old patient with no specific past medical history presented with colicky abdominal pain, distention, bilious vomiting, stool and gas cessation of three days' duration, with a history of similar episodes over the previous three months that had resolved spontaneously. He had no history of constitutional symptoms and no significant past surgical or medical history. His vital signs were within normal limits. Abdominal examination revealed abdominal distension and a tender central abdominal mass. However, the abdomen was soft and there were no clinical signs of peritonitis. Rectal examination was unremarkable. The white blood cell count was 22,000/μL with a predominance of neutrophils (85.7 %), thrombocytosis was 494,000, C-reactive was 122, haemoglobin, protein, serum electrolytes, urea, creatinine and liver function tests were within normal limits. A CT scan of the abdomen showed a thick dilated sac-like membrane containing a cluster of small intestinal loops giving the appearance of a cocoon, multiple deep collections at the root of the mesentery and an intraperitoneal effusion. Intraperitoneal effusion ( Fig. 1 , Fig. 2 ). Fig. 1 CT image showing abdominal cocoon in the anterior part of abdomen adherent to the anterior abdominal wall circumferential thickening of the intestinal wall (arrow), as well as several submucosal nodules (arrowheads). Fig. 1 Fig. 2 CT image showing small intestinal loops are encased in a sac of thick peritoneal membrane circumferential thickening of the intestinal wall (arrow), as well as several submucosal nodules (arrowheads). Fig. 2 CT image showing abdominal cocoon in the anterior part of abdomen adherent to the anterior abdominal wall circumferential thickening of the intestinal wall (arrow), as well as several submucosal nodules (arrowheads). CT image showing small intestinal loops are encased in a sac of thick peritoneal membrane circumferential thickening of the intestinal wall (arrow), as well as several submucosal nodules (arrowheads). The diagnosis and management were discussed with the patient. An exploratory midline laparotomy revealed the presence of generalized peritoneal nodules, multiple peritoneal adhesions, moderate ascites, a thick membrane containing the small intestine and no evidence of abscess or fluid collection ( Fig. 3 , Fig. 4 ). Fig. 3 intraoperative image showing the encapsulated small bowel . Fig. 3 Fig. 4 intraoperative image showing generalized peritoneal nodules (Blue arrowed), multiple peritoneal adhesions. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.) Fig. 4 intraoperative image showing the encapsulated small bowel . intraoperative image showing generalized peritoneal nodules (Blue arrowed), multiple peritoneal adhesions. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.) Adhesiolysis was performed and the sac was excised from the small bowel to the root of the mesentery with biopsy of a peritoneal nodule. Mycobacterial culture (acid-alcohol culture) showed no growth after eight weeks. Histopathology showed a necrotizing granulomatous inflammation in the peritoneal tissue composed of many granulomas. The upper granuloma contains a central necrotic zone surrounded by epithelioid histiocytes with a few multinucleated giant cells. No malignant cells were found ( Fig. 5 ). Fig. 5 Microphotography showing necrotizing granulomatous inflammation in biopsied peritoneal tissue, composed of many granulomas (Red arrows). The upper granuloma contains a central necrotic zone surrounded by epithelioid histiocytes with few multinucleated giant cells. (HE, 200×). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.) Fig. 5 Microphotography showing necrotizing granulomatous inflammation in biopsied peritoneal tissue, composed of many granulomas (Red arrows). The upper granuloma contains a central necrotic zone surrounded by epithelioid histiocytes with few multinucleated giant cells. (HE, 200×). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.) The patient tolerated the operation and was discharged in good condition 7 days after the operation, then referred to the Centre for Diagnosis and Treatment of Tuberculosis (CDTMR), where he was given antibacterial treatment for 6 months. At the 1-year follow-up, the patient is doing well, with no active complaints or relapses.

Pr Madani Ayoub, Pr Ouzni mohammed: Data collection, Data analysis and interpretation, article writing. Pr Soufi Mehdi: the leader surgeon of the case. Pr Achraf Mery, Pr Abbaoui Sanae: Interpretation of histological data.

Not required for this case report.

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Dr. Madani Ayoub.

Encapsulating peritoneal sclerosis is a rare but devastating disease associated with high morbidity and mortality. A high index of suspicion is warranted in patients with unexplained recurrent symptoms of bowel obstruction. A careful history should be taken to identify patients with known risk factors. The diagnosis is clinical and can be confirmed by radiography or laparotomy. Treatment should be directed to the underlying disease. Surgical enterolysis should be considered in patients who have failed conservative medical therapy. In principle, abdominal tuberculosis should be treated medically and surgery should only be used when indicated, usually as a last resort to establish a diagnosis or to treat complications. Globally, 20–40 % of cases of abdominal TB present acutely and require emergency surgery. Indications for surgery include intestinal obstruction, perforation and bleeding [ 32 ].

Sclerosing encapsulating peritonitis (SEP) is a chronic inflammatory disease that results from recurrent low-grade or subclinical peritonitis without specific abdominal signs; it eventually progresses to sclerosis and membrane formation with subsequent cocooning [ [1] , [2] , [3] ]. The disease is characterised by a thick, grey-white fibrous membrane surrounding the contents of the abdomen, mainly the small intestine [ [1] , [2] , [3] ]. Primary SEP, also known as abdominal cocon, has no obvious associated conditions; however, SEP may be secondary to conditions that cause peritoneal inflammation and fibroblastic proliferation [ 4 ]. SEP is thought to occur when a peritoneal inflammatory process (inciting factor) occurs in patients with a predisposing condition. In the peritoneal dialysis (PD) literature, the predisposing condition is non-inflammatory peritoneal sclerosis resulting from repeated dialysis sessions. In support of this, the cumulative incidence of SEP increases dramatically with time on PD [ 4 ], triggers a cascade of proinflammatory [transforming growth factor β1 (TGFβ1), interleukin-6 (IL-6), CCN2] and proangiogenic [vascular endothelial growth factor (VEGF)] cytokines [ 6 ]. TGFβ1 promotes transdifferentiation of peritoneal mesothelial cells into mesenchymal cells, leading to mesothelial cell depletion, increased production of extracellular matrix components [collagen type 1, alpha 1] and fibrogenesis, resulting in a fibrocollagenous cocoon [ 7 ]. SEP can be classified as primary (idiopathic) or secondary, where a trigger for the inflammatory process can be identified. In secondary SEP, a local or systemic factor can be identified as the trigger for peritoneal inflammation. Medications have been implicated as triggers: Methotrexate, antiepileptic drugs. Infection: Tuberculosis, non-tuberculosis, mycobacteria, bacterial peritonitis, cytomegalovirus, fungi, parasites, mechanical or chemical intraperitoneal irritants: Ventriculoperitoneal shunt Peritoneovenous shunt, Intraperitoneal iodine, Abdominal trauma, Intra-abdominal surgery, Foreign body, Talcum powder, Asbestos, Silica, Cirrhosis, Organ transplant, Endometriosis, Gynaecological neoplasms, Dermoid cyst rupture, Systemic rheumatological and inflammatory disorders: Sarcoidosis, Systemic Lupus Erythematosus and [ 8 ]. There are no symptoms in the early stages because the capsule is thin, but symptoms of bowel obstruction develop as the capsule thickens and shortens over time, narrowing the intestinal tract. In many cases, symptoms of bowel obstruction develop slowly rather than abruptly as the entire small bowel becomes narrowed [ 9 ]. The presenting signs and symptoms of SEP are usually vague and non-localising. The patient usually presents with vomiting, abdominal pain or distension and other features of subacute bowel obstruction. Patients usually have had previous episodes with similar symptoms that resolved spontaneously. Malnutrition may occur due to recurrent episodes. Clinically, the abdomen is soft to palpation. A soft, non-tender mass may be palpable in the central part of the abdomen, which actually represents the clumped bowel loops [ 10 ]. CT is currently the best studied and most commonly used imaging modality for the diagnosis of SEP. The coves of the small bowel are often joined by a thickened, enveloping peritoneum, typically associated with dilatation of the proximal bowel [ 11 ]. Other radiographic features include localised ascites, increased mesenteric fat density and localised or diffuse peritoneal calcifications [ 11 ]. Although the presence of complex foci may be due to intra-abdominal haemorrhage, they should raise suspicion of perforation or sepsis, particularly if they contain gas [ 12 ]. Magnetic resonance imaging is less commonly used for diagnosis, but the results are likely to be similar. The advantages are the absence of ionising radiation and better delineation of the bowel wall and peritoneal thickening [ 13 ]. There is no evidence-based consensus on the optimal therapeutic approach for patients with idiopathic SEP [ 14 ]. Conservative treatment for as long as possible is the best approach for patients with mild abdominal symptoms. In these patients, bowel rest, nasogastric decompression and nutritional support (enteral or parenteral) are the most appropriate therapeutic options [ 15 ]. Malnutrition and weight loss are the most common symptoms in patients with SEP following recurrent episodes of bowel obstruction [ 16 ]. Patients whose symptoms are refractory to conservative treatment may be treated with drugs such as tamoxifen, steroids, colchicine, azathioprine and mycophenolate mofetil [ 17 ]. Corticosteroids are thought to inhibit collagen synthesis and maturation by suppressing the inflammatory process within the peritoneal membrane. They also completely eliminate the thickened membrane [ 18 ]. Tamoxifen is a selective estrogen receptor modulator that inhibits the production of growth factors by fibroblasts. It is therefore often used to treat certain fibrosclerotic conditions, such as retroperitoneal fibrosis. Colchicine inhibits the expression of growth factors and therefore has an anti-inflammatory effect [ 18 ]. In contrast to asymptomatic or mildly symptomatic patients, patients with severe signs of bowel obstruction or an intraoperative diagnosis of SEP have several surgical options. Partial membrane excision and adhesiolysis; resection and anastomosis; resection and protective enterostomy; exploratory laparotomy may be used alone or in combination depending on patient factors [ 19 , 20 ]. The most appropriate procedure is to detach the membrane from the intestinal surface and excise the dense adhesions between the intestinal loops [ 21 ]. Membrane excision and adhesiolysis should be performed on all affected bowel segments if there are no other contraindications to this procedure. The risk of recurrence is quite low if the membrane can be completely removed from the bowel surface [ 19 ]. Inserting an anti-adhesive substance between the bowel loops before closing the abdomen may prevent the development of postoperative adhesive obstruction of the small bowel [ 22 ]. To avoid complications such as anastomotic leakage and short bowel syndrome in SEP patients, bowel resection is only indicated when necrosis has developed [ 20 ]. Resection is generally unnecessary and may increase patient morbidity and mortality if performed without a clear indication [ 23 ]. Kawanishi et al. reported a mortality rate of 4 % in patients with PD-related secondary SEP who underwent adhesiolysis alone, compared with 82 % in those who underwent enterectomy and anastomosis in a separate study [ 24 ]. Laparoscopy is not part of the standard surgical approach in patients with SEP. A limited number of case reports have described successful laparoscopic membrane excision and adhesiolysis [ 25 ]. The advantage of laparoscopy is that it can be used for diagnostic and therapeutic purposes in patients whose diagnosis is unclear after appropriate investigations. Cocoons secondary to tuberculosis can occur at any age or sex and usually present with complete small bowel obstruction that does not respond to conservative treatment [ 26 ]. During surgery, in addition to the covering membrane, there are dense inter-intestinal adhesions that must be released to relieve the obstruction, and the risk of iatrogenic complications is therefore high. Other manifestations of abdominal tuberculosis, such as mesenteric abscesses, enlarged and caseous mesenteric lymph nodes, and nodules on the intestinal serosa, are also common and may suggest a tuberculous aetiology [ 27 ]. Histological examination of the membranous tissue shows proliferation of fibrous connective tissue with a chronic non-specific inflammatory response. Once the diagnosis of tuberculosis is established, these patients should receive standard anti-tuberculosis treatment [ 28 ]. Undiagnosed and untreated tuberculous peritonitis can result in a mortality rate of 50–60 % [ 29 ], but the disease is usually curable if properly treated. Anti-tuberculosis therapy (ATT) without surgery can lead to recovery in most patients with tuberculous abdominal cocooning [ 30 ], and surgery is usually unnecessary if a definitive diagnosis can be made preoperatively. In a previous report of tuberculous bowel obstruction associated with strictures, clinical improvement occurred in 91 % of patients treated with ATT, while complete radiological resolution was noted in 70 % of patients, suggesting a role for ATT in the management of tuberculous strictures [ 31 ].

Sclerosing encapsulating peritonitis (SEP), commonly known as abdominal cocoon syndrome (ACS), is considered one of the rare causes of intestinal obstruction, (SEP) is a chronic inflammatory disease that results from recurrent low-grade or subclinical peritonitis without specific abdominal signs; it eventually progresses to sclerosis and membrane formation with subsequent cocooning, SEP may be primary or secondary to conditions causing inflammation of the peritoneum and fibroblastic proliferation [ 1 ]. We present the case of a 20-year-old male patient admitted with recurrent episodes of abdominal pain and small bowel obstruction due to SEP. In this article we present the clinical manifestations, evaluation, management and prognosis of this type of disease. This work was reported according to the SCARE 2023 criteria [ 2 ].

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