Acute presentation of cocoon abdomen as intestinal obstruction mimicking with strangulated eventration: A case report.

We report the case of an 87-year-old Moroccan female patient with a medical history of cholecystectomy at the age of 35, performed via a subcostal approach due to acute multilithiasic cholecystitis. At the age of 55, she underwent a right hemicolectomy with ileocolic anastomosis via a midline approach to address localized adenocarcinoma of the right colon. The postoperative course was uncomplicated, and subsequent follow-up revealed no signs of recurrence. Notably, the patient also presented with a right ectopic kidney. She presented to our emergency department with abdominal pain and obstructive syndrome that occurred 4 days before admission. She described paraumbilical pain, abdominal distention, nausea, multiple episodes of vomiting, and an inability to defecate or pass gas. On physical examination ( Table 1 ), the patient appeared cachectic, with a weight of 48.5 kg and a BMI of 18.9. She was conscious, hemodynamically stable, afebrile, with a distended abdomen. Two palpable, tender, and irreducible abdominal masses were noted—one midline below the umbilicus and the other in the right iliac fossa, indicative of a strangulated layered eventration. Rectal examination revealed an empty rectal ampoule. Other system examinations were normal, and laboratory values, including electrolytes, renal function tests, and hemoglobin, were within normal ranges. Table 1 Patient characteristics. Table 1 Variables Results Age, y 87 Gender Female BMI, kg/m 2 18.9 BP, mmHg 110/80 GCS 15/15 PR, beats/min 90 RR, cycle/min 16 SpO 2 , % in RA 98 Body temperature, C° 37.4 WBC, /μl 8700 Hb, g/dl 12.4 Creatinine, mg/l 6.1 Na, mEq/l 137 K, mEq/l 3.8 CRP, mg/l 40 X-Ray radiography Air-fluid levels Physical examination Acute abdomen Patient characteristics. Erect abdominal X-ray depicted multiple air-fluid levels ( Fig. 1 ). Subsequently, the patient was hospitalized, a nasogastric tube was inserted, and she was maintained NPO with IV fluids and analgesics. A CT scan was performed suggesting a strangulated ventral hernia with ischemic intestinal loops ( Fig. 2 ). Fig. 1 Erect abdominal X-Ray showing multiple air-fluids levels (arrows), consistent with the clinical picture of acute small bowel obstruction. Fig. 1 Fig. 2 Axial contrast material-enhanced CT images showing a thick peritoneal capsule encasing bowel loops (calipers) responsible for acute intestinal obstruction. Fig. 2 Erect abdominal X-Ray showing multiple air-fluids levels (arrows), consistent with the clinical picture of acute small bowel obstruction. Axial contrast material-enhanced CT images showing a thick peritoneal capsule encasing bowel loops (calipers) responsible for acute intestinal obstruction. The patient was promptly taken to the operating room. An iterative midline incision was made, and intraoperatively, extensive adhesions were found between the small bowel loops and carefully released in a limited region. A fibrocollagenous cocoon-like sac was identified adjacent to the right iliac fossa, partially incarcerating two hypoperfused but viable small bowel loops ( Fig. 3 ). The thick peritoneal membrane was excised, and the abdominal wall was reinforced with separate X-shaped sutures. Fig. 3 Intraoperative photography after meticulous adhesiolysis showing a fibrocollagenous cocoon-like sac (arrow). Fig. 3 Intraoperative photography after meticulous adhesiolysis showing a fibrocollagenous cocoon-like sac (arrow). Retrospective interpretation of the initial CT scan confirmed the presence of SEP's characteristic radiological signs. The postoperative period was unremarkable with good overall recovery. Sips of water were started on postoperative day (POD) 1, and gradually advanced to regular diet. She had a bowel movement on POD 2 and was discharged on POD 5. During interval outpatient clinic follow-up at day 15 and 2 months post discharge the patient appeared in good health with no recurrent symptoms.

S.B wrote the article and did the review and editing. O.B, A.M, M.Z revised the manuscript. E.E and H.A revised and supervised the manuscript.

The institution exempts the case report from ethical approval.

No funding.

Not applicable.

Soukayna Bourabaa MD.

Accurately diagnosing SEP prior to surgery presents considerable challenges, especially given the common presentation of nonspecific obstructive symptoms in patients. However, those with a history of predisposing factors and chronic symptoms should prompt clinicians to consider SEP in the differential diagnosis. One distinguishing hallmark of abdominal cocoon syndrome is the encapsulation of either the entire or a segment of the small bowel within a thick, glossy membrane. In cases of mild symptoms, a conservative management approach often yields positive outcomes. However, when symptoms become pronounced, surgical intervention is frequently warranted. Choosing a minimally invasive surgical strategy is advisable to reduce the risk of potential complications, such as anastomosis leaks and the onset of short bowel syndrome.

SEP is a rare condition, with approximately 200 reported cases in the English literature [ 10 ]. Despite its rarity, the exact pathogenesis remains elusive, theorized to be secondary to cytokine release and fibroblast activation during peritoneal inflammation, resulting in the formation of a thick fibrocollagenous peritoneal membrane [ 10 ]. 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 ]. Our case presents a unique feature, as the patient exhibited a right ectopic kidney ( Fig. 2 ), a finding not previously described in the existing literature. SEP has gained increasing attention, particularly in the context of peritoneal dialysis (PD) [ 18 ]. A multicenter study in Japan reported an overall incidence of SEP in PD patients as 2.5% [ 19 ]. Notably, this incidence rises with prolonged PD durations, reaching 1.9 %, 6.4 %, 10.8 %, and 19.4 % after two, five, six, and eight years, respectively [ 20 ]. The mortality rate varies from 56 % to 93 % despite various therapeutic modalities [ 21 ]. The pathophysiology of PD-related SEP is described by the “two-hit” hypothesis [ 22 ], where non-inflammatory peritoneal sclerosis accumulates in repeated PD (“first hit”), followed by a cascade of pro-inflammatory and pro-angiogenic cytokines (“second-hit”), leading to mesothelial cell transdifferentiation and the formation of inflammatory fibro-collagenous membranes [ 23 ]. Secondary causes of SEP encompass various rare etiologies such as abdominal tuberculosis, previous surgeries, sarcoidosis, gastrointestinal tumors, systemic lupus erythematosus (SLE), familial Mediterranean fever (FMF), fibrogenic foreign body, beta-blocker use, ventriculoperitoneal and peritoneovenous shunts, orthotopic liver transplantation, and recurrent peritonitis [ [24] , [25] ] ( Table 2 ). In our presented case, the patient's history of prior abdominal surgeries led to the diagnosis of secondary SEP. Table 2 Literature overview of causative factors for secondary sclerosing encapsulating peritonitis. Table 2 Causes Primary (idiopathic) Secondary  Related to peritoneal dialysis  Unrelated to peritoneal dialysis, including tubercular peritonitis 

 Associations Surgery or surgical shunts  Laparotomy for carcinoma or benign disorders  Abdominal lavage with povidone iodine  LeVeen peritoneovenous shunt  Ventriculoperitoneal shunt  Intraperitoneal perfusion chemotherapy (IPC) and hyperthermic intraperitoneal chemotherapy (HIPEC) Peritonitis  Bacterial peritonitis  Meconium peritonitis Malignancies  Gastric  Pancreatic  Midgut neuroendocrine  Renal  Ovarian  Lymphoma Diseases of the female reproductive tract  Luteinized thecoma of the ovary  Endometriosis  Adenomyosis, leiomyoma  Dermoid cyst rupture Bowel perforation Administration of β-blockers (Practolol) Cirrhosis Systemic rheumatologic and inflammatory disorders  Autoimmune disease (systemic lupus erythematosus)  Familial mediterranean fever  Peritoneal sarcoidosis After organ transplantation  Liver  Kidney  Intestine Acute appendicitis Radiation enteritis Literature overview of causative factors for secondary sclerosing encapsulating peritonitis. 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 ). Fig. 4 Types of abdominal cocoon syndrome 57; (A) Type I, (B) Type II, and (C) Type III. In Type I and Type II, the membrane (grey shading) encompasses a part and the entirety of the small intestine, respectively. Type III SEP entails a membrane (grey shading) that encloses the entire small bowel along with other organs, such as the ovaries and colon. Fig. 4 Types of abdominal cocoon syndrome 57; (A) Type I, (B) Type II, and (C) Type III. In Type I and Type II, the membrane (grey shading) encompasses a part and the entirety of the small intestine, respectively. Type III SEP entails a membrane (grey shading) that encloses the entire small bowel along with other organs, such as the ovaries and colon. 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. In the current report, the patient was diagnosed with type I SEP, demonstrating partial encapsulation of the small bowel. Clinically, SEP is a rare cause of acute or subacute intestinal obstruction, acknowledged in 81 % of reported cases of abdominal cocoon [ 27 ]. While a palpable bowel mass may manifest in 63 % of cases, the accurate preoperative diagnosis is often elusive [ 28 ]. Weight loss and ascites have also been documented in subset of patients. Saqib et al. [ 29 ] documented a relatively unusual emergency presentation of SEP marked by signs of focal peritonitis and sepsis. Our patient presented with classical symptoms of small bowel obstruction and palpable abdominal mass that was suspected to be a strangulated midline eventration. 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 ]. Computed Tomography (CT) scan is the imaging modality of choice [ [10] , [11] , [12] , [13] , [14] , [15] , [16] ], and allows for the identification of the thickened contrast material–enhanced abnormal peritoneal membrane and the encapsulated clumped bowel loops. Beyond this, CT scan can potentially help identify the cause of SEP, such as omental granuloma in tuberculosis, and detecting associated complications like bowel obstruction. Radiological features in our case were atypical, showcasing an enhancing membrane encapsulating small bowel loops. The differential diagnosis of SEP includes congenital peritoneal encapsulation, peritonitis carcinomatosa, pseudomyxoma peritonei, tuberculous peritonitis, and internal hernia [ 30 ]. Histopathological examination of biopsied peritoneal tissue may uncover fibroconnective tissue proliferation, inflammatory infiltration, and dilated lymphatic vessels [ 10 ]. Although these features are not pathognomonic for SEP, they contribute to the diagnosis when combined with operative findings. Importantly, characteristic features such as foreign body granulomas, giant cells, and foreign material are notably absent, facilitating the differentiation of SEP from other conditions, such as tuberculosis [ [13] , [14] , [15] , [16] , [17] , [18] , [18] , [19] ]. The small intestine is primarily affected, with the length of involvement varying. Prudent follow-up suffices for asymptomatic cases with idiopathic SEP. Conservative management emerges as the most suitable therapy in mildly symptomatic cases. Such cases are best managed by intestinal rest, nasogastric decompression, and nutritional support (enteral or parenteral). Patients with no regression of symptoms may be treated with anti-inflammatory or antifibrogenic like tamoxifen, steroids, colchicine, azathioprine, mycophenolate mofetil (MMF) and mammalian target of rapamycin (mTOR). In cases marked by poor oral intake or malnutrition, total parenteral nutrition might become necessary. However, it's important to note that these treatment protocols have mainly been utilized in cases of secondary SEP, and there are no sufficient data to justify their use in idiopathic SEP. The debate over surgical therapy persists for markedly symptomatic cases or those with a confirmed SEP diagnosis during laparotomy. Various options such as excision of thickened peritoneum and adhesiolysis, resection and anastomosis in the presence of gangrenous or perforated bowel, and protective enterostomy, can be used alone or in combination based on the patient's condition. Caution is advised against intestinal resection due to potential complications such as anastomosis leaks and short bowel syndrome, given that resection is usually unnecessary and can increase morbidity and mortality. The most common postoperative complications include early postoperative small bowel obstruction (EPSBO), wound site infections, intra-abdominal collections, enterocutaneous fistulas, short-bowel syndrome, and bowel perforation [ [10] , [11] , [12] , [13] , [13] , [14] , [15] , [16] , [17] , [18] , [19] , [20] , [21] , [22] , [23] , [24] , [25] , [26] , [27] , [28] , [29] , [30] , [31] ]. These complications can be attributed to several factors, including acute presentation, delayed intraoperative diagnosis, extensive adhesiolysis, and bowel manipulation [ [16] , [17] , [18] , [19] , [20] , [21] , [22] , [23] , [24] , [25] , [26] , [27] , [28] , [29] , [30] , [31] , [32] ]. EPSBO can be managed conservatively with bowel rest, total parenteral nutrition (TPN), and somatostatin combined with a low dose of steroids. Some authors have proposed prophylactic appendectomy to avoid potential challenges with future appendectomy [ 33 ]. While diagnostic laparoscopy is generally confirmatory and rules out other causes [ 34 , 35 ], it is not deemed beneficial in managing this condition, and carries an increased risk of iatrogenic bowel injury during trocar insertion [ 10 ]. Machado [ 16 ] reported the chance of recurrence in patients who have failed complete or partial excision of the sac.

SEP, initially documented by Owtschinnikow [ 1 ] in 1907 as “ peritonitis chronica fibrosa incapsulata ”, saw its eartly nomenclature evolve. In 1921, Winnen [ 2 ] coined the term “ Zuckergussdarm ”, or “ icing gut ”, describing the condition's characteristic white appearance due to the covering membrane. Over time, SEP has been referred to by various names such as “ peritoneal fibrosis ”, “ peritoneal sclerosis ”, “ sclerotic thickening of the peritoneal membrane ” [ 3 ], “ sclerosing peritonitis ” [ 4 ], “ sclerosing obstructive peritonitis ” [ 5 ], “ encapsulating peritonitis ” [ 6 ], “ chronic encapsulating fibrous peritonitis ” [ 7 ], and “ calcific peritonitis ” [ 8 ]. In 1978, Foo et al. [ 9 ] used the term “ abdominal cocoon ” to describe a primary or idiopathic form observed in adolescent girls from tropical or subtropical countries, such as China, India, Turkey, Nigeria, Malaysia, Singapore, Pakistan, Kinya, and Saudi Arabia [ 10 ]. They associated it with fallopian tube inflammation or irregular menstruation presumably due to chemical peritonitis induced by retrograde infection [ 11 ]. However, the term has become synonymous with “ sclerosing encapsulating peritonitis ” [ 12 ], recognized globally and occurring secondary to diverse causes. The etiology of SEP is multifactorial, with long-term peritoneal dialysis and bacterial peritoneal infections, especially tuberculosis, among the prime predisposing factors. Patients typically present with acute, subacute, or chronic intestinal obstruction attacks [ 13 ]. The preoperative diagnosis is quite challenging, often requiring intraoperative confirmation [ 13 ], although advances in radiological imaging allow some preoperative diagnoses [ 14 ]. Asymptomatic idiopathic SEP often warrants conservative management, while symptomatic cases may require various surgical interventions. This report outlines an exceptional case involving total small bowel obstruction attributed to SEP. Initially, the suspicion arose from a midline strangulated eventration during preoperative assessment, representing the first documented case within our university hospital to the best of our knowledge. This case report was written in line with the SCARE guidelines [ 15 ].

The authors declare that they have no competing interests.