Accurate definition and management of idiopathic sclerosing encapsulating peritonitis

Abstract

Aim: To review the literature on idiopathic sclerosing encapsulating peritonitis (SEP), also known as abdominal cocoon syndrome.

Methods: The PubMed, MEDLINE, Google Scholar, and Google databases were searched using specific key words to identify articles related to idiopathic SEP. These key words were "sclerosing encapsulating peritonitis," "idiopathic sclerosing encapsulating peritonitis," "abdominal cocoon," and "abdominal cocoon syndrome." The search included letters to the editor, case reports, review articles, original articles, and meeting presentations published in the English-language literature from January 2000 to May 2014. Articles or abstracts containing adequate information about age, sex, symptom duration, initial diagnosis, radiological tools, and surgical approaches were included in the study. Papers with missing or inadequate data were excluded.

Results: The literature search yielded 73 articles on idiopathic (primary) SEP published in 23 countries. The four countries that published the greatest number of articles were India (n = 21), Turkey (n = 14), China (n = 8) and Nigeria (n = 3). The four countries that reported the greatest number of cases were China (n = 104; 53.88%), India (n = 35; 18.13%), Turkey (n = 17; 8.80%) and Nigeria (n = 5; 2.59%). The present study included 193 patients. Data on age could be obtained for 184 patients (range: 7-87 years; mean ± SD, 34.7 ± 19.2 years), but were unavailable for nine patients. Of the 184 patients, 122 were male and 62 were female; sex data could not be accessed in the remaining nine patients. Of the 149 patients whose preoperative diagnosis information could be obtained, 65 (43.6%) underwent operations for abdominal cocoon, while the majority of the remaining patients underwent operations for a presumed diagnosis of intestinal obstruction and/or abdominal mass. Management information could be retrieved for 115 patients. Of these, 68 underwent excision + adhesiolysis (one laparoscopic); 24 underwent prophylactic appendectomy in addition to excision + adhesiolysis. Twenty patients underwent various resection and repair techniques along with excision + adhesiolysis. The remaining three patients were managed with antituberculosis therapy (n = 2) and immunosuppressive therapy (n = 1).

Conclusion: Idiopathic SEP is a rare disorder characterized by frequently recurring bouts of intestinal obstruction. Surgical therapy is the gold standard management strategy.

Keywords: Abdominal cocoon syndrome; Idiopathic; Intestinal obstruction; Primary; Sclerosisis encapsulation peritonitis.

Figure 1

Bowel encased in a membranous sac suggestive of peritoneal encapsulation. A: The overall appearance of the membranous sac is shown. All intestines are localized behind the accessory peritoneal membrane; B: The appearance of the opened membranous sac is shown.

Figure 2

Intraoperative photograph showing the encapsulated small bowel (dense, cocoon-like fibrous membrane).

Figure 3

Small bowel transit. Procubitus image with localized compression. Liquid distension of the gastroduodenum (asterisks) and adhesion of the small intestinal loops (arrows) are persistent despite localized compression, producing a “cauliflower” appearance[24]; B: Upper gastrointestinal images reveal dilatation of the duodenum and jejunal loops, delayed bowel transit, and failure of the oral contrast to pass distally[38].

Figure 4

Contrast-enhanced abdominal computed tomography[24]. Small intestinal loops are encased in a sac of thick peritoneal membrane (continuous arrows) with a small volume of peritoneal liquid effusion (discontinuous arrow). Gastroduodenal distension is also present (asterisks). A: Axial slice; B: Multiplanar coronal reconstruction.

Figure 5

Comparison of diagnostic features on computed tomography and magnetic resonance images[5]. A: Computed tomography scan in the axial plane showing a subtotal conglomeration of small bowel loops coiled in a concertina-like fashion and encased by a thick membrane (yellow arrows); B: T2-weighted magnetic resonance imaging sequence in the axial plane showing bowel loops aggregated in a festoon-like shape and encased by a thick membrane (yellow arrows); C: Computed tomography scan in the coronal plane showing the conglomeration of small bowls loops (yellow arrows); a few free loops are present in the upper quadrant (red arrow); D: T2-weighted magnetic resonance imaging sequence in the coronal plane showing the same conglomerated small bowel loops (yellow arrows) and a few free bowel loops (red arrow).

Figure 6

Laparoscopic view of the entire bowel segment encased with a fibrocollagenous membrane[37].