Computed Tomography Features of Encapsulating Peritoneal Sclerosis

Abstract

Encapsulating peritoneal sclerosis (EPS) is an uncommon but destructive complication of continuous peritoneal dialysis. EPS is a chronic inflammatory pathology classified as primary and secondary EPS. The classification depends on the pathologic findings of the encasing membrane and etiopathogenesis. Idiopathic EPS is known as abdominal cocoon syndrome. However, several etiologies, such as gynaecological infection, resulting in immunologic tissue damage, retrograde peritonitis, retrograde menstruation with viral infection, vascular anomalies, and omental hypoplasia, have been defined as secondary etiopathogenesis. Progressive peritoneal thickening, enclosure of the small bowel in fibrous tissue that results in small bowel obstruction, is a clinical presentation of EPS. Clinical findings are nonspecific. Radiological evaluation of EPS provides an early diagnosis and treatment and computed tomography (CT) scanning is a useful diagnostic method. The early diagnosis of EPS allows the detection of early-stage disease. In this paper, we present the demonstrative CT findings of EPS and evaluate radiological mimics of the disease.

Progressive Small Bowel Obstruction in a Patient With the Autoimmune Syndrome APECED

“…If no infection is present, immunosuppressive therapy with mammalian target of rapamycin inhibitors (ie, sirolimus, everolimus) has been favored on the basis of both its immunomodulatory and antifibrotic beneficial effects. [1][2][3] If EPS is diagnosed after SBO develops, as in our patient, surgical intervention is required. [1][2][3] Keywords: Small Bowel Obstruction; Encapsulating Peritoneal Sclerosis; Abdominal Cocoon.…”

“…1 EPS is most frequently associated with peritoneal dialysis, but it can also be caused by infections (eg, tuberculosis, recurrent peritonitis), cirrhosis, drugs (eg, practolol), or autoimmune disease (eg, sarcoidosis, systemic lupus erythematosus) as in our patient. [1][2][3] The pathogenesis of EPS is poorly understood; a "2-hit" process has been proposed by which an inciting trigger promotes peritoneal inflammation in a predisposed individual. 1,3 Patients with EPS present with anorexia, vomiting, abdominal pain or distention, and/or weight loss and may have an elevated CRP.…”

“…1,3 Patients with EPS present with anorexia, vomiting, abdominal pain or distention, and/or weight loss and may have an elevated CRP. 1,2 Computed tomography findings of peritoneal thickening and enhancement, small bowel dilation with bowel wall thickening, loculated ascitic fluid collection, and/or tissue calcifications should raise suspicion for EPS. 2 If EPS is diagnosed early, treatment should be directed toward addressing the underlying inciting condition.…”

“…1,2 Computed tomography findings of peritoneal thickening and enhancement, small bowel dilation with bowel wall thickening, loculated ascitic fluid collection, and/or tissue calcifications should raise suspicion for EPS. 2 If EPS is diagnosed early, treatment should be directed toward addressing the underlying inciting condition. If no infection is present, immunosuppressive therapy with mammalian target of rapamycin inhibitors (ie, sirolimus, everolimus) has been favored on the basis of both its immunomodulatory and antifibrotic beneficial effects.…”

Peritoneal Imaging may be the Last Piece of the Puzzle for Precision Evaluation of Peritoneal Function

“…The main CT features of EPS patients can be divided into the following four aspects: (1) peritoneal changes: e.g. thickening, calcification and marked enhancement (in case of contrast application); (2) small bowel abnormalities: thickening, encapsulation or dilatation of the intestine, which in severe cases can form a "cocoon" appearance; (3) fluid collections: local fluid accumulation and recurrent blood ascites; And (4) calcifications: intestinal, mesenteric and parietal peritoneal calcification [7]. Stafford-Johnson et al [8] first compared CT scans of patients with EPS with other PD patients (vintage ranging from 1 month to 7 years) and concluded that peritoneal calcification, peritoneal thickening, localized fluid collections, and bowel tethering were diagnostic of EPS.…”