Published online Apr 27, 2022. doi: 10.4240/wjgs.v14.i4.352
Peer-review started: November 28, 2021
First decision: December 26, 2021
Revised: January 6, 2022
Accepted: March 26, 2022
Article in press: March 26, 2022
Published online: April 27, 2022
Primary encapsulating peritoneal sclerosis (EPS) is a rare but devastating disease that causes fibrocollagenous cocoon-like encapsulation of the bowel, resulting in bowel obstruction. The pathogenesis, prevention, and treatment strategies of EPS remain unclear so far. Since most patients are diagnosed during exploratory laparotomy, for the non-surgically diagnosed patients with primary EPS, the surgical timing is also uncertain.
A 44-year-old female patient was referred to our center on September 6, 2021, with complaints of abdominal distention and bilious vomiting for 2 d. Physical examination revealed that the vital signs were stable, and the abdomen was slightly distended. Computerized tomography scan showed a conglomerate of multiple intestinal loops encapsulated in a thick sac-like membrane, which was surrounded by abdominal ascites. The patient was diagnosed with idiopathic EPS. Recovery was observed after abdominal paracentesis, and the patient was discharged on September 13 after the resumption of a normal diet. This case raised a question: When should an exploratory laparotomy be performed on patients who are non-surgically diagnosed with EPS. As a result, we conducted a review of the literature on the clinical manifestations, intraoperative findings, surgical methods, and therapeutic effects of EPS.
Recurrent intestinal obstructions and abdominal mass combined with the imaging of encapsulated bowel are helpful in diagnosing idiopathic EPS. Small intestinal resection should be avoided.
Core Tip: Primary encapsulating peritoneal sclerosis (EPS), also called an abdominal cocoon, is so rare that the etiology, pathogenesis, treatment strategies of primary EPS remain vague. We reported a case of primary EPS and carried out a comprehensive literature analysis. The data indicated for the first time that recurrent intestinal obstructions and abdominal mass combined with the imaging of encapsulated bowel are helpful in diagnosing primary EPS. Surgical treatments are promising, but care should be taken to avoid small intestinal resection. Elective abdominal exploration might decrease complications of patients with primary EPS, but further research is required to substantiate this.