Encapsulating peritoneal sclerosis in an AMA-M2 positive patient:A case report

BACKGROUND Encapsulating peritoneal sclerosis(EPS)is hard to diagnose because of nonspecific symptoms and signs.It is a general consensus that EPS is classified as primary and secondary.There have been several studies discovering some highrisk factors such as liver cirrhosis,of which AMA-M2 is a biomarker,and intraabdominal surgery such as laparoscopic surgery.Imaging studies help to diagnose EPS and exploratory laparotomy might be an alternative if imaging fails.Nowadays,laparotomy plays a key role in treating EPS,especially when medical treatments do not work and medical therapy fails to ease patients’symptoms.CASE SUMMARY A 58-year-old man complained of unexplained vomiting and abdominal distension 2 mo after laparoscopic cholecystectomy.Increased alkaline phosphatase and liver enzymes were discovered.An autoimmune liver disease test showed that AMA-M2 was positive.A gastroscopy revealed bile reflux gastritis.A magnetic resonance imaging scan showed a slight dilatation of the intrahepatic bile duct.A colonoscopy showed that there was a mucosal eminence lesion in the sigmoid colon(24 cm away from the anus),with a size of 3 cm×3 cm and erosive surface.At last,the small intestine and the stomach were found to be encased in a cocoon-like membrane during the surgery.The membrane was dissected and adhesiolysis was done to release the trapped organs.The patient recovered and was discharged 44 d after the operation,and there was no recurrence during a follow-up period of 3 mo.CONCLUSION AMA-M2 is a marker of primary biliary sclerosis and may help to make a preoperative diagnosis of EPS.

Surgical timing for primary encapsulating peritoneal sclerosis: A case report and review of literature

BACKGROUND 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.CASE SUMMARY 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.CONCLUSION 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.

Abdominal cocoon in children: A case report and review of literature

BACKGROUND Abdominal cocoon or“encapsulating peritoneal sclerosis”(EPS)is an uncommon and rare cause of intestinal obstruction.Only a few cases have been reported in paediatric patients.Typically,EPS is described as the primary form in young adolescent girls from tropical and subtropical countries because of viral peritonitis due to retrograde menstruation or a history of peritoneal dialysis.Most patients are asymptomatic or present with abdominal pain,which is likely to occur secondary to subacute bowel obstruction.Findings at imaging,such as ultrasound,computed tomography,and magnetic resonance imaging,are often nonspecific.When diagnosed,EPS is characterized by total or partial encasement of the bowel within a thick fibrocollagenous membrane that envelopes the small intestine in the form of a cocoon because of chronic intraabdominal fibroinflammatory processes.The membrane forms a fibrous tissue sheet that covers,fixes,and finely constricts the gut,compromising its motility.CASE SUMMARY We present a case of EPS in a 12-year-old boy 8 wk after primary surgery for resection of symptomatic jejunal angiodysplasia.There was no history of peritoneal dialysis or drug intake.CONCLUSION In this report,we sought to highlight the diagnostic,surgical,and histopathological characteristics and review the current literature on EPS in paediatric patients.