Abdominal cocoon syndrome-a rare culprit behind small bowel ischemia and obstruction:Three case reports

BACKGROUND Abdominal cocoon syndrome(ACS)represents a category within sclerosing encapsulating peritonitis,characterized by the encapsulation of internal organs with a fibrous,cocoon-like membrane of unknown origin,resulting in bowel obstruction and ischemia.Diagnosing this condition before surgery poses a cha-llenge,often requiring confirmation during laparotomy.In this context,we depict three instances of ACS:One linked to intestinal obstruction,the second exclu-sively manifesting as intestinal ischemia without any obstruction,and the final case involving a discrepancy between the radiologist and the surgeon.CASE SUMMARY Three male patients,aged 53,58,and 61 originating from Northern Thailand,arrived at our medical facility complaining of abdominal pain without any prior surgeries.Their vital signs remained stable during the assessment.The diagnosis of abdominal cocoon was confirmed through abdominal computed tomography(CT)before surgery.In the first case,the CT scan revealed capsules around the small bowel loops,showing no enhancement,along with mesenteric congestion affecting both small and large bowel loops,without a clear obstruction.The second case showed intestinal obstruction due to an encapsulated capsule on the CT scan.In the final case,a patient presented with recurring abdominal pain.Initially,the radiologist suspected enteritis as the cause after the CT scan.However,a detailed review led the surgeon to suspect encapsulating peritoneal sclerosis(ACS)and subsequently perform surgery.The surgical procedure involved complete removal of the encapsulating structure,resection of a portion of the small bowel,and end-to-end anastomosis.No complications occurred during surgery,and the patients had a smooth recovery after surgery,eventually discharged in good health.The histopathological examination of the fibrous membrane(cocoon)across all cases consistently revealed the presence of fibro-collagenous tissue,without any indications of malignancy.CONCLUSION Individuals diagnosed with abdominal cocoons commonly manifest vague symptoms of abdominal discomfort.An elevated degree of clinical suspicion,combined with the application of appropriate radiological evaluations,markedly improves the probability of identifying the abdominal cocoon before surgical intervention.In cases of complete bowel obstruction or ischemia,the established norm is the comprehensive removal of the peritoneal sac as part of standard care.Resection with intestinal anastomosis is advised solely when ischemia and gangrene have been confirmed.

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.