Idiopathic Encapsulating Peritoneal Sclerosis Mimicking Acute Appendicitis: A Case Report and Systematic Literature...

Area of Science:

• Gastroenterology
• Abdominal Surgery
• Pathology

Background:

• Encapsulating peritoneal sclerosis (EPS), also known as abdominal cocoon syndrome (ACS), is a rare condition with challenging diagnosis.
• It can be idiopathic or secondary, often linked to peritoneal dialysis or abdominal tuberculosis.
• Patients may present with subacute intestinal obstruction or acute ileus.

Purpose of the Study:

• To report an unusual case of idiopathic EPS misdiagnosed as acute appendicitis.
• To review the literature for diagnosis and treatment strategies of ACS.
• To increase awareness of idiopathic EPS and its varied clinical presentations.

Main Methods:

• Case report of a 63-year-old male with misdiagnosed appendicitis.
• Intraoperative diagnosis of EPS after initial misdiagnosis by CT scan.
• Literature review of 52 studies (82 patients) on idiopathic EPS.

Main Results:

• Idiopathic EPS was diagnosed intraoperatively in a patient initially suspected of acute appendicitis.
• Literature review identified 82 patients with idiopathic EPS across 52 studies.
• Clinical presentations of idiopathic EPS are variable, necessitating high preoperative suspicion.

Conclusions:

• Idiopathic EPS is a rare but important differential diagnosis in patients with abdominal symptoms.
• Clinicians must maintain a high index of suspicion for EPS due to its varied presentations.
• Management should be individualized based on the cause, presentation, and severity of symptoms.