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

BACKGROUND Encapsulating peritoneal sclerosis (EPS), also known as abdominal cocoon syndrome (ACS), is a rare pathological entity that is difficult to diagnose. The syndrome can be idiopathic or secondary, and is mainly associated with peritoneal dialysis or abdominal tuberculosis. Patients with EPS can display symptoms of subacute intestinal obstruction or acute symptoms of ileus. We present a case of idiopathic EPS, misdiagnosed as acute appendicitis. Here, we report an unusual presentation of ACS and review the literature to delineate its diagnosis and treatment. CASE REPORT A 63-year-old man presented to the emergency department with abdominal pain, mainly located in the right iliac fossa. Acute appendicitis was misdiagnosed by contrast-enhanced CT findings. The diagnosis of EPS was established intraoperatively. Secondary causes of EPS were ruled out and the diagnosis of idiopathic EPS was made. We identified 52 studies (82 patients) of idiopathic EPS in the most recently published literature and studied their features. CONCLUSIONS Idiopathic EPS is rare, but clinicians should be aware of this uncommon entity and the variability of its clinical symptoms upon manifestation. High suspicion should be raised preoperatively. The management should be tailored to each specific patient, according to cause, manifestation, and severity of clinical symptoms.