Abdominal Cocoon Syndrome (Idiopathic Sclerosing Encapsulating Peritonitis): How Easy Is Its Diagnosis Preoperatively? A Case Report

Abstract

The abdominal cocoon syndrome (or idiopathic encapsulating peritonitis) is a rare cause of intestinal obstruction. It has been reported predominantly in adolescent girls living in tropical/subtropical region in which diagnosis is only made at laparotomy in most cases. The cause and pathogenesis of the condition have not been elucidated. Prolonged administration of practalol, meconium peritonitis, and tuberculous infection of the female genital tract have been incriminated as possible causes. The author reports a case of a female patient with recurrent intestinal obstruction treated for years but failed to settle down on conservative treatment during her last hospital admission and had to undergo surgery. Preoperative diagnosis of this syndrome as the cause of her intestinal obstruction was not made until at laparotomy, when a thick fibrotic peritoneal wrapping of the bowel in a concertina-like fashion with some adhesions was found. Excision of this membrane and adhesiolysis were carried out without any need for bowel resection, and this led to relief of the obstruction and patient's complete recovery. Awareness of this benign condition in the differential diagnosis of intestinal obstruction will result in early diagnosis and correct management and prevent unnecessary bowel resections and bad outcomes.

Abdominal cocoon: a surgical challenge

“…Plain abdominal X-ray will reveal dilated small bowel loops, multiple air fluid levels, clumping of bowel loops, peritoneal and bowel wall calcifications. 13,14…”

“…However, the radiologist needs to be fully aware of this peculiar condition in order to propose a provisional diagnosis of EPS on ultra sound. 14,15…”

Idiopathic abdominal cocoon syndrome: Preoperative diagnosis with computed tomography

“…CECT with multiplanar reconstruction images, which clearly shows peritoneal thickening, signs of intestinal obstruction, clustering and fixation of intestinal loops, should be the preferred imaging technique (4). The definite diagnosis of ACS is generally made on the basis of intraoperative and histopathological findings, similar to the present case (5). Treatment should consist of complete surgical excision of the encapsulating peritoneal sac with adhesiolysis.…”

Abdominal cocoon: An enigmatic entity

“…These were: intestinal obstruction in a young girl without a definite etiology, presentation with abdominal pain and vomiting but no definite obstruction, history of previous spontaneously resolving episodes of obstruction and presentation with soft non tender abdominal lump. 33,34 These may not hold true for the patients with secondary EPS or even for all patients with primary EPS. Table 1 lists various etiologic factors that must be sought in a patient with abdominal cocoon.…”