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Mahajan N, Agarwal M, Khatri A, Siddiqui Y M B, Sengar M. Abdominal Cocoon Syndrome in a Child With Intestinal Obstruction: A Case Report and Literature Review. J. Pediatr. Rev 2020; 8 (3) :195-200
URL: http://jpr.mazums.ac.ir/article-1-310-en.html

Abdominal cocoon syndrome or idiopathic Sclerosing Encapsulating Peritonitis (SEP) is an extremely uncommon cause of intestinal obstruction. Its etiology is explainable through numerous theories.
An eleven-year-old girl referred to the pediatric surgery OPD with complaints of abdominal pain for the past two weeks and vomiting for two days. Her family history for tuberculosis was positive. On examination, the abdomen was distended and slightly firm on palpation. The X-ray of her abdomen revealed multiple air-fluid levels. The CECT of the abdomen indicated dilated abdomen, duodenum, and proximal bowel loops. Some trapped inter bowel free fluid was also observed. The clinical presentation of subacute intestinal obstruction and the radiological features suggested a differential diagnosis of tubercular peritonitis versus pseudomyxoma peritonei. The obstructive symptoms demonstrated that the patient underwent an explorative laparotomy. Preoperatively, a thin membranous sac was identified enclosing multiple dilated small bowel loops. The sac was released by blunt dissection and part of the sac was provided for histopathological examination. Based on clinical, histopathological, and radiological findings, a diagnosis of abdominal cocoon syndrome was determined. The postoperative follow-up period of 6 months was uneventful.
We presented a rare case of primary sclerosing encapsulating peritonitis, also reported as abdominal cocoon syndrome. It is among the rare potentially devastating causes of intestinal obstruction in children. A very high index of suspicion is imperative to arrive at its pre-operative diagnosis alone by clinical and radiological findings.