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Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon)
© Serafimidis et al; licensee BioMed Central Ltd. 2006
Received: 18 July 2005
Accepted: 13 February 2006
Published: 13 February 2006
Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon) is a rare cause of small bowel obstruction, especially in adult population. Diagnosis is usually incidental at laparotomy. We discuss one such rare case, outlining the fact that an intra-operative surprise diagnosis could have been facilitated by previous investigations.
A 56 year-old man presented in A&E department with small bowel ileus. He had a history of 6 similar episodes of small bowel obstruction in the past 4 years, which resolved with conservative treatment. Pre-operative work-up did not reveal any specific etiology. At laparotomy, a fibrous capsule was revealed, in which small bowel loops were encased, with the presence of interloop adhesions. A diagnosis of abdominal cocoon was established and extensive adhesiolysis was performed. The patient had an uneventful recovery and follow-up.
Idiopathic sclerosing encapsulating peritonitis, although rare, may be the cause of a common surgical emergency such as small bowel ileus, especially in cases with attacks of non-strangulating obstruction in the same individual. A high index of clinical suspicion may be generated by the recurrent character of small bowel ileus combined with relevant imaging findings and lack of other plausible etiologies. Clinicians must rigorously pursue a preoperative diagnosis, as it may prevent a "surprise" upon laparotomy and result in proper management.
Sclerosing encapsulating peritonitis is a rare cause of small bowel obstruction, and can be classified as idiopathic or secondary (most importantly and most frequently due to chronic ambulatory peritoneal dialysis). The idiopathic form (also known as abdominal cocoon) was first described by Foo et al in 1978[1, 2]. It affects mainly young females from tropical and subtropical regions, but adult case reports from temperate zones can be encountered in literature. It is characterized by a thick, fibrotic, cocoon-like membrane, partially or totally encasing the small bowel. Clinically, it presents with recurrent episodes of acute or subacute small bowel obstruction, weight loss, nausea and anorexia, and at times with a palpable abdominal mass. Most cases are diagnosed incidentally at laparotomy, as in the case presented, although a preoperative diagnosis is purported feasible by a combination of barium follow-through (concertina pattern or cauliflower sign and delayed transit of contrast medium) and computed tomography of the abdomen (small bowel loops congregated to the center of the abdomen encased by a soft-tissue density mantle)[3, 4]. However, preoperative diagnosis requires a high index of clinical suspicion. Surgery (membrane dissection and extensive adhesiolysis) is the treatment of choice, and there is usually no need for bowel loop resection, especially when a preoperative diagnosis is feasible. Resection of the bowel is unnecessary and it increases morbidity and mortality. Resection is indicated only if the bowel is non-viable. An excellent long-term postoperative prognosis is most of the times guaranteed. Recently, in a series of 5 patients, in addition to adhesiolysis, small bowel intubation was performed with good results.
In the case presented, an intra-operative diagnosis of idiopathic sclerosing peritonitis was made in an adult male patient with a history of recurrent bouts of small bowel ileus. Pre-operative findings were inconclusive in his current admission. Although final surgical management would not have been modified, if results from imaging investigations during prior admissions were accessible in time, a pre-operative diagnosis could have been made possible.
Idiopathic sclerosing encapsulating peritonitis or abdominal cocoon, although a rare cause of a common surgical emergency such as small bowel ileus, may be responsible, especially in cases with recurrent attacks of non-strangulating obstruction in the same individual. A high index of clinical suspicion may be generated by the recurrent presentation of small bowel ileus combined with relevant imaging findings and lack of other etiologies Clinicians must rigorously pursue a preoperative diagnosis, as it may prevent a "surprise" upon laparotomy and unnecessary procedures for the patient, such as bowel resection.
Written consent from the patient was obtained for the publication of the case (relevant findings from history, examination and investigations and intra-operative photographs).
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