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Rare disease
CASE REPORT
Abdominal cocoon syndrome: an obstructive adhesiolytic metamorphosis
  1. Abagayle E Renko1,
  2. Samantha R Witte2 and
  3. Amanda B Cooper2
  1. 1 The Pennsylvania State University, College of Medicine, Hershey, Pennsylvania, USA
  2. 2 Department of Surgery, Milton S Hershey Medical Center, Hershey, Pennsylvania, USA
  1. Correspondence to Dr Amanda B Cooper, acooper2{at}pennstatehealth.psu.edu

Abstract

Abdominal cocoon syndrome (ACS), also known as idiopathic sclerosing peritonitis and primary sclerosing peritonitis, is a rare condition causing small bowel obstruction first described in 1978 by Foo et al. It is characterised by total or partial encasement of the small bowel in a fibrocollagenous cocoon-like sac accompanied by extensive intrinsic small bowel adhesions. While the aetiology of this condition remains largely unknown, ACS can be divided into two subtypes: primary or idiopathic, which is often accompanied by cryptorchidism, and secondary to another cause such as congenital dysplasia or medications. Definitive diagnosis can only be achieved following laparotomy with extensive lysis of adhesions to alleviate the obstruction. However, preoperative diagnosis is possible if clinicians are aware of the condition and its radiologic signs.

  • general surgery
  • emergency medicine
  • small intestine
  • pathology
  • radiology

https://doi.org/10.1136/bcr-2018-228593

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    Footnotes

    • Contributors AER contributed to the conceptualisation, literature review, original draft preparation and revision of the manuscript. SRW contributed to the conceptualisation, revision and editing of the manuscript. ABC contributed to the conceptualisation, reviewing and editing and supervision of this manuscript and project. There was no data collection for this project. All authors have approved the manuscript. All authors also agreed to be accountable for the article and to ensure that all questions regarding the integrity of the article are investigated and resolved.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Patient consent for publication Obtained.