Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. May 21, 2019; 25(19): 2294-2307
Published online May 21, 2019. doi: 10.3748/wjg.v25.i19.2294
Congenital peritoneal encapsulation: A review and novel classification system
Aneesh Dave, James McMahon, Assad Zahid
Aneesh Dave, James McMahon, Assad Zahid, Department of Colorectal Surgery, Royal Prince Alfred Hospital, Camperdown 2050, New South Wales, Australia
Aneesh Dave, Assad Zahid, Sydney Medical School, Edward Ford Building, the University of Sydney, Camperdown 2006, New South Wales, Australia
Author contributions: All authors equally contributed to this paper with conception and design of the study, literature review and analysis, drafting and critical revision and editing, and final approval of the final version.
Conflict-of-interest statement: No potential conflicts of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Assad Zahid, BSc, MBBS, MPhil, Surgeon, Department of Colorectal Surgery, Royal Prince Alfred Hospital, Missenden Road, PO Box 4270, Camperdown 2050, New South Wales, Australia.
Telephone: +61-295156111
Received: February 15, 2019
Peer-review started: February 17, 2019
First decision: March 14, 2019
Revised: March 23, 2019
Accepted: March 29, 2019
Article in press: March 30, 2019
Published online: May 21, 2019

Congenital peritoneal encapsulation (CPE) is a very rare, congenital condition characterised by the presence of an accessory peritoneal membrane which encases a variable extent of the small bowel. It is unclear how CPE develops, however it is currently understood to be a result of an aberrant adhesion in the peritoneal lining of the physiological hernia in foetal mid-gut development. The condition was first described in 1868, and subsequently there have been only 45 case reports of the phenomenon. No formal, systematised review of CPE has yet been performed, meaning the condition remains poorly understood, underdiagnosed and mismanaged. Diagnosis of CPE remains clinical with important adjuncts provided by imaging and diagnostic laparoscopy. Two thirds of patients present with abdominal pain, likely secondary to sub-acute bowel obstruction. A fixed, asymmetrical distension of the abdomen and differential consistency on abdominal palpation are more specific clinical features present in approximately 10% of cases. CPE is virtually undetectable on plain imaging, and is only detected on 40% of patients with computed tomography scan. Most patients will undergo diagnostic laparotomy to confirm the diagnosis. Management of CPE includes both medical management of the critically-unstable patient and surgical laparotomy, partial peritonectomy and adhesiolysis. Prognosis following prompt surgical treatment is excellent, with a majority of patients being symptom free at follow up. This review summarises the current literature on the aetiology, diagnosis and treatment of this rare disease. We also introduce a novel classification system for encapsulating bowel diseases, which may distinguish CPE from the commoner, more morbid conditions of abdominal cocoon and encapsulating peritoneal sclerosis.

Keywords: Congenital, Encapsulation, Peritoneum, Cocoon, Sclerosis

Core tip: Congenital peritoneal encapsulation (CPE) is a very rare congenital disorder characterised by the presence of an accessory peritoneal membrane surrounding the entirety of the small intestine. Though not fully understood, it is thought to arise due to an aberrant peritoneal adhesion during foetal mid-gut development. It is a rare but important cause of undifferentiated abdominal pain and sub-acute small bowel obstruction. We present a comprehensive review of CPE including an international epidemiological focus, diagnosis and treatment. We also describe a novel classification system for encapsulating bowel diseases.