The enigma of primary and secondary encapsulating peritoneal sclerosis

BMC Surgery, Dec 2016

Background Encapsulating Peritoneal Sclerosis (EPS) describes a variety of diseases that are frequently confused with different names and different etiopathogeneses. The aim of this article is to report personal experience of focusing on correct classification and the status of current diagnosis and treatment. Methods A retrospective analysis was performed. Age, sex, ethnic origin, past medical history, symptoms and their duration, radiological tools and signs, laboratory tests, preoperative diagnosis, surgical approach, intraoperative findings, pathological findings, hospital stay, morbidity and mortality were studied. Results A total of seven patients, including six males and one female, aged from 24 to 72 years were observed. Four patients had recurrent abdominal colic pain for 3 months, 1, 2 and 9 years; two patients also reported recurrent attacks but without any specification of the duration. All seven patients presented at the emergency department with abdominal pain that was mainly diffused over the entire abdomen. Six patients were submitted to a CT scan. Only in two patients was the diagnosis of EPS made preoperatively. All seven patients were submitted to open surgery. The hospital stay was between 4 and 60 days. One patient had morbidity, and one patient died of MOF. Conclusions Currently, the correct identification of EPS is more easily possible than in the past, but the diagnosis is still a challenge. Surgery must be performed as soon as possible to avoid a poorer quality of life.

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The enigma of primary and secondary encapsulating peritoneal sclerosis

Allam et al. BMC Surgery The enigma of primary and secondary encapsulating peritoneal sclerosis Hisham Allam 0 Omer Al Yahri 0 Sharon Mathew 0 Adham Darweesh 3 Ahmed Nafea Suliman 0 Sherif Abdelaziem 0 Mohamed Khairat 0 Adriana Toro 2 Isidoro Di Carlo 0 1 0 Departments of General Surgery, Hamad General Hospital , Al Rayyan Road, 3050 Doha , Qatar 1 Department of Surgical Sciences and Advanced Technologies “G.F. Ingrassia”, University of Catania , Catania , Italy 2 Department of General Surgery, Barone I. Romeo Hospital , Patti, Messina , Italy 3 Department of Radiology, Hamad General Hospital , Doha , Qatar Background: Encapsulating Peritoneal Sclerosis (EPS) describes a variety of diseases that are frequently confused with different names and different etiopathogeneses. The aim of this article is to report personal experience of focusing on correct classification and the status of current diagnosis and treatment. Methods: A retrospective analysis was performed. Age, sex, ethnic origin, past medical history, symptoms and their duration, radiological tools and signs, laboratory tests, preoperative diagnosis, surgical approach, intraoperative findings, pathological findings, hospital stay, morbidity and mortality were studied. Results: A total of seven patients, including six males and one female, aged from 24 to 72 years were observed. Four patients had recurrent abdominal colic pain for 3 months, 1, 2 and 9 years; two patients also reported recurrent attacks but without any specification of the duration. All seven patients presented at the emergency department with abdominal pain that was mainly diffused over the entire abdomen. Six patients were submitted to a CT scan. Only in two patients was the diagnosis of EPS made preoperatively. All seven patients were submitted to open surgery. The hospital stay was between 4 and 60 days. One patient had morbidity, and one patient died of MOF. Conclusions: Currently, the correct identification of EPS is more easily possible than in the past, but the diagnosis is still a challenge. Surgery must be performed as soon as possible to avoid a poorer quality of life. Encapsulating peritoneal sclerosis; Peritoneal encapsulation; Abdominal cocoon syndrome - Background Encapsulating peritoneal sclerosis (EPS) is an uncommon chronic syndrome, usually presenting clinically as intermittent, acute or sub-acute gastrointestinal obstruction [1]. EPS can be divided into primary and secondary [2]. Primary EPS is also defined as idiopathic [3]. Primary EPS have been also defined in 1978 as abdominal cocoon syndrome [4]. The cause of primary EPS is still unknown; [4, 5]. Secondary EPS is related to many conditions and causes; the most common is peritoneal dialysis (Table 1). Both forms (primary and secondary) are different from peritoneal encapsulation (PE). This disease was reported for the first time in 1868 by Cleland [6], and it is represented by an accessory peritoneal membrane. More than the result of inflammation should be considered with a duplication of the peritoneum; this is found incidentally in the majority of cases. The peritoneal membrane in this disease is secured laterally to the ascending and descending colon, cranially to the transverse mesocolon, and caudally to the posterior parietal peritoneum. It has two openings: one at the entrance of the intestine at the duodenojejunal junction, and the other at the ileocecal junction for the exit of the last loop. The absence of sclerosis represents the difference with the EPS, in fact the coverage of the intestine is only a layer of serosa membrane like the peritoneum. Many tools can be used to diagnose EPS. Instead of utilizing the past medical history and the radiological finding, preoperative diagnosis remains challenging, and in a high percentage of cases, the diagnosis is reached at the time of the surgical procedure. © The Author(s). 2016 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. 1) Peritoneal encapsulation 2) Sclerosing encpsulating peritonitis a) Primary or idiopatic or abdominal cocoon syndrome b) Secondary The aim of this article is to report a valuable number of patients who were observed in our hospital, focusing on correct classification and the status of current diagnosis and treatment. Methods A retrospective analysis was performed on patients admitted to the Hamad General Hospital Department of Surgery, General Surgery section, in the last 10 years from Janua (...truncated)


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Hisham Allam, Omer Al Yahri, Sharon Mathew, Adham Darweesh, Ahmed Suliman, Sherif Abdelaziem, Mohamed Khairat, Adriana Toro, Isidoro Di Carlo. The enigma of primary and secondary encapsulating peritoneal sclerosis, BMC Surgery, 2016, pp. 81, 16, DOI: 10.1186/s12893-016-0198-2