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
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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
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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)