Concept definition:
Idoipathic sclerosing encapsulating peritonitis (SEP) is a chronic inflammatory process in which the small intestines are encased by a dense fibrocolagenous membrane,
first defined over a 100 years ago as "peritonitis chronica fibrosa incapsulata" by Owtschinnikv and latter also named by Foo in 1978 as "abdominal cocoon syndrome"
SEP can be divided into primary (idiopathic) or secondary forms if an underlying etiolgy is identified but until recently there has been much confusion between the names given to the dissease and terms such as abdominal cocoon,
peritoneal encapsulation ,
sclerosing encapsulating peritonitis or even abdominal cocoon syndrome have been used indisticly to name various entities.
Peritoneal encapsulation is a developmental anomaly in which there is a congenital acccesory peritoneal membrane classically found between the mesocolon and the omentum,
unrelated to any inflamatory condition.
SEP is considered to be primary (idiopathic) or secondary if an underlying cause can be demonstrated.
Idiopathic SEP is very rare,
with less than 200 reported cases in the literature.
It is more often described in tropical or subtropical countries and seems to have 2 forms,
one in adolescents and one in adults,
with a male:female ratio of 3.1.
According to the amount of intestine afected it can be divided into 3 types as shown on the image bellow.
![](https://epos.myesr.org/posterimage/esr/ecr2019/146905/media/807266?maxheight=300&maxwidth=300)
Fig. 3: Types of SEP
Secondary SEP is more frecuent and a number of "toxics" have been related with the triggering of the entity (table 1) but the most frequent etiologies of secondary SEP are peritoneal dialisis and peritoneal tuberculosis,
but there are case presentations of other etiologies such as abdominal surgery,
recurrent peritonitis,
autoinmune dissorders and others.
![](https://epos.myesr.org/posterimage/esr/ecr2019/146905/media/807335?maxheight=300&maxwidth=300)
Fig. 4: Secondary SEP: underlying causes
Clinical presentation:
Most patients with SEP attend the emergency room with signs and symptoms of intestinal obstruction but a wide range of symptoms such as nausea,
vomiting,
anorexia,
weight loss or malnutrition can appear,
depending of the severity and chronicity of the gastrointestinal obstruction.
Ascites and distension are some other common clinical findings,
but the palpation of an abdominal mass,
although reported,
is rare.
Anemia,
low albumin and high C-reative protein levels can be found but are not specific.
The past medical history is the most powerful tool for the clinical suspicion of EPS as most of the patients will describe previous atacks of colic pain.
A thorough history must be done to outrule associated risk factors.
Unusual conditions causing gastrointestinal obstruction are diagnosed in about 6% of affected individuals and idiopathic SEP is one of the more unusual between them.
Treatment:
There is no consensus about the treatment of EPS.
A conservative management with nasogastric tube decompresion,
bowel rest and nutritional support can be used to restore the nutritional status before surgery.
Secondary EPS can be specifically treated but there is no medical therapy for idiopathic EPS.
Peritonectomy and lysis with resection of the fibrous tissue represents the treatment of choice although it has a high rate of post operative mortality (19-34,5%) and a high rate of recurrence,
around 25%.
An inmediate postoperative small bowel obstruction can occur and should be treated by bowel rest and prenteral nutrition.
![](https://epos.myesr.org/posterimage/esr/ecr2019/146905/media/807829?maxheight=300&maxwidth=300)
Fig. 5: Post surgical intestinal obstruction