sclerosing encapsulating peritonitis ( sep ) is characterized by partial or complete encasement of small intestine by a thick fibrocollagenous membrane . a 90-year - old male patient presented to our emergency department with signs and symptoms of intestinal obstruction and dehydration . physical examination findings , patient 's age and plain abdominal radiography were consistent with tumoral obstruction or viscus perforation . explorative laparotomy revealed a fibrous capsule encasing intestines as well as dense adhesions between intestinal loops . since the overall condition of the patient was not well enough to allow a wide dissection and membrane excision , the operation was terminated after performing a limited loop ileostomy . despite advances in radiological techniques , the exact diagnosis in many cases is still made according to intraoperative findings and histopathological properties of the excised membrane . while some cases of sep remain asymptomatic for years , most cases are characterized by recurrent bouts of acute , subacute or chronic intestinal obstruction . to our knowledge , sclerosing encapsulating peritonitis ( sep ) is an entity characterized by encasement of small intestine by a fibrocollagenous membrane ( 1 - 5 ) . it was first described as  peritonitis chronica fibrosa incapsulata  ( 1 , 2 ) . depending on the underlying cause , the disorder is divided into primary ( idiopathic ) and secondary forms ( 1 ) . the idiopathic form was named as abdominal cocoon syndrome ( acs ) ( 2 , 5 ) . affected patients most commonly present with acute , subacute or chronic attacks of intestinal obstruction ( 1 , 3 ) . the preoperative diagnosis is quite challenging and most cases are diagnosed intraoperatively ( 3 ) . on the other hand , recent advances in radiological imaging modalities have now allowed preoperative diagnosis of sep in some cases ( 4 ) . a 90-year - old turkish male patient ( diyarbakir , turkey ) presented to our emergency department in april 2011 with severe abdominal pain , nausea , vomiting and inability to defecate or pass gas . according to the information obtained from patient relatives , the patient had been admitted to separate healthcare facilities for a couple of times with abdominal pain and constipation for five years , but no pathology had been found by a variety of tests and examinations . his medical history was unremarkable except for hypertension controlled by a salt - free diet . vital signs were as follows : blood pressure as 90/50 mmhg , pulse rate of 95 bpm and body temperature of 37.9c . on physical examination , his laboratory tests were as follows ; wbc 8.890 ( neutrophil : 86.1% ) , hemoglobin 14 gr / dl , bun 38 mg / dl and creatinine 0.8 mg / dl . there were diffuse air - fluid levels in the abdomen on upright plain abdominal x - ray ( figure 1 , table 1 ) . the physical examination findings , patient 's age and plain abdominal radiography were consistent with tumoral obstruction or viscus perforation ; therefore , patient was urgently taken into operation . a laparotomy was performed via midline incision , followed by abdominal exploration that revealed encapsulation of all intestinal segments by a dense , plate - like fibrous membrane that encased whole small intestine and caused dense adhesions between intestinal loops ( figure 2 ) . taken into consideration our previous experiences , we regarded this appearance as abdominal cocoon syndrome . as the fibrocollagenous membrane was densely adhered to both intestinal surface and interloop distance , excision and adhesiolysis could only be applied to a limited region . considering the overall medical condition of the patient , we did not perform membrane excision and adhesiolysis that would prolong the operation time . the patient was admitted to intensive care unit owing to his poor general status . despite aggressive fluid and electrolyte replacement attempts and administration of wide - spectrum antibiotics , the patient died as a consequence of septic shock and multiorgan failure ( wbc 1880/l , bun 123 mg / dl creatinine 2.92 mg / dl and albumin 2.4 gr / dl ) three days after the operation . depending on the underlying causes , sep is divided into primary ( idiopathic ) and secondary forms ( 2 , 6 - 9 ) . the idiopathic form of the disease was named as  abdominal cocoon syndrome in 1978 ( 2 , 3 , 8 , 10 ) . idiopathic sep is mostly observed in female children of adolescent age living in tropical and subtropical countries , although may be seen in children living in temperate regions or adults of advanced age ( 1 ) . in a study that presented the youngest case series to date , the disease was 7 times more common in men compared to women ( 3 ) . although many theories have been put forward to explain the etiology of the disease , the exact etiology has remained to be proven . in women , retrograde menstruation , retrograde extension of fallopian infections or viral gynecological infections the most common known cause of secondary sep worldwide is peritoneal dialysis ( 2 ) . the main objective of numerous articles on secondary sep in the literature is the association between peritoneal dialysis and sep . abdominal tuberculosis is responsible for a sizeable part of secondary sep in underdeveloped countries ( 9 ) . previous surgeries , sarcoidosis , gastrointestinal tumors , sle , fmf , fibrogenic foreign body , beta - blocker use , ventriculoperitoneal and peritoneovenous shunts , orthotopic liver transplantation and recurrent peritonitis are among other rare causes ( 5 - 7 , 9 ) . however , the patient presented here had no apparent cause , neither in medical history nor in the microbiological tests . thus , we considered the case as idiopathic sep . while some cases of sep have an asymptomatic course , most exhibit symptoms characterized by recurrent acute , subacute or chronic attacks of gastrointestinal obstruction ( 1 , 2 , 7 ) . among the symptoms of the disease , abdominal pain , nausea , vomiting , loss of appetite , weight loss , distention and inability to defecate or pass gas are the primary symptoms ( 1 , 2 ) . occasionally , a soft and painless mass lesion can be palpated in some patients ( 2 , 6 ) . to date , two cases of intestinal perforation secondary to sep have been reported , one being idiopathic and the other secondary to tuberculosis ( 1 ) . the first and most important steps towards diagnosis of sep are awareness and clinical suspicion proportional to experience and knowledge of the disease ( 1 , 7 ) . among patients presenting to emergency department with intestinal obstruction , 60 - 80% are caused by postoperative adhesions , while only 6% are caused by unusual causes ( 2 ) . idiopathic sep forms only a small part of unusual cases leading to intestinal obstruction ( 2 ) . apart from clinical history and suspicion , the most frequently diagnostic tools are x - ray films , barium passage radiographies , ultrasonography ( usg ) and computed tomography ( ct ) ( 1 , 2 ) . as the case presented here , cases with signs and symptoms of intestinal obstruction have small bowel dilatation and air - fluid levels on abdominal x - ray films . air - fluid levels in elderly patients who have not been operated previously suggest tumor obstruction or chronic mesenteric ischemia ( 1 ) . an accordion pattern and cauliflower appearance on barium passage radiographies of small intestine may give a clue of sep ( 1 , 2 , 7 ) . abdominal usg may demonstrate dilated small bowel segments surrounded by a thick , hypoechogenic fibrous membrane ( 2 , 7 ) . ct is currently the most useful radiological method for diagnosis of sep ( 2 ) . especially multidetector ct with excellent image quality on coronal , sagittal and axial planes may provide the best results . the characteristic ct sign is the appearance of loops of small intestine that conglomerate at midline and are encased by a dense mantle without peripheral contrast uptake ( 2 ) . the major obstacles before ct include presentation of many patients to emergency departments with signs and symptoms of intestinal obstruction , physical examination findings consistent with acute abdomen and lack of ct in many centers . considering all these factors , it can be concluded that the preoperative diagnosis is still difficult in symptomatic cases with sep and most patients are diagnosed by intraoperative signs and histopathological findings ( 5 ) . prudent follow - up is all that is needed in asymptomatic cases with idiopathic sep ( 7 ) . such cases are best managed by intestinal rest , nasogastric decompression and nutritional support ( enteral or parenteral ) ( 3 , 11 ) . cases with no regression of symptoms may be treated with antiinflammatory or antifibrogenic drugs such as tamoxifen , steroids , colchicine , azathioprine and mycophenolate mofetil ( 11 ) . however , these treatment protocols have mainly been used in secondary sep and there are no sufficient data to justify their use in idiopathic sep . to my opinion and experience surgical therapy is used in markedly symptomatic cases or cases with sep diagnosis confirmed during laparotomy is still debated . different options such as membrane excision + adhesiolysis , resection + anastomosis , resection + anastomosis + protective enterostomy can be used alone or in combination , depending on the condition of patient . intestinal resection should be avoided owing to certain complications including anastomosis leaks and short bowel syndrome since resection is usually not essential and unnecessary resections increase morbidity and mortality ( 3 , 7 ) . the role of laparoscopy in the management of sep is unclear . in the literature , there are a limited number of case reports in which the authors reportedly performed successful membrane excision and adhesiolysis . one additional advantage of laparoscopy is that it can be used for diagnostic purposes in cases with unclear diagnosis ( 8) . in conclusion , preoperative diagnosis is a true challenge and most reported cases have been incidentally diagnosed during laparotomy . fortunately , clinicians possess some diagnostic radiological methods that aid in diagnostic process . while a conservative approach is the best in mildly symptomatic cases , a minimally invasive approach should be the norm to avert troublesome complications such as anastomosis leaks and short bowel syndrome .
introduction : sclerosing encapsulating peritonitis ( sep ) is characterized by partial or complete encasement of small intestine by a thick fibrocollagenous membrane . depending on underlying causes 
 , sep is divided into primary and secondary forms . 
 idiopathic sep is also called idiopathic or abdominal cocoon syndrome . 
 herein we presented a case of idiopathic sep.case presentation : a 90-year - old male patient presented to our emergency department with signs and symptoms of intestinal obstruction and dehydration . 
 physical examination findings , patient 's age and plain abdominal radiography were consistent with tumoral obstruction or viscus perforation . 
 explorative laparotomy revealed a fibrous capsule encasing intestines as well as dense adhesions between intestinal loops . 
 since the overall condition of the patient was not well enough to allow a wide dissection and membrane excision , the operation was terminated after performing a limited loop ileostomy . 
 unfortunately , the patient was lost due to organ failure at the postoperative period.conclusions:despite advances in radiological techniques , the exact diagnosis in many cases is still made according to intraoperative findings and histopathological properties of the excised membrane . 
 while some cases of sep remain asymptomatic for years , most cases are characterized by recurrent bouts of acute , subacute or chronic intestinal obstruction . to our knowledge , 
 the case presented here is the oldest patient with idiopathic sep in the literature .