Peritonitis chronica fibrosa incapsulata

Encapsulating peritoneal sclerosis is a rare benign cause of acute or subacute small bowel obstruction. It is characterized by total or partial encasement of the small bowel within a thick fibrocollagenous membrane.

Terminology

The condition was originally termed abdominal cocoon. The condition is popularly known as sclerosing encapsulating peritonitis, however, this is somewhat of a misnomer as inflammation is not always present . It has also been known as sclerosing peritonitis, encapsulating peritonitis, and peritonitis chronica fibrosa incapsulata.

Epidemiology

Encapsulating peritoneal sclerosis can occur at any age, with reports ranging from 2-day neonate to 82 years .

Clinical presentation

The presentation is non-specific and patients may present with vomiting, abdominal pain and/or a subacute bowel obstruction .

Pathology

It can be idiopathic or secondary due to:

• continuous ambulatory peritoneal dialysis (prevalence ~0.7%)
• tuberculosis
• peritoneovenous or ventriculoperitoneal shunts
• treatment with practolol

Various abdominal disorders such as tuberculosis, sarcoidosis, familial Mediterranean fever, gastrointestinal malignancy, ovarian carcinoma , protein S deficiency, liver transplantation, fibrogenic foreign material, and luteinised ovarian thecomas are the other rare causes.

Radiographic features

Plain radiograph

Abdominal radiographic appearances are nonspecific and may be normal or may show:

• gas-fluid levels similar to those in patients with any other cause of small-bowel obstruction
• the wall of the "cocoon" may calcify

Ultrasound

• clumped bowel loops
• trilaminar appearance of a hyperechoic membrane, hypoechoic bowel wall and hyperechoic bowel contents
• ascites may be present

CT

In the appropriate clinical setting, recognition of the entire dilated small bowel at the center of the abdomen and encased within a thick fibrocollagenous membrane, as though it were in a cocoon, is diagnostic of sclerosing encapsulating peritonitis. The other imaging findings may include:

• enhancing peritoneum, thickened >2 mm
• signs of small intestinal obstruction
• fixation of intestinal loops
• ascites or localized fluid collections (especially interbowel)
• bowel wall thickening
• peritoneal or mural calcification
• calcified and/or reactive adenopathy

MRI

MRI will demonstrate the same features as CT, although it may better discriminate between thickened bowel and the peritoneal membrane than CT .

Differential diagnosis

Encapsulating peritoneal sclerosis may be confused with congenital peritoneal encapsulation, which is characterized by a thin accessory peritoneal sac surrounding the small bowel.

Siehe auch:

• intraabdominelle Tuberkulose
• ventrikuloperitonealer Shunt
• Peritonitis
• peritoneale Dialyse
• practolol
• Peritonitis bei Peritonealdialyse
• zystische peritoneale Veränderungen

und weiter:

• Oreopoulos-Zellermann-Katheter
• Tenckhoff catheter
• peritoneale Verkalkungen