Sclerosing encapsulating peritonitis (SEP) is a rare disorder that is characterized by encapsulation of bowel loops by thick fibrinogenous case. Most patients present with vague abdominal symptoms. It is challenging to diagnose the condition preoperatively. Surgical management is preserved for patients with small bowel obstruction with no improvement on conservative measures or for those with signs of bowel ischemia (Li et al., 2014; Habib et al. 2011). Herein, we discuss the clinical signs and symptoms, the radiological features, the surgical management, and outcome of SEP based on a patient who underwent surgery in our hospital.
Sclerosing encapsulating peritonitis, or cocoon syndrome, is a rare disorder, which is usually seen in patients on continuous ambulatory peritoneal dialysis (CAPD). The exact known of the condition is still unknown. The gross pathology resembles a cocoon case encapsulating the bowel loops, hence the name. Patients with SEP often present with vague abdominal symptoms. A computed tomography in a patient with intestinal obstruction is likely to raise the suspicion of SEP; however, the case is usually diagnosed intraoperatively. Many patients would resolve with only conservative medical managements, but some may require surgical intervention. It is still unclear whether early surgical intervention has an advantage over conservative management, but, in most reviewed case reports, surgeons preferred to preserve the surgical management for those who do not respond to conservative measures [
A 50-year-old Pakistani gentleman, who was previously healthy, was admitted to our surgical department with generalized abdominal pain, absolute constipation, and abdominal distention. He had multiple admissions during the previous eighteen months with similar episodes, which were diagnosed as small bowel obstruction, by the clinical picture, and multiple air-fluid levels on abdominal X-rays, and always resolved on conservative measures. He was afebrile and hemodynamically stable. His abdomen was distended with no scars. He had generalized abdominal tenderness and exaggerated bowel sounds. His rectum had remnant of stools on PR examination. He had neutrophilic leukocytosis, but the rest of his labs were within normal limits. His axial abdominal X-ray showed multiple air-fluid levels. A computed tomographic scan of his abdomen, which was performed on the same day of admission, showed evidence of multiple bowel loops dilated in the left side of the abdomen with distal collapse of bowel (transition zone) with swirling sign of the vessels. Additionally, an encasement of the small bowel within a membrane-like sac was observed, with a few calcifications (Figures
Sclerosing encapsulating peritonitis, also known as cocoon syndrome, is a poorly understood and rarely described condition. The pathogenesis is thought to be by the release of fibrin-like material by fibrinogenic cytokines [
The clinical presentation of patients with SEP is usually vague, with nonspecific abdominal symptoms, including bloating, nausea, abdominal discomfort, constipation, or vomiting [
The preoperative diagnosis of SEP is usually challenging. It takes high experience and knowledge of the disease to suspect its presence. To properly diagnose SEP preoperatively, imaging studies are of crucial importance. These include erect abdominal X-ray films, barium passage radiography, ultrasonography (USG), and computed tomography (CT). Abdominal X-rays would show air-fluid levels and dilated bowel loops in a patient with symptoms of intestinal obstruction. A barium swallow would show an accordion pattern and cauliflower appearance [
The management of SEP depends on the presentation of the patient. In asymptomatic patients with idiopathic SEP, regular follow-up is all that is required [
In conclusion, SEP is a rare, poorly understood condition in which a fibrous membrane encapsulates the bowel, possibly leading to intestinal obstruction and its consequences. Preoperative diagnosis is usually challenging. The use of the CT scans along with other imaging techniques may assist in reaching a correct diagnosis and subsequently the proper management. Although conservative management remains preferred in mildly symptomatic patients, surgical intervention is often required to prevent or deal with the complications of intestinal obstruction. A minimally invasive approach should be attempted to avoid troublesome complications.
The authors declare no financial conflicts of interest related to the material in this manuscript.